ACHD Featured Articles of September 2017

ACHD Reviews of September 2017 Manuscripts, provided by ISACHD

 

Retrospective UK multicentre study of the pregnancy outcomes of women with a Fontan repair.

Cauldwell M, Steer PJ, Bonner S, Asghar O, Swan L, Hodson K, Head CEG, Jakes AD, Walker N, Simpson M, Bolger AP, Siddiqui F, English KM, Maudlin L, Abraham D, Sands AJ, Mohan AR, Curtis SL, Coats L, Johnson MR.

Heart. 2017 Sep 27. pii: heartjnl-2017-311763. doi: 10.1136/heartjnl-2017-311763. [Epub ahead of print]

PMID: 28954835

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Take Home Points:

 

  • Patients with a Fontan circulation have high rates of miscarriage
  • The obstetric and neonatal complication rate is high in those who do not miscarry.
  • In women who went on to have a live birth heart failure occurred in 13.2% and arrhythmias in 11.2%.

 

Blanche CupidoCommentary by Dr. Blanche Cupido (Cape Town), section editor of ACHD Journal WatchAn increasing number of women with Fontan circulations reach adulthood and desire pregnancy. This study represents a retrospective review of pregnancy outcomes in women with Fontan circulations delivering at 10 specialized centers in the UK between January 2005 and November 2016.

 

Fifty women had 124 pregnancies. 34% had one pregnancy, the rest of the patients had multiple pregnancies. 77% of pregnancies were planned and 82% of women had received preconception counseling.

 

Only 42.7% (n=53) of pregnancies resulted in live births. There were 68 miscarriages, 2 terminations, 1 intrauterine death and 4 neonatal deaths.

 

At baseline, most women had NYHA class I (96%), normal ventricular function (80%) and a CARPREG score of 0 or 1. Only 30% of patients had a baseline saturation of <92%. In 70.5% of pregnancies, women were taking warfarin pre-pregnancy. The anticoagulation practices during pregnancy varied widely.

Sept ACHD 1

 

Maternal heart failure and arrhythmias were the most common maternal complications (13.2% and 11.2% respectively).  In those who developed heart failure, 6/7 were asymptomatic prior to pregnancy. No maternal deaths occurred.

Sept ACHD 2

 

Fifty-eight percent of all pregnancies ended in miscarriage. A baseline saturation rate of <85% carried a significantly increased risk of miscarriage (100% vs 51.7%, P=0.008) The baseline hemoglobin in those who miscarried was higher than those who had live births (Hb 14.4g% vs 13.8g%) and both are higher than compared to normal pregnancies (Hb 12.0g%).

 

Median gestation at live birth was 33.5 weeks. Preterm births occurred in 71.7% of pregnancies. Over 40% of patients experienced blood loss of 500ml or more. Major obstetric haemorrhage occurred in 7 women.

 

In summary, Fontan patients have high rates of miscarriage, neonatal and obstetric complications. Most women who have a live birth, the most common complications were heart failure (13.2%) and arrhythmias (11.2%).

 

Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease.

Evangelista A, Gallego P, Calvo-Iglesias F, Bermejo J, Robledo-Carmona J, Sánchez V, Saura D, Arnold R, Carro A, Maldonado G, Sao-Avilés A, Teixidó G, Galian L, Rodríguez-Palomares J, García-Dorado D.

Heart. 2017 Sep 1. pii: heartjnl-2017-311560. doi: 10.1136/heartjnl-2017-311560. [Epub ahead of print]

PMID: 28864719

 

 

Take Home Points:

 

  • Bicuspid aortic valve is heterogeneous with complex interactions between valve morphology, age, gender, and cardiovascular risk factors.
  • Aortic stenosis is more frequent in the right-non-coronary morphotype and associated with the presence of a raphe, older age, and cardiovascular risk factors.
  • Aortic regurgitation was more common in males, younger age, and the presence of valve prolapse.
  • Aortic root dilation was associated with younger age, male sex, and aortic regurgitation and less frequent in patients with fusion of the right and non-coronary cusps.
  • Ascending aorta dilation was similar among valvular morphotype and less frequent in normal functional valve patients.
  • Aortic arch is less likely to be dilated in right – left morphotype compared with the others.
  • The right-non-coronary morphotype is associated with aortic stenosis, less aortic root dilation and more aortic arch dilation.

 

jokhadar-maanCommentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch:

Bicuspid aortic valve (BAV) is the most common congenital heart abnormality and is associated with aortic stenosis (AS) and/or aortic regurgitation (AR) and/or aortopathy with high heterogeneity.

 

Dr. Evangelista and colleagues recruited 852 consecutive adults diagnosed with BAV at 8 tertiary Spanish hospitals between 2012 and 2015. Patients with prior valvuloplasty, aortic surgery, endocarditis, coarctation, or other congenital aortic disease were excluded. Patients who did not have a definitive diagnosis of BAV because of image quality were also excluded.

 

The mean age was about 47years. About 18% did not have a raphe.

 

BAV morphotype was categorized as follows:

Valvular dysfunction was categorized as none, mild, moderate, or severe AS or AR.  Patients with mixed disease were categorized according to the predominant lesion. The presence of a raphe, calcification, or sigmoid prolapse were reported in all cases.  The ascending aorta was measured with 2-D echo using conventional methods.

Valve dysfunction:

RN fusion patients had more valve prolapse (22%) and valve calcification (32%).  As would be expected, calcification was more common with increased age and the presence of cardiac risk factors, including hypertension, dyslipidemia, diabetes, and smoking.

 

RL fusion patients had less calcification. AS was related to age, dyslipidemia, smoking, raphe presence, and RN morphotype. AR was related to male sex and the presence of sigmoid prolapse. AR was more prevalent in young patients and AS in older patients. About 26% of patients with raphe had AS compared to 11% without a raphe.

 

Aortic dilation:

Aortic root dilation was present in about 34% and/or ascending aorta in about 76%.  There was no association between BAV morphotype and ascending aorta dilation. However, RN patients had smaller aortic root and larger arch. Root diameter was larger in AR when compared with AS or patients without significant valve dysfunction. In patients with aorta dilation, about 18% had aortic root dilation and about 82% had ascending aorta dilation.  Aortic root dilation was associated with younger age, male sex, and AR.

 

This is the largest prospective multi-center study of the associations between BAV morphology, valvular dysfunction, and aorta dilation.  This study highlights the heterogeneity of BAV and the complex interactions between valve morphology, age, gender, and cardiovascular risk factors. This was a cross-sectional study that could not assess the impact of these associations with disease progression. This information is an important step forward, but additional studies are needed to help better tailor imaging, screening, medical therapy, and surgical strategy in these patients.

 

Management of Marfan Syndrome during pregnancy: A real world experience from a Joint Cardiac Obstetric Service.

Lim JCE, Cauldwell M, Patel RR, Uebing A, Curry RA, Johnson MR, Gatzoulis MA, Swan L.

Int J Cardiol 2017 Sep 15; 243: 180-184. Doi: 10.1016/j.ijcard.2017.05.077. Epub 2017 May 22.

PMID : 28606654

 

Take Home Points:

 

  • The European Society of Cardiology drew up guidelines on the management of pregnant patients with Marfan Syndrome.
  • Non-compliance to the guidelines is not uncommon despite the presence of a dedicated Joint Cardiac and Obstetric Service.

 

Leong Ming ChernCommentary from Dr. MC Leong (Kuala Lumpur), section editor of ACHD Journal Watch:  The European Society of Cardiology has drawn up guidelines on the management of patients with Marfan Syndrome with dilated aortic root (aortic root > 4cm) [1]. Specific recommendations include: preconception counseling, imaging surveillance and the use of beta-blocker to reduce the risk of aortic dissection. The authors conducted a single center audit examining the management of pregnant women with Marfan Syndrome under the care of the Joint Cardiac and Obstetric Service at their hospital compared to the guidelines over a period of 10 years (2005-2015) specifically looking at the three aspects stated above.

 

The audit standards [ESC Guidelines] to which the clinical practice was assessed against are summarized in Table 2.

Twenty-three pregnancies were reported in 15 women, with a median conception age of 34 years. In 9 of the 23 pregnancies aortic root dimensions were greater than 4cm at baseline.

Sept ACHD 3 1

Three important cardiovascular complications occurred: Type A dissection 10 days post-partum; significant LV dysfunction and aortic root dilation at 35 weeks gestation, and progressive LV dysfunction in 1.

Counseling :

Pre-conception counseling were documented in 19/23 (83%) cases. Three of the 4 patients who did not have pre-pregnancy counselling had a moderate or high risk of cardiac event, of whom 2 developed cardiovascular complications.

 

Imaging :

Pre-conception imaging was performed in 12/23 (52%) cases. While most cases occurred prior to the publication of the guidelines, 3 cases occurred after the guidelines were made known to the team. Despite aortic dilatation being noted in 7/23 (30%) pregnancies, none had further follow up MRI scans performed.

 

In only 7/23 (30%) pregnancies were there appropriate aortic imaging documented during pregnancy. In 22 of  23 (96%) pregnancies, did women have a pre-discharge echocardiography. In 19 of 23 (83%) pregnancies, the women had at least 1 echocardiogram within 6 months postpartum.

 

Beta- blocker :

 

Seventeen 17 of 23 (74%) of pregnancies were managed with beta-blocker therapy.

 

Non-compliance with guidelines is not uncommon. Areas of non-compliance are mainly seen (1) late presentation of patients during pregnancy and (2) and the recommended post-partum follow- up imaging.  Though there may be various reasons for non-compliance, the blame should come from both sides.

 

Ref :

1. V. Regitz-Zagrosek, C. Blomstrom Lunquist, C. Borghi, et al., ESC guidelines on management of cardiovascular disease during pregnancy, Eur Heart J. 32 (2011) 3147-3197.

 

 

Elevated sympathetic activity, endothelial dysfunction, and late hypertension after repair of coarctation of the aorta.

Lee MGY, Hemmes RA, Mynard J, Lambert E, Head GA, Cheung MMH, Konstantinov IE, Brizard CP, Lambert G, d’Udekem Y.

Int J Cardiol. 2017 Sep 15;243:185-190. doi: 10.1016/j.ijcard.2017.05.075. Epub 2017 May 19.

PMID: 28545853

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Take Home Points:

 

  • Hypertension, affecting up to 75% of young adults after coarctation repair, is a common cause of early morbidity and mortality in coarctation patients, even without residual obstruction of the arch.
  • Direct measurement of sympathetic neural activity in patients after coarctation repair may identify patients at higher risk of vascular complications.
  • Measurement of endothelial function may also be helpful in identifying patients at risk of hypertension and other vascular events.
  • This study evaluated several parameters of sympathetic activity, including direct measurement of muscle sympathetic nerve activity, plasma renin activity, sympathetic baroreflex function, endothelial dysfunction, and ambulatory arterial stiffness index.
  • “White Coat” hypertension in coarctation patients likely is not benign based on results of this small study.

Kay_William_MD.16.CVaCommentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch: 

In this small pilot study from Melbourne, Australia (n = 23 subjects), patients late after coarctation repair were matched to 17 healthy control subjects and underwent a variety of tests to measure parameters of sympathetic activity and endothelial function. 

 

A novel technique used in this study, not previously studied in coarctation patients, was muscle sympathetic nerve activity testing (MSNA), which is a direct measurement of sympathetic activity of muscle vasoconstrictor nerve fibers, which has been linked to systemic hypertension in other populations in previous studies.  The investigators performed this by inserting an electrode into the right peroneal nerve at the fibular head with a reference subcutaneous electrode 2-3 cm away from the recording site.

 

Parameters other than MSNA which were studied included evaluation of plasma renin activity, spontaneous arterial baroreflex measurements, and ambulatory arterial stiffness index.  All patients also underwent echocardiography, clinic blood pressure measurement, and 24-h ambulatory BP measurement.  Overall, amongst the coarctation patients, the incidence of hypertension at rest in clinic was 6% (44% with prehypertension) and the incidence of hypertension on 24-h BP monitor was 15% (20% with prehypertension).  Interestingly, only one patient with hypertension on 24-h BP monitor had hypertension at rest in the clinic.  The entire control group had normal blood pressure on 24-h monitoring.  Despite the fairly low incidence of hypertension, nearly half of patients had LVH on echocardiogram.

 

In this study, several comparisons were made.  Variables were compared between the coarctation and control group.  Additionally, amongst the coarctation group, comparisons were made between patients with and without resting clinic hypertension as well as with and without hypertension revealed on a 24 H ambulatory blood pressure monitor.

 

MSNA was found to be markedly different in the coarctation group compared with control group.  Additionally, MSNA was higher in coarctation patients with an elevated resting BP measurement in clinic than those that were normotensive in clinic.  However, there was no difference in the MSNA levels amongst between coarctation patients with and without elevated BP on 24-h monitoring.

 

There were no differences in renin activity between coarctation patients and controls; between coarctation patients with and without elevated clinic BP measurements, or between coarctation patients with and without elevated 24-h BP measurements.  Additionally, there were no differences found amongst any of the groups regarding baroreflexes.

 

Endothelial function as assessed by digital pulse amplitude tonometry was impaired in coarctation patients compared to controls, and was also impaired in patients with coarctation with elevated BP in clinic (but of note this impairment was not seen in patients who had HTN only on 24 h BP monitoring).

 

The authors conclude that this study reveals potential etiologies for as well as targets for therapy for hypertension in the coarctation population.  Of note, elevated MSNA as well as impaired endothelial function were particularly evident in patients with elevated blood pressure in clinic, but with normal 24-h blood pressure, calling into question if “white coat hypertension” is benign in the coarctation population.  Further studies of the repaired coarctation patient population with larger sample sizes would be quite interesting, and more research is clearly needed.

 Sept ACHD 4

ACHD Sept 2017

 

 

  1. Retrospective UK multicentre study of the pregnancy outcomes of women with a Fontan repair.

Cauldwell M, Steer PJ, Bonner S, Asghar O, Swan L, Hodson K, Head CEG, Jakes AD, Walker N, Simpson M, Bolger AP, Siddiqui F, English KM, Maudlin L, Abraham D, Sands AJ, Mohan AR, Curtis SL, Coats L, Johnson MR.

Heart. 2017 Sep 27. pii: heartjnl-2017-311763. doi: 10.1136/heartjnl-2017-311763. [Epub ahead of print]

PMID: 28954835

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Select item 28954834

 

  1. Exertional dyspnoea in a 28-year-old woman.

Carlson SD, Steinberg ZL, Krieger EV.

Heart. 2017 Sep 27. pii: heartjnl-2017-312174. doi: 10.1136/heartjnl-2017-312174. [Epub ahead of print]

PMID: 28954831

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Select item 28952403

 

  1. Burden and contributing factors associated with tricuspid regurgitation: a hospital-based study.

Yiu KH, Chen Y, Liu JH, Lin Q, Liu M, Wu M, Wang R, Zhen Z, Zou Y, Lam YM, Ng MY, Lau CP, Tse HF.

Hosp Pract (1995). 2017 Sep 27. doi: 10.1080/21548331.2017.1384688. [Epub ahead of print]

PMID: 28952403

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  1. Prognostic value of galectin-3 in adults with congenital heart disease.

Baggen VJM, van den Bosch AE, Eindhoven JA, Menting ME, Witsenburg M, Cuypers JAAE, Boersma E, Roos-Hesselink JW.

Heart. 2017 Sep 23. pii: heartjnl-2017-312070. doi: 10.1136/heartjnl-2017-312070. [Epub ahead of print]

PMID: 28942393

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  1. New imaging tools in cardiovascular medicine: computational fluid dynamics and 4D flow MRI.

Itatani K, Miyazaki S, Furusawa T, Numata S, Yamazaki S, Morimoto K, Makino R, Morichi H, Nishino T, Yaku H.

Gen Thorac Cardiovasc Surg. 2017 Sep 19. doi: 10.1007/s11748-017-0834-5. [Epub ahead of print]

PMID: 28929446

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  1. Exercise in Adult with Congenital Heart Disease: Not a chimaera anymore.

Montanaro C, Limongelli G.

Int J Cardiol. 2017 Sep 15;243:209-210. doi: 10.1016/j.ijcard.2017.05.054. No abstract available.

PMID: 28747023

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  1. Patient reported outcomes are associated with physical activity level in adults with congenital heart disease.

Bay A, Dellborg M, Berghammer M, Sandberg C, Engström G, Moons P, Johansson B.

Int J Cardiol. 2017 Sep 15;243:174-179. doi: 10.1016/j.ijcard.2017.03.137.

PMID: 28747022

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  1. Mortality in pulmonary arterial hypertension due to congenital heart disease: Serial changes improve prognostication.

Schuijt MTU, Blok IM, Zwinderman AH, van Riel ACMJ, Schuuring MJ, de Winter RJ, Duijnhouwer AL, van Dijk APJ, Mulder BJM, Bouma BJ.

Int J Cardiol. 2017 Sep 15;243:449-453. doi: 10.1016/j.ijcard.2017.05.101. Epub 2017 Jun 1.

PMID: 28606655

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  1. Management of Marfan Syndrome during pregnancy: A real world experience from a Joint Cardiac Obstetric Service.

Lim JCE, Cauldwell M, Patel RR, Uebing A, Curry RA, Johnson MR, Gatzoulis MA, Swan L.

Int J Cardiol. 2017 Sep 15;243:180-184. doi: 10.1016/j.ijcard.2017.05.077. Epub 2017 May 22.

PMID: 28606654

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  1. Detection of mechanical complications related to the potential risk of sudden cardiac death in patients with pulmonary arterial hypertension by computed tomography.

Lee SE, Im JH, Sung JM, Cho IJ, Shim CY, Hong GR, Chung N, Jung JW, Chang HJ.

Int J Cardiol. 2017 Sep 15;243:460-465. doi: 10.1016/j.ijcard.2017.05.090. Epub 2017 May 25.

PMID: 28576625

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  1. Effect of phosphodiesterase-5 inhibition with Tadalafil on SystEmic Right VEntricular size and function – A multi-center, double-blind, randomized, placebo-controlled clinical trial – SERVE trial – Rational and design.

Tobler D, Bouchardy J, Reto E, Heg D, Müller C, Frenk A, Gabriel H, Schwitter J, Rutz T, Buechel RR, Willhelm M, Trachsel L, Freese M, Greutmann M, Schwerzmann M; SERVE trial.

Int J Cardiol. 2017 Sep 15;243:354-359. doi: 10.1016/j.ijcard.2017.05.079. Epub 2017 May 23.

PMID: 28566262

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Select item 28545853

 

  1. Elevated sympathetic activity, endothelial dysfunction, and late hypertension after repair of coarctation of the aorta.

Lee MGY, Hemmes RA, Mynard J, Lambert E, Head GA, Cheung MMH, Konstantinov IE, Brizard CP, Lambert G, d’Udekem Y.

Int J Cardiol. 2017 Sep 15;243:185-190. doi: 10.1016/j.ijcard.2017.05.075. Epub 2017 May 19.

PMID: 28545853

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Select item 28539208

 

  1. Effect of maternal age and cardiac disease severity on outcome of pregnancy in women with congenital heart disease.

Furenäs E, Eriksson P, Wennerholm UB, Dellborg M.

Int J Cardiol. 2017 Sep 15;243:197-203. doi: 10.1016/j.ijcard.2017.04.100. Epub 2017 May 1.

PMID: 28539207

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  1. Educational level and employment status in adults with congenital heart disease.

Pfitzer C, Helm PC, Rosenthal LM, Walker C, Ferentzi H, Bauer UMM, Berger F, Schmitt KRL.

Cardiol Young. 2017 Sep 13:1-7. doi: 10.1017/S104795111700138X. [Epub ahead of print]

PMID: 28899436

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  1. The CRASH report: emergency management dilemmas facing acute physicians in patients with pulmonary arterial hypertension.

Price LC, Dimopoulos K, Marino P, Alonso-Gonzalez R, McCabe C, Kemnpy A, Swan L, Boutsikou M, Al Zahrani A, Coghlan GJ, Schreiber BE, Howard LS, Davies R, Toshner M, Pepke-Zaba J, Church AC, Peacock A, Corris PA, Lordan JL, Gaine S, Condliffe R, Kiely DG, Wort SJ.

Thorax. 2017 Sep 12. pii: thoraxjnl-2016-209725. doi: 10.1136/thoraxjnl-2016-209725. [Epub ahead of print]

PMID: 28904006

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  1. The Relationship between Parental Rearing Behavior, Resilience, and Depressive Symptoms in Adolescents with Congenital Heart Disease.

Moon JR, Song J, Huh J, Kang IS, Park SW, Chang SA, Yang JH, Jun TG.

Front Cardiovasc Med. 2017 Sep 8;4:55. doi: 10.3389/fcvm.2017.00055. eCollection 2017.

PMID: 28944224 Free PMC Article

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  1. Homoarginine-A prognostic indicator in adolescents and adults with complex congenital heart disease?

Raedle-Hurst T, Mueller M, Meinitzer A, Maerz W, Dschietzig T.

PLoS One. 2017 Sep 8;12(9):e0184333. doi: 10.1371/journal.pone.0184333. eCollection 2017.

PMID: 28886170 Free PMC Article

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  1. Environmental and lifestyle factors influencing risk of congenital heart block during pregnancy in anti-Ro/SSA-positive women.

Meisgen S, Tingström J, Skog Andreasson A, Sonesson SE, Kockum I, Wahren-Herlenius M.

RMD Open. 2017 Sep 7;3(2):e000520. doi: 10.1136/rmdopen-2017-000520. eCollection 2017.

PMID: 28955500

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  1. The Center for Epidemiologic Studies Depression Scale is an adequate screening instrument for depression and anxiety disorder in adults with congential heart disease.

Moon JR, Huh J, Song J, Kang IS, Park SW, Chang SA, Yang JH, Jun TG.

Health Qual Life Outcomes. 2017 Sep 5;15(1):176. doi: 10.1186/s12955-017-0747-0.

PMID: 28874154 Free PMC Article

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  1. Functional Capacity in Congenital Heart Disease: A Systematic Review and Meta-Analysis.

Schaan CW, Macedo ACP, Sbruzzi G, Umpierre D, Schaan BD, Pellanda LC.

Arq Bras Cardiol. 2017 Sep 4. pii: S0066-782X2017005017101. doi: 10.5935/abc.20170125. [Epub ahead of print] Portuguese, English.

PMID: 28876372 Free Article

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  1. Acquired cardiovascular disease in adult patients with congenital heart disease.

Giannakoulas G, Ntiloudi D.

Heart. 2017 Sep 4. pii: heartjnl-2017-311997. doi: 10.1136/heartjnl-2017-311997. [Epub ahead of print] No abstract available.

PMID: 28870981

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  1. Pregnancy outcome in women with congenital heart disease: A single center experience.

Ntiloudi D, Zegkos T, Bazmpani MA, Parcharidou D, Panagiotidis T, Hadjimiltiades S, Karvounis H, Giannakoulas G.

Hellenic J Cardiol. 2017 Sep 2. pii: S1109-9666(17)30272-5. doi: 10.1016/j.hjc.2017.08.008. [Epub ahead of print]

PMID: 28873334 Free Article

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  1. Inferior Vena Cava Thrombus in a Postpartum Patient With Fontan Physiology: A Case Report.

Tashjian JA, Fraint H, DiNardo J, Rouine-Rapp K.

A A Case Rep. 2017 Sep 1;9(5):136-139. doi: 10.1213/XAA.0000000000000548.

PMID: 28542047

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  1. [Congenital heart disease in adolescents and adults: Management in a general cardiology department in Senegal].

Mbaye A, Bodian M, Ngaïdé AA, Abdourafiq H, Leye MCBO, Savodogo S, Aw F, Ndiaye M, Kouamé I, Babaka K, Dioum M, Gaye ND, Sarr SA, Ndiaye MB, Kane AD, Kane A.

Ann Cardiol Angeiol (Paris). 2017 Sep;66(4):217-222. doi: 10.1016/j.ancard.2017.02.003. Epub 2017 May 12. French.

PMID: 28506578

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  1. Attitudes and perceptions of pregnant women with CHD: results of a single-site survey.

Sabanayagam A, Briston D, Zaidi AN.

Cardiol Young. 2017 Sep;27(7):1257-1264. doi: 10.1017/S1047951116002857. Epub 2017 Apr 18.

PMID: 28416028

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  1. Effect of iloprost on biomarkers in patients with congenital heart disease-pulmonary arterial hypertension.

Li XY, Zheng Y, Long Y, Zhang X, Zhang L, Tian D, Zhou D, Lv QZ.

Clin Exp Pharmacol Physiol. 2017 Sep;44(9):914-923. doi: 10.1111/1440-1681.12796.

PMID: 28608969

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  1. A case of Alagille syndrome presenting with chronic cholestasis in an adult.

Kim J, Yang B, Paik N, Choe YH, Paik YH.

Clin Mol Hepatol. 2017 Sep;23(3):260-264. doi: 10.3350/cmh.2016.0057. Epub 2017 Jul 7.

PMID: 28683534 Free Article

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  1. Imaging of the pulmonary valve in the adults.

Pignatelli RH, Noel C, Reddy SCB.

Curr Opin Cardiol. 2017 Sep;32(5):529-540. doi: 10.1097/HCO.0000000000000436.

PMID: 28678047

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  1. Adult congenital heart disease: magnitude of the problem.

Thakkar AN, Chinnadurai P, Lin CH.

Curr Opin Cardiol. 2017 Sep;32(5):467-474. doi: 10.1097/HCO.0000000000000429.

PMID: 28617685

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  1. Imaging adult patients with Fontan circulation.

Ginde S, Goot BH, Frommelt PC.

Curr Opin Cardiol. 2017 Sep;32(5):521-528. doi: 10.1097/HCO.0000000000000422.

PMID: 28548989

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  1. Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting.

Gravholt CH, Andersen NH, Conway GS, Dekkers OM, Geffner ME, Klein KO, Lin AE, Mauras N, Quigley CA, Rubin K, Sandberg DE, Sas TCJ, Silberbach M, Söderström-Anttila V, Stochholm K, van Alfen-van derVelden JA, Woelfle J, Backeljauw PF; International Turner Syndrome Consensus Group.

Eur J Endocrinol. 2017 Sep;177(3):G1-G70. doi: 10.1530/EJE-17-0430. Review.

PMID: 28705803 Free Article

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  1. Anatomical and clinical predictors of valve dysfunction and aortic dilation in bicuspid aortic valve disease.

Evangelista A, Gallego P, Calvo-Iglesias F, Bermejo J, Robledo-Carmona J, Sánchez V, Saura D, Arnold R, Carro A, Maldonado G, Sao-Avilés A, Teixidó G, Galian L, Rodríguez-Palomares J, García-Dorado D.

Heart. 2017 Sep 1. pii: heartjnl-2017-311560. doi: 10.1136/heartjnl-2017-311560. [Epub ahead of print]

PMID: 28864719

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  1. Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977-2012.

Hjortshøj CS, Jensen AS, Sørensen K, Nagy E, Johansson B, Kronvall T, Dellborg M, Estensen ME, Holmstrøm H, Turanlahti M, Thilén U, Søndergaard L.

Heart. 2017 Sep;103(17):1353-1358. doi: 10.1136/heartjnl-2016-310979. Epub 2017 Apr 27.

PMID: 28450553

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  1. Improving on the diagnostic characteristics of echocardiography for pulmonary hypertension.

Broderick-Forsgren K, Davenport CA, Sivak JA, Hargett CW, Foster MC, Monteagudo A, Armour A, Rajagopal S, Arges K, Velazquez EJ, Samad Z.

Int J Cardiovasc Imaging. 2017 Sep;33(9):1341-1349. doi: 10.1007/s10554-017-1114-2. Epub 2017 Mar 24.

PMID: 28337558

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  1. Isolated Right Juxtaposition of the Atrial Appendages in Adult With Bicuspid Aortic Valve Stenosis.

Boutayeb A, Kenassi F, Bensouda A, Marmade L.

World J Pediatr Congenit Heart Surg. 2017 Sep;8(5):635-636. doi: 10.1177/2150135117720687.

PMID: 28901226

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  1. The Lymphatic System: The Achilles Heel of the Fontan-Kreutzer Circulation.

Kreutzer C, Kreutzer G.

World J Pediatr Congenit Heart Surg. 2017 Sep;8(5):613-623. doi: 10.1177/2150135117720685.

PMID: 28901223

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  1. Reference values for blood pressure response to cycle ergometry in the first two decades of life: comparison with patients with a repaired coarctation of the aorta.

Kaafarani M, Schroer C, Takken T.

Expert Rev Cardiovasc Ther. 2017 Sep 26. doi: 10.1080/14779072.2017.1385392. [Epub ahead of print]

PMID: 28949265

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  1. Hepatic Stiffness Using Shear Wave Elastography and the Related Factors for a Fontan Circulation.

Kim SO, Lee SY, Jang SI, Park SJ, Kwon HW, Kim SH, Lee CH, Choi ES, Cho SK, Hong SH, Kim YM.

Pediatr Cardiol. 2017 Sep 21. doi: 10.1007/s00246-017-1727-1. [Epub ahead of print]

PMID: 28932977

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  1. Imaging the adult with transposition of the great arteries.

Anderson JH, Cetta F.

Curr Opin Cardiol. 2017 Sep;32(5):482-489. doi: 10.1097/HCO.0000000000000420.

PMID: 28537999

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ACHD Featured Articles of July 2017

Adult Congenital Heart Reviews of July/August 2017 Manuscripts

 

Physical activity in adults with congenital heart disease and associations with functional outcomes.

Müller J, Amberger T, Berg A, Goeder D, Remmele J, Oberhoffer R, Ewert P, Hager A.

Heart. 2017 Jul;103(14):1117-1121. doi: 10.1136/heartjnl-2016-310828. Epub 2017 Mar 8.

PMID: 28274955

Similar articles

Select item 28183791

 

 

Take Home Points:

 

  • Utilizing the International Physical Activity Questionnaire (IPAQ) divided patients into the 3 activity categories: health-enhancing physical activity (HEPA) – 50%, minimal activity (30.2%)and inactivity (19.5%) – the results of the questionnaire correlated well with objectively measures exercise capacity and health-related quality of life.
  • The group partaking in health enhancing physical activity was associated with a higher exercise capacity and better mental and physical health related quality of life scores when compared to minimally active and inactive patients.
  • Pacemakers were independently associated with poorer quality of life scores.

 

 

Blanche CupidoCommentary by Dr. Blanche Cupido (Cape Town), section editor of ACHD Journal Watch:

A good exercise capacity predicts favorable long-term morbidity and mortality in patients with CHD. Cardiopulmonary exercise testing (CPET) and accelerometry currently represent the gold standard for physical activity but is costly and not readily available. In this study, the authors utilize the International Physical Activity Questionnaire (IPAQ) to assess self-reported physical activity within the last 7 days. Exercise capacity is also objectively measured with cardiopulmonary exercise testing and assess quality of life using the SF-36(Health related quality of life) questionnaire.

 

786 patients filled out the IPAQ and SF-36 questionnaires. The mean age was 31 year and the mean BMI 23.7kg/m2.  A wide variety of pathologies were included. IPAQ identified 3 levels of activity: health-enhancing physical activity, minimally active and inactive.  Cyanotic patients (those with oxygen saturations of <90% at rest or on exercise) were included in the analysis irrespective of heart rate or RER.

 

50% of patients reported health enhancing physical activity (HEPA), 30.2% minimal activity and 19.8% inactivity. Men had higher activity levels compared to women (p=0,086) but no significant differences were observed between the different pathology subgroups or their level of disease severity. Compared to the HEPA group, the inactive group had lower peak oxygen uptake (74.2% vs 86.7%, p<0.001). They also had lower mental (97.1% vs 104.1%, p<0.001) and physical (91% vs 97.4%, p<0.001) health related quality of life scores. (see attached figure)

July 2017 ACHD 1

Betablocker use, male gender, pacemaker, oxygen saturation at rest and severity class were associated with impaired exercise capacity. Pacemakers were associated with lower physical quality of life (p=0.021) and a lower mental quality of life as well (p=0.014).

 

Long-term survival after the Fontan operation: Twenty years of experience at a single center.

Downing TE, Allen KY, Glatz AC, Rogers LS, Ravishankar C, Rychik J, Faerber JA, Fuller S, Montenegro LM, Steven JM, Spray TL, Nicolson SC, Gaynor JW, Goldberg DJ.

J Thorac Cardiovasc Surg. 2017 Jul;154(1):243-253.e2. doi: 10.1016/j.jtcvs.2017.01.056. Epub 2017 Mar 6.

PMID: 28341469

Similar articles

Select item 28320512

  

Take Home Points:

 

  • Despite the early improvements in post-operative outcomes, long-term survival after the Fontan has not improved appreciably over the last decade
  • In this contemporary Fontan cohort, late survival with an intact Fontan was as follows: 94% at 1 year (95% confidence interval 92% -95%), 90% at 10 years (CI, 88% -92% ), 85%  at 15 years (CI, 82% -88% ), and 74%  at 20 years (CI, 67% -80% ). Risk factors were identifiable as exerting an early influence, i.e. within the 1st year or late influence, after 1 year.  Specific early risk factors identified included prolonged pleural drainage, intensive care unit stay> 1 week, Fontan before 1997, preoperative atrioventricular valve regurgitation, and longer cross-clamp time. Only independent late risk factor was pre-operative AV-valve regurgitation
  • Early mortality was determined by the presence of higher pre-operative pulmonary artery pressures >15mm Hg, prolonged pleural drainage, longer aortic cross clamp time, and earlier surgical era. Late mortality was determined by the presence of atrioventricular valve regurgitation only. There no correlations between ventricular morphology and outcomes.

 

Moe_Tabitha-WEBCommentary by Dr. Tabitha G. Moe (Phoenix), section editor of ACHD Journal WatchThis is a large single center retrospective presentation of Fontan outcomes in 773 patients.  Patients underwent the Fontan procedure between 1992 and 2009, and all had a cavo-pulmonary connection. Late outcomes were determined from local medical records, and utilization of the National Death Index, and the Scientific Registry of Transplant Recipients for patients that were cared for remote from CHOP. In this cohort greater than 50% of patients carried the diagnosis of hypoplastic left heart syndrome.   A total of 114 (13%) reached the common endpoint of death (n=79), heart transplantation 9n=25) or Fontan takedown (n=10). Of the 79 total deaths, 27 occurred during the initial surgical hospitalization, and 37 within the first post-operative year, and the remaining 15 occurred late.  This accounted for a 94%- 1 year mortality, 90%- 10 year mortality, and 85%- 15yr mortality. The 20 year estimate for survival with an intact Fontan circulation was 74%, and overall 20-year survival was 79%. Ventricular morphology and type of Fontan operation (lateral tunnel vs. extracardiac) were not associated with either early or late outcomes.

 

Interestingly risk factors identified for morality appeared to be active within the 1 post-operative year only, these include prolonged pleural effusions >2 weeks, prolonged aortic cross clamp time, and earlier surgical era’s. Pre-operative pulmonary artery pressures > 15mmHg was able to identify those at greatest risk in the early post-operative period.

The presence of Atrioventricular valve regurgitation > mild was able to identify those at risk for late mortality.

 

It should be noted that of the 25 patients who underwent heart transplantation, there was a 50% survival at 5 years.  As this is a single-center study local clinical practices may affect outcomes, for example 90% of this cohort was fenestrated, and the current status of the fenestration is unknown. Further study of late patency of the fenestration, and the burden of pre-Fontan AP collaterals will be important in further follow-up studies.

 

Long-term survival after the Fontan operation: Twenty years of experience at a single center.

Downing TE, Allen KY, Glatz AC, Rogers LS, Ravishankar C, Rychik J, Faerber JA, Fuller S, Montenegro LM, Steven JM, Spray TL, Nicolson SC, Gaynor JW, Goldberg DJ.

J Thorac Cardiovasc Surg. 2017 Jul;154(1):243-253.e2. doi: 10.1016/j.jtcvs.2017.01.056. Epub 2017 Mar 6.

PMID: 28341469

Similar articles

Select item 28320512

  

Take Home Points:

 

  • Despite the early improvements in post-operative outcomes, long-term survival after the Fontan has not improved appreciably over the last decade
  • In this contemporary Fontan cohort, late survival with an intact Fontan was as follows: 94% at 1 year (95% confidence interval 92% -95%), 90% at 10 years (CI, 88% -92% ), 85%  at 15 years (CI, 82% -88% ), and 74%  at 20 years (CI, 67% -80% ). Risk factors were identifiable as exerting an early influence, i.e. within the 1st year or late influence, after 1 year.  Specific early risk factors identified included prolonged pleural drainage, intensive care unit stay> 1 week, Fontan before 1997, preoperative atrioventricular valve regurgitation, and longer cross-clamp time. Only independent late risk factor was pre-operative AV-valve regurgitation
  • Early mortality was determined by the presence of higher pre-operative pulmonary artery pressures >15mm Hg, prolonged pleural drainage, longer aortic cross clamp time, and earlier surgical era. Late mortality was determined by the presence of atrioventricular valve regurgitation only. There no correlations between ventricular morphology and outcomes.

 

Commentary by Dr. Tabitha G. Moe (Phoenix), section editor of ACHD Journal Watch:  This is a large single center retrospective presentation of Fontan outcomes in 773 patients.  Patients underwent the Fontan procedure between 1992 and 2009, and all had a cavo-pulmonary connection. Late outcomes were determined from local medical records, and utilization of the National Death Index, and the Scientific Registry of Transplant Recipients for patients that were cared for remote from CHOP. In this cohort greater than 50% of patients carried the diagnosis of hypoplastic left heart syndrome.   A total of 114 (13%) reached the common endpoint of death (n=79), heart transplantation 9n=25) or Fontan takedown (n=10). Of the 79 total deaths, 27 occurred during the initial surgical hospitalization, and 37 within the first post-operative year, and the remaining 15 occurred late.  This accounted for a 94%- 1 year mortality, 90%- 10 year mortality, and 85%- 15yr mortality. The 20 year estimate for survival with an intact Fontan circulation was 74%, and overall 20-year survival was 79%. Ventricular morphology and type of Fontan operation (lateral tunnel vs. extracardiac) were not associated with either early or late outcomes.

 

Interestingly risk factors identified for morality appeared to be active within the 1 post-operative year only, these include prolonged pleural effusions >2 weeks, prolonged aortic cross clamp time, and earlier surgical era’s. Pre-operative pulmonary artery pressures > 15mmHg was able to identify those at greatest risk in the early post-operative period.

The presence of Atrioventricular valve regurgitation > mild was able to identify those at risk for late mortality.

 

It should be noted that of the 25 patients who underwent heart transplantation, there was a 50% survival at 5 years.  As this is a single-center study local clinical practices may affect outcomes, for example 90% of this cohort was fenestrated, and the current status of the fenestration is unknown. Further study of late patency of the fenestration, and the burden of pre-Fontan AP collaterals will be important in further follow-up studies.

 

Number of thoracotomy predicts impairment in lung function and exercise capacity in patients with congenital heart disease.

Muller J, Ewert P, Hager A.

J Cardiol. 2017 Jul 4. pii: S0914-5087(17)30157-0. doi: 10.1016/j.jjcc.2017.05.005. [Epub ahead of print]

PMID:28687271

 

Take Home Points:

 

  • Thoracotomies at the time of cardiac surgery may lead to restrictive lung function and decreased exercise capacity.
  • For every thoracotomy, there is a reciprocal 1.8 –fold increase in the prevalence of restrictive lung function.
  • Multiple thoracotomies did not lead to a reduced exercise capacity; rather, it leads to restrictive lung function which leads to reduced exercise capacity.

 

Leong Ming ChernCommentary by Dr. M.C. Leong (Kuala Lumpur), section editor of ACHD Journal Watch: Thoracotomies, performed at the time of cardiac surgeries, may cause chest deformities and adversely altered chest wall compliances, accumulation of fibrotic tissue from surgical scars, weakened inspiratory muscles and diaphragmatic palsy. They contribute to restrictive lung function and decreased exercise capacity. This study aimed to study the relationship between the number of thoracotomies and lung function, exercise capacity and survival.

 

The authors retrospectively analyzed 1372 adolescents (624 females, 746 males; age : 32.4 ± 11.5 years) with congenital heart disease who were referred for cardiopulmonary testing and spirometry at their center from January 2010 to September 2015. 563 (41%) patients had restrictive ventilator pattern.July 2017 ACHD 2 In multivariate analysis, for every thoracotomy and the presence of cyanosis, the odds ratio for a restrictive ventilatory pattern was increased by 81.5% (OR: 1.815, CI: 1.606–2.050, p < 0.001) and 3.0-fold (OR: 3.021, CI: 1.812– 5.038, p < 0.001).

July 2017 ACHD 3
The complexity of the congenital heart disease, cyanosis and the need for beta-blocker, which causes chronotropic incompetence, and older age were associated with poorer exercise capacity while having a higher body mass index, female sex and a higher forced vital capacity were noted to be associated with better exercise capacity. Interestingly, the authors found that multiple thoracotomies did not directly cause limitation in exercise capacity, but they caused a restrictive ventilatory pattern which led to limited exercise capacity.

July 2017 ACHD 4

During a mean follow-up of 2.1 ± 1.6 years, 21 patients died. The number of thoracotomies, oxygen saturation at rest and forced vital capacity were not predictive of mortality. Probability of survival was increased by 6.3% (CI: 1.032–1.095; p < 0.001) for every percentage increase in peak oxygen uptake.

July 2017 ACHD 5

 

Impaired exercise tolerance and poor lung function have previously been linked to poorer survival. Exercise intolerance is, in turn, affected not only by cardiac haemodynamics but also by lung function. Many patients with congenital heart disease, have both chest deformities and history of thoracotomies. Future studies teasing out the relative effects of congenital chest wall deformity vs. surgically induced restrictive changes may be informative as to the exact aetiological mechanisms.

 

ACHD July 2017

 

  1. Infective endocarditis in adults with congenital heart disease remains a lethal disease.

Tutarel O, Alonso-Gonzalez R, Montanaro C, Schiff R, Uribarri A, Kempny A, Grübler MR, Uebing A, Swan L, Diller GP, Dimopoulos K, Gatzoulis MA.

Heart. 2017 Jul 28. pii: heartjnl-2017-311650. doi: 10.1136/heartjnl-2017-311650. [Epub ahead of print]

PMID: 28754810

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Select item 28754809

 

  1. Identification of adults with congenital heart disease of moderate or great complexity from administrative data.

Steiner JM, Kirkpatrick JN, Heckbert SR, Habib A, Sibley J, Lober W, Randall Curtis J.

Congenit Heart Dis. 2017 Jul 24. doi: 10.1111/chd.12524. [Epub ahead of print]

PMID: 28736836

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Select item 28736822

 

  1. Organisation of care for pregnancy in patients with congenital heart disease.

Roos-Hesselink JW, Budts W, Walker F, De Backer JFA, Swan L, Stones W, Kranke P, Sliwa-Hahnle K, Johnson MR.

Heart. 2017 Jul 24. pii: heartjnl-2017-311758. doi: 10.1136/heartjnl-2017-311758. [Epub ahead of print] Review.

PMID: 28739807

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Select item 28728659

 

  1. Strengths, Limitations, and Geographical Discrepancies in the Eligibility Criteria for Sport Participation in Young Patients With Congenital Heart Disease.

Cantinotti M, Giordano R, Assanta N, Murzi B, Melo M, Franchi E, Crocetti M, Iervasi G, Kutty S.

Clin J Sport Med. 2017 Jul 21. doi: 10.1097/JSM.0000000000000474. [Epub ahead of print]

PMID: 28742603

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Select item 28730319

 

  1. Cardiac defects, morbidity and mortality in patients affected by RASopathies. CARNET study results.

Calcagni G, Limongelli G, D’Ambrosio A, Gesualdo F, Digilio MC, Baban A, Albanese SB, Versacci P, De Luca E, Ferrero GB, Baldassarre G, Agnoletti G, Banaudi E, Marek J, Kaski JP, Tuo G, Russo MG, Pacileo G, Milanesi O, Messina D, Marasini M, Cairello F, Formigari R, Brighenti M, Dallapiccola B, Tartaglia M, Marino B.

Int J Cardiol. 2017 Jul 21. pii: S0167-5273(17)31761-8. doi: 10.1016/j.ijcard.2017.07.068. [Epub ahead of print]

PMID: 28768581

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Select item 28733436

 

  1. Erratum for “Lesion-Specific Factors Contributing to In-hospital Costs in Adults With Congenital Heart Disease” Am J Cardiol 2016;117:1821-1825.
[No authors listed]

Am J Cardiol. 2017 Jul 15;120(2):339. doi: 10.1016/j.amjcard.2016.10.004. Epub 2016 Nov 28. No abstract available.

PMID: 27908445

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Select item 28178426

 

  1. Are adults with congenital heart disease informed about their risk for infective endocarditis and treated in accordance to current guidelines?

Bauer UMM, Helm PC, Diller GP, Asfour B, Schlensak C, Schmitt K, Ewert P, Tutarel O.

Int J Cardiol. 2017 Jul 15. pii: S0167-5273(17)31833-8. doi: 10.1016/j.ijcard.2017.07.040. [Epub ahead of print]

PMID: 28743483

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Select item 28560967

 

  1. Adult congenital heart disease in Greece: Preliminary data from the CHALLENGE registry.

Giannakoulas G, Vasiliadis K, Frogoudaki A, Ntellos C, Tzifa A, Brili S, Manginas A, Papaphylactou M, Parcharidou D, Kampouridis N, Pitsis A, Chamaidi A, Kolios M, Papadopoulos G, Douras A, Davlouros P, Ntiloudi D, Karvounis H, Kalangos A, Tsioufis C, Rammos S; CHALLENGE investigators.

Int J Cardiol. 2017 Jul 13. pii: S0167-5273(17)32552-4. doi: 10.1016/j.ijcard.2017.07.024. [Epub ahead of print]

PMID: 28743482

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Select item 28702718

 

  1. Comprehensive Multi-Dimensional MRI for the Simultaneous Assessment of Cardiopulmonary Anatomy and Physiology.

Cheng JY, Zhang T, Alley MT, Uecker M, Lustig M, Pauly JM, Vasanawala SS.

Sci Rep. 2017 Jul 13;7(1):5330. doi: 10.1038/s41598-017-04676-8.

PMID: 28706270 Free PMC Article

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Select item 28711428

 

  1. Employment after heart transplantation among adults with congenital heart disease.

Tumin D, Chou H, Hayes D Jr, Tobias JD, Galantowicz M, McConnell PI.

Congenit Heart Dis. 2017 Jul 12. doi: 10.1111/chd.12513. [Epub ahead of print]

PMID: 28703426

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Select item 28703367

 

  1. A case of Alagille syndrome presenting with chronic cholestasis in an adult.

Kim J, Yang B, Paik N, Choe YH, Paik YH.

Clin Mol Hepatol. 2017 Jul 7. doi: 10.3350/cmh.2016.0057. [Epub ahead of print]

PMID: 28683534 Free Article

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Select item 28688027

 

  1. Factors associated with aortic root dilatation after surgically repaired ventricular septal defect.

Saito C, Fukushima N, Fukushima K, Matsumura G, Ashihara K, Hagiwara N.

Echocardiography. 2017 Jul 6. doi: 10.1111/echo.13622. [Epub ahead of print]

PMID: 28681451

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Select item 28684436

 

  1. Heart Disease and Pregnancy.

Ashrafi R, Curtis SL.

Cardiol Ther. 2017 Jul 5. doi: 10.1007/s40119-017-0096-4. [Epub ahead of print] Review.

PMID: 28681178

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Select item 28675906

 

  1. Clinical implications of eicosapentaenoic acid/arachidonic acid ratio (EPA/AA) in adult patients with congenital heart disease.

Kanoh M, Inai K, Shinohara T, Tomimatsu H, Nakanishi T.

Heart Vessels. 2017 Jul 5. doi: 10.1007/s00380-017-1015-2. [Epub ahead of print]

PMID: 28681101

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Select item 28681132

 

  1. Number of thoracotomies predicts impairment in lung function and exercise capacity in patients with congenital heart disease.

Müller J, Ewert P, Hager A.

J Cardiol. 2017 Jul 4. pii: S0914-5087(17)30157-0. doi: 10.1016/j.jjcc.2017.05.005. [Epub ahead of print]

PMID: 28687271

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Select item 28614966

 

  1. Myocardial bridges: Overview of diagnosis and management.

Rogers IS, Tremmel JA, Schnittger I.

Congenit Heart Dis. 2017 Jul 3. doi: 10.1111/chd.12499. [Epub ahead of print]

PMID: 28675696

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Select item 28678047

 

  1. Imaging of the pulmonary valve in the adults.

Pignatelli RH, Noel C, Reddy SCB.

Curr Opin Cardiol. 2017 Jul 3. doi: 10.1097/HCO.0000000000000436. [Epub ahead of print]

PMID: 28678047

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Select item 28672079

 

  1. Evaluating the Performance of Ultrasound Screening for Congenital Heart Disease: A Descriptive Cohort Study.

Froehlich RJ, Maggio L, Has P, Werner EF, Rouse DJ.

Am J Perinatol. 2017 Jul;34(9):905-910. doi: 10.1055/s-0037-1601309. Epub 2017 Mar 16.

PMID: 28301892

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Select item 28264208

 

  1. Cardiopulmonary Exercise Testing in Adult Congenital Heart Disease.

Mantegazza V, Apostolo A, Hager A.

Ann Am Thorac Soc. 2017 Jul;14(Supplement_1):S93-S101. doi: 10.1513/AnnalsATS.201611-876FR.

PMID: 28375677

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Select item 28701619

 

  1. Cognitive dysfunction in adult CHD with different structural complexity.

Tyagi M, Fteropoulli T, Hurt CS, Hirani SP, Rixon L, Davies A, Picaut N, Kennedy F, Deanfield J, Cullen S, Newman SP.

Cardiol Young. 2017 Jul;27(5):851-859. doi: 10.1017/S1047951116001396. Epub 2016 Oct 18.

PMID: 27751192

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Select item 27680401

 

  1. Gerbode defect and multivalvular dysfunction: Complex complications in adult congenital heart disease.

Ruivo C, Guardado J, Montenegro Sá F, Saraiva F, Antunes A, Correia J, Morais J.

Echocardiography. 2017 Jul;34(7):1099-1101. doi: 10.1111/echo.13561. Epub 2017 May 18.

PMID: 28517030

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Select item 28434921

 

  1. Adolescents with congenital heart disease: their opinions about the preparation for transfer to adult care.

Burström Å, Bratt EL, Frenckner B, Nisell M, Hanséus K, Rydberg A, Öjmyr-Joelsson M.

Eur J Pediatr. 2017 Jul;176(7):881-889. doi: 10.1007/s00431-017-2917-9. Epub 2017 May 16.

PMID: 28508990 Free PMC Article

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Select item 28672919

 

  1. Complete isolation of right subclavian artery supplied by the thoracic aorta and bilateral patent ductus arteriosus.

Ghasemi A, Serati AR, Emami S, Movahed MR.

Future Cardiol. 2017 Jul;13(4):337-344. doi: 10.2217/fca-2016-0062. Epub 2017 Jun 16.

PMID: 28621552

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Select item 28525297

 

  1. Physical activity in adults with congenital heart disease and associations with functional outcomes.

Müller J, Amberger T, Berg A, Goeder D, Remmele J, Oberhoffer R, Ewert P, Hager A.

Heart. 2017 Jul;103(14):1117-1121. doi: 10.1136/heartjnl-2016-310828. Epub 2017 Mar 8.

PMID: 28274955

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Select item 28183791

 

  1. Arterial stiffness and arterial function in adult cyanotic patients with congenital heart disease.

Trojnarska O, Szczepaniak-Chicheł L, Gabriel M, Bartczak-Rutkowska A, Rupa-Matysek J, Tykarski A, Grajek S.

J Cardiol. 2017 Jul;70(1):62-67. doi: 10.1016/j.jjcc.2016.09.003. Epub 2016 Oct 15.

PMID: 27756510

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Select item 27428818

 

  1. Association Between Depressive Symptoms and Exercise Capacity in Patients With Heart Disease: A META-ANALYSIS.

Papasavvas T, Alhashemi M, Micklewright D.

J Cardiopulm Rehabil Prev. 2017 Jul;37(4):239-249. doi: 10.1097/HCR.0000000000000193.

PMID: 27428818

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Select item 28449285

 

  1. Research Review: Childhood chronic physical illness and adult emotional health – a systematic review and meta-analysis.

Secinti E, Thompson EJ, Richards M, Gaysina D.

J Child Psychol Psychiatry. 2017 Jul;58(7):753-769. doi: 10.1111/jcpp.12727. Epub 2017 Apr 27. Review.

PMID: 28449285

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Select item 28540525

 

  1. Survival in portopulmonary hypertension: Outcomes of the United Kingdom National Pulmonary Arterial Hypertension Registry.

Sithamparanathan S, Nair A, Thirugnanasothy L, Coghlan JG, Condliffe R, Dimopoulos K, Elliot CA, Fisher AJ, Gaine S, Gibbs JSR, Gatzoulis MA, E Handler C, Howard LS, Johnson M, Kiely DG, Lordan JL, Peacock AJ, Pepke-Zaba J, Schreiber BE, Sheares KKK, Wort SJ, Corris PA; National Pulmonary Hypertension Service Research Collaboration of the United Kingdom and Ireland.

J Heart Lung Transplant. 2017 Jul;36(7):770-779. doi: 10.1016/j.healun.2016.12.014. Epub 2016 Dec 31.

PMID: 28190786

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Select item 28140493

 

  1. Long-term survival after the Fontan operation: Twenty years of experience at a single center.

Downing TE, Allen KY, Glatz AC, Rogers LS, Ravishankar C, Rychik J, Faerber JA, Fuller S, Montenegro LM, Steven JM, Spray TL, Nicolson SC, Gaynor JW, Goldberg DJ.

J Thorac Cardiovasc Surg. 2017 Jul;154(1):243-253.e2. doi: 10.1016/j.jtcvs.2017.01.056. Epub 2017 Mar 6.

PMID: 28341469

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Select item 28320512

 

  1. An Overview of Cardiac Computed Tomography in Adults With Congenital Heart Disease.

Suranyi P, Varga-Szemes A, Hlavacek AM.

J Thorac Imaging. 2017 Jul;32(4):258-273. doi: 10.1097/RTI.0000000000000281.

PMID: 28632654

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Select item 28632653

 

  1. Imaging Adults With Congenital Heart Disease Part II: Advanced CMR Techniques.

Sahu A, Slesnick TC.

J Thorac Imaging. 2017 Jul;32(4):245-257. doi: 10.1097/RTI.0000000000000274.

PMID: 28632653

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Select item 28632652

 

  1. Utility of Cardiac Magnetic Resonance Imaging in the Management of Adult Congenital Heart Disease.

Muscogiuri G, Secinaro A, Ciliberti P, Fuqua M, Nutting A.

J Thorac Imaging. 2017 Jul;32(4):233-244. doi: 10.1097/RTI.0000000000000280.

PMID: 28632652

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Select item 28632651

 

  1. Imaging in Adult Congenital Heart Disease.

Gaydos SS, Varga-Szemes A, Judd RN, Suranyi P, Gregg D.

J Thorac Imaging. 2017 Jul;32(4):205-216. doi: 10.1097/RTI.0000000000000282.

PMID: 28632651

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Select item 28632650

 

  1. Advanced Cardiac Imaging in Adults With Congenital Heart Disease: The Great Wave.

Hlavacek AM, Schoepf UJ.

J Thorac Imaging. 2017 Jul;32(4):203-204. doi: 10.1097/RTI.0000000000000283. No abstract available.

PMID: 28632650

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Select item 28549022

 

  1. Aneurysms of the azygos vein.

Kreibich M, Siepe M, Grohmann J, Pache G, Beyersdorf F.

J Vasc Surg Venous Lymphat Disord. 2017 Jul;5(4):576-586. doi: 10.1016/j.jvsv.2016.12.012. Epub 2017 Apr 5. Review.

PMID: 28624000

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Select item 28514670

 

  1. Three-dimensional echocardiography in adult congenital heart disease.

Yang HS.

Korean J Intern Med. 2017 Jul;32(4):577-588. doi: 10.3904/kjim.2016.251. Epub 2017 Jun 30. Review.

PMID: 28704916 Free PMC Article

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Select item 27808411

 

  1. Status and Challenges of Care in Africa for Adults With Congenital Heart Defects.

Edwin F, Zühlke L, Farouk H, Mocumbi AO, Entsua-Mensah K, Delsol-Gyan D, Bode-Thomas F, Brooks A, Cupido B, Tettey M, Aniteye E, Tamatey MM, Gyan KB, Tchoumi JCT, Elgamal MA.

World J Pediatr Congenit Heart Surg. 2017 Jul;8(4):495-501. doi: 10.1177/2150135117706340.

PMID: 28696875

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Select item 28696873

 

  1. A Rare Case of Embolic ST-Elevation Myocardial Infarction in an Adult Patient With Repaired Hypoplastic Left Heart Syndrome.

Shamoon R, Habib H, Rampal U, Hamdan A, Bikkina M, Shamoon F.

World J Pediatr Congenit Heart Surg. 2017 Jul;8(4):543-549. doi: 10.1177/2150135116651860. Epub 2016 Jun 13.

PMID: 27297083

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Select item 28697829

 

  1. Intermediate term thrombotic risk in contemporary total cavo-pulmonary connection for single ventricle circulations.

Faircloth JM, Roe O, Alsaied T, Palumbo JS, Vinks A, Veldtman GR.

J Thromb Thrombolysis. 2017 Jul 31. doi: 10.1007/s11239-017-1530-0. [Epub ahead of print]

PMID: 28761995

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Select item 28762167

 

  1. Elevated Myocardial Extracellular Volume Fraction in Duchenne Muscular Dystrophy.

Starc JJ, Moore RA, Rattan MS, Villa CR, Gao Z, Mazur W, Jefferies JL, Taylor MD.

Pediatr Cardiol. 2017 Jul 31. doi: 10.1007/s00246-017-1690-x. [Epub ahead of print]

PMID: 28762167

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Select item 28762166

 

  1. Management of Kawasaki disease in adults.

Denby KJ, Clark DE, Markham LW.

Heart. 2017 Jul 27. pii: heartjnl-2017-311774. doi: 10.1136/heartjnl-2017-311774. [Epub ahead of print] Review.

PMID: 28751537

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Select item 28750632

 

  1. Impact of bileaflet mitral valve prolapse on quantification of mitral regurgitation with cardiac magnetic resonance: a single-center study.

Vincenti G, Masci PG, Rutz T, De Blois J, Prša M, Jeanrenaud X, Schwitter J, Monney P.

J Cardiovasc Magn Reson. 2017 Jul 27;19(1):56. doi: 10.1186/s12968-017-0362-6.

PMID: 28750632 Free PMC Article

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Select item 28749010

 

  1. Exercise Performance and 22q11.2 Deletion Status Affect Quality of Life in Tetralogy of Fallot.

Goldmuntz E, Cassedy A, Mercer-Rosa L, Fogel MA, Paridon SM, Marino BS.

J Pediatr. 2017 Jul 19. pii: S0022-3476(17)30899-5. doi: 10.1016/j.jpeds.2017.06.049. [Epub ahead of print]

PMID: 28734657

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Select item 28724437

 

  1. Non-invasive Evaluation of Right Ventricular Function with Real-Time 3-D Echocardiography.

Chen R, Zhu M, Amacher K, Wu X, Sahn DJ, Ashraf M.

Ultrasound Med Biol. 2017 Jul 14. pii: S0301-5629(17)30202-8. doi: 10.1016/j.ultrasmedbio.2017.05.007. [Epub ahead of print]

PMID: 28716430

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Select item 28707371

 

  1. Cervical artery dissection expands the cardiovascular phenotype in FBN1-related Weill-Marchesani syndrome.

Newell K, Smith W, Ghoshhajra B, Isselbacher E, Lin A, Lindsay ME.

Am J Med Genet A. 2017 Jul 11. doi: 10.1002/ajmg.a.38353. [Epub ahead of print]

PMID: 28696036

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Select item 28696274

 

  1. Editorial commentary: Keeping the congenitally malformed heart in shape.

van den Berg G, Krasemann T, Postma AV.

Trends Cardiovasc Med. 2017 Jul 10. pii: S1050-1738(17)30106-8. doi: 10.1016/j.tcm.2017.06.019. [Epub ahead of print] No abstract available.

PMID: 28735782

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Select item 28691263

 

  1. Clinical implications of eicosapentaenoic acid/arachidonic acid ratio (EPA/AA) in adult patients with congenital heart disease.

Kanoh M, Inai K, Shinohara T, Tomimatsu H, Nakanishi T.

Heart Vessels. 2017 Jul 5. doi: 10.1007/s00380-017-1015-2. [Epub ahead of print]

PMID: 28681101

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Select item 28743382

 

  1. Characterization of micro-RNA Profile in the Blood of Patients with Marfan’s Syndrome.

Abu-Halima M, Ludwig N, Rädle-Hurst T, Keller A, Motsch L, Marsollek I, El Rahman MA, Abdul-Khaliq H, Meese E.

Thorac Cardiovasc Surg. 2017 Jul 5. doi: 10.1055/s-0037-1604083. [Epub ahead of print] No abstract available.

PMID: 28679133

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Select item 28676038

 

  1. Imaging of the pulmonary valve in the adults.

Pignatelli RH, Noel C, Reddy SCB.

Curr Opin Cardiol. 2017 Jul 3. doi: 10.1097/HCO.0000000000000436. [Epub ahead of print]

PMID: 28678047

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Select item 28672079

 

  1. Observations on obesity patterns in tetralogy of Fallot patients from childhood to adulthood.

Briston DA, Sabanayagam A, Zaidi AN.

Cardiol Young. 2017 Jul;27(5):890-894. doi: 10.1017/S1047951116001530. Epub 2016 Sep 22.

PMID: 27655153

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Select item 27640521

 

  1. An Overview of Cardiac Computed Tomography in Adults With Congenital Heart Disease.

Suranyi P, Varga-Szemes A, Hlavacek AM.

J Thorac Imaging. 2017 Jul;32(4):258-273. doi: 10.1097/RTI.0000000000000281.

PMID: 28632654

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Select item 28632651

 

  1. Time in therapeutic range as a marker for thrombotic and bleeding outcomes in Fontan patients.

Faircloth JM, Miner KM, Alsaied T, Nelson N, Ciambarella J, Mizuno T, Palumbo JS, Vinks AA, Veldtman GR.

J Thromb Thrombolysis. 2017 Jul;44(1):38-47. doi: 10.1007/s11239-017-1499-8.

PMID: 28429248

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Select item 28624000

 

  1. Challenges of Cardiac Transplantation Following the Fontan Procedure.

Kirklin JK, Pearce FB, Dabal RJ, Carlo WF Jr, Mauchley DC.

World J Pediatr Congenit Heart Surg. 2017 Jul;8(4):480-486. doi: 10.1177/2150135117714460. No abstract available.

PMID: 28696868

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Select item 27257015

 

 

 

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ACHD Featured Articles of June 2017

ACHD Manuscripts of June 2017 curated by members of the International Society of Adult Congenital Herat Disease

 

Platelet count and mean platelet volume predict outcome in adults with Eisenmenger syndrome.

Martin-Garcia AC, Arachchillage DR, Kempny A, Alonso-Gonzalez R, Martin-Garcia A, Uebing A, Swan L, Wort SJ, Price LC, McCabe C, Sanchez PL, Dimopoulos K, Gatzoulis MA.

Heart. 2017 Jun 29. pii: heartjnl-2016-311144. doi: 10.1136/heartjnl-2016-311144. [Epub ahead of print]

PMID: 28663364

 

Take Home Points:

  • Thrombocytopenia predicts mortality in Eisenmenger syndrome. Patients with a platelet count of <100×10⁹/L had double the mortality of those with higher platelet counts.
  • Raised mean platelet volume, very high hemoglobin (>220 g/L) or very low hemoglobin (<130 g/L), but not platelet count were associated with increased thrombotic events

 

jokhadar-maanCommentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch:   Eisenmenger syndrome (pulmonary hypertension with cyanosis (sat < 90%) and presence of a large intracardiac or extracardiac shunt) is associated high hemoglobin (erythrocytosis) and thrombocytopenia. Erythrocytosis as an expected compensatory response to cyanosis. Eisenmenger syndrome is also associated with thrombocytopenia that is caused by whole megakaryocytes bypassing the lungs via right to left shunting.  Megakaryocytes are normally fragmented into platelets in the pulmonary circulation.  Mean platelet volume (MPV) reflects platelet size and higher MPV is associated with higher platelet aggregation. Larger platelet may have more aggregation capacity and higher MPV is an independent risk factor for myocardial infarction and stroke in patients with coronary disease.

To further elucidate the role of erythrocytosis, thrombocytopenia, and MPV in Eisenmenger syndrome, Dr. Martin-Garcia and colleagues from the UK and Spain performed a retrospective analysis of 226 Eisenmenger syndrome patients who were seen at the Royal Brompton Hospital between 2005 in 2016 for median follow-up of about 8 years.  About a third of patients were male and the mean age was about 35 years.  Fifty percent of patients had complex anatomy, 43% had post tricuspid shunt, and about 7% of patients had a pre-tricuspid shunt.  About a quarter of patients were on pulmonary vasodilator therapy at baseline and about 58% of patients were started on pulmonary visit dilator therapy during follow-up.

About 44% of patients had Down syndrome, who tended to be younger (mean age 30), more complex anatomy (72%), similar oxygen saturations, similar aspirin use, and similar clopidogrel use.  However, Down syndrome patients were less likely to be on warfarin, novel anticoagulants, iron supplementation (despite more frequent iron deficiency), pulmonary vasodilator therapy, and also tended to have higher hemoglobin and lower platelet concentrations but similar MPV.

The mean hemoglobin concentration was 193.8 g/L; 90% of patients had an hemoglobin > 153.0 g/L, 75% > 172.3 g/L and 46.5% > 200 g/L. The mean platelet count was at low-normal levels (152.6×10⁹/L). About a quarter of patients had a platelet count of <100×10⁹/L, whereas 5.3% had platelet counts of <50×10⁹/L. The mean MPV was 9.6±1.2 fL. However, only about 5% of patients had MPV >11.5 fL.

As one would expect, lower oxygen saturations were associated with higher hemoglobin, lower platelet count was associated with higher hemoglobin, and lower platelet count was also associated with higher MPV.  MPV and hemoglobin concentrations had a positive correlation.  These patterns were present in both Down syndrome patients and non-Down syndrome patients.

Of interest, iron deficiency was present and about 45% of patients in this study, who had lower hemoglobin and higher platelet counts but similar MPV to those patients without iron deficiency.

Patients with a platelet count of <100×10⁹/L had double the mortality. MPV was not predictive of mortality. Patient with very high (>220 g/L) or very low (<130 g/L) Hb concentration were both associated with about a 4-fold increase in thrombotic events. (HR about 3.9 and 4.8, respectively). MPV >9.5 fL was associated with a 3-fold increase in thrombotic events (HR 3.5).

An interesting point made in the discussion of this paper was that iron deficiency, which is common in Eisenmenger syndrome patients, is associated with increased mortality in this population.  Iron replacement, though recommended, could worsen thrombocytopenia though this was not associated with increasing bleeding events.

Hemoglobin, platelet count, and MPV may be helpful in Eisenmenger syndrome risk stratification and could guide treatment. However, additional studies are needed.

 

 

 

How Pregnancy Impacts Adult Cyanotic Congenital Heart Disease: A Multicenter Observational Study.

Ladouceur M, Benoit L, Basquin A, Radojevic J, Hauet Q, Hascoet S, Moceri P, Le Gloan L, Amedro P, Lucron H, Richard A, Gouton M, Nizard J.

Circulation. 2017 Jun 13;135(24):2444-2447. doi: 10.1161/CIRCULATIONAHA.116.027152. No abstract available.

PMID: 28606952

 

Take Home Points:

  • Selected patients with cyanotic CHD can have successful pregnancies, especially when there’s adequate pre-pregnancy counseling and good follow-up with a multi-disciplinary team.
  • Although the risks to the mother are lower in this cohort than previously described, the potential for late chronic heart failure remains.
  • Further larger cohort studies are required to validate these findings.

 

Blanche CupidoCommentary by Dr. Blanche Cupido (Cape Town), section editor of ACHD Journal WatchMore women with unrepaired cyanotic CHD are surviving into adulthood. They are at high risk for both maternal and fetal complications during pregnancy. This study represents a retrospective review of 51 cyanotic patients presenting between 1997 and 2015 from 11 referral centers in the M3C French network (Center de reference des Malformations Cardiaques Congenitales Complexes). Patients with central cyanosis at rest or exercise and with an underlying ‘compatible’ lesion were included. Twenty patients were excluded because of pulmonary hypertension. The remaining 31 patients were divided into 4 categories according to their mechanism for cyanosis: 1. Ventricular right to left shunt and systemic LV, 2. Univentricular heart, 3. Ventricular right to left shunt and systemic RV, Atrial right to left shunt.

 

Seventy-one pregnancies occurred in 31 patients. 48% (n=15) had previous palliative shunts but had residual cyanosis. All but 2 patients had normal systemic ventricular function.  Cardiovascular complications (in 13 pregnancies) documented were: 3 with heart failure, 2 with de novo SVT, 7 with worsening hypoxemia. There was no distinct association between baseline maternal characteristics and outcomes.

No thromboembolic complications occurred during the pregnancies (13 patients received anticoagulation and 5 received antiplatelet agents) – these numbers are too small to draw any definitive conclusions. One patient developed infective endocarditis.

There were no deaths during the follow-up period (median 3.8 years).  Thirteen percent (n=4) developed late heart failure with a deterioration in systemic ventricular function. The study did not comment on fetal loss or fetal complications.

 

Longitudinal Outcomes of Patients with Single Ventricle after the Fontan Procedure.

Atz AM, Zak V, Mahony L, Uzark K, D’agincourt N, Goldberg DJ, Williams RV, Breitbart RE, Colan SD, Burns KM, Margossian R, Henderson HT, Korsin R, Marino BS, Daniels K, McCrindle BW; Pediatric Heart Network Investigators.

J Am Coll Cardiol. 2017 Jun 6;69(22):2735-2744. doi: 10.1016/j.jacc.2017.03.582.

PMID: 28571639

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Select item 28571637

 

Take Home Points:

  • In the Fontan 3 study, a large, longitudinal cohort of Fontan patients (n=373 who consented to present study out of initial 546 enrolled in Fontan 1 study) followed by the Pediatric Heart Network (PHN) over 12 years, transplant-free survival was 90% at a mean age of 21.2 +5 years (mean 17.8 years after Fontan procedure).
  • Exercise capacity, on average, decreased from the initial Fontan 1 to Fontan 3 study, from a peak VO2 of 69% predicted to 61% predicted.
  • Risk of death or cardiac transplantation from Fontan 1 to Fontan 3 study was associated with poorer ventricular performance (either reduced EF or significant ventricular dilation), but was independent of ventricular morphology, age of the patient, or type of Fontan connection.
  • Worse measured exercise capacity correlated well with a lower Pediatric Quality of Life Inventory physical summary score.
  • Among the seven centers in the PHN study, there was significant variability regarding mortality outcome as well as percentage of patients receiving transplantation. A total of 21 patients in the study died without transplant, and 23 underwent transplantation.  Rate of transplantation ranged from 0% to 21.1%.  The center with zero transplants had the highest overall mortality rate (13.6%).  The center with the highest transplant rate (21.1%) had the second highest death rate (10.5%).  This level of discrepancy highlights need for more standardization in criteria for cardiac transplantation in the Fontan population.

 

Kay_William_MD.16.CVaCommentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch:  Long term data in Fontan patients is lacking as most case series either have very small numbers of patients and/or have limited number of years of follow-up.  A few larger series encompass several surgical strategies and eras of surgery, and as a result using conclusions from these studies to care for younger patients in the modern surgical era may not be appropriate.

Over a decade ago, the Pediatric Heart Network (PHN) sought to start a longitudinal study of Fontan outcomes.  Out of 7 centers, the PHN initially enrolled 546 patients in the Fontan 1 study; this data was published long ago, and interval data of 416 patients was published in the Fontan 2 study more recently.  The present study, the Fontan 3 study, included 373 surviving patients who consented to remain enrolled in the study, or a total of 80% of patients still eligible to be in the study.  The present study population was enrolled at 21.2 + 3.5 years, at an average of 17.8 + 3.4 years from initial Fontan procedure.

Several outcomes were studied, including hard outcome of death or cardiac transplantation, quality of life (QOL) measures, exercise capacity variables, measurements of ventricular performance.  QOL was measured on a standardized test, the Pediatric Quality of Life Inventory, and/or the SF-36 for those > 19 years of age upon enrollment.  Other variables need for interval procedures, including need for medical or other treatment of arrhythmia, surgery, placement of cardiac rhythm device, or catheter intervention, as well as presence of significant noncardiac comorbidities including stroke, seizures, thrombus, protein-losing enteropathy, cirrhosis, or plastic bronchitis.  There was a significant increase from Fontan 1 to Fontan 3 of all comorbidties as well as need for catheterization and cardiac surgery.  Cirrhosis was found in only 8% of the cohort; however this paper does not mention how exactly cirrhosis was defined, nor did it mention any detail about liver biopsy, elastography, or imaging assessment of cirrhosis.

Most patients completed a QOL inventory (n=364), of which 74% had all laboratory testing performed, and 223 had maximal effort cardiopulmonary exercise testing.  The most common cardiac surgical procedure was placement or revision of a cardiac rhythm device, the majority of which were pacemakers (only 9/373 patients underwent interval placement of an ICD).

Overall systemic ventricle ejection fraction went down slightly during the study, from 58 + 11% to 55 + 10% (p < 0.001).  A lower QOL score correlated with poorer exercise performance.  Mean BNP levels were low in the population, although they were significantly higher at time of Fontan 3 compared with Fontan 1 cohort (18.0 pg/ml vs 13.0 pg/ml; p < 0.001).  Although overall levels were low, interestingly, mean BNP levels were statistically high in patients with atriopulmonary connection compared with intracardiac lateral tunnel, which in turn was higher than that seen in extracardiac conduit.  When the QOL scores were normalized on a 0-100 scale (with 100 representing best QOL), there was an association between higher logBNP and lower QOL score.  No association was found between QOL score and echocardiographic variables.

Hard outcomes of death or transplant represented approximately 10% of the initial Fontan study population, with a total of 54 patients having died (n=31) or undergoing transplant (n=23) by time of Fontan 3 study.  The authors found no association of high risk of death/transplant with patient sex, age, race, ethnicity, ventricular morphology, or type of Fontan procedure.  On univariate analysis, a poorer QOL physical summary score, higher BNP value, lack of completion of exercise test, lower resting oxygen saturation, lower predicted VO2 at anaerobic threshold, and poorer ventricular performance were associated with a higher risk of death/transplant.

Some information regarding socioeconomic status was included.  As a continuous variable, family income did not correlate with risk of death/transplantation.  However, when the population was dichotomized into low-income and high-income groups, a family income > $100,000 annually had better outcomes.  Overall, 96% of the study population maintained either private or public health insurance during the study.

Most of the study population is entering the age of “transition” from pediatrics to adulthood.  Patients at time of Fontan 3 were more likely to have seen an adult cardiologist in the past 2 years (35% versus 30%) as well as to have seen an adult congenital cardiologist (33% to 24%).  Female patients had a higher likelihood of having seen an OB-Gyn (41% versus 32%), but patients were found to receive less educational support compared to the prior study (decrease from 36% to 26%).

There was significant discrepancy from center to center regarding percentage of patients undergoing transplantation; this finding emphasizes the need for further research to better refine criteria for heart transplant evaluation in the Fontan population.

June ACHD 1

June ACHD 2

 

Mortality in pulmonary arterial hypertension due to congenital heart disease: serial changes improve prognostication.

Schuijt MTU1Blok IM2Zwinderman AH1van Riel ACMJ2Schuuring MJ2de Winter RJ1Duijnhouwer AL3van Dijk APJ3Mulder BJM2Bouma BJ4.

Int J Cardiol. 2017 Jun 1. pii: S0167-5273(17)30321-2. doi: 10.1016/j.ijcard.2017.05.101. [Epub ahead of print]

PMID: 28606655

 

Take Home Points:

  • Prognostic value of baseline parameters in patients with congenital heart disease and pulmonary hypertension weakens over time as the patient’s condition changes.
  • Serial changes in WHO functional class, 6-minute-walk distance, SaO2 at peak exercise, NT pro BNP and TAPSE were more potent than baseline parameters at predicting mortality.

 

Leong Ming ChernCommentary from Dr. MC Leong (Kuala Lumpur), section editor of ACHD Journal Watch: Baseline parameters of patients with congenital heart disease and pulmonary arterial hypertension (PAH-CHD) have been shown to be associated with mortality. However, the prognostic values of these baseline parameters weaken over time as the patient’s clinical condition changes and hence serial changes in clinical parameters should be a more apt way to prognosticate patients with PAH-CHD. This study aimed to investigate the prognostic value of serial changes in standard clinical parameters in PAD-CHD.

92 patients with PAH-CHD, either repaired or not, who were referred to 2 study centres in the Netherlands between January 2005 and May 2016 were included. Patients were given a standard monotherapy of either Bosentan (before 2014) or Macitentan (after 2014) after the first clinic visit and they were followed up 6 monthly with standard evaluation protocol which included SaO2 at peak exercise, six-minute-walk distance, biochemical (haemoglobin, urea, NT pro BNP and creatinine) and echocardiographic (TAPSE, RV S’ and echocardiogram-derived systolic pulmonary arterial pressure) parameters.

During a median follow up of 6.0 (IQR: 3.7-9.3) years, 35 (38%) patients died. Onset of WHO functional class IV (HR : 18.34), decrease of six-minute-walk distance (HR : 0.65 per 50m), SaO2 at peak exercise (HR : 0.74 per 5%), NT pro BNP (HR : 2.25 per 1000ng/l) and TAPSE (HR: 0.80 per 0.5cm) were significantly associated with higher mortality. Interestingly, a survival model, based on serial changes of these parameters in time-dependent univariate Cox regression, showed 25% mortality in 0.06 years with WHO functional class IV reached; 1.3 years with an increase of NT pro BNP of 1000ng/l; 3.7 years with TAPSE decreased 0.5cm; 4.4 years with six-minute-walk distance decreasing 50m; and 4.5 years with SaO2 at peak exercise decreasing 5%. (Figure 1) Impact of the serial changes was also shown in Table 3.

June ACHD 3 June ACHD 4 June ACHD 5

Undoubtedly, the use of serial changes in the parameters during follow up better reflects mortality in this group of patient and these parameters were shown to be useful to aid risk stratification of patients and subsequently guide medical therapy.

In this study, first year mortality was unusually high. The authors reasoned that this may due to the late initiation of PAH targeted therapy and older aged cohort. Meanwhile, the practice of starting all PAH-CHD on endothelin receptor antagonist at the first visit, which is practiced in the study centre is not conventional, especially in patients who are haemodynamically balanced. It would be interesting if the authors could analyse difference in serial changes in parameters in the patients with corrected PAH-CHD compared to those without, as patients with corrected PAH-CHD tended to fare worse than those without correction.

 

 

Effect of Fontan-Associated Morbidities on Survival with Intact Fontan Circulation.

Allen KY, Downing TE, Glatz AC, Rogers LS, Ravishankar C, Rychik J, Fuller S, Montenegro LM, Steven JM, Spray TL, Nicolson SC, Gaynor JW, Goldberg DJ.

Am J Cardiol. 2017 Jun 1;119(11):1866-1871. doi: 10.1016/j.amjcard.2017.03.004. Epub 2017 Mar 16.

PMID: 28385177

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Select item 28161345

 

Take Home Points:

  • Substantial mild plus pre-Fontan AV valve insufficiency is a significant risk factor for development of long-term comorbidities.
  • Ventricular morphology does not appear to differentially contribute to increased risks of cardiovascular comorbidities.
  • 20-year survival without comorbidities in TCPC is reported as 47% in this cohort.

 

 

Moe_Tabitha-WEBCommentary by Dr. Tabitha G. Moe (Phoenix), section editor of ACHD Journal WatchSurvival after the Fontan operation has improved over the course of time. Fontan operations can reasonably be categorized into separate and distinct eras. This paper from Children’s Hospital of Philadelphia [CHOP] outlines medical comorbidities in those patients in the second and third eras of Fontan or total cavopulmonary connections, specifically there are not any subjects who underwent atrio-pulmonary Fontan.  This review again emphasizes that ventricular morphology does not seem to be associated with long-term outcomes. They report a 20-year post-Fontan survival of 47% without PLE, PB, thrombosis, or tachyarrhythmia, consistent with previously reposted single-center studies at Mayo Clinic, and Australia-New Zealand. This study is valuable in that it defines the outcomes of 773 modern era Fontans.  Nearly 50% of the patients did not have any recent follow up at CHOP requiring the re-identification and contact of a significant number of patients, and in fact the records were collected from 70 centers worldwide. They report 79 deaths, 25 heart transplants, and 10 Fontan takedowns.  Of this cohort 163 experienced at least one component of the composite medical morbidity: PLE, PB, thrombosis, or tachyarrhythmia. Pre-operative AV valve insufficiency more than mild, post-op pleural drainage >14 days, and longer cross clamp times were associated with an increased risk of the composite risk factors. For those subjects with the ideal outcomes, and none of the composite risks experienced freedom from Fontan failure at 20 years was 98%.  The Fontan cohort is relatively young with average age at Fontan completion of 2.3 years, 50% of the cohort were HLHS. 90% of their cohort were initially fenestrated.

 June ACHD 6

 

 

 

The central figure for this article represents the remarkable outcomes of those without any reported comorbidities.  There is a marked decline in long-term outcomes with the addition of any one morbidity.  One of the unfortunate characteristics of this cohort was the reported 229 subjects who were lost to follow up.  This is a significant number of single number of single ventricle subjects, operated on at a prestigious institution with highly organized follow-up plans, who still managed to be lost to follow up. Further analysis of this subcohort of patients may reveal opportunities to maintain life-time congenital cardiac care at all centers and represents a valuable tool for identifying patients who may be at risk to being lost to follow up. Although thrombosis remains a risk factor long-term the highest risk time frame is perioperatively, and maintaining anti-platelet or anti-thrombotic management during this high-risk window continues to be of importance. Lateral tunnel, and extracardiac conduits seem to have similar risks for development of atrial arrhythmias, however, this may be further elucidated with a longer follow up period. AV-valve insufficiency is a more significant risk factor than ventricular morphology in the long-term outcomes, again these variables may diverge with longer term follow up.  Interestingly, the hemodynamic findings on pre-Fontan catheterization with pulmonary artery pressure variables did not contribute to long-term outcomes. This composite outcome allows for additional speculation in an otherwise very complex and heterogenous cohort to evaluate and the authors are to be commended for this unique approach.

 

ACHD June 2017

 

  1. Platelet count and mean platelet volume predict outcome in adults with Eisenmenger syndrome.

Martin-Garcia AC, Arachchillage DR, Kempny A, Alonso-Gonzalez R, Martin-Garcia A, Uebing A, Swan L, Wort SJ, Price LC, McCabe C, Sanchez PL, Dimopoulos K, Gatzoulis MA.

Heart. 2017 Jun 29. pii: heartjnl-2016-311144. doi: 10.1136/heartjnl-2016-311144. [Epub ahead of print]

PMID: 28663364

 

Select item 28669434

 

  1. Determinants of quality of life in adults with CHD: an Australian cohort.

Eaton SL, Wang Q, Menahem S.

Cardiol Young. 2017 Jun 27:1-6. doi: 10.1017/S1047951117000816. [Epub ahead of print]

PMID: 28651679

Similar articles

Select item 28653469

 

  1. Illness perceptions in adult congenital heart disease: A multi-center international study.

Rassart J, Apers S, Kovacs AH, Moons P, Thomet C, Budts W, Enomoto J, Sluman MA, Wang JK, Jackson JL, Khairy P, Cook SC, Subramanyan R, Alday L, Eriksen K, Dellborg M, Berghammer M, Johansson B, Rempel GR, Menahem S, Caruana M, Veldtman G, Soufi A, Fernandes SM, White KS, Callus E, Kutty S, Luyckx K; APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD).

Int J Cardiol. 2017 Jun 26. pii: S0167-5273(17)30122-5. doi: 10.1016/j.ijcard.2017.06.072. [Epub ahead of print]

PMID: 28669511

 

Select item 28652574

 

  1. Impact on Survival of Warfarin in Patients with Pulmonary Arterial Hypertension Receiving Subcutaneous Treprostinil.

Ascha M, Zhou X, Rao Y, Minai OA, Tonelli AR.

Cardiovasc Ther. 2017 Jun 23. doi: 10.1111/1755-5922.12281. [Epub ahead of print]

PMID: 28643420

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Select item 28643441

 

  1. Impact of growing cohorts of adults with con-genital heart disease on clinical workload: a 20-year experience at a tertiary care centre.

Padrutt M, Bracher I, Bonassin F, Santos Lopes B, Gruner C, Stämpfli SF, Wolber T, Kretschmar O, Oxenius A, De Pasquale G, Seeliger T, Lüscher TF, Attenhofer Jost C, Greutmann M.

Swiss Med Wkly. 2017 Jun 21;147:w14443. doi: smw.2017.14443. eCollection 2017 Jun 21.

PMID: 28634971 Free Article

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Select item 28645012

 

  1. Learning and Memory in Adolescents With Critical Biventricular Congenital Heart Disease.

Cassidy AR, Newburger JW, Bellinger DC.

J Int Neuropsychol Soc. 2017 Jun 20:1-13. doi: 10.1017/S1355617717000443. [Epub ahead of print]

PMID: 28629487

Similar articles

Select item 28632506

 

  1. Impact of age and sex on survival and causes of death in adults with congenital heart disease.

Oliver JM, Gallego P, Gonzalez AE, Garcia-Hamilton D, Avila P, Alonso A, Ruiz-Cantador J, Peinado R, Yotti R, Fernandez-Aviles F.

Int J Cardiol. 2017 Jun 19. pii: S0167-5273(17)32510-X. doi: 10.1016/j.ijcard.2017.06.060. [Epub ahead of print]

PMID: 28648355

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Select item 28631106

 

  1. Transthoracic 3D echocardiographic left heart chamber quantification in patients with bicuspid aortic valve disease.

van den Hoven AT, Mc-Ghie JS, Chelu RG, Duijnhouwer AL, Baggen VJM, Coenen A, Vletter WB, Dijkshoorn ML, van den Bosch AE, Roos-Hesselink JW.

Int J Cardiovasc Imaging. 2017 Jun 19. doi: 10.1007/s10554-017-1192-1. [Epub ahead of print]

PMID: 28631106

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Select item 28625174

 

  1. The Role of Cardiopulmonary Exercise Testing for Decision Making in Patients with Repaired Tetralogy of Fallot.

Dallaire F, Wald RM, Marelli A.

Pediatr Cardiol. 2017 Jun 16. doi: 10.1007/s00246-017-1656-z. [Epub ahead of print] Review.

PMID: 28623384

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Select item 28622179

 

  1. Effects of persistent Fontan fenestration patency on cardiopulmonary exercise testing variables.

Heal ME, Jackson LB, Atz AM, Butts RJ.

Congenit Heart Dis. 2017 Jun 15. doi: 10.1111/chd.12451. [Epub ahead of print]

PMID: 28618202

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Select item 28618149

 

  1. Adult congenital heart disease: magnitude of the problem.

Thakkar AN, Chinnadurai P, Lin CH.

Curr Opin Cardiol. 2017 Jun 15. doi: 10.1097/HCO.0000000000000429. [Epub ahead of print]

PMID: 28617685

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Select item 28318658

 

  1. How Pregnancy Impacts Adult Cyanotic Congenital Heart Disease: A Multicenter Observational Study.

Ladouceur M, Benoit L, Basquin A, Radojevic J, Hauet Q, Hascoet S, Moceri P, Le Gloan L, Amedro P, Lucron H, Richard A, Gouton M, Nizard J.

Circulation. 2017 Jun 13;135(24):2444-2447. doi: 10.1161/CIRCULATIONAHA.116.027152. No abstract available.

PMID: 28606952

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Select item 28608969

 

  1. Effect of iloprost on biomarkers in patients with congenital heart disease-pulmonary arterial hypertension.

Li XY, Zheng Y, Long Y, Zhang X, Zhang L, Tian D, Zhou D, Lv QZ.

Clin Exp Pharmacol Physiol. 2017 Jun 13. doi: 10.1111/1440-1681.12796. [Epub ahead of print]

PMID: 28608969

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Select item 28608519

 

  1. Pregnancy, Marfan Syndrome and Type B Aortic Dissection.

Johnson MR, Roos Hesselink JW.

BJOG. 2017 Jun 10. doi: 10.1111/1471-0528.14781. [Epub ahead of print]

PMID: 28600893

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Select item 28601813

 

  1. Atrial fibrillation in pregnancy: a growing challenge.

Katsi V, Georgiopoulos G, Marketou M, Oikonomou D, Parthenakis F, Makris T, Nihoyannopoulos P, Vardas P, Tousoulis D.

Curr Med Res Opin. 2017 Jun 8:1-8. doi: 10.1080/03007995.2017.1330257. [Epub ahead of print]

PMID: 28498066

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Select item 28596305

 

  1. Genotype-phenotype correlations in Marfan syndrome.

Landis BJ, Veldtman GR, Ware SM.

Heart. 2017 Jun 8. pii: heartjnl-2017-311513. doi: 10.1136/heartjnl-2017-311513. [Epub ahead of print] No abstract available.

PMID: 28596305

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Select item 28532196

 

  1. Left ventricular thrombus or pseudothrombus? A rare cardiac CT artifact.

Rodrigues JCL, Yeong M, Curtis SL, Lyen SM.

Eur Heart J Cardiovasc Imaging. 2017 Jun 6. doi: 10.1093/ehjci/jex149. [Epub ahead of print] No abstract available.

PMID: 28586404

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Select item 28571640

 

  1. Cardiorespiratory Fitness, Not the Severity of the Condition, Dictates Late Outcomes After Fontan Procedures.

d’Udekem Y.

J Am Coll Cardiol. 2017 Jun 6;69(22):2745-2747. doi: 10.1016/j.jacc.2017.03.581. No abstract available.

PMID: 28571640

Similar articles

Select item 28571639

 

  1. Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure.

Atz AM, Zak V, Mahony L, Uzark K, D’agincourt N, Goldberg DJ, Williams RV, Breitbart RE, Colan SD, Burns KM, Margossian R, Henderson HT, Korsin R, Marino BS, Daniels K, McCrindle BW; Pediatric Heart Network Investigators.

J Am Coll Cardiol. 2017 Jun 6;69(22):2735-2744. doi: 10.1016/j.jacc.2017.03.582.

PMID: 28571639

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Select item 28571637

 

  1. Mitral valve prolapse and Marfan syndrome.

Thacoor A.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12467. [Epub ahead of print] Review.

PMID: 28580713

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Select item 28580712

 

  1. Clinical analyses of 383 cases with maternal cardiac diseases.

Aydin E, Ozyuncu O, Kasapoglu D, Orgul G, Ozer N, Yurdakok M, Beksac MS.

J Perinat Med. 2017 Jun 5. pii: /j/jpme.ahead-of-print/jpm-2017-0023/jpm-2017-0023.xml. doi: 10.1515/jpm-2017-0023. [Epub ahead of print]

PMID: 28622145

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Select item 28582432

 

  1. Feasibility of exercise stress echocardiography and myocardial response in patients with repaired congenital heart disease.

Hasan BS, Lunze FI, Alvi N, Shafer KM, Rhodes J.

Am Heart J. 2017 Jun;188:1-10. doi: 10.1016/j.ahj.2017.02.029. Epub 2017 Feb 23.

PMID: 28577664

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  1. Transition of Care in Congenital Heart Disease: Ensuring the Proper Handoff.

Lee A, Bailey B, Cullen-Dean G, Aiello S, Morin J, Oechslin E.

Curr Cardiol Rep. 2017 Jun;19(6):55. doi: 10.1007/s11886-017-0859-5. Review.

PMID: 28477319

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Select item 28432659

 

  1. Current Role of Blood and Urine Biomarkers in the Clinical Care of Adults with Congenital Heart Disease.

Rajpal S, Alshawabkeh L, Opotowsky AR.

Curr Cardiol Rep. 2017 Jun;19(6):50. doi: 10.1007/s11886-017-0860-z. Review.

PMID: 28432659

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Select item 28306681

 

  1. Evaluation of athletes with complex congenital heart disease.

Bates BA, Richards C, Hall M, Kerut EK, Campbell W, McMullan MR.

Echocardiography. 2017 Jun;34(6):934-936. doi: 10.1111/echo.13562. Epub 2017 May 12.

PMID: 28497583

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Select item 28386957

 

  1. Hypoplasia of the posterior mitral leaflet: A rare cause of mitral regurgitation in adulthood.

Bacich D, Braggion G, Faggian G.

Echocardiography. 2017 Jun;34(6):949-950. doi: 10.1111/echo.13537. Epub 2017 Apr 7.

PMID: 28386957

Similar articles

Select item 28370416

 

  1. Right ventricular function and N-terminal pro-brain natriuretic peptide levels in adult patients with simple dextro-transposition of the great arteries.

Martínez-Quintana E, Marrero-Negrín N, Gopar-Gopar S, Rodríguez-González F.

Echocardiography. 2017 Jun;34(6):876-880. doi: 10.1111/echo.13526. Epub 2017 Mar 29.

PMID: 28370416

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Select item 28533195

 

  1. Valvular Heart Disease in Adults: Etiologies, Classification, and Diagnosis.

Hollenberg SM.

FP Essent. 2017 Jun;457:11-16.

PMID: 28671804

 

Select item 28620608

 

  1. Heart transplantation for adults with congenital heart disease: current status and future prospects.

Matsuda H, Ichikawa H, Ueno T, Sawa Y.

Gen Thorac Cardiovasc Surg. 2017 Jun;65(6):309-320. doi: 10.1007/s11748-017-0777-x. Epub 2017 Apr 24. Review.

PMID: 28439697

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Select item 27831545

 

  1. Aortopathies in adult congenital heart disease and genetic aortopathy syndromes: management strategies and indications for surgery.

Kuijpers JM, Mulder BJ.

Heart. 2017 Jun;103(12):952-966. doi: 10.1136/heartjnl-2015-308626. Epub 2017 Mar 7. No abstract available.

PMID: 28270426

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Select item 28259844

 

  1. Cyanotic congenital heart disease and atherosclerosis.

Tarp JB, Jensen AS, Engstrøm T, Holstein-Rathlou NH, Søndergaard L.

Heart. 2017 Jun;103(12):897-900. doi: 10.1136/heartjnl-2016-311012. Epub 2017 Mar 4. Review.

PMID: 28259844

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Select item 27993911

 

  1. The management of the third stage of labour in women with heart disease.

Cauldwell M, Steer PJ, Swan L, Uebing A, Gatzoulis MA, Johnson MR.

Heart. 2017 Jun;103(12):945-951. doi: 10.1136/heartjnl-2016-310607. Epub 2016 Dec 19.

PMID: 27993911

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Select item 28528723

 

  1. Prognostic predictive value of gene mutations in Japanese patients with hypertrophic cardiomyopathy.

Chida A, Inai K, Sato H, Shimada E, Nishizawa T, Shimada M, Furutani M, Furutani Y, Kawamura Y, Sugimoto M, Ishihara J, Fujiwara M, Soga T, Kawana M, Fuji S, Tateno S, Kuraishi K, Kogaki S, Nishimura M, Ayusawa M, Ichida F, Yamazawa H, Matsuoka R, Nonoyama S, Nakanishi T.

Heart Vessels. 2017 Jun;32(6):700-707. doi: 10.1007/s00380-016-0920-0. Epub 2016 Nov 24.

PMID: 27885498

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Select item 28239760

 

  1. Mortality in pulmonary arterial hypertension due to congenital heart disease: Serial changes improve prognostication.

Schuijt MTU, Blok IM, Zwinderman AH, van Riel ACMJ, Schuuring MJ, de Winter RJ, Duijnhouwer AL, van Dijk APJ, Mulder BJM, Bouma BJ.

Int J Cardiol. 2017 Jun 1. pii: S0167-5273(17)30321-2. doi: 10.1016/j.ijcard.2017.05.101. [Epub ahead of print]

PMID: 28606655

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Select item 28132780

 

  1. Early mortality and concomitant procedures related to Fontan conversion: Quantitative analysis.

Brida M, Baumgartner H, Gatzoulis MA, Diller GP.

Int J Cardiol. 2017 Jun 1;236:132-137. doi: 10.1016/j.ijcard.2017.01.111. Epub 2017 Jan 25.

PMID: 28132780

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Select item 28635060

 

  1. Endothelin inhibitors lower pulmonary vascular resistance and improve functional capacity in patients with Fontan circulation.

Agnoletti G, Gala S, Ferroni F, Bordese R, Appendini L, Pace Napoleone C, Bergamasco L.

J Thorac Cardiovasc Surg. 2017 Jun;153(6):1468-1475. doi: 10.1016/j.jtcvs.2017.01.051. Epub 2017 Feb 10.

PMID: 28283234

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Select item 28268011

 

  1. Pregnancy in Adult Congenital Heart Disease: Special Delivery.

Davidson WR Jr.

JAMA Cardiol. 2017 Jun 1;2(6):671-672. doi: 10.1001/jamacardio.2017.0365. No abstract available.

PMID: 28403448

Similar articles

Select item 28403428

 

  1. Maternal and Fetal Outcomes of Admission for Delivery in Women With Congenital Heart Disease.

Hayward RM, Foster E, Tseng ZH.

JAMA Cardiol. 2017 Jun 1;2(6):664-671. doi: 10.1001/jamacardio.2017.0283.

PMID: 28403428

Similar articles

Select item 28241248

 

  1. Value of Cardiovascular Magnetic Resonance Imaging in Noninvasive Risk Stratification in Tetralogy of Fallot.

Bokma JP, de Wilde KC, Vliegen HW, van Dijk AP, van Melle JP, Meijboom FJ, Zwinderman AH, Groenink M, Mulder BJM, Bouma BJ.

JAMA Cardiol. 2017 Jun 1;2(6):678-683. doi: 10.1001/jamacardio.2016.5818.

PMID: 28241248

Similar articles

Select item 28384795

 

  1. Transition of Adolescents and Young Adults with Congenital Heart Disease: Challenges, Progress, and Future Improvements.

Nicolarsen J.

Pediatr Ann. 2017 Jun 1;46(6):e224-e228. doi: 10.3928/19382359-20170519-02.

PMID: 28599027

Similar articles

Select item 28500413

 

  1. Impaired Pulmonary Function is an Additional Potential Mechanism for the Reduction of Functional Capacity in Clinically Stable Fontan Patients.

Turquetto ALR, Canêo LF, Agostinho DR, Oliveira PA, Lopes MICS, Trevizan PF, Fernandes FLA, Binotto MA, Liberato G, Tavares GMP, Neirotti RA, Jatene MB.

Pediatr Cardiol. 2017 Jun;38(5):981-990. doi: 10.1007/s00246-017-1606-9. Epub 2017 May 12.

PMID: 28500413

 

  1. Red Flags for Maltese Adults with Congenital Heart Disease: Poorer Dental Care and Less Sports Participation Compared to Other European Patients-An APPROACH-IS Substudy.

Caruana M, Apers S, Kovacs AH, Luyckx K, Thomet C, Budts W, Sluman M, Eriksen K, Dellborg M, Berghammer M, Johansson B, Soufi A, Callus E, Moons P, Grech V; APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD).

Pediatr Cardiol. 2017 Jun;38(5):965-973. doi: 10.1007/s00246-017-1604-y. Epub 2017 Mar 24.

PMID: 28341902

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Select item 28210768

 

  1. The Lymphatic Circulation in Adaptations to the Fontan Circulation.

Menon S, Chennapragada M, Ugaki S, Sholler GF, Ayer J, Winlaw DS.

Pediatr Cardiol. 2017 Jun;38(5):886-892. doi: 10.1007/s00246-017-1576-y. Epub 2017 Feb 16. Review.

PMID: 28210768

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Select item 28199782

 

  1. Social independence of adult congenital heart disease patients in Japan.

Ochiai R, Ikeda Y, Kato H, Shiraishi I; Parents’ Association of Heart Disease Children.

Pediatr Int. 2017 Jun;59(6):675-681. doi: 10.1111/ped.13260. Epub 2017 Apr 17.

PMID: 28199782

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Select item 27816335

 

  1. A Rare Case of Anomalous Left Coronary Artery From the Pulmonary Artery (Bland-White-Garland Syndrome) in a 68-Year-Old Woman.

Roberts SM, Banbury T, Mehta A.

Semin Cardiothorac Vasc Anesth. 2017 Jun;21(2):186-190. doi: 10.1177/1089253216659146. Epub 2016 Jul 9.

PMID: 27401860

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Select item 27688032

 

  1. TGFβ1- and BMP2/PPARγ-regulated miRNAs in Pulmonary Arterial Hypertension.

Calvier L, Chouvarine P, Legchenko E, Hansmann G.

Am J Respir Crit Care Med. 2017 Jun 27. doi: 10.1164/rccm.201705-0923LE. [Epub ahead of print] No abstract available.

PMID: 28653861

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Select item 28651677

 

  1. Development of Pulmonary Hypertension During Treatment with Diazoxide: A Case Series and Literature Review.

Timlin MR, Black AB, Delaney HM, Matos RI, Percival CS.

Pediatr Cardiol. 2017 Jun 22. doi: 10.1007/s00246-017-1652-3. [Epub ahead of print]

PMID: 28642988

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Select item 28639339

 

  1. A case of intracardiac echinococcosis.

Kara SS, Gullu UU.

Cardiol Young. 2017 Jun 20:1-2. doi: 10.1017/S1047951117001196. [Epub ahead of print]

PMID: 28631585

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Select item 28631576

 

  1. Assessment of early diastolic intraventricular pressure gradient in the left ventricle among patients with repaired tetralogy of Fallot.

Kobayashi M, Takahashi K, Yamada M, Yazaki K, Matsui K, Tanaka N, Shigemitsu S, Akimoto K, Kishiro M, Nakanishi K, Kawasaki S, Nii M, Itatani K, Shimizu T.

Heart Vessels. 2017 Jun 20. doi: 10.1007/s00380-017-1011-6. [Epub ahead of print]

PMID: 28634695

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  1. Effective Cardiac Index and Systemic-Pulmonary Collaterals Evaluated by Cardiac Magnetic Resonance Late After Fontan Palliation.

Ait Ali L, Cadoni A, Rossi G, Keilberg P, Passino C, Festa P.

Am J Cardiol. 2017 Jun 15;119(12):2069-2072. doi: 10.1016/j.amjcard.2017.03.040. Epub 2017 Mar 29.

PMID: 28454633

Similar articles

Select item 28430547

 

  1. Enhancing Insights into Pulmonary Vascular Disease through a Precision Medicine Approach. A Joint NHLBI-Cardiovascular Medical Research and Education Fund Workshop Report.

Newman JH, Rich S, Abman SH, Alexander JH, Barnard J, Beck GJ, Benza RL, Bull TM, Chan SY, Chun HJ, Doogan D, Dupuis J, Erzurum SC, Frantz RP, Geraci M, Gillies H, Gladwin M, Gray MP, Hemnes AR, Herbst RS, Hernandez AF, Hill NS, Horn EM, Hunter K, Jing ZC, Johns R, Kaul S, Kawut SM, Lahm T, Leopold JA, Lewis GD, Mathai SC, McLaughlin VV, Michelakis ED, Nathan SD, Nichols W, Page G, Rabinovitch M, Rich J, Rischard F, Rounds S, Shah SJ, Tapson VF, Lowy N, Stockbridge N, Weinmann G, Xiao L.

Am J Respir Crit Care Med. 2017 Jun 15;195(12):1661-1670. doi: 10.1164/rccm.201701-0150WS.

PMID: 28430547

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Select item 28618202

 

  1. Results of a phase I/II multi-center investigation of udenafil in adolescents after fontan palliation.

Goldberg DJ, Zak V, Goldstein BH, Chen S, Hamstra MS, Radojewski EA, Maunsell E, Mital S, Menon SC, Schumacher KR, Payne RM, Stylianou M, Kaltman JR, deVries TM, Yeager JL, Paridon SM; Pediatric Heart Network Investigators.

Am Heart J. 2017 Jun;188:42-52. doi: 10.1016/j.ahj.2017.02.030. Epub 2017 Mar 6.

PMID: 28577680

Similar articles

Select item 28385177

 

  1. Effect of Fontan-Associated Morbidities on Survival With Intact Fontan Circulation.

Allen KY, Downing TE, Glatz AC, Rogers LS, Ravishankar C, Rychik J, Fuller S, Montenegro LM, Steven JM, Spray TL, Nicolson SC, Gaynor JW, Goldberg DJ.

Am J Cardiol. 2017 Jun 1;119(11):1866-1871. doi: 10.1016/j.amjcard.2017.03.004. Epub 2017 Mar 16.

PMID: 28385177

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Select item 28161345

 

  1. Associations of Age and Sex With Marfan Phenotype: The National Heart, Lung, and Blood Institute GenTAC (Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions) Registry.

Roman MJ, Devereux RB, Preiss LR, Asch FM, Eagle KA, Holmes KW, LeMaire SA, Maslen CL, Milewicz DM, Morris SA, Prakash SK, Pyeritz RE, Ravekes WJ, Shohet RV, Song HK, Weinsaft JW; GenTAC Investigators*.

Circ Cardiovasc Genet. 2017 Jun;10(3). pii: e001647. doi: 10.1161/CIRCGENETICS.116.001647.

PMID: 28600386

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Select item 28468790

 

  1. Erratum to: Decreased Diastolic Ventricular Kinetic Energy in Young Patients with Fontan Circulation Demonstrated by Four-Dimensional Cardiac Magnetic Resonance Imaging.

Sjöberg P, Heiberg E, Wingren P, Johansson JR, Malm T, Arheden H, Liuba P, Carlsson M.

Pediatr Cardiol. 2017 Jun;38(5):1087. doi: 10.1007/s00246-017-1617-6. No abstract available.

PMID: 28456830

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Select item 28456829

 

  1. Dopamine as a potential rescue therapy for refractory protein-losing enteropathy in Fontan-palliated patients.

Friedland-Little JM, Gajarski RJ, Schumacher KR.

Pediatr Transplant. 2017 Jun;21(4). doi: 10.1111/petr.12925. Epub 2017 Mar 30.

PMID: 28370952

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Select item 28295946

 

  1. Mazindol: a risk factor for pulmonary arterial hypertension?

Konofal E, Benzouid C, Delclaux C, Lecendreux M, Hussey E.

Sleep Med. 2017 Jun;34:168-169. doi: 10.1016/j.sleep.2017.02.020. Epub 2017 Mar 18.

PMID: 28522087

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Select item 27860542

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ACHD Featured Articles of May 2017

Adult Congenital Heart Reviews of May 2017 Manuscripts

 

Diagnostic value of Doppler echocardiography for identifying hemodynamic significant pulmonary valve regurgitation in tetralogy of Fallot: comparison with cardiac MRI.

Beurskens NEG, Gorter TM, Pieper PG, Hoendermis ES, Bartelds B, Ebels T, Berger RMF, Willems TP, van Melle JP.

Int J Cardiovasc Imaging. 2017 May 31. doi: 10.1007/s10554-017-1165-4. [Epub ahead of print]

PMID: 28567705

 

 

Take Home Points:

 

  • The echo parameters of pulmonary regurgitation pressure half time (PHT) < 100 ms and pulmonary regurgitation jet width/annulus ratio ≥1/3 were sensitive and specific at identifying significant pulmonary regurgitation and correlated well with cardiac MRI derived pulmonary regurgitant fraction ≥20%. These 2 echo parameters were particularly accurate when combined, with a sensitivity and specificity of 97% and 100%, respectively.

 

 

jokhadar-maanCommentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch:

Complete repair of tetralogy of Fallot (TOF) often results in pulmonary regurgitation (PR) and gradual right ventricular volume overload and its consequences of right ventricle dilation, dysfunction, and arrhythmia.  Eventual pulmonary valve replacement is often necessary to preserve right ventricle function and improve functional outcome. Therefore, the reliable quantification of PR is a critical component in the decision making for pulmonary valve replacement. Currently, PR quantification after TOF repair is most reliably assessed with phase contrast magnetic resonance imaging (MRI).  More reliable PR quantification with echocardiography has obvious advantages that include cost savings, mobility, comfort, and safety in the setting pacemakers and devices.

 

Dr. Beurskens and colleagues from the Groningen in The Netherlands performed a retrospective analysis of 97 patients with repaired TOF who had a cardiac MRI and echocardiogram within 3-months of each other between 2007 and 2013. The mean age was about 28 years.

 

Four echo parameters were compared to cardiac MRI derived PR fraction (PRF), which was considered the gold standard. Mild PR was a PRF <20% and significant PR was PRF ≥20%. The mean PRF was 29.4±15.7%. The findings were as follows:

 

Pressure half-time (PHT) <100 ms had a sensitivity and specificity of 93% and 75%, respectively for identifying significant PR (C-statistic 0.82).  PHT was measured by drawing a line along the continuous wave (CW) Doppler PR signal.

 

PR Jet/annulus ratio ≥1/3 had a sensitivity and specificity of 96% and 75%, respectively. (C-statistic 0.87). The jet diameter was measured during early diastole using Color Doppler while avoiding aliasing by adjusting the Nyquist limit. The annulus diameter was measured in the same view using 2D images without color. Both measurements were used to calculate the jet/annulus ratio.

 

Combined jet/annulus ratio ≥1/3 and PHT <100 ms was highly accurate in identifying PRF≥20%, with sensitivity and specificity of 97% and 100%, respectively.

 

Less helpful parameters:

 

PR index (PRi) <0.77 had a sensitivity and specificity of 66% and 54%, respectively (C-statistic 0.63). PRi was calculated using the PR time as percentage of the total diastolic time, derived from the CW Doppler tracings. Lower PRi corresponds with more severe PR.

 

Diastolic to systolic time velocity integral (DSTVI) >0.49 had sensitivity and specificity of 84% and 33%, respectively, (C-statistic 0.56). DSTVI was calculated by dividing the surface area of the pulse-wave (PW) Doppler diastolic flow curve by the surface area of the systolic flow curve. PW Doppler sampling was at the level of the main pulmonary artery.

 

Conclusions:

PHT and jet/annulus ratio appear to be useful tools in the echo assessment of PR after TOF repair. However, these findings and criteria require validation and possible refinement and additional studies are needed to determine whether using these echo parameters translates to clinical benefit, particularly as it applies to timing of pulmonary valve replacement.

 

 

 

Renal resistive index reflects Fontan pathophysiology and predicts mortality.

Ohuchi H, Negishi J, Hayama Y, Miyazaki A, Shiraishi I, Ichikawa H.

Heart 2017; 0: 1-7. Doi : 10.1136/heartjnl-2016-310812

PMID: 28465331

 

 

Take Home Points:

 

  • Higher renal resistive index (RRI), currently used and validated to monitor end-organ damage in patients with diabetes and hypertension, is associated with poorer functional capacity, neuro-hormonal activation, and diminished renal function in Fontan subjects.
  • RRI ≥ 0.81 has a strong prognostic value for all–cause mortality in Fontan patients.

 

 

Leong Ming ChernCommentary from Dr. MC Leong (Kuala Lumpur), section editor of ACHD Journal Watch: Renal resistive index (RRI), which is calculated using the formula: RRI = 100 x (1- end diastolic velocity/ peak systolic velocity), has been shown to be a marker of cardiovascular damage and is a useful tool for monitoring end-organ damage as well as the predicted mortality for patients with hypertension. In patients with congenital heart disease, it has been demonstrated to be a useful tool for identifying severe heart failure, and identify those with a worse prognosis. This study aimed to analyze the significance of RRI in patients with Fontan with respect to invasive hemodynamics, neurohumoral activation (plasma norepinephrine concentration, brain natriuretic peptide and plasma renin activity), exercise capacity (peak VO2), renal function (24-hour creatinine clearance), liver function and glucose intolerance.

 

280 clinically stable Fontan patients, aged 6 years and above were assessed between March 2007 and September 2016. Mean follow up duration is 32±22 months. Eighteen patients died during follow up. Higher RRI was correlated with higher NYHA functional class, presence of protein losing enteropathy, pulmonary AVM’s, arrhythmia, BNP, plasma renin activity and norepinephrine levels.  RRI was however inversely correlated with Peak VO2 and serum Albumin levels. A cut-off value of RRI ≥ 0.81 was strongly predictive of mortality. On multivariate analyses, RRI ≥ 0.81 was the only independent predictor of mortality (HR: 12.0; 95% CI: 3.4-50.1; p<0.0001).

ACHD 1 May

May ACHD 2

May ACHD 3

Effects of long-term iloprost treatment on right ventricular function in patients with Eisenmenger syndrome.

Chon MK, Cho KI, Cha KS, Seo JS, Kim DS.

J Cardiol. 2017 May;69(5):741-746. doi: 10.1016/j.jjcc.2016.07.002. Epub 2016 Aug 1.

PMID: 27492657

Similar articles

Select item 28245348

 

 

Take Home Points:

 

  • Eisenmenger syndrome [ES] is associated with significant morbidity and mortality.
  • Long term [after 48 weeks of treatment] inhaled iloprost therapy significantly improved both clinical and echocardiographic parameters.
  • These included significant improvements in mean and systolic pulmonary artery pressures, RV function parameters (Improved RV MPI, RV FAC, tricuspid annular peak systolic velocity), WHO functional classification, 6MWD and NT-proBNP.

 

 

Blanche CupidoCommentary by Dr. Blanche Cupido (Cape Town), section editor of ACHD Journal WatchThis is a prospective multicenter study that evaluated the effect of inhaled iloprost therapy (given for a 48-week period) on RV function and clinical status in patients with ES. Although ES generally carries a better prognosis than idiopathic PAH, morbidity and mortality remain significant in this group as well. Although a number of drugs currently exist, few of them have been demonstrated beneficial in ES.

 

Eleven patients with ES (right to left shunt present at rest) were enrolled between December 2010 and November 2012. Patients who had concomitant severe LV dysfunction (EF <40%), pulmonary venous congestion, RV outflow obstruction, glibenclamide and cyclosporine therapy, coronary artery disease, systolic BP <85mmHg, renal impairment (serum creatinine >1.5mg/dL, those on other PAH treatments within a one month period of enrollment, anticoagulation therapy, planned surgical procedures during the study period and inability to perform the study protocol were excluded.

 

All patients received the full 48 weeks of treatment. The dose of inhaled iloprost was gradually uptitrated to the target dose of 2.5-5mcg divided by 6-9 times per day.  No adverse drug-related events occurred during the study period. Patients were assessed at baseline and at 48 weeks of therapy – various clinical, laboratory and echocardiographic measures were assessed. The mean age of the cohort was 44.2 years. All patients were WHO functional class III-IV at enrollment.

 

On echocardiography, the following parameters showed a significant improvement after therapy:

  • Reduction in mean pulmonary artery pressure (42.5 to 36.5mmHG, p=0.004)
  • Reduction in systolic pulmonary artery pressure (92.6 to 74.5mmHg, p=0.005)
  • Small improvement in RV fractional area change (36.4 to 44.4%, p=0.032)
  • Significant improvement in RV MPI (0.68 to 0.52, p<0.05)
  • Significant improvement in tricuspid annular peak systolic velocity (9.1 to 11.0, p<0.05)

May ACHD 4

May ACHD 5

 

Furthermore, as depicted in figure 1 above, both global and regional strain parameters were significantly improved.

 

These echocardiographic improvements in RV function and pulmonary pressure were accompanied by functional improvement on clinical grounds. The 6MWD (310.6 to 399.7m, p<0.001) and SaO2 (90.9 to 92.5 %, p=0.022) showed a significant improvement after the 48 weeks on iloprost.  There was a significant improvement in the WHO functional class of the cohort (mainly from class III to class II, p=0.006). NT-proBNP levels also improved significantly (802 to 687 ng/L, p=0.008).

 

 

 

The study may not be generalizable due to its small study sample and the lack of a placebo arm. These pulmonary vascular measures were derived from echo parameters, and these were not validated against the gold standard of cardiac catheterization in this population. Further larger studies are warranted to verify these results.

 

 

Mortality Risk Stratification in Fontan Patients Who Underwent Heart Transplantation.

Berg CJ, Bauer BS, Hageman A, Aboulhosn JA, Reardon LC.

Am J Cardiol. 2017 May 15;119(10):1675-1679. doi: 10.1016/j.amjcard.2017.02.005. Epub 2017 Mar 1.

PMID: 28341356

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Take Home Points:

 

  • Better risk stratification is needed for patient selection to improve post OHT outcomes for Fontan patients.
  • In this study, all-cause mortality in a Fontan population, including adults and children, was 44% after OHT during a mean follow-up time of 3.5 years (n=36)
  • All patients in this study who required pre-OHT renal replacement therapy or pre-OHT ECMO died post OHT, mostly in the immediate perioperative period.
  • Risk factors for post OHT mortality include: young age at time of OHT (<18 years), short interval between Fontan and OHT (<10 years), severe systolic dysfunction (EF < 20%), moderate or severe atrioventricular valve regurgitation, an elevated MELD-XI score > 19, pre-OHT renal replacement therapy, or pre-OHT ECMO.
  • The authors suggest a risk score of 0-12 points based on presence of risk factors. In their retrospective analysis, a score > 5 points was associated with 88% mortality; 2-5 points with 50% mortality, and 0-1 points with 0% mortality.

 

 

Kay_William_MD.16.CVaCommentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch: 

The Fontan procedure, which has been the final common pathway for most single ventricle patients for well over 20 years now, has a number of drawbacks, and a significant number of patients will ultimately require orthotopic heart transplantation (OHT).  However, OHT is a scarce resource and federal guidelines are quite strict regarding organ allocation, and Fontan survivors, thought to be at higher risk of periprocedural complications, must compete against other heart failure patients, many of whom have never undergone prior open heart surgery.

 

This paper is a retrospective review of all patients (children and adults) with failing Fontan physiology who underwent transplantation from 1991 to 2014 at UCLA (n=36).  Average follow-up time was 3.5 years, with 44% patients having a postoperative mortality.  The authors evaluated potential risk factors for Fontan-OHT mortality and came up with a risk score that will need to be evaluated in newer multicenter, ideally prospective, studies.

 

Risk factors found to correlate with mortality including being <18 years old at time of OHT, a short interlude <10 years between Fontan and OHT, severe systolic dysfunction of single ventricle (EF < 20%), moderate or severe atrioventricular valve regurgitation, an elevated Model of End-stage Liver Disease eXcluding INR score (MELD-XI), need for renal replacement therapy, or need for advanced mechanical support prior to OHT.

 

All patients who required preoperative renal replacement therapy or ECMO died after OHT.  Interestingly, in this study, moderate or severe atrioventricular valve regurgitation was a stronger predictor of mortality than a severely depressed EF < 20%.  A MELD-XI score > 19 was correlated with increased odds of all-cause mortality after OHT.  Interestingly, there was no correlation between serum creatinine, bilirubin, or MELD-XI with documented presence of chronic kidney disease, cirrhosis, or ascites.  The authors note that there is evidence in the literature that patients with protein losing enteropathy (PLE) may have lower bilirubin due to wasting of the bilirubin in intestinal effluent, which may result in a falsely reassuring MELD-XI score.

 

A scoring scheme was composed by the authors including the 6 preoperative factors found to have elevated hazard ratios.  Risk factors were assigned 1,2, or 3 points based on HR <4, 4-6, or >6, respectively, with a maximum total score of 12.  No patient with a score of 0 or 1 points died post OHT during the follow-up period.  Patients with 2-5 points had 50% mortality, and patients with scores > 5 points had 88% mortality.

ACHD May 2017

 

  1. Increased risk of thromboembolic events in adult congenital heart disease patients with atrial tachyarrhythmias: Bias due to the data sparsity.

Ayubi E, Safiri S, Mansournia MA.

Int J Cardiol. 2017 Jul 15;239:20. doi: 10.1016/j.ijcard.2017.02.133. No abstract available.

PMID: 28560967

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  1. Cognitive dysfunction in adult CHD with different structural complexity.

Tyagi M, Fteropoulli T, Hurt CS, Hirani SP, Rixon L, Davies A, Picaut N, Kennedy F, Deanfield J, Cullen S, Newman SP.

Cardiol Young. 2017 Jul;27(5):851-859. doi: 10.1017/S1047951116001396. Epub 2016 Oct 18.

PMID: 27751192

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  1. Arterial stiffness and arterial function in adult cyanotic patients with congenital heart disease.

Trojnarska O, Szczepaniak-Chicheł L, Gabriel M, Bartczak-Rutkowska A, Rupa-Matysek J, Tykarski A, Grajek S.

J Cardiol. 2017 Jul;70(1):62-67. doi: 10.1016/j.jjcc.2016.09.003. Epub 2016 Oct 15.

PMID: 27756510

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  1. Cardiorespiratory Fitness, Not the Severity of the Condition, Dictates Late Outcomes After Fontan Procedures.

d’Udekem Y.

J Am Coll Cardiol. 2017 Jun 6;69(22):2745-2747. doi: 10.1016/j.jacc.2017.03.581. No abstract available.

PMID: 28571640

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  1. Longitudinal Outcomes of Patients With Single Ventricle After the Fontan Procedure.

Atz AM, Zak V, Mahony L, Uzark K, D’agincourt N, Goldberg DJ, Williams RV, Breitbart RE, Colan SD, Burns KM, Margossian R, Henderson HT, Korsin R, Marino BS, Daniels K, McCrindle BW; Pediatric Heart Network Investigators.

J Am Coll Cardiol. 2017 Jun 6;69(22):2735-2744. doi: 10.1016/j.jacc.2017.03.582.

PMID: 28571639

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  1. Knowledge-based reconstruction for measurement of right ventricular volumes on cardiovascular magnetic resonance images in a mixed population.

Pieterman ED, Budde RPJ, Robbers-Visser D, van Domburg RT, Helbing WA.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12484. [Epub ahead of print]

PMID: 28580763

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  1. Mitral valve prolapse and Marfan syndrome.

Thacoor A.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12467. [Epub ahead of print] Review.

PMID: 28580713

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  1. Long-term outcomes after aortic coarctation repair in Maltese patients: A population-based study.

Caruana M, Grech V.

Congenit Heart Dis. 2017 Jun 5. doi: 10.1111/chd.12488. [Epub ahead of print]

PMID: 28580660

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  1. Transition of Care in Congenital Heart Disease: Ensuring the Proper Handoff.

Lee A, Bailey B, Cullen-Dean G, Aiello S, Morin J, Oechslin E.

Curr Cardiol Rep. 2017 Jun;19(6):55. doi: 10.1007/s11886-017-0859-5. Review.

PMID: 28477319

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  1. Current Role of Blood and Urine Biomarkers in the Clinical Care of Adults with Congenital Heart Disease.

Rajpal S, Alshawabkeh L, Opotowsky AR.

Curr Cardiol Rep. 2017 Jun;19(6):50. doi: 10.1007/s11886-017-0860-z. Review.

PMID: 28432659

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  1. Anesthesia in adults with congenital heart disease.

Baehner T, Ellerkmann RK.

Curr Opin Anaesthesiol. 2017 Jun;30(3):418-425. doi: 10.1097/ACO.0000000000000468.

PMID: 28306681

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  1. EuroEcho-Imaging 2016: highlights.

Magne J, Popescu BA, Bucciarelli-Ducci C, Cosyns B, Donal E, Gimelli A, Miller O, Badano L, Habib G.

Eur Heart J Cardiovasc Imaging. 2017 Jun 1;18(6):621-628. doi: 10.1093/ehjci/jex063.

PMID: 28407053

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  1. Heart transplantation for adults with congenital heart disease: current status and future prospects.

Matsuda H, Ichikawa H, Ueno T, Sawa Y.

Gen Thorac Cardiovasc Surg. 2017 Jun;65(6):309-320. doi: 10.1007/s11748-017-0777-x. Epub 2017 Apr 24. Review.

PMID: 28439697

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  1. Aortopathies in adult congenital heart disease and genetic aortopathy syndromes: management strategies and indications for surgery.

Kuijpers JM, Mulder BJ.

Heart. 2017 Jun;103(12):952-966. doi: 10.1136/heartjnl-2015-308626. Epub 2017 Mar 7. No abstract available.

PMID: 28270426

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  1. Cyanotic congenital heart disease and atherosclerosis.

Tarp JB, Jensen AS, Engstrøm T, Holstein-Rathlou NH, Søndergaard L.

Heart. 2017 Jun;103(12):897-900. doi: 10.1136/heartjnl-2016-311012. Epub 2017 Mar 4. Review.

PMID: 28259844

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  1. The management of the third stage of labour in women with heart disease.

Cauldwell M, Steer PJ, Swan L, Uebing A, Gatzoulis MA, Johnson MR.

Heart. 2017 Jun;103(12):945-951. doi: 10.1136/heartjnl-2016-310607. Epub 2016 Dec 19.

PMID: 27993911

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  1. Echocardiography-Derived Left Ventricular Outflow Tract Gradient and Left Ventricular Posterior Wall Thickening Are Associated with Outcomes for Anatomic Repair in Congenitally Corrected Transposition of the Great Arteries.

Moodley S, Balasubramanian S, Tacy TA, Chan F, Hanley FL, Punn R.

J Am Soc Echocardiogr. 2017 Jun 1. pii: S0894-7317(17)30219-5. doi: 10.1016/j.echo.2017.03.019. [Epub ahead of print]

PMID: 28579248

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  1. Endothelin inhibitors lower pulmonary vascular resistance and improve functional capacity in patients with Fontan circulation.

Agnoletti G, Gala S, Ferroni F, Bordese R, Appendini L, Pace Napoleone C, Bergamasco L.

J Thorac Cardiovasc Surg. 2017 Jun;153(6):1468-1475. doi: 10.1016/j.jtcvs.2017.01.051. Epub 2017 Feb 10.

PMID: 28283234

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  1. Red Flags for Maltese Adults with Congenital Heart Disease: Poorer Dental Care and Less Sports Participation Compared to Other European Patients-An APPROACH-IS Substudy.

Caruana M, Apers S, Kovacs AH, Luyckx K, Thomet C, Budts W, Sluman M, Eriksen K, Dellborg M, Berghammer M, Johansson B, Soufi A, Callus E, Moons P, Grech V; APPROACH-IS consortium and the International Society for Adult Congenital Heart Disease (ISACHD).

Pediatr Cardiol. 2017 Jun;38(5):965-973. doi: 10.1007/s00246-017-1604-y. Epub 2017 Mar 24.

PMID: 28341902

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  1. The Lymphatic Circulation in Adaptations to the Fontan Circulation.

Menon S, Chennapragada M, Ugaki S, Sholler GF, Ayer J, Winlaw DS.

Pediatr Cardiol. 2017 Jun;38(5):886-892. doi: 10.1007/s00246-017-1576-y. Epub 2017 Feb 16. Review.

PMID: 28210768

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  1. A Rare Case of Anomalous Left Coronary Artery From the Pulmonary Artery (Bland-White-Garland Syndrome) in a 68-Year-Old Woman.

Roberts SM, Banbury T, Mehta A.

Semin Cardiothorac Vasc Anesth. 2017 Jun;21(2):186-190. doi: 10.1177/1089253216659146. Epub 2016 Jul 9.

PMID: 27401860

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  1. Outcomes of pulmonary arterial hypertension therapy in Australia: is monotherapy adequate?

Moonen A, Garsia R, Youssef P, Torzillo P, Corte T, Boehm C, Cordina R, Celermajer D, Lau E.

Intern Med J. 2017 May 31. doi: 10.1111/imj.13495. [Epub ahead of print]

PMID: 28560817

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  1. Imaging adult patients with Fontan circulation.

Ginde S, Goot BH, Frommelt PC.

Curr Opin Cardiol. 2017 May 25. doi: 10.1097/HCO.0000000000000422. [Epub ahead of print]

PMID: 28548989

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  1. Detection of mechanical complications related to the potential risk of sudden cardiac death in patients with pulmonary arterial hypertension by computed tomography.

Lee SE, Im JH, Sung JM, Cho IJ, Shim CY, Hong GR, Chung N, Jung JW, Chang HJ.

Int J Cardiol. 2017 May 25. pii: S0167-5273(17)31912-5. doi: 10.1016/j.ijcard.2017.05.090. [Epub ahead of print]

PMID: 28576625

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  1. Pictorial Review of Surgical Anatomy in Adult Congenital Heart Disease.

De Cecco CN, Muscogiuri G, Madrid Pérez JM, Eid M, Suranyi P, Lesslie VW, Bastarrika G.

J Thorac Imaging. 2017 May 25. doi: 10.1097/RTI.0000000000000273. [Epub ahead of print]

PMID: 28549022

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  1. Pregnancy-associated plasma protein-A (PAPP-A) and the proform of the eosinophil major basic protein (ProMBP) are associated with increased risk of death in heart failure patients.

Dembic M, Hedley PL, Torp-Pedersen C, Køber L, Christiansen M.

Scand J Clin Lab Invest. 2017 May 24:1-6. doi: 10.1080/00365513.2017.1325926. [Epub ahead of print]

PMID: 28537443

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  1. Inferior Vena Cava Thrombus in a Postpartum Patient With Fontan Physiology: A Case Report.

Tashjian JA, Fraint H, DiNardo J, Rouine-Rapp K.

A A Case Rep. 2017 May 23. doi: 10.1213/XAA.0000000000000548. [Epub ahead of print]

PMID: 28542047

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  1. Effect of phosphodiesterase-5 inhibition with Tadalafil on SystEmic Right VEntricular size and function – A multi-center, double-blind, randomized, placebo-controlled clinical trial – SERVE trial – Rational and design.

Tobler D, Bouchardy J, Reto E, Heg D, Müller C, Frenk A, Gabriel H, Schwitter J, Rutz T, Buechel RR, Willhelm M, Trachsel L, Freese M, Greutmann M, Schwerzmann M; SERVE trial.

Int J Cardiol. 2017 May 23. pii: S0167-5273(17)32122-8. doi: 10.1016/j.ijcard.2017.05.079. [Epub ahead of print]

PMID: 28566262

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  1. The use of Macitentan in Fontan circulation: a case report.

Demetriades P, Aziz A, Condliffe R, Bowater SE, Clift PF.

BMC Cardiovasc Disord. 2017 May 22;17(1):131. doi: 10.1186/s12872-017-0567-5.

PMID: 28532389 Free PMC Article

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  1. Preparing adolescents with heart problems for transition to adult care, 2009-2010 National Survey of Children with Special Health Care Needs.

Downing KF, Oster ME, Farr SL.

Congenit Heart Dis. 2017 May 19. doi: 10.1111/chd.12476. [Epub ahead of print]

PMID: 28523852

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  1. Transition in Patients with Congenital Heart Disease in Germany: Results of a Nationwide Patient Survey.

Helm PC, Kaemmerer H, Breithardt G, Sticker EJ, Keuchen R, Neidenbach R, Diller GP, Tutarel O, Bauer UMM.

Front Pediatr. 2017 May 19;5:115. doi: 10.3389/fped.2017.00115. eCollection 2017.

PMID: 28580351 Free Article

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  1. Elevated sympathetic activity, endothelial dysfunction, and late hypertension after repair of coarctation of the aorta.

Lee MGY, Hemmes RA, Mynard J, Lambert E, Head GA, Cheung MMH, Konstantinov IE, Brizard CP, Lambert G, d’Udekem Y.

Int J Cardiol. 2017 May 19. pii: S0167-5273(17)30820-3. doi: 10.1016/j.ijcard.2017.05.075. [Epub ahead of print]

PMID: 28545853

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  1. Gerbode defect and multivalvular dysfunction: Complex complications in adult congenital heart disease.

Ruivo C, Guardado J, Montenegro Sá F, Saraiva F, Antunes A, Correia J, Morais J.

Echocardiography. 2017 May 18. doi: 10.1111/echo.13561. [Epub ahead of print]

PMID: 28517030

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  1. Effect of Fontan geometry on exercise haemodynamics and its potential implications.

Tang E, Wei ZA, Whitehead KK, Khiabani RH, Restrepo M, Mirabella L, Bethel J, Paridon SM, Marino BS, Fogel MA, Yoganathan AP.

Heart. 2017 May 18. pii: heartjnl-2016-310855. doi: 10.1136/heartjnl-2016-310855. [Epub ahead of print]

PMID: 28522658

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  1. Impaired atrioventricular transport in patients with transposition of the great arteries palliated by atrial switch and preserved systolic right ventricular function: A magnetic resonance imaging study.

Ladouceur M, Kachenoura N, Soulat G, Bollache E, Redheuil A, Azizi M, Delclaux C, Chatellier G, Boutouyrie P, Iserin L, Bonnet D, Mousseaux E.

Congenit Heart Dis. 2017 May 16. doi: 10.1111/chd.12472. [Epub ahead of print]

PMID: 28508510

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  1. Adolescents with congenital heart disease: their opinions about the preparation for transfer to adult care.

Burström Å, Bratt EL, Frenckner B, Nisell M, Hanséus K, Rydberg A, Öjmyr-Joelsson M.

Eur J Pediatr. 2017 May 16. doi: 10.1007/s00431-017-2917-9. [Epub ahead of print]

PMID: 28508990

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  1. Lymphodynamics in Congenital HeartDisease: The Forgotten Circulation.

Kreutzer J, Kreutzer C.

J Am Coll Cardiol. 2017 May 16;69(19):2423-2427. doi: 10.1016/j.jacc.2017.03.559. No abstract available.

PMID: 28494979

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  1. National Trends and In-Hospital Outcomes in Pregnant Women With Heart Disease in the United States.

Lima FV, Yang J, Xu J, Stergiopoulos K.

Am J Cardiol. 2017 May 15;119(10):1694-1700. doi: 10.1016/j.amjcard.2017.02.003. Epub 2017 Feb 28.

PMID: 28343597

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  1. Mortality Risk Stratification in Fontan Patients Who Underwent Heart Transplantation.

Berg CJ, Bauer BS, Hageman A, Aboulhosn JA, Reardon LC.

Am J Cardiol. 2017 May 15;119(10):1675-1679. doi: 10.1016/j.amjcard.2017.02.005. Epub 2017 Mar 1.

PMID: 28341356

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  1. How often is congenital heart disease recognized as a significant comorbidity among hospitalized adults with congenital heart disease?

Robbins JM, Onukwube J, Goudie A, Collins RT 2nd.

Int J Cardiol. 2017 May 15;235:42-48. doi: 10.1016/j.ijcard.2017.02.100. Epub 2017 Feb 22.

PMID: 28279500

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  1. Original Findings and Updated Meta-Analysis for the Association Between Maternal Diabetes and Risk for Congenital Heart Disease Phenotypes.

Hoang TT, Marengo LK, Mitchell LE, Canfield MA, Agopian AJ.

Am J Epidemiol. 2017 May 13:1-11. doi: 10.1093/aje/kwx033. [Epub ahead of print]

PMID: 28505225

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  1. Predictors of procedural complications in adult Fontan patients undergoing non-cardiac procedures.

Egbe AC, Khan AR, Ammash NM, Barbara DW, Oliver WC, Said SM, Akintoye E, Warnes CA, Connolly HM.

Heart. 2017 May 13. pii: heartjnl-2016-311039. doi: 10.1136/heartjnl-2016-311039. [Epub ahead of print]

PMID: 28501797

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  1. [Congenital heart disease in adolescents and adults: Management in a general cardiology department in Senegal].

Mbaye A, Bodian M, Ngaïdé AA, Abdourafiq H, Leye MCBO, Savodogo S, Aw F, Ndiaye M, Kouamé I, Babaka K, Dioum M, Gaye ND, Sarr SA, Ndiaye MB, Kane AD, Kane A.

Ann Cardiol Angeiol (Paris). 2017 May 12. pii: S0003-3928(17)30020-3. doi: 10.1016/j.ancard.2017.02.003. [Epub ahead of print] French.

PMID: 28506578

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  1. Hospital Inpatient Costs for Single Ventricle Patients Surviving the Fontan Procedure.

Huang L, Schilling C, Dalziel KM, Xie S, Celermajer DS, McNeil JJ, Winlaw D, Hornung TS, Radford DJ, Grigg LE, Bullock A, Wheaton GR, Justo RN, Blake J, Bishop R, Du Plessis K, d’Udekem Y.

Am J Cardiol. 2017 May 11. pii: S0002-9149(17)30785-3. doi: 10.1016/j.amjcard.2017.04.049. [Epub ahead of print]

PMID: 28583678

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  1. Congenital Heart Disease and High Altitude: Is Chronic Hypoxia a Common Factor in Intellectual Impairment?

Ghimire LV.

High Alt Med Biol. 2017 May 9. doi: 10.1089/ham.2017.0043. [Epub ahead of print] No abstract available.

PMID: 28485995

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Select item 28485227

 

  1. Predicting long-term mortality after Fontan procedures: A risk score based on 6707 patients from 28 studies.

Alsaied T, Bokma JP, Engel ME, Kuijpers JM, Hanke SP, Zuhlke L, Zhang B, Veldtman GR.

Congenit Heart Dis. 2017 May 8. doi: 10.1111/chd.12468. [Epub ahead of print]

PMID: 28480627

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Select item 28534022

 

  1. Mental Health Problems in Parents of Children with Congenital Heart Disease.

Kolaitis GA, Meentken MG, Utens EMWJ.

Front Pediatr. 2017 May 8;5:102. doi: 10.3389/fped.2017.00102. eCollection 2017. Review.

PMID: 28534022 Free PMC Article

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  1. Extracardiac Conduit Fontan – Outcome Data in Early Adulthood.

Zentner D, Cheshire C, Grigg L.

Heart Lung Circ. 2017 May 3. pii: S1443-9506(17)30332-3. doi: 10.1016/j.hlc.2017.03.162. [Epub ahead of print]

PMID: 28545820

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Select item 28469241

 

  1. Forced vital capacity predicts morbidity and mortality in adults with repaired tetralogy of Fallot.

Cohen KE, Buelow MW, Dixon J, Brazauskas R, Cohen SB, Earing MG, Ginde S.

Congenit Heart Dis. 2017 May 2. doi: 10.1111/chd.12470. [Epub ahead of print]

PMID: 28464509

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  1. Managing subfertility in patients with heart disease: What are the choices?

Cauldwell M, Patel RR, Steer PJ, Swan L, Norman-Taylor J, Gatzoulis M, Johnson MR.

Am Heart J. 2017 May;187:29-36. doi: 10.1016/j.ahj.2017.02.007. Epub 2017 Feb 15. Review.

PMID: 28454805

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  1. Noninvasive Screening for Pulmonary Hypertension by Exercise Testing in Congenital Heart Disease.

Müller J, Heck PB, Ewert P, Hager A.

Ann Thorac Surg. 2017 May;103(5):1544-1549. doi: 10.1016/j.athoracsur.2016.09.038. Epub 2016 Dec 1.

PMID: 27914635

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  1. An adult case of Kommerell’s diverticulum with a right-sided aortic arch.

Tsutsumi K, Nishida K, Tsukamoto J, Iijima N, Ishida O.

Asian Cardiovasc Thorac Ann. 2017 May;25(4):307-309. doi: 10.1177/0218492317707146. Epub 2017 Apr 29.

PMID: 28457170

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  1. A congenital diverticulum of the left ventricular apex manifested by stroke and recurrent ventricular tachycardia.

Dostálová G, Paleček T, Kuchynka P, Havránek Š, Mašek M, Hlubocká Z, Karetová D, Wichterle D, Dušková J, Lindner J, Linhart A.

Cardiovasc Pathol. 2017 May – Jun;28:3-6. doi: 10.1016/j.carpath.2017.01.009. Epub 2017 Feb 4.

PMID: 28219755

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  1. Congenital Heart Disease: Multimodal Imaging Is Every Day’s Routine.

Beerbaum PBJ.

Circ Cardiovasc Imaging. 2017 May;10(5). pii: e006589. doi: 10.1161/CIRCIMAGING.117.006589. No abstract available.

PMID: 28495825

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Select item 28495824

 

  1. How to Image the Dilated Right Ventricle.

Partington SL, Kilner PJ.

Circ Cardiovasc Imaging. 2017 May;10(5). pii: e004688. doi: 10.1161/CIRCIMAGING.116.004688. Review. No abstract available.

PMID: 28495824

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Select item 28495823

 

  1. How to Image the Adult Patient With Fontan Circulation.

Hauser JA, Taylor AM, Pandya B.

Circ Cardiovasc Imaging. 2017 May;10(5). pii: e004273. doi: 10.1161/CIRCIMAGING.116.004273. Review. No abstract available.

PMID: 28495823

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Select item 28495821

 

  1. How to Image Repaired Tetralogy of Fallot.

Valente AM, Geva T.

Circ Cardiovasc Imaging. 2017 May;10(5). pii: e004270. doi: 10.1161/CIRCIMAGING.116.004270. Review. No abstract available.

PMID: 28495821

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Select item 27659940

 

  1. Improving the quality of transition and transfer of care in young adults with congenital heart disease.

Everitt IK, Gerardin JF, Rodriguez FH 3rd, Book WM.

Congenit Heart Dis. 2017 May;12(3):242-250. doi: 10.1111/chd.12463. Review.

PMID: 28580608

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Select item 28371362

 

  1. Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature.

Smith K, Gros B.

Congenit Heart Dis. 2017 May;12(3):251-260. doi: 10.1111/chd.12465. Epub 2017 Apr 2. Review.

PMID: 28371362

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Select item 28244680

 

  1. Correlates of posttraumatic stress disorder in adults with congenital heart disease.

Eslami B.

Congenit Heart Dis. 2017 May;12(3):357-363. doi: 10.1111/chd.12452. Epub 2017 Feb 20.

PMID: 28217850

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Select item 28211263

 

  1. Residents’ understanding of adult congenital heart disease.

Cooper P, Hindes M, Maul TM, Cook SC.

Congenit Heart Dis. 2017 May;12(3):309-314. doi: 10.1111/chd.12441. Epub 2017 Jan 16.

PMID: 28092429

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Select item 27893194

 

  1. A first population-based long-term outcome study in adults with repaired tetralogy of Fallot in Malta.

Caruana M, Grech V.

Congenit Heart Dis. 2017 May;12(3):301-308. doi: 10.1111/chd.12439. Epub 2016 Nov 28.

PMID: 27893189

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Select item 27885832

 

  1. The power in being together for young adults who have heart disease – the photoshoot experience.

Gallagher R, Potter E, Thomson Mangnall L, Ladak L, Gallagher P, Neubeck L.

Heart Lung. 2017 May – Jun;46(3):199-204. doi: 10.1016/j.hrtlng.2017.02.004. Epub 2017 Mar 31.

PMID: 28366291

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Select item 28237272

 

  1. Maternal Antihypertensive Medication Use and Congenital Heart Defects: Updated Results From the National Birth Defects Prevention Study.

Fisher SC, Van Zutphen AR, Werler MM, Lin AE, Romitti PA, Druschel CM, Browne ML; and the National Birth Defects Prevention Study.

Hypertension. 2017 May;69(5):798-805. doi: 10.1161/HYPERTENSIONAHA.116.08773. Epub 2017 Apr 3.

PMID: 28373593

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Select item 28584431

 

  1. Effect of maternal age and cardiac disease severity on outcome of pregnancy in women with congenital heart disease.

Furenäs E, Eriksson P, Wennerholm UB, Dellborg M.

Int J Cardiol. 2017 May 1. pii: S0167-5273(17)30391-1. doi: 10.1016/j.ijcard.2017.04.100. [Epub ahead of print]

PMID: 28539207

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Select item 28238509

 

  1. Preconception counseling, predicting risk and outcomes in women with mWHO 3 and 4 heart disease.

Cauldwell M, Ghonim S, Uebing A, Swan L, Steer PJ, Gatzoulis M, Johnson MR.

Int J Cardiol. 2017 May 1;234:76-80. doi: 10.1016/j.ijcard.2017.02.003. Epub 2017 Feb 4.

PMID: 28238509

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Select item 28209388

 

  1. ACR Appropriateness Criteria<sup>®</sup> Known or Suspected Congenital Heart Disease in the Adult.

Expert Panel on Cardiac Imaging:, Woodard PK, Ho VB, Akers SR, Beache G, Brown RKJ, Cummings KW, Greenberg SB, Min JK, Stillman AE, Stojanovska J, Jacobs JE.

J Am Coll Radiol. 2017 May;14(5S):S166-S176. doi: 10.1016/j.jacr.2017.02.036.

PMID: 28473073

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Select item 27492657

 

  1. Effects of long-term iloprost treatment on right ventricular function in patients with Eisenmenger syndrome.

Chon MK, Cho KI, Cha KS, Seo JS, Kim DS.

J Cardiol. 2017 May;69(5):741-746. doi: 10.1016/j.jjcc.2016.07.002. Epub 2016 Aug 1.

PMID: 27492657

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Select item 28245348

 

  1. Isolated Aneurysms of the Membranous Ventricular Septum Without Residual Shunts: Systematic Review and Description of 3 Cases in Adults.

Abdul Jabbar A, Mufti O, Mazur W, Quraishi MB, Srivastava BK, Tivakaran V.

J Ultrasound Med. 2017 May;36(5):869-878. doi: 10.7863/ultra.16.02087. Epub 2017 Feb 23. Review.

PMID: 28230264

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Select item 28370380

 

  1. Clinical Characteristics of Adult Dogs More Than 5 Years of Age at Presentation for Patent Ductus Arteriosus.

Boutet BG, Saunders AB, Gordon SG.

J Vet Intern Med. 2017 May;31(3):685-690. doi: 10.1111/jvim.14689. Epub 2017 Apr 3.

PMID: 28370380 Free PMC Article

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  1. Myocardial cytochrome oxidase activity increases with age and hypoxemia in patients with congenital heart disease.

Onwugbufor M, Levy RJ, Zurakowski D, Jonas RA, Sinha P.

Perfusion. 2017 May;32(4):306-312. doi: 10.1177/0267659116681435. Epub 2016 Dec 5.

PMID: 27913766 Free PMC Article

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  1. [Adult congenital heart disease: Medical and psychosocial issues].

Ladouceur M, Pontnau F, Iserin L.

Presse Med. 2017 May;46(5):523-529. doi: 10.1016/j.lpm.2017.02.003. Epub 2017 Mar 14. French.

PMID: 28314442

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  1. [Cardiac rehabilitation for children and adults with congenital heart disease].

Amedro P, Gavotto A, Bredy C, Guillaumont S.

Presse Med. 2017 May;46(5):530-537. doi: 10.1016/j.lpm.2016.12.001. Epub 2017 Jan 23. French.

PMID: 28126509

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  1. Real-world, long-term survival of incident patients with pulmonary arterial hypertension.

Marques-Alves P, Baptista R, Marinho da Silva A, Pêgo M, Castro G.

Rev Port Pneumol (2006). 2017 May – Jun;23(3):124-131. doi: 10.1016/j.rppnen.2017.01.006. Epub 2017 Mar 7.

PMID: 28283462 Free Article

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  1. Diagnostic value of Doppler echocardiography for identifying hemodynamic significant pulmonary valve regurgitation in tetralogy of Fallot: comparison with cardiac MRI.

Beurskens NEG, Gorter TM, Pieper PG, Hoendermis ES, Bartelds B, Ebels T, Berger RMF, Willems TP, van Melle JP.

Int J Cardiovasc Imaging. 2017 May 31. doi: 10.1007/s10554-017-1165-4. [Epub ahead of print]

PMID: 28567705

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  1. Right ventricular-vascular coupling in heart failure with preserved ejection fraction and pre- vs. post-capillary pulmonary hypertension.

Gorter TM, van Veldhuisen DJ, Voors AA, Hummel YM, Lam CSP, Berger RMF, van Melle JP, Hoendermis ES.

Eur Heart J Cardiovasc Imaging. 2017 May 22. doi: 10.1093/ehjci/jex133. [Epub ahead of print]

PMID: 28531295

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  1. Imaging the adult with transposition of the great arteries.

Anderson JH, Cetta F.

Curr Opin Cardiol. 2017 May 19. doi: 10.1097/HCO.0000000000000420. [Epub ahead of print]

PMID: 28537999

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  1. Frequency of Development of Aortic Valve Disease in Unrepaired Perimembranous Ventricular Septal Defects.

Padiyath A, Makil ES, Braley KT, Bolin EH, Tang X, Gossett JM, Collins RT 2nd.

Am J Cardiol. 2017 May 15;119(10):1670-1674. doi: 10.1016/j.amjcard.2017.02.004. Epub 2017 Feb 28.

PMID: 28325571

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Select item 28502268

 

  1. Cardiopulmonary exercise test in adults with prior Fontan operation: The prognostic value of serial testing.

Egbe AC, Driscoll DJ, Khan AR, Said SS, Akintoye E, Berganza FM, Connolly HM.

Int J Cardiol. 2017 May 15;235:6-10. doi: 10.1016/j.ijcard.2017.02.140. Epub 2017 Feb 28.

PMID: 28284501

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  1. [Congenital heart disease in adolescents and adults: Management in a general cardiology department in Senegal].

Mbaye A, Bodian M, Ngaïdé AA, Abdourafiq H, Leye MCBO, Savodogo S, Aw F, Ndiaye M, Kouamé I, Babaka K, Dioum M, Gaye ND, Sarr SA, Ndiaye MB, Kane AD, Kane A.

Ann Cardiol Angeiol (Paris). 2017 May 12. pii: S0003-3928(17)30020-3. doi: 10.1016/j.ancard.2017.02.003. [Epub ahead of print] French.

PMID: 28506578

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  1. Renal resistive index reflects Fontan pathophysiology and predicts mortality.

Ohuchi H, Negishi J, Hayama Y, Miyazaki A, Shiraishi I, Ichikawa H.

Heart. 2017 May 2. pii: heartjnl-2016-310812. doi: 10.1136/heartjnl-2016-310812. [Epub ahead of print]

PMID: 28465331

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  1. Anomalous origin of right coronary artery causing myocardial ischemia in a young patient.

Singhi AK, Bari EA, Banerjee S.

Ann Pediatr Cardiol. 2017 May-Aug;10(2):212-214. doi: 10.4103/apc.APC_151_16.

PMID: 28566835 Free PMC Article

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Select item 28566834

 

  1. Adult CHD: the ongoing need for physician counselling about heredity and contraceptive options.

Londono-Obregon C, Goldmuntz E, Davey BT, Zhang X, Slap GB, Kim YY.

Cardiol Young. 2017 May;27(4):671-676. doi: 10.1017/S1047951116001013. Epub 2016 Jul 7.

PMID: 27383541

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Select item 27373527

 

  1. Improving the quality of transition and transfer of care in young adults with congenital heart disease.

Everitt IK, Gerardin JF, Rodriguez FH 3rd, Book WM.

Congenit Heart Dis. 2017 May;12(3):242-250. doi: 10.1111/chd.12463. Review.

PMID: 28580608

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Select item 28244680

 

 

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ACHD Featured Articles of April 2017

Adult Congenital Heart Reviews of April 2017 Manuscripts, sponsored by the International Society for Adult Congenital Heart Disease

 

  1. Maternal and Fetal Outcomes of Admission for Delivery in Women With Congenital Heart Disease. Hayward RM, Foster E, Tseng ZH. JAMA Cardiol. 2017 Apr 12. doi: 10.1001/jamacardio.2017.0283. [Epub ahead of print] PMID: 28403428 Similar articles Select item 28412020

 

Take Home Points:

  • California delivery hospitals demonstrated an association with congenital heart disease, and congestive heart failure admissions, atrial arrhythmias, and fetal growth restriction.
  • Maternal complex congenital heart disease is associated with ventricular arrhythmias and maternal in hospital mortality, though rare.
  • Increased monitoring at the time of delivery may be recommended as a result.
  • Post delivery there is an increased likelihood of readmission that is markedly increased when compared to pregnant women without CHD.

 

Moe_Tabitha-WEBCommentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch:

This investigation from January 1st 2005 to December 31st, 2011 reviewed more than 3 million deliveries in the state of California with its diverse population, and a variety of centers.  Of those 3 million deliveries, 3189 women had noncomplex congenital heart disease and 262 had complex congenital heart disease. Women with CHD were more likely to undergo Caesarian delivery (39%), compared with women without CHD (32%). Interestingly 54.9% of the women in this cohort who had  Tetralogy of Fallot underwent C-section. This marked increase above baseline C-section rates clashes with the maternal indications for C-section, suggesting that the number of C-sections performed on women with TOF is out of proportion with those that are considered necessary by WHO risk stratification criteria. Complex congenital heart disease was associated with greater adjusted odds of serious ventricular arrhythmias and maternal in hospital mortality. Both simple and complex congenital heart disease women were at higher risk for readmission, atrial arrhythmias, heart failure, and fetal growth restriction as compared with the women without congenital heart disease.

This study expertly molds a large publicly available dataset from the HCUP data at UCSF allowing the authors to investigate an important question relating to outcomes associated with pregnancy in CHD.  Though valuable overall, this large database is limited by the specificity of coding classifications, and the transition from ICD 9 to ICD 10 in recent years, which occurred following the data collected in this paper.  Complex CHD included endocardial cushion defects, hypoplastic left heart syndrome, tetralogy of Fallot, transposition of the great arteries, truncus arteriosus, and univentricular heart defects.  All other defects were classified as noncomplex.

ACHD1 v2

The figure above alludes to the increasing likelihood of readmissions for patients with congenital heart disease over the course of the 6 years following delivery. Though this data does not differentiate non pregnancy related complications from overall cardiac complications, it does suggest that pregnancy may present an important milestone determining later outcomes. However, no comparative non-pregnant data is presented, making proper interpretation of such data somewhat speculative

This is nevertheless an important study as it demonstrates an overall decrease in maternal comorbidities compared with the previously reported data by Siu et al. More study as well as collaboration is certainly needed.

 

 

NT-proBNP predicts mortality in adults with transposition of the great arteries late after Mustard or Senning correction.

Popelová JR, Tomková M, Tomek J.

Congenit Heart Dis. 2017 Apr 17. doi: 10.1111/chd.12466. [Epub ahead of print]

PMID: 28419713

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Take Home Points:

 

  • Patients with a Mustard or Senning operation are at risk of unexpected deaths due to heart failure and arrhythmias
  • NT-proBNP was the most useful independent predictor of mortality (HR 281.39), using univariate Cox Regression. A value of 1000 pg/mL had a 88% sensitivity and 95% specificity for the prediction of mortality.
  • Surprisingly, mitral E velocity of 0.68 m/s or greater was associated with improved survival i.e. in the subpulmonary ventricle.

 

Blanche CupidoCommentary from Dr Blanche Cupido (Cape Town, South Africa), section editor of ACHD Journal Watch: Mustard and Senning corrections for transposition of the great arteries have largely been replaced by the arterial switch operation. However a number of such operations continue to be performed in the developing world for simple transposition of the great arteries. Although most patients remain asymptomatic for many years, they may die unexpectedly, usually as a result of heart failure of arrhythmias.  As risk factors for mortality, especially sudden cardiac death remain elusive, the authors of this study sought to investigate the predictors for mortality in adult patients with Mustard or Senning repairs.

 

Eighty-seven patients (39 Senning and 48 Mustard) were followed in an outpatient setting over a 10 years (2005-2015).  They underwent serial clinical [all], echocardiographic [all] and NT-proBNP measurements. CMR data was available in 18% and VO2max measurements in 44%.

 

The majority of patients were male (n=64, 74%). Those with a Mustard correction were significantly older (age 30 vs 21, p<0.0001) and had higher NT-proBNP levels (287 vs 144, p=0.00058) when compared to Senning patients.

 

The overall mortality was 9% (n=8) with no significant differences between the two groups. One of the patients died of early rejection post transplantation, the rest were due to heart failure.

ACHD2

 

NT-proBNP and the mitral E velocity were the only two independent predictors for death that emerged on multivariable analysis (NT-proBNP HR 281.39 (CI 13.06-6063, p=0.00048; and mitral E velocity HR 0.95 (CI 0.90-0.99, p=0.01815).

ACHD3

 

An NT-proBNP level of <1000 pg/mL correlated with improved survival.

ACHD4

 

A baseline mitral E velocity of >0.68m/s was associated with improved survival. Further validation is required. NYHA class and doppler and tissue doppler parameters relating to the systemic RV, surprisingly, did not show significant differences.

The findings of this study need verification however in larger datasets. Because of the relatively small numbers in this particular study, and the small number of outcomes, multivariable time dependent analysis was not possible.

 

Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977-2012.

Heart. 2017 Apr 27. pii: heartjnl-2016-310979. doi: 10.1136/heartjnl-2016-310979. [Epub ahead of print]

Hjortshøj CS1Jensen AS1Sørensen K2Nagy E3Johansson B4Kronvall T5Dellborg M6Estensen ME7Holmstrøm H8Turanlahti M9Thilén U10Søndergaard L1.

PMID: 28450553

Take Home Points:
• During the last 4 decades, the incidence and prevalence of Eisenmenger syndrome in the Nordic region has decreased.
• During the last 4 decades, the age of death increased, which may not necessarily reflect improved survival, but is more likely the result of decreased incidence and shifting of age distribution.
• Median survival of Eisenmenger syndrome patients is significantly lower than the background population.
• Down syndrome patients with Eisenmenger syndrome appear to have worse survival.

jokhadar-maanCommentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch: Eisenmenger syndrome (ES) is a late complication of unrepaired large systemic to pulmonary shunt that is characterized by increased pulmonary vascular resistance, pulmonary hypertension, and cyanosis with its associated features of digital clubbing and exertional dyspnea. ES was more common before modern diagnostic methods and surgical correction became available.
Though several studies report ES survival rates, the results are often influenced by so-called “immortal time bias” also known as survivor bias. Because recruit starts at time of diagnosis of start of late follow-up, there is an inherent bias toward survivors as earlier events such as mortality are not normally capture in such analyses. ES late survival is therefore likely to be overestimated when age is used as the time scale variable for Kaplan-Meier survival analyses without accounting for all events that have happened from the time of birth, which for most studies is not feasible.
Therefore, the time from diagnosis to start of follow-up should be removed from the analysis, by accounting for the age at entry to the study.
With this in mind, Dr. Hjortshøj and colleagues from Denmark, Sweden, Norway, and Finland performed a retrospective population based analysis through an extensive search of national registries and found 714 patients with ES between 1977 and 2012. It should be noted that patients with surgically corrected shunts but with persistent pulmonary hypertension were not included in the study.
The results were as follows:
ES incidence decreased from 2.5 to 0.23/million inhabitants/year.
ES prevalence decreased from 24.6 to 12.0/million inhabitants.
The mean age of the study population increased from about 19.9 in 1977 to 40.4 in 2012. The rate of deaths per million inhabitants increased as well. The absolute number of children with ES decreased throughout the study period.
The annual mortality rate was about 2.3%
The median survival was about 38.4 years, which is lower than the median survival of 81.6 years for the matched background control population.
Patients with simple shunts appear to survive longer than patients with complex lesions.
Down syndrome patients appeared to have worse mortality, particularly after factoring in the high preoperative mortality following shunt corrective surgery.
The mean age of death was 27.7 during the first era of the study (1977 to 1992), which was 38.8 during the second era (1993 to 2006), and was 46.3 during the last era (2006 to 2012). Though the study showed an increase in the age of death throughout the study, this may not represent improved longevity, but rather the result of decreasing incidence on age distribution that leads to an older study population.
The results of this study suggest that a reduced incidence of ES is due to earlier detection and treatment of patients with congenital heart disease. However, there will likely remain a small subset of patients with complex lesions who are unsuitable for surgical correction and will go on to develop ES. These findings in this Nordic population are likely similar to other developed countries, but quite different from developing countries where early access to diagnosis and treatment is less common.
Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in Eisenmenger syndrome.

Hascoët S1, Baruteau AE2, Humbert M3, Simonneau G3, Jais X3, Petit J2, Laux D2, Sitbon O3, Lambert V4, Capderou A5.

J Heart Lung Transplant. 2017 Apr;36(4):386-398. doi: 10.1016/j.healun.2016.10.006. Epub 2016 Oct 15.

 

 

Take Home Points:

 

  • There is little long-term data in usefulness of PAH-specific drug therapy (PAH-SDT) in Eisenmenger syndrome
  • In this study, 69 patients with Eisenmenger syndrome were followed over 20 years using a structured follow-up including thorough hemodynamics (with measured VO2) and clinical status
  • PAH-SDT resulted in improvement in measured hemodynamics in 68.0% of patients; however after a median of 4.9 years, changes in hemodynamic measures were no longer significant
  • Even with PAH-SDT, there was a high event rate (death, heart-lung transplantation, or listing for transplantation), with a 15-year cumulative event rate of 49.2%
  • Independent predictors of events were superior vena cava oxygen saturation and hemodynamic reponse to PAH-SDT

 

Kay_William_MD.16.CVaCommentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch: This study from France quite thoroughly evaluated a cohort of 69 patients with Eisenmenger syndrome who were treated with PAH-specific drug therapy (PAH-SDT).  All patients underwent thorough invasive hemodynamics, including a measured, rather than calculated, VO2, as well as 6-minute walk distance and WHO functional status.    All patients had measurement of indexed pulmonary output (Qpi), pulmonary vascular index (PVRi), and superior vena cava oxygen saturation.  All patients had an initial PVRi > 8 Woods units * m2 at the start of the study, and all patients were on PAH-SDT (i.e. there was no control group).

Measured hemodynamics improved in the majority of patients, 68.0% of the sample. The hemodynamic response and clinical response was generally initially good.  Overall increase in Qpi was 0.4 LPM/m2.  Overall increase in 6MW distane was +49 m.   Changes in hemodynamics and 6MW distance were assessed after each change in treatment (i.e. intensification or addition of a 2nd or 3rd drug).  The treatment effect was found not to be sustained over long-term follow-up, with changes no longer being significant after a median of 4.9 years.

Events were common; 33.3% of patients met the composite endpoint of death, heart-lung transplant, or listing for transplantation after a median of 7.2 years.  The 15-year cumulative event rate was 49.2%.  Median time to event was 8.1 years.  Patients with events had worse hemodynamics at baseline.  On univariate analysis, predictors of events were a PVRi > 30 Woods Units * m2, right atrial pressure > 4 mmHg, and SVC saturation < 65%, and pulmonary artery saturation < 70%.  On multivariate analysis,  SVC saturation was found to correlate with event rate.  Each 1% decrease in SVC saturation was associated with a 9.8% increase in hazard ratio for events.  SVC saturation in Eisenmenger syndrome reflects both arterial saturation as well as systemic cardiac output.

This study showed some evidence that patients with pretricuspid shunts may have more benefit from PAH-SDT than those with posttricuspid shunts; however the authors caution this may be artifact from “immortal survival bias.”  Atrial septal defects were the most common underlying CHD in this cohort (n=43, 62.3%).

The authors conclude that PAH-SDT can be helpful in Eisenmenger syndrome, but the regimen may need to be individualized base on hemodynamic changes for each patient.

ACHD5

 

 

Figure 4 Kaplan-Meier cumulative event curves in the sub-group (E),aortic oxygen saturation, (F) SVC oxygen saturation, (G),and pattern of hemodynamic change under PAH-SDT(H).

 

 

 

Is the presence of end-diastolic forward flow specific for restrictive right ventricular physiology in repaired tetralogy of Fallot?

 

Mori Y, Murakami T, Inoue N, Kaneko S, Nakashima Y, Koide M.

Int J Cardiol. 2017 Apr 11. pii: S0167-5273(16)34242-5. doi: 10.1016/j.ijcard.2017.04.021. [Epub ahead of print]

PMID

 

Take Home Points:

 

  • From past publications, end diastolic forward flow (EDFF) in repaired tetralogy of Fallot [TOF] has been considered to be synonymous with restrictive right ventricular (RV) physiology.
  • In the present study patients with EDFF have different hemodynamics as compared to those without: higher RA pressure, higher RA to PA pressures gradient, and had higher ANP levels
  • Not all patients who demonstrate EDFF, have restrictive RV physiology when taking invasive hemodynamics into account
  • Patients with a small RV and EDFF demonstrate restrictive RV physiology, and demonstrate associated invasive hemodynamics
  • Patients with large RV and EDFF do not demonstrate restrictive RV physiology. Instead, they may have very low pulmonary diastolic pressure, which is lower than right atrial pressure, resulting in EDFF during RA contraction.

 

Leong Ming ChernCommentary from Dr. M.C. Leong (Kuala Lumpur), section editor of ACHD Journal Watch:  The presence of end diastolic forward flow (EDFF) in the pulmonary arteries on echocardiography or cardiac magnetic resonance has been regarded as the hallmark echocardiopgraphic feature of restrictive right ventricular (RV) physiology in repaired Tetralogy of Fallot. However not all such patients have good late outcomes. A recent study found that TOF patients with EDFF have good effort tolerance only if associated with small right ventricles. The authors of the current study therefore sought to demonstrate that not all patients with EDFF on echocardiography have higher right ventricular filling pressures via cardiac catheterizations.

 

62 patients with repaired TOF were retrospectively reviewed. They were aged 15.7 ± 11.6 years, and underwent cardiac catheterization. Patients were then divided into Group 1 (n=23) with and Group 2 (n=39) without EDFF; Group A (n=53) with small RVs (<150ml/m2) and Group B (n=9) with larger RV (≥150ml/m2). Group 1 had a higher right atrial pressure, pressure gradient between the right atrium and pulmonary diastolic pressure and atrial natriuretic peptide levels. Group A had better RV and left ventricular ejection fraction and smaller LV size.

 

However, on further subgroup analysis, patients with EDFF and smaller RV (Group 1A) had higher right atrial pressure and less pulmonary regurgitation than the rest of the groups. Patients with large RV and EDFF (Group 1B) had right atrial pressure and RV end diastolic pressure similar to those without EDFF. This group of patients had higher pressure gradient between the right atrium and pulmonary diastolic pressure compared to patients without EDFF.

ACHD6ACHD7-1

 

The authors hence postulate that patients in Group 1A corresponds to “true” restrictive RV physiology. However, patients in Group 1B may not have true restrictive RV physiology, despite EDFF. Instead, they may have severe pulmonary regurgitation which results in very low pulmonary diastolic pressure, which created a high gradient between the right atrium and pulmonary diastolic pressure and hence EDFF.

    

 

ACHD April 2017

 

  1. Epidemiological changes in Eisenmenger syndrome in the Nordic region in 1977-2012.

Hjortshøj CS, Jensen AS, Sørensen K, Nagy E, Johansson B, Kronvall T, Dellborg M, Estensen ME, Holmstrøm H, Turanlahti M, Thilén U, Søndergaard L.

Heart. 2017 Apr 27. pii: heartjnl-2016-310979. doi: 10.1136/heartjnl-2016-310979. [Epub ahead of print]

PMID: 28450553

Similar articles

Select item 28449285

 

  1. Research Review: Childhood chronic physical illness and adult emotional health – a systematic review and meta-analysis.

Secinti E, Thompson EJ, Richards M, Gaysina D.

J Child Psychol Psychiatry. 2017 Apr 27. doi: 10.1111/jcpp.12727. [Epub ahead of print] Review.

PMID: 28449285

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Select item 28450386

 

  1. Heart transplantation for adults with congenital heart disease: current status and future prospects.

Matsuda H, Ichikawa H, Ueno T, Sawa Y.

Gen Thorac Cardiovasc Surg. 2017 Apr 24. doi: 10.1007/s11748-017-0777-x. [Epub ahead of print] Review.

PMID: 28439697

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  1. Critical involvement of ZEB2 in collagen fibrillogenesis: the molecular similarity between Mowat-Wilson syndrome and Ehlers-Danlos syndrome.

Teraishi M, Takaishi M, Nakajima K, Ikeda M, Higashi Y, Shimoda S, Asada Y, Hijikata A, Ohara O, Hiraki Y, Mizuno S, Fukada T, Furukawa T, Wakamatsu N, Sano S.

Sci Rep. 2017 Apr 19;7:46565. doi: 10.1038/srep46565.

PMID: 28422173 Free PMC Article

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Select item 28422570

 

  1. Attitudes and perceptions of pregnant women with CHD: results of a single-site survey.

Sabanayagam A, Briston D, Zaidi AN.

Cardiol Young. 2017 Apr 18:1-8. doi: 10.1017/S1047951116002857. [Epub ahead of print]

PMID: 28416028

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Select item 28430913

 

  1. Person-centred transition programme to empower adolescents with congenital heart disease in the transition to adulthood: a study protocol for a hybrid randomised controlled trial (STEPSTONES project).

Acuña Mora M, Sparud-Lundin C, Bratt EL, Moons P.

BMJ Open. 2017 Apr 17;7(4):e014593. doi: 10.1136/bmjopen-2016-014593.

PMID: 28420661 Free Article

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Select item 28412993

 

  1. Paediatric-onset coronary artery anomalies in pregnancy: a single-centre experience and systematic literature review.

Keir M, Bhagra C, Vatenmakher D, Arancibia-Galilea F, Jansen K, Toh N, Silversides CK, Colman J, Siu SC, Sermer M, Crean AM, Wald RM.

Cardiol Young. 2017 Apr 17:1-9. doi: 10.1017/S1047951117000658. [Epub ahead of print]

PMID: 28412993

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Select item 28419713

 

  1. NT-proBNP predicts mortality in adults with transposition of the great arteries late after Mustard or Senning correction.

Popelová JR, Tomková M, Tomek J.

Congenit Heart Dis. 2017 Apr 17. doi: 10.1111/chd.12466. [Epub ahead of print]

PMID: 28419713

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Select item 28417257

 

  1. Stroke in adults with Down syndrome.

Marto JP, Dias M, Alves JN, Montes V, Beato-Coelho J, Marques-Matos C, de Sousa A, Bernardo F, Calado S, Viana-Baptista M.

J Neurol Sci. 2017 Apr 15;375:279-280. doi: 10.1016/j.jns.2017.02.014. Epub 2017 Feb 8. No abstract available.

PMID: 28320148

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Select item 28408455

 

  1. Pregnancy in Adult Congenital Heart Disease: Special Delivery.

Davidson WR Jr.

JAMA Cardiol. 2017 Apr 12. doi: 10.1001/jamacardio.2017.0365. [Epub ahead of print] No abstract available.

PMID: 28403448

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Select item 28403428

 

  1. Maternal and Fetal Outcomes of Admission for Delivery in Women With Congenital Heart Disease.

Hayward RM, Foster E, Tseng ZH.

JAMA Cardiol. 2017 Apr 12. doi: 10.1001/jamacardio.2017.0283. [Epub ahead of print]

PMID: 28403428

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Select item 28412020

 

  1. Is Takotsubo Syndrome a benign condition?

Drakopoulou MI, Gatzoulis MA.

Hellenic J Cardiol. 2017 Apr 8. pii: S1109-9666(17)30141-0. doi: 10.1016/j.hjc.2017.03.009. [Epub ahead of print]

PMID: 28400292 Free Article

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Select item 28385091

 

  1. Hypoplasia of the posterior mitral leaflet: A rare cause of mitral regurgitation in adulthood.

Bacich D, Braggion G, Faggian G.

Echocardiography. 2017 Apr 7. doi: 10.1111/echo.13537. [Epub ahead of print]

PMID: 28386957

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Select item 28396253

 

  1. Plastic bronchitis: An unusual complication of acute chest syndrome in adult.

Feray S, Mora P, Decavele M, Pham T, Hafiani EM, Fartoukh M.

Respir Med Case Rep. 2017 Apr 7;21:93-95. doi: 10.1016/j.rmcr.2017.04.006. eCollection 2017.

PMID: 28443234 Free PMC Article

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Select item 28343629

 

  1. Cardiopulmonary Exercise Testing in Adult Congenital Heart Disease.

Mantegazza V, Apostolo A, Hager A.

Ann Am Thorac Soc. 2017 Apr 4. doi: 10.1513/AnnalsATS.201611-876FR. [Epub ahead of print]

PMID: 28375677

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Select item 28395882

 

  1. Incidence and predictors of obstetric and fetal complications in women with structural heart disease.

van Hagen IM, Roos-Hesselink JW, Donvito V, Liptai C, Morissens M, Murphy DJ, Galian L, Bazargani NM, Cornette J, Hall R, Johnson MR.

Heart. 2017 Apr 4. pii: heartjnl-2016-310644. doi: 10.1136/heartjnl-2016-310644. [Epub ahead of print]

PMID: 28377476

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Select item 28377475

 

  1. Diagnosis, imaging and clinical management of aortic coarctation.

Dijkema EJ, Leiner T, Grotenhuis HB.

Heart. 2017 Apr 4. pii: heartjnl-2017-311173. doi: 10.1136/heartjnl-2017-311173. [Epub ahead of print] Review.

PMID: 28377475

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Select item 28359509

 

  1. Clinical Characteristics of Adult Dogs More Than 5 Years of Age at Presentation for Patent Ductus Arteriosus.

Boutet BG, Saunders AB, Gordon SG.

J Vet Intern Med. 2017 Apr 3. doi: 10.1111/jvim.14689. [Epub ahead of print]

PMID: 28370380 Free Article

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Select item 28384795

 

  1. Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature.

Smith K, Gros B.

Congenit Heart Dis. 2017 Apr 2. doi: 10.1111/chd.12465. [Epub ahead of print] Review.

PMID: 28371362

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Select item 28441822

 

  1. [Assessment of undiagnosed critical congenital heart disease before discharge from the maternity hospital].

Zhao QM, Liu F, Wu L, Ye M, Jia B, Ma XJ, Huang GY.

Zhonghua Er Ke Za Zhi. 2017 Apr 2;55(4):260-266. doi: 10.3760/cma.j.issn.0578-1310.2017.04.006. Chinese.

PMID: 28441821

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Select item 28441820

 

  1. National In-Hospital Outcomes of Pregnancy in Women With Single Ventricle Congenital Heart Disease.

Collins RT 2nd, Chang D, Sandlin A, Goudie A, Robbins JM.

Am J Cardiol. 2017 Apr 1;119(7):1106-1110. doi: 10.1016/j.amjcard.2016.12.015. Epub 2017 Feb 16.

PMID: 28242012

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Select item 28328118

 

  1. High prevalence of fatigue in adults with a 22q11.2 deletion syndrome.

Vergaelen E, Claes S, Kempke S, Swillen A.

Am J Med Genet A. 2017 Apr;173(4):858-867. doi: 10.1002/ajmg.a.38094. Epub 2017 Feb 12.

PMID: 28190295

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Select item 27956203

 

  1. Biomarkers of Calcific Aortic Valve Disease.

Small A, Kiss D, Giri J, Anwaruddin S, Siddiqi H, Guerraty M, Chirinos JA, Ferrari G, Rader DJ.

Arterioscler Thromb Vasc Biol. 2017 Apr;37(4):623-632. doi: 10.1161/ATVBAHA.116.308615. Epub 2017 Feb 2. Review.

PMID: 28153876

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Select item 28212920

 

  1. Bicuspid aortic valve outcomes.

Rodrigues I, Agapito AF, de Sousa L, Oliveira JA, Branco LM, Galrinho A, Abreu J, Timóteo AT, Rosa SA, Ferreira RC.

Cardiol Young. 2017 Apr;27(3):518-529. doi: 10.1017/S1047951116002560. Epub 2016 Dec 12.

PMID: 27938448

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Select item 27766996

 

  1. Comparison of participants and non-participants in patient-reported outcome surveys: the case of Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease – International Study.

Berghammer MC, Mattsson E, Johansson B, Moons P, Dellborg M.

Cardiol Young. 2017 Apr;27(3):427-434. doi: 10.1017/S1047951116000676. Epub 2016 May 11.

PMID: 27167242

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Select item 27416545

 

  1. Tricuspid annular plane systolic excursion is preserved in young patients with pulmonary hypertension except when associated with repaired congenital heart disease.

Hauck A, Guo R, Ivy DD, Younoszai A.

Eur Heart J Cardiovasc Imaging. 2017 Apr 1;18(4):459-466. doi: 10.1093/ehjci/jew068.

PMID: 27099276

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Select item 28329112

 

  1. Does functional capacity depend on the size of the shunt? A prospective, cohort study of adults with small, unrepaired ventricular septal defects.

Maagaard M, Heiberg J, Asschenfeldt B, Ringgaard S, Hjortdal VE.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):722-727. doi: 10.1093/ejcts/ezw420.

PMID: 28204298

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Select item 28199509

 

  1. The effect of a resilience improvement program for adolescents with complex congenital heart disease.

Lee S, Lee J, Choi JY.

Eur J Cardiovasc Nurs. 2017 Apr;16(4):290-298. doi: 10.1177/1474515116659836. Epub 2016 Jul 10.

PMID: 27400701

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Select item 28431063

 

  1. Recommendations on the Echocardiographic Assessment of Aortic Valve Stenosis: A Focused Update from the European Association of Cardiovascular Imaging and the American Society of Echocardiography.

Baumgartner H, Hung J, Bermejo J, Chambers JB, Edvardsen T, Goldstein S, Lancellotti P, LeFevre M, Miller F Jr, Otto CM.

J Am Soc Echocardiogr. 2017 Apr;30(4):372-392. doi: 10.1016/j.echo.2017.02.009.

PMID: 28385280

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Select item 28306488

 

  1. Clinical recommendations of cardiac magnetic resonance, Part II: inflammatory and congenital heart disease, cardiomyopathies and cardiac tumors: a position paper of the working group ‘Applicazioni della Risonanza Magnetica’ of the Italian Society of Cardiology.

Pontone G, Di Bella G, Silvia C, Maestrini V, Festa P, Ait-Ali L, Masci PG, Monti L, di Giovine G, De Lazzari M, Cipriani A, Guaricci AI, Dellegrottaglie S, Pepe A, Marra MP, Aquaro GD.

J Cardiovasc Med (Hagerstown). 2017 Apr;18(4):209-222. doi: 10.2459/JCM.0000000000000499.

PMID: 28092290

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Select item 27986330

 

  1. Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in Eisenmenger syndrome.

Hascoët S, Baruteau AE, Humbert M, Simonneau G, Jais X, Petit J, Laux D, Sitbon O, Lambert V, Capderou A.

J Heart Lung Transplant. 2017 Apr;36(4):386-398. doi: 10.1016/j.healun.2016.10.006. Epub 2016 Oct 15.

PMID: 27866929

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Select item 28415932

 

  1. [The Relationships among Perceived Parental Bonding, Illness Perception, and Anxiety in Adult Patients with Congenital Heart Diseases].

Shin N, Jang Y, Kang Y.

J Korean Acad Nurs. 2017 Apr;47(2):178-187. doi: 10.4040/jkan.2017.47.2.178. Korean.

PMID: 28470155

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Select item 28302382

 

  1. Use of Cardiac Computed Tomography for Ventricular Volumetry in Late Postoperative Patients with Tetralogy of Fallot.

Kim HJ, Mun DN, Goo HW, Yun TJ.

Korean J Thorac Cardiovasc Surg. 2017 Apr;50(2):71-77. doi: 10.5090/kjtcs.2017.50.2.71. Epub 2017 Apr 5.

PMID: 28382264 Free PMC Article

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Select item 26891904

 

  1. Readiness for Transition to Adult Health Care for Young Adolescents with Congenital Heart Disease.

Stewart KT, Chahal N, Kovacs AH, Manlhiot C, Jelen A, Collins T, McCrindle BW.

Pediatr Cardiol. 2017 Apr;38(4):778-786. doi: 10.1007/s00246-017-1580-2. Epub 2017 Feb 10.

PMID: 28184978

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Select item 28184976

 

  1. Decreased Diastolic Ventricular Kinetic Energy in Young Patients with Fontan Circulation Demonstrated by Four-Dimensional Cardiac Magnetic Resonance Imaging.

Sjöberg P, Heiberg E, Wingren P, Ramgren Johansson J, Malm T, Arheden H, Liuba P, Carlsson M.

Pediatr Cardiol. 2017 Apr;38(4):669-680. doi: 10.1007/s00246-016-1565-6. Epub 2017 Feb 10. Erratum in: Pediatr Cardiol. 2017 Apr 29;:.

PMID: 28184976 Free PMC Article

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Select item 28184975

 

  1. Magnetic resonance imaging 4-D flow-based analysis of aortic hemodynamics in Turner syndrome.

Arnold R, Neu M, Hirtler D, Gimpel C, Markl M, Geiger J.

Pediatr Radiol. 2017 Apr;47(4):382-390. doi: 10.1007/s00247-016-3767-8. Epub 2017 Feb 9.

PMID: 28184962

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Select item 28132084

 

  1. Relationship Between Fragmented QRS Complexes and Cardiac Status in Duchenne Muscular Dystrophy: Multimodal Validation Using Echocardiography, Magnetic Resonance Imaging, and Holter Monitoring.

Cho MJ, Lee JW, Lee J, Shin YB, Lee HD.

Pediatr Cardiol. 2017 Apr 29. doi: 10.1007/s00246-017-1616-7. [Epub ahead of print]

PMID: 28456833

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Select item 28456832

 

  1. Validation of numerical simulation methods in aortic arch using 4D Flow MRI.

Miyazaki S, Itatani K, Furusawa T, Nishino T, Sugiyama M, Takehara Y, Yasukochi S.

Heart Vessels. 2017 Apr 25. doi: 10.1007/s00380-017-0979-2. [Epub ahead of print]

PMID: 28444501

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Select item 28377612

 

  1. Natural history of aortic root dilation through young adulthood in a hypermobile Ehlers-Danlos syndrome cohort.

Ritter A, Atzinger C, Hays B, James J, Shikany A, Neilson D, Martin L, Weaver KN.

Am J Med Genet A. 2017 Apr 24. doi: 10.1002/ajmg.a.38243. [Epub ahead of print]

PMID: 28436618

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Select item 28436679

 

  1. Topsy-turvy heart and associated imaging findings.

Bayramoglu Z, Yılmaz R, Demir AA, Yekeler E, Dursun M, Dindar A, Nisli K, Omeroglu R.

J Cardiovasc Comput Tomogr. 2017 Apr 20. pii: S1934-5925(17)30093-X. doi: 10.1016/j.jcct.2017.04.008. [Epub ahead of print] No abstract available.

PMID: 28438441

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Select item 28366040

 

  1. Is the presence of end-diastolic forward flow specific for restrictive right ventricular physiology in repaired tetralogy of Fallot?

Mori Y, Murakami T, Inoue N, Kaneko S, Nakashima Y, Koide M.

Int J Cardiol. 2017 Apr 11. pii: S0167-5273(16)34242-5. doi: 10.1016/j.ijcard.2017.04.021. [Epub ahead of print]

PMID: 28457561

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Select item 28410946

 

  1. Aortopathy in an Adult With Tricuspid Atresia and Left Ventricular Non-Compaction After Fontan Procedure.

Murakami T, Mori Y, Inoue N, Kaneko S, Nakashima Y.

Circ J. 2017 Apr 5. doi: 10.1253/circj.CJ-17-0016. [Epub ahead of print] No abstract available.

PMID: 28381694 Free Article

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Select item 28389305

 

  1. Presentation of missed childhood Kawasaki disease in adults: Experience from a tertiary care center in north India.

Vijayvergiya R, Bhattad S, Varma S, Singhal M, Gordon J, Singh S.

Int J Rheum Dis. 2017 Apr 5. doi: 10.1111/1756-185X.13073. [Epub ahead of print]

PMID: 28378434

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Select item 28381550

 

  1. Revisiting the utility of technical performance scores following tetralogy of Fallot repair.

Lodin D, Mavrothalassitis O, Haberer K, Sunderji S, Quek RGW, Peyvandi S, Moon-Grady A, Karamlou T.

J Thorac Cardiovasc Surg. 2017 Apr 5. pii: S0022-5223(17)30640-2. doi: 10.1016/j.jtcvs.2017.02.066. [Epub ahead of print]

PMID: 28461051

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Select item 28382463

 

  1. Fontan survival: As good as it gets?

Thankavel P, Jaquiss RDB.

J Thorac Cardiovasc Surg. 2017 Apr 4. pii: S0022-5223(17)30633-5. doi: 10.1016/j.jtcvs.2017.03.114. [Epub ahead of print] No abstract available.

PMID: 28434620

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Select item 28378408

 

  1. Decreased false-positive adolescent pre-athletic screening with Seattle Criteria-interpreted electrocardiograms.

Colombo JN, Samson RA, Valdes SO, Meziab O, Sisk D, Klewer SE.

Cardiol Young. 2017 Apr;27(3):512-517. doi: 10.1017/S104795111600086X. Epub 2016 Jun 20.

PMID: 27322729

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Select item 27161562

 

  1. Mechanism of Progressive Heart Failure and Significance of Pulmonary Hypertension in Obstructive Hypertrophic Cardiomyopathy.

Covella M, Rowin EJ, Hill NS, Preston IR, Milan A, Opotowsky AR, Maron BJ, Maron MS, Maron BA.

Circ Heart Fail. 2017 Apr;10(4):e003689. doi: 10.1161/CIRCHEARTFAILURE.116.003689.

PMID: 28396501

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Select item 27905124

 

  1. Resilience and personal growth: A potential resource for therapeutic programmes in people with congenital heart disease.

Dorka R.

Eur J Cardiovasc Nurs. 2017 Apr;16(4):270-271. doi: 10.1177/1474515116687223. Epub 2017 Jan 10. No abstract available.

PMID: 28071940

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Select item 27400701

 

  1. Loss-of-function mutations in the X-linked biglycan gene cause a severe syndromic form of thoracic aortic aneurysms and dissections.

Meester JA, Vandeweyer G, Pintelon I, Lammens M, Van Hoorick L, De Belder S, Waitzman K, Young L, Markham LW, Vogt J, Richer J, Beauchesne LM, Unger S, Superti-Furga A, Prsa M, Dhillon R, Reyniers E, Dietz HC, Wuyts W, Mortier G, Verstraeten A, Van Laer L, Loeys BL.

Genet Med. 2017 Apr;19(4):386-395. doi: 10.1038/gim.2016.126. Epub 2016 Sep 15.

PMID: 27632686 Free PMC Article

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Select item 28279417

 

  1. Does Body Mass Index Predict Premature Cardiomyopathy Onset for Duchenne Muscular Dystrophy?

McKane M, Soslow JH, Xu M, Saville BR, Slaughter JC, Burnette WB, Markham LW.

J Child Neurol. 2017 Apr;32(5):499-504. doi: 10.1177/0883073816687422. Epub 2017 Jan 13.

PMID: 28084148

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Select item 28324046

 

  1. Clinical and magnetic resonance evolution of “infarct-like” myocarditis.

Faletti R, Gatti M, Baralis I, Bergamasco L, Bonamini R, Ferroni F, Imazio M, Stola S, Gaita F, Fonio P.

Radiol Med. 2017 Apr;122(4):273-279. doi: 10.1007/s11547-016-0723-5. Epub 2017 Jan 9.

PMID: 28070840

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Select item 28096476

 

  1. The importance of the neutrophil-to-lymphocyte ratio in patients with hypertrophic cardiomyopathy.

Ozyilmaz S, Akgul O, Uyarel H, Pusuroglu H, Gul M, Satilmisoglu MH, Bolat I, Ozyilmaz I, Uçar H, Yildirim A, Bakir I.

Rev Port Cardiol. 2017 Apr;36(4):239-246. doi: 10.1016/j.repc.2016.09.014. Epub 2017 Mar 18. English, Portuguese.

PMID: 28318851

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Select item 28142228

 

 

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