CHD Intervention Featured Articles of April 2017

Cardiac Catheterization Reviews of April 2017 Manuscripts

 

Tricuspid Regurgitation Does Not Impact Right Ventricular Remodeling After Percutaneous Pulmonary Valve Implantation.

Tanase D, Ewert P, Georgiev S, Meierhofer C, Pabst von Ohain J, McElhinney DB, Hager A, Kühn A, Eicken A.

JACC Cardiovasc Interv. 2017 Apr 10;10(7):701-708. doi: 10.1016/j.jcin.2017.01.036.

 

Take Home Points:

 

  • Percutaneous pulmonary valve implantation (PPVI) improves right ventricular (RV) size and exercise performance regardless of the severity of tricuspid regurgitation (TR).
  • Severity of TR improves following PPVI, and therefore the presence of significant TR should not preclude attempts at PPVI.

 

Whiteside, WendyComment from Dr. Wendy Whiteside (Cincinnati), section editor of Congenital Heart Disease Interventions Journal Watch:  Percutaneous pulmonary valve implantation (PPVI) has become the therapy of choice for treatment of RV outflow tract (RVOT) conduit dysfunction.  In patients presenting for PPVI, TR is a frequent associated finding, occurring in as many as 1/3 of patients.  There are multiple etiologies and mechanisms for TR in these patients, including annular dilation in the setting of a dilated right ventricle, valvar dysplasia, or surgical or pacemaker lead disruption, however the presence of significant TR may impact the decision to perform pulmonary valve replacement via a percutaneous or surgical approach and may affect the ability for the RV to remodel following pulmonary valve replacement.  If significant TR is present and the tricuspid valve is felt to require surgical intervention at the time of valve replacement, these patients may be referred exclusively for surgical intervention without consideration for a less invasive approach.  However, if the TR can be improved just by improving the RVPA conduit dysfunction and subsequent RV remodeling, these patients may be able to have a less invasive percutaneous procedure rather than a surgical one.

 

To investigate the impact of moderate or severe TR on RV remodeling after PPVI, Tanase et al conducted a matched cohort study comparing patients who underwent percutaneous pulmonary valve implantation (PPVI) either with significant TR (moderate or severe) or without significant TR, assessing the outcomes of TR severity by echocardiogram, RV size by cardiac MRI, and exercise performance by objective measures from cardiopulmonary exercise testing.

 

Among the 173 patients at their center who underwent PPVI over an 8-year period, patients with moderate to severe TR by echocardiographic criteria were identified, and represented 13% of their PPVI population.  Ultimately, the study included 18 patients with RVOT dysfunction and significant TR and 18 matched control subjects with RVOT dysfunction but no TR.  Patients were matched to have the same pulmonary valve pathology (primary stenosis, insufficiency, mixed), similar indexed RV end-diastolic volume, and similar NYHA functional classification).  Data were obtained at baseline, 6 months post-PPVI, and at latest follow-up (median of 6.5 years post-PPVI, range 8 months to 9.3 years).  All patients with significant TR were identified by serial echocardiography (looking back at least 8 years prior to PPVI) and found to have gradually increasing TR over time with no patients having a sudden increase in TR after surgery to suggest a primary anatomic valve issue.

 

Median patient age was 22±8 years, majority had NYHA functional class II symptoms, median peak oxygen uptake (VO2 peak) was 28.5 mlO2/kg/min, and median RVEDVi was 100 ml/m2 (range 61-185 ml/m2).  After PPVI, the degree of TR improved in 15 of 18 patients (83%) and was unchanged in the remaining 3 patients at 6-month follow-up.  At latest follow-up, no patient had significant TR (15 trivial and 3 mild).  There was a significant decrease in RVEDVi from pre- to post-PPVI with no difference between TR groups.  Similarly, while VO2 increased following PPVI, there was no difference in this increase between TR groups.  Tanase et al therefore conclude that in most patients with RVOT conduit dysfunction and significant TR, PPVI leads to a reduction in TR.  PPVI also decreases RV volume and improves exercise tolerance without a difference between baseline TR severity groups

 

These findings help to provide insight into a clinically very meaningful question—whether the presence of significant TR in a patient with a dysfunctional RVOT conduit should play into the decision for type and timing of intervention.  While they provide convincing evidence to suggest that secondary TR will improve following PPVI, there some important limitations to consider. The mechanism of TR is an important variable in the ability to improve TR.  Tanase et al attempted to exclude patients with a primary tricuspid valve abnormality (by showing a slow progression of TR over time in the included study patients).  Generalizing these results, therefore, to patients with primary valve abnormalities (dysplastic or surgically manipulated valves) should be done with caution.  Secondly, the median RVEDVi of included patients is small (100 ml/m2) and may not be representative of most PPVI populations.  This may lessen the effect of the additional volume load of TR on RV remodeling following PPVI.  Despite these limitations and small patient size, this study is of interest and should be considered in decision making for patients with RVOT conduit dysfunction and concomitant TR.

 

 

 

 

 

 

Impacts of early cardiac catheterization for children with congenital heart disease supported by extracorporeal membrane oxygenation.

Kato A, Lo Rito M, Lee KJ, Haller C, Guerguerian AM, Sivarajan VB, Honjo O.

Catheter Cardiovasc Interv. 2017 Apr;89(5):898-905. doi: 10.1002/ccd.26632. Epub 2016 Jul 14.

PMID: 27416545

 

Take Home Points:

  • Early catheterization is associated with shorter duration of ECMO support and higher survival probability at 30 days after ECMO cannulation.
  • ECMO-related end-organ dysfunction is a significantly poor prognostic factor for successful decannulation.

 

Averin , KonstantinCommentary from Dr. Konstantin Averin (Edmonton), section editor of Congenital Heart Disease Interventions Journal Watch: Extracorporeal membrane oxygenation (ECMO) has been widely used in the pediatric population for cases of peri-operative hemodynamic instability, failure to wean from cardiopulmonary bypass, and cardiopulmonary resuscitation. Cardiac catheterization in patients on ECMO support poses significant challenges but can potentially be useful in improving clinical outcomes. This single center retrospective cohort study focused on patients with congenital heart disease (CHD) and hypothesized that timing of cardiac catheterization may be a predictor of clinical outcomes. The specific aims of the study were: (1) to analyze the institution’s experience with cardiac catheterization on pediatric patients with CHD supported by cardiac ECMO and (2) to determine factors associated with successful weaning from ECMO and short-term outcomes.

 

Three hundred and forty-two patients required ECMO support between 2000 and 2014. Of these, 47 underwent 49 cardiac catheterizations that met inclusion criteria with a median patient age of 65 days and median weight 4.2kg. ECMO was successfully weaned in 33 patients (70%) after a median support time of 4 days with 51% of patients surviving to hospital discharge. Cardiac catheterizations were performed a median of 1 day after ECMO initiation and during 27 of them an intervention was performed (balloon/stent angioplasty and balloon atrial septostomy). There was no procedure related mortality but 9 (18%) procedure-related serious complications with 8 occurring during interventional catheterization (pulmonary artery rupture, stent dislodgement, arrhythmia, pulmonary hemorrhage, and sheath tip migration). In a multivariate analysis, absence of renal or respiratory complications were prognostic of successful ECMO weaning. Kaplan-Meier analysis demonstrated that patient who underwent earlier catheterization (<48 hours after cannulation) had a higher survival probability at 30 days after ECMO cannulation compared with late catheterization.

 

The authors conclude that early cardiac catheterization in pediatric patients with CHD who require ECMO support may be associated with better short-term survival. Early cardiac catheterization should be considered in this cohort, especially if there are unresolved anatomic or physiologic questions. The absence of ECMO-related complications is a predictor for successful weaning off ECMO.

cath 1 april

 

 

Impact of imaging approach on radiation dose and associated cancer risk in children undergoing cardiac catheterization.

Hill KD, Wang C, Einstein AJ, Januzis N, Nguyen G, Li JS, Fleming GA, Yoshizumi TK.

Catheter Cardiovasc Interv. 2017 Apr;89(5):888-897. doi: 10.1002/ccd.26630. Epub 2016 Jun 17.

PMID: 27315598

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Take Home Points:

  • Congenital cardiac catheterizations utilize much higher doses of ionizing radiation than other imaging modalities, such as computed tomography, increasing the lifetime cancer risk for patients
  • Traditional measures of radiation exposure (air kerma or dose-area product) do not accurately measure the true tissue exposure to radiation
  • Altering the methods of image acquisition (collimation, magnification, minimizing source to image distance, removal of antiscatter grids, lower acquisition frame rates) lead to marked variation in effective radiation doses and are easily modifiable by the interventional cardiologist

 

Seckler, MikeCommentary from Dr. Michael Seckeler (Tucson), section editor of Congenital Heart Disease Interventions Journal Watch: The authors have undertaken a very important study for any practitioner who utilizes ionizing radiation for imaging pediatric patients. Using two patient phantoms representing a newborn and a 5-year-old child, they systematically compared the relative effects of different alterable parameters of fluoroscopic imaging used in standard congenital catheterization laboratories on effective radiation doses in the phantoms. In addition, they were able to estimate lifetime cancer risks based on the effective radiation doses. This is the first study to look at this in pediatric patients.

 

The modifiable factors included collimation, magnification, minimizing source to image distance, removal of antiscatter grids and lower acquisition frame rates. These were tested in various combinations and effective radiation doses measured. Optimizing the imaging for minimal radiation exposure (lower magnification, maximal collimation, lowest source to image distance, removal of antiscatter grids and lower acquisition frame rates) all led to significant reductions in the effective radiation doses and lower predicted lifetime cancer risks (Figure 1).

 

The authors do report several limitations to their conclusions. First, optimizing the radiation exposure does not always lead to the best image quality, which has the potential to compromise patient safety during a procedure. However, by highlighting how each of several different factors can improve radiation exposure, this data will allow interventional cardiologists to make as many modifications as possible to find the optimal balance between safety and image quality. Second, the radiation exposures were derived from phantoms and mathematical simulations; however, the intention was not to provide exact doses, but the relative changes in exposure with changes in imaging modality.

This is a very important paper for our field and provides tools to help interventional cardiologists minimize the radiation exposure for our patients.

cath 2 april

CHD Interventions April 2017

 

  1. Real-time three dimensional CT and MRI to guide interventions for congenital heart disease and acquired pulmonary vein stenosis.

Suntharos P, Setser RM, Bradley-Skelton S, Prieto LR.

Int J Cardiovasc Imaging. 2017 Apr 28. doi: 10.1007/s10554-017-1151-x. [Epub ahead of print]

PMID: 28455631

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  1. Clinical Trial Principles and Endpoint Definitions for Paravalvular Leaks in Surgical Prosthesis: An Expert Statement.

Ruiz CE, Hahn RT, Berrebi A, Borer JS, Cutlip DE, Fontana G, Gerosa G, Ibrahim R, Jelnin V, Jilaihawi H, Jolicoeur EM, Kliger C, Kronzon I, Leipsic J, Maisano F, Millan X, Nataf P, O’Gara PT, Pibarot P, Ramee SR, Rihal CS, Rodes-Cabau J, Sorajja P, Suri R, Swain JA, Turi ZG, Tuzcu EM, Weissman NJ, Zamorano JL, Serruys PW, Leon MB; Paravalvular Leak Academic Research Consortium..

J Am Coll Cardiol. 2017 Apr 25;69(16):2067-2087. doi: 10.1016/j.jacc.2017.02.038. Review.

PMID: 28427582

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  1. In vivo intracardiac vector velocity imaging using phased array transducers for pediatric cardiology.

Fadnes S, Wigen M, Nyrnes SA, Lovstakken L.

IEEE Trans Ultrason Ferroelectr Freq Control. 2017 Apr 24. doi: 10.1109/TUFFC.2017.2689799. [Epub ahead of print]

PMID: 28436859

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  1. Prediction of adverse events after catheter-based procedures in adolescents and adults with congenital heart disease in the IMPACT registry.

Stefanescu Schmidt AC, Armstrong A, Kennedy KF, Nykanen D, Aboulhosn J, Bhatt AB.

Eur Heart J. 2017 Apr 18. doi: 10.1093/eurheartj/ehx200. [Epub ahead of print]

PMID: 28430913

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  1. Clinical Trial Principles and Endpoint Definitions for Paravalvular Leaks in Surgical Prosthesis: An Expert Statement.

Ruiz CE, Hahn RT, Berrebi A, Borer JS, Cutlip DE, Fontana G, Gerosa G, Ibrahim R, Jelnin V, Jilaihawi H, Jolicoeur EM, Kliger C, Kronzon I, Leipsic J, Maisano F, Millan X, Nataf P, O’Gara PT, Pibarot P, Ramee SR, Rihal CS, Rodes-Cabau J, Sorajja P, Suri R, Swain JA, Turi ZG, Tuzcu EM, Weissman NJ, Zamorano JL, Serruys PW, Leon MB; of the Paravalvular Leak Academic Research Consortium..

Eur Heart J. 2017 Apr 18. doi: 10.1093/eurheartj/ehx211. [Epub ahead of print]

PMID: 28430909

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  1. Cell Therapy Trials in Congenital Heart Disease.

Oh H.

Circ Res. 2017 Apr 14;120(8):1353-1366. doi: 10.1161/CIRCRESAHA.117.309697. Review.

PMID: 28408455

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  1. A systematic review of 3-D printing in cardiovascular and cerebrovascular diseases.

Sun Z, Lee SY.

Anatol J Cardiol. 2017 Apr 10. doi: 10.14744/AnatolJCardiol.2017.7464. [Epub ahead of print]

PMID: 28430115 Free Article

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  1. Avoidable costs of stenting for aortic coarctation in the United Kingdom: an economic model.

Salcher M, Mcguire A, Muthurangu V, Kelm M, Kuehne T, Naci H; CARDIOPROOF..

BMC Health Serv Res. 2017 Apr 10;17(1):258. doi: 10.1186/s12913-017-2215-2.

PMID: 28395657 Free PMC Article

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  1. Coincidental Significant Tricuspid Regurgitation at the Time of Right Ventricle-to-Pulmonary Artery Conduit Intervention: Should We Address it, Ignore it, or Take a More Nuanced Approach?

Hebson CL, Ephrem G, Rodriguez FH 3rd.

JACC Cardiovasc Interv. 2017 Apr 10;10(7):709-711. doi: 10.1016/j.jcin.2017.02.032. No abstract available.

PMID: 28385409

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  1. Coronary Sinus Defect Following Transcatheter Closure of ASD Using Amplatzer Septal Occluder: Potential Erosion by the Device.

Mohammad Nijres B, Al-Kubaisi M, Bokowski J, Abdulla RI, Awad S.

Pediatr Cardiol. 2017 Apr 10. doi: 10.1007/s00246-017-1613-x. [Epub ahead of print]

PMID: 28396933

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  1. Impacts of early cardiac catheterization for children with congenital heart disease supported by extracorporeal membrane oxygenation.

Kato A, Lo Rito M, Lee KJ, Haller C, Guerguerian AM, Sivarajan VB, Honjo O.

Catheter Cardiovasc Interv. 2017 Apr;89(5):898-905. doi: 10.1002/ccd.26632. Epub 2016 Jul 14.

PMID: 27416545

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  1. Patent foramen ovale with right atrial septal pouch.

Kijima Y, Bokhoor P, Tobis JM.

Catheter Cardiovasc Interv. 2017 Apr;89(5):E169-E171. doi: 10.1002/ccd.26357. Epub 2015 Dec 29.

PMID: 26711371

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  1. In-hospital cost comparison between percutaneous pulmonary valve implantation and surgery.

Andresen B, Mishra V, Lewandowska M, Andersen JG, Andersen MH, Lindberg H, Døhlen G, Fosse E.

Eur J Cardiothorac Surg. 2017 Apr 1;51(4):747-753. doi: 10.1093/ejcts/ezw378.

PMID: 28007875 Free PMC Article

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  1. Evolution of hybrid interventions for congenital heart disease.

Agrawal H, Alkashkari W, Kenny D.

Expert Rev Cardiovasc Ther. 2017 Apr;15(4):257-266. doi: 10.1080/14779072.2017.1307733. Epub 2017 Mar 23. Review.

PMID: 28293976

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  1. Minimally invasive endoscopic surgery versus catheter-based device occlusion for atrial septal defects in adults: reconsideration of the standard of care.

Schneeberger Y, Schaefer A, Conradi L, Brickwedel J, Reichenspurner H, Kozlik-Feldmann R, Detter C.

Interact Cardiovasc Thorac Surg. 2017 Apr 1;24(4):603-608. doi: 10.1093/icvts/ivw366.

PMID: 28040751

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  1. The Medtronic Micro Vascular Plug™ for Vascular Embolization in Children With Congenital Heart Diseases.

Sathanandam S, Justino H, Waller BR 3rd, Gowda ST, Radtke W, Qureshi AM.

J Interv Cardiol. 2017 Apr;30(2):177-184. doi: 10.1111/joic.12369. Epub 2017 Feb 16.

PMID: 28211168

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  1. Pulmonary artery dissection following balloon valvuloplasty in a dog with pulmonic stenosis.

Grint KA, Kellihan HB.

J Vet Cardiol. 2017 Apr;19(2):182-189. doi: 10.1016/j.jvc.2016.09.005. Epub 2016 Nov 30.

PMID: 27913078

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  1. Bronchial artery embolization for the treatment of haemoptysis in pulmonary hypertension.

Rasciti E, Sverzellati N, Silva M, Casadei A, Attinà D, Palazzini M, Galiè N, Zompatori M.

Radiol Med. 2017 Apr;122(4):257-264. doi: 10.1007/s11547-016-0714-6. Epub 2016 Dec 26.

PMID: 28025781

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  1. Three-dimensional Printed Cardiac Models: Applications in the Field of Medical Education, Cardiovascular Surgery, and Structural Heart Interventions.

Valverde I.

Rev Esp Cardiol (Engl Ed). 2017 Apr;70(4):282-291. doi: 10.1016/j.rec.2017.01.012. Epub 2017 Feb 8. English, Spanish.

PMID: 28189544

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  1. Seven Coils in 1 Heart: Therapeutic Option for Multiple VSD.

Sabiniewicz R, Weryński P.

JACC Cardiovasc Interv. 2017 Apr 24;10(8):837-838. doi: 10.1016/j.jcin.2017.02.016. Epub 2017 Mar 29. No abstract available.

PMID: 28365261

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  1. Are the AMPLATZER Duct Occluder II Additional Sizes devices dedicated only for smaller children?

Fiszer R, Chojnicki M, Szkutnik M, Haponiuk I, Chodór B, Białkowski J.

EuroIntervention. 2017 Apr 20;12(17):2100-2103. doi: 10.4244/EIJ-D-15-00238.

PMID: 27867138 Free Article

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  1. Targeted endomyocardial biopsy guided by real-time cardiovascular magnetic resonance.

Unterberg-Buchwald C, Ritter CO, Reupke V, Wilke RN, Stadelmann C, Steinmetz M, Schuster A, Hasenfuß G, Lotz J, Uecker M.

J Cardiovasc Magn Reson. 2017 Apr 19;19(1):45. doi: 10.1186/s12968-017-0357-3.

PMID: 28424090 Free PMC Article

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  1. Transcatheter closure of a residual aorto-left ventricular tunnel: report of a case with a 6-year follow-up.

Djukic M, Djordjevic SA, Dähnert I.

Cardiol Young. 2017 Apr 17:1-4. doi: 10.1017/S1047951117000701. [Epub ahead of print]

PMID: 28414001

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  1. Radiation dose reduction in pediatric great vessel stent computed tomography using iterative reconstruction: A phantom study.

den Harder AM, Suchá D, van Doormaal PJ, Budde RPJ, de Jong PA, Schilham AMR, Breur JMPJ, Leiner T.

PLoS One. 2017 Apr 14;12(4):e0175714. doi: 10.1371/journal.pone.0175714. eCollection 2017.

PMID: 28410386 Free PMC Article

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  1. Clinical Impact of Stent Implantation for Coarctation of the Aorta with Associated Hypoplasia of the Transverse Aortic Arch.

Lu WH, Fan CS, Chaturvedi R, Lee KJ, Manlhiot C, Benson L.

Pediatr Cardiol. 2017 Apr 10. doi: 10.1007/s00246-017-1611-z. [Epub ahead of print]

PMID: 28396934

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  1. Percutaneous Mechanical Circulatory Support Using Impella® Devices for Decompensated Cardiogenic Shock: A Pediatric Heart Center Experience.

Parekh D, Jeewa A, Tume SC, Dreyer WJ, Pignatelli R, Horne D, Justino H, Qureshi AM.

ASAIO J. 2017 Apr 6. doi: 10.1097/MAT.0000000000000581. [Epub ahead of print]

PMID: 28394814

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  1. Intractable Back Pain After Coil Embolization of Giant Veno-Venous Collaterals in a Patient With Fontan Circulation.

Okada S, Kamada M, Nakagawa N, Ishiguchi Y, Moritoh Y, Shohi M, Okamoto K, Hasegawa S, Ohga S.

Int Heart J. 2017 Apr 6;58(2):298-301. doi: 10.1536/ihj.16-194. Epub 2017 Mar 21.

PMID: 28320993 Free Article

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  1. Impact of imaging approach on radiation dose and associated cancer risk in children undergoing cardiac catheterization.

Hill KD, Wang C, Einstein AJ, Januzis N, Nguyen G, Li JS, Fleming GA, Yoshizumi TK.

Catheter Cardiovasc Interv. 2017 Apr;89(5):888-897. doi: 10.1002/ccd.26630. Epub 2016 Jun 17.

PMID: 27315598

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Select item 27473470

 

  1. Early Cardiac Catheterization Leads to Shortened Pediatric Extracorporeal Membrane Oxygenation Run Duration.

Burke CR, Chan T, Rubio AE, McMullan DM.

J Interv Cardiol. 2017 Apr;30(2):170-176. doi: 10.1111/joic.12368. Epub 2017 Mar 8.

PMID: 28271557

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  1. Percutaneous Coronary Intervention With Bioresorbable Scaffolds in a Young Child.

Nazif TM, Kalra S, Ali ZA, Karmpaliotis D, Turner ME, Starc TJ, Cao Y, Marboe CC, Collins MB, Leon MB, Kirtane AJ.

JAMA Cardiol. 2017 Apr 1;2(4):430-434. doi: 10.1001/jamacardio.2016.4954.

PMID: 28030655

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  1. Multicenter Off-Label Use of Nit-Occlud Coil in Retrograde Closure of Small Patent Ductus Arteriosus.

Zanjani KS, Sobhy R, El-Kaffas R, El-Sisi A.

Pediatr Cardiol. 2017 Apr;38(4):828-832. doi: 10.1007/s00246-017-1589-6. Epub 2017 Feb 21.

PMID: 28224170

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  1. Preprocedural Risk Assessment Prior to PPVI with CMR and Cardiac CT.

Malone L, Fonseca B, Fagan T, Gralla J, Wilson N, DiMaria M, Truong U, Browne LP.

Pediatr Cardiol. 2017 Apr;38(4):746-753. doi: 10.1007/s00246-017-1574-0. Epub 2017 Feb 16.

PMID: 28210769

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Pediatric Cardiology Featured Articles of March 2017

Pediatric Cardiology Featured Reviews of February-March 2017 Publications

 

Altered aortic 3D hemodynamics and geometry in pediatric Marfan syndrome patients.

van der Palen RL, Barker AJ, Bollache E, Garcia J, Rose MJ, van Ooij P, Young LT, Roest AA, Markl M, Robinson JD, Rigsby CK.

J Cardiovasc Magn Reson. 2017 Mar 17;19(1):30. doi: 10.1186/s12968-017-0345-7.

PMID: 28302143

Free PMC Article

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Take Home Points:

 

  • Marfan syndrome patients have lower mean wall shear stress and altered blood flow patterns in the proximal ascending and proximal descending aorta, both areas at risk for aortic dissection and rupture.
  • 4D flow CMR derived hemodynamic parameters may be helpful to monitor disease progression and severity in Marfan syndrome patients.

 
Voges IngaCommentary from Dr. Inga Voges (London UK) section editor of Congenital Heart Surgery Journal Watch:
 This interesting prospective study examined aortic blood flow patterns and geometry in 25 patients with Marfan syndrome (MFS) and 21 healthy controls by using cardiovascular magnetic resonance (CMR) imaging including 4D flow CMR. CMR cine imaging and contrast-enhanced magnetic resonance angiographic data were used to assess thoracic aortic diameters. After postprocessing of 4D flow datasets, blood flow patterns and regional thoracic aortic wall shear stress (in 10 aortic segments) were additionally analyzed.

The authors found abnormal flow patterns and reduced wall shear stress in the aortic root/proximal ascending aorta and the proximal descending aorta (see figure below), both areas that were dilated in MFS patients compared to the controls.  Mean wall shear stress was inversely related with aortic diameters and aortic size change in both MFS patients and controls.

The authors conclude that the hemodynamic parameters, aortic flow patterns and wall shear stress, may serve as additional valuable markers of disease severity.

Pediatric media 1

 

Rates of autism and potential risk factors in children with congenital heart defects.

Bean Jaworski JL, Flynn T, Burnham N, Chittams JL, Sammarco T, Gerdes M, Bernbaum JC, Clancy RR, Solot CB, Zackai EH, McDonald-McGinn DM, Gaynor JW.

Congenit Heart Dis. 2017 Mar 16. doi: 10.1111/chd.12461. [Epub ahead of print]

PMID:28299880

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Take Home Points:

 

  • Children with CHD are at increased risk for autism spectrum disorders compared to the national average. 1 in 30.9 (3.2%) children with serious CHD were diagnosed with autism spectrum disorder compared to the national rate of 1 in 68 (1.47%).
  • Abnormalities noted on neurodevelopmental testing at age 4 were the only independent predictor of a diagnosis of autism spectrum disorder in this sample of children with CHD.
  • These findings emphasize the importance of neurodevelopmental testing in children with CHD.

 

Abarbanell picture smallCommentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: Recent research has indicated the children with congenital heart disease (CHD) are at risk for neurodevelopmental delays and are more likely to need special education services.  This study from Philadelphia evaluated whether children with CHD are also at increased risk for autism spectrum disorders.  Children included in this study were part of a larger prospective study on the “Neurodevelopmental outcomes in children with apo-lipoprotein E polymorphisms after infant cardiac surgery.”  195 children with serious CHD requiring cardiac surgery within first 6 months of life were included in this study and researchers in particular focused on the results of neurodevelopmental testing performed at age 4 years and the social communication questionnaire (SCQ).  The SCQ is a parent reported questionnaire used as a screening tool for autistic spectrum disorders.  Researchers found that among their sample that children with CHD were more likely to either have a parent report a diagnosis of autism spectrum disorder or screen positive using the SCQ for autism spectrum disorder compared to a national rate of autism spectrum diagnoses.  1 in 30.9 (3.2%) children with CHD were reported to have autism spectrum disorder compared to the national rate of 1 in 68 (1.47%).  Univariate analysis suggested that medical factors such as delayed sternal closure, presence of a genetic abnormality and parental report of developmental problems and symptoms of ADHD were associated with increased odds of autism septum disorder.  The only independent predictor on multivariable analysis for autism spectrum disorder in this sample of children with CHD was abnormal neurodevelopmental testing with particular deficits in social skills, intellectual function and pervasive developmental problems.  These results would confer that children with CHD are at risk for neurodevelopmental delays and in particular at increased risk for autism spectrum disorder and underscores the importance of neurodevelopmental screening in children with CHD.

pediatric media 2

 

Right-Dominant Unbalanced Atrioventricular Septal Defect: Echocardiography in Surgical Decision Making.

Arunamata A, Balasubramanian S, Mainwaring R, Maeda K, Selamet Tierney ES.

J Am Soc Echocardiogr. 2017 Mar;30(3):216-226. doi: 10.1016/j.echo.2016.10.017. Epub 2016 Dec 6.

PMID: 27939051

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Select item 27909838 269.

 

Take Home Points:

 

  • In this small study (n=46) of infants with right dominant AVSD over 60% underwent single ventricle palliation.
  • Survival among infants with right dominant AVSD in this study was 76% at 7 years.
  • Single ventricle palliation may need to be considered in infants with right dominant AVSD and echocardiographic findings of an atrioventricular valve index (AVV) £7 and a right ventricle/left ventricle inflow angle of £ 114°.

 

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: It can sometimes be a surgical dilemma as to when to purse a biventricular vs a single ventricle surgical repair in the setting of unbalanced atrioventricular septal defect (AVSD).  Researchers in this study evaluated echocardiographic markers that maybe helpful in assessing surgical planning in particular whether single ventricle palliation should be pursed in patients with right-dominant AVSD.  46 patients with right dominant AVSD were included in this study.  Survival was 76% at 7 years and 61% (n=28) of patients underwent single ventricle palliation.  Of the infants who underwent single ventricle palliation they had small left ventricular dimensions and volumes as well as a decreased atrioventricular valve index (AVVI) and right ventricle(RV)/left ventricle (LV) inflow angle. A left ventricular end-diastolic volume (LVEDV) Z score £ -2.5, AVVI £ 0.7 and a RV/LV inflow angle of £ 114° was found to have a moderate association with single ventricle palliation.  See Figure 2.  Researchers validated their results on 12 additional infants with right dominant AVSD and found that a RV/LV inflow angle £114° had a sensitivity of 100% and AVVI £ 0.70 had a sensitivity of 88% for predicting single ventricle palliation.  This study would suggest that echocardiographic measurements of LVEDV, AVVI and RV/LV inflow angle could be helpful tools to assess whether single ventricle palliation vs biventricular palliation in right dominant AVSD is needed.

Pediatric media 3 Pediatric media 4

 

 

Surgical rescue after transcatheter interventional procedures in congenital heart disease patients: an existing problem.

Varrica A, Lo Rito M, Generali T, Satriano A, D’Oria V, Conforti E, Pluchinotta F, Chessa M, Butera G, Frigiola A, Carminati M, Giamberti A.

EuroIntervention. 2017 Feb 20;12(14):1724-1729. doi: 10.4244/EIJ-D-16-00031.

PMID:27773863

Free Article

Similar articles

 

Take Home Points:

 

  • Despite improvements in catheterization equipment and techniques, there is still the potential need for surgical intervention after cath-based intervention in immediate and short-term follow-up.
  • This study stresses the importance of the availability of surgical colleagues, particularly during challenging cath-based interventions, as well as long term follow-up to look for future complications.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: Varrica and colleagues present a retrospective review of their single-center experience with interventional cardiac catheterizations and assess the rate of surgical interventions following catheterization. They divided the cases into early complications – during the catheterization or prior to hospital discharge, and latecomplications – surgery after hospital discharge. Of nearly 7,000 catheterizations during the review period, 3,205 involved an intervention (Table 1). A total of 40 patients (1.2%) required surgical intervention and 55% of interventions were early.pediatric media v42

 

Atrial septal defect (ASD) closure required the highest number of surgical interventions, but was also the most commonly performed cath-based intervention. Of the early ASD surgical interventions, 79% were related to device embolization or erosion. The highest need for surgical intervention occurred in the percutaneous pulmonary valve implantation patients – early interventions were for coronary artery compression and device embolization and later interventions were for infective endocarditis involving the implanted valve. Of all late interventions, ASD device complications were the most common cause leading to 72% of surgeries.

 

Despite the limitation of being a single-center study, the large number of patients and long follow-up period provide additional insight into the risks of cath-based interventions in the current era as well as the importance of continued follow-up after interventions are performed.

Cardiac 3D Printing and its Future Directions.

Vukicevic M, Mosadegh B, Min JK, Little SH.

JACC Cardiovasc Imaging. 2017 Feb;10(2):171-184. doi: 10.1016/j.jcmg.2016.12.001. Review.

PMID: 28183437

Similar articles

 

Take Home Points:

 

  • 3-D printing is being increasingly utilized in congenital and structural cardiac interventions.
  • Methods are expanding beyond simple anatomic visualization and now include the potential to physically model and assess hemodynamics in vitro to more accurately guide appropriate interventions and development of new interventional devices.

 

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: The authors provide an overview of the available 3-D printing technologies and their different capabilities in regards to model resolution and material availability. They also outline the steps of generating a 3-D model from initial image acquisition of a 3-D volumetric dataset (typically CT or MR data), through image

pediatric media v6 1

segmentation (the process of highlighting the pertinent anatomical structures), 3-D reconstruction, generating a digital 3-D model and finally printing the physical model.

 

The multiple uses of 3-D printed models that have been developed so far are also presented. These include:

  1. Trainee and patient education, particularly of complex congenital heart defects
  2. Functional flow models which have been used to model aortic stenosis and coronary artery flow
  3. Surgical and catheterization procedural planning, including double outlet right ventricle repair, complex percutaneous pulmonary valve implantation and aortic coarctation stenting
  4. Development of new devices that are more appropriate for human anatomy rather than using animal models

 

They conclude with a discussion of currently available printing materials and their different mechanical properties followed by a review of critical developments in the literature for different aspects of creating 3-D printed models. Importantly, limitations of the currently available technology are presented, including accuracy of mechanical properties of the materials to simulate cardiac muscle and a lack of validation of model accuracy across a variety of generation methods and 3-D printing hardware.

 

Pediatric cardiology March 2017

 

  1. The United States Pediatric Cardiology 2015 Workforce Assessment: A Survey of Current Training and Employment Patterns: A Report of the American College of Cardiology, American Heart Association, American Academy of Pediatrics Section on Cardiology and Cardiac Surgery, and Society for Pediatric Cardiology Training Program Directors.

Ross RD, Srivastava S, Cabrera AG, Ruch-Ross HS, Radabaugh CL, Minich LL, Mahle WT, Brown DW.

J Am Coll Cardiol. 2017 Mar 14;69(10):1347-1352. doi: 10.1016/j.jacc.2016.09.921. Epub 2016 Nov 28. No abstract available.

PMID:

 

27908576

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  1. Clinical Progress in Cell Therapy for Single VentricleCongenital Heart Disease.

Bittle GJ, Wehman B, Karathanasis SK, Kaushal S.

Circ Res. 2017 Mar 31;120(7):1060-1062. doi: 10.1161/CIRCRESAHA.117.310702. No abstract available.

PMID:

 

28360342

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  1. Trends in National Institutes of Health-FundedCongenital Heart DiseaseResearch from 2005 to 2015.

Burns KM, Pemberton VL, Schramm CA, Pearson GD, Kaltman JR.

Pediatr Cardiol. 2017 Mar 27. doi: 10.1007/s00246-017-1605-x. [Epub ahead of print]

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28349207

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  1. A Rare Case of Cor Triatriatum Leading to Respiratory Failure and Shock in an 8-Month-Old.

Burger C.

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28350721

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Indian J Pediatr. 2017 Mar 24. doi: 10.1007/s12098-017-2333-1. [Epub ahead of print]

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J Pediatr (Rio J). 2017 Mar 20. pii: S0021-7557(17)30218-8. doi: 10.1016/j.jped.2016.11.007. [Epub ahead of print]

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28302743

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  1. Neurodevelopmental Abnormalities andCongenital Heart Disease: Insights Into Altered Brain Maturation.

Morton PD, Ishibashi N, Jonas RA.

Circ Res. 2017 Mar 17;120(6):960-977. doi: 10.1161/CIRCRESAHA.116.309048.

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28302742

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  1. Has the Congenitally MalformedHeartChanged Its Face? Journey From Understanding Morphology to Surgical Cure in Congenital Heart Disease.

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Circ Res. 2017 Mar 17;120(6):901-903. doi: 10.1161/CIRCRESAHA.116.310229. No abstract available.

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28302737

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  1. Perspective onCongenital Heart DiseaseResearch.

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28302736

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

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

Am J Perinatol. 2017 Mar 16. doi: 10.1055/s-0037-1601309. [Epub ahead of print]

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28301892

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  1. Lower Circulating Folate Induced by a Fidgetin Intronic Variant is Associated with ReducedCongenital Heart DiseaseSusceptibility.

Wang D, Wang F, Shi KH, Tao H, Li Y, Zhao R, Lu H, Duan W, Qiao B, Zhao SM, Wang H, Zhao JY.

Circulation. 2017 Mar 16. pii: CIRCULATIONAHA.116.025164. doi: 10.1161/CIRCULATIONAHA.116.025164. [Epub ahead of print]

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28302752

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Bean Jaworski JL, Flynn T, Burnham N, Chittams JL, Sammarco T, Gerdes M, Bernbaum JC, Clancy RR, Solot CB, Zackai EH, McDonald-McGinn DM, Gaynor JW.

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Heart. 2017 Mar 16. pii: heartjnl-2016-311105. doi: 10.1136/heartjnl-2016-311105. [Epub ahead of print] No abstract available.

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28302684

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Burden AF.

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28302683

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Rutz T, Meierhofer C, Naumann S, Martinoff S, Ewert P, Stern HC, Fratz S.

J Magn Reson Imaging. 2017 Mar 16. doi: 10.1002/jmri.25701. [Epub ahead of print]

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El-Alameey IR, Ahmed HH, Tawfik SM, Hassaballa F, Gawad AM, Eltahlawy E.

Open Access Maced J Med Sci. 2017 Mar 15;5(1):58-63. doi: 10.3889/oamjms.2017.026. Epub 2017 Feb 12.

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28293318

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Satou GM, Rheuban K, Alverson D, Lewin M, Mahnke C, Marcin J, Martin GR, Mazur LS, Sahn DJ, Shah S, Tuckson R, Webb CL, Sable CA; American Heart Association Congenital Cardiac Disease Committee of the Council on Cardiovascular Disease in the Young and Council on Quality Care and Outcomes Research..

Circulation. 2017 Mar 14;135(11):e648-e678. doi: 10.1161/CIR.0000000000000478. Epub 2017 Feb 13. Review. No abstract available.

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28193604

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Bavare AC, Rafie KS, Bastero PX, Hagan JL, Checchia PA.

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28296663

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Loke YH, Harahsheh AS, Krieger A, Olivieri LJ.

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Zhu PF, Zhang Y, Ban J, Li TT, Shi XM.

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  1. Maternal acceptability of pulse oximetry screening at home after home birth or very early discharge.

Narayen IC, Kaptein AA, Hogewoning JA, Blom NA, Te Pas AB.

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28359576

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Cardiovasc Pathol. 2017 Mar – Apr;27:51-53. doi: 10.1016/j.carpath.2016.12.005. Epub 2017 Jan 2.

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Farhadi R, Rafiei A, Hamdamian S, Zamani H, Yazdani J.

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Clin Genet. 2017 Mar;91(3):349-354. doi: 10.1111/cge.12835. Epub 2016 Sep 5. Review.

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  1. Ciliopathies.

Braun DA, Hildebrandt F.

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PMID:

 

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  1. Congenitaluni-leaflet mitral valve with severe stenosis: A case report with literature review.

Zhang W, Wang Y, Ma C, Zhang Z, Yang J.

Echocardiography. 2017 Mar;34(3):468-471. doi: 10.1111/echo.13473. Epub 2017 Feb 9.

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  1. Persistent left superior vena cava accompanyingcongenital heart diseasein children: Experience of a tertiary care center.

Ari ME, Doğan V, Özgür S, Ceylan Ö, Ertuğrul İ, Kayalı Ş, Yoldaş T, Örün UA, Kaya Ö, Karademir S.

Echocardiography. 2017 Mar;34(3):436-440. doi: 10.1111/echo.13447. Epub 2017 Jan 3.

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Tian T, Yang Y, Zhou L, Luo F, Li Y, Fan P, Dong X, Liu Y, Cui J, Zhou X.

Heart Lung Circ. 2017 Mar 1. pii: S1443-9506(17)30078-1. doi: 10.1016/j.hlc.2017.01.013. [Epub ahead of print]

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  1. Compound heterozygous GATA5 mutations in a girl with hydrops fetalis,congenitalheartdefects and genital anomalies.

Hempel M, Casar Tena T, Diehl T, Burczyk MS, Strom TM, Kubisch C, Philipp M, Lessel D.

Hum Genet. 2017 Mar;136(3):339-346. doi: 10.1007/s00439-017-1762-2. Epub 2017 Feb 8.

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28180938

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  1. Defining the Risk and Associated Morbidity and Mortality of Severe Respiratory Syncytial Virus Infection Among Infants withCongenital Heart Disease.

Checchia PA, Paes B, Bont L, Manzoni P, Simões EA, Fauroux B, Figueras-Aloy J, Carbonell-Estrany X.

Infect Dis Ther. 2017 Mar;6(1):37-56. doi: 10.1007/s40121-016-0142-x. Epub 2017 Jan 9. Review.

PMID:

 

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  1. Pressure-volume loop-derived cardiac indices during dobutamine stress: a step towards understanding limitations in cardiac output in children with hypoplastic leftheartsyndrome.

Wong J, Pushparajah K, de Vecchi A, Ruijsink B, Greil GF, Hussain T, Razavi R.

Int J Cardiol. 2017 Mar 1;230:439-446. doi: 10.1016/j.ijcard.2016.12.087. Epub 2016 Dec 22.

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  1. The promises and challenges of exome sequencing in familial, non-syndromiccongenital heart disease.

Blue GM, Humphreys D, Szot J, Major J, Chapman G, Bosman A, Kirk EP, Sholler GF, Harvey RP, Dunwoodie SL, Winlaw DS.

Int J Cardiol. 2017 Mar 1;230:155-163. doi: 10.1016/j.ijcard.2016.12.024. Epub 2016 Dec 13.

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  1. Normative Values of Aortic Arch Structures in Premature Infants.

Dijkema EJ, Molenschot MC, Breur JM, de Vries WB, Slieker MG.

J Am Soc Echocardiogr. 2017 Mar;30(3):227-232. doi: 10.1016/j.echo.2016.11.016. Epub 2017 Jan 27.

PMID:

 

28139441

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  1. Infundibular Pulmonic Stenosis in a Moluccan Cockatoo (Cacatua moluccensis).

Zoller G, Guzman DS, Summa N, Keller KA, Silverman SJ, Stern JA.

J Avian Med Surg. 2017 Mar;31(1):53-61. doi: 10.1647/2015-126.

PMID:

 

28358604

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  1. Is maternal smoking during pregnancy associated with an increased risk ofcongenitalheartdefects among offspring? A systematic review and meta-analysis of observational studies.

Zhang D, Cui H, Zhang L, Huang Y, Zhu J, Li X.

J Matern Fetal Neonatal Med. 2017 Mar;30(6):645-657. doi: 10.1080/14767058.2016.1183640. Epub 2016 May 16.

PMID:

 

27126055

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Select item 27072884 286.

 

  1. A novel, more efficient, staged approach for criticalcongenital heart diseasescreening.

Mouledoux J, Guerra S, Ballweg J, Li Y, Walsh W.

J Perinatol. 2017 Mar;37(3):288-290. doi: 10.1038/jp.2016.204. Epub 2016 Nov 10.

PMID:

 

27831548

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  1. Pathology and molecular mechanisms of coarctation of the aorta and its association with the ductus arteriosus.

Yokoyama U, Ichikawa Y, Minamisawa S, Ishikawa Y.

J Physiol Sci. 2017 Mar;67(2):259-270. doi: 10.1007/s12576-016-0512-x. Epub 2016 Dec 20. Review.

PMID:

 

28000176

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  1. Use of Model-based Iterative Reconstruction to Improve Detection ofCongenitalCardiovascular Anomalies in Infants Undergoing Free-breathing Computed Tomographic Angiography.

Kligerman S, Bolster F, Mitchell J, Henry T, Jeudy J, White CS.

J Thorac Imaging. 2017 Mar;32(2):127-135. doi: 10.1097/RTI.0000000000000257.

PMID:

 

28221263

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  1. Down syndrome and the complexity of genome dosage imbalance.

Antonarakis SE.

Nat Rev Genet. 2017 Mar;18(3):147-163. doi: 10.1038/nrg.2016.154. Epub 2016 Dec 28. Review.

PMID:

 

28029161

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  1. FDG-PET Identification of Infected Pulmonary Artery Conduit Following Tetralogy of Fallot (TOF) Repair.

Zhang Y, Williams H, Pucar D.

Nucl Med Mol Imaging. 2017 Mar;51(1):86-87. doi: 10.1007/s13139-016-0424-y. Epub 2016 Jun 8.

PMID:

 

28250862

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  1. Cardiorespiratory Interactions in Paediatrics: ‘It’s (almost always) the circulation stupid!’

Rigby ML, Rosenthal M.

Paediatr Respir Rev. 2017 Mar;22:60-65. doi: 10.1016/j.prrv.2016.08.003. Epub 2016 Aug 12. Review.

PMID:

 

28029591

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  1. The Impact of the Right Ventricular Outflow Tract Patch on Right Ventricular Strain in Tetralogy of Fallot: A Comparison with Valvar Pulmonary Stenosis Utilizing Cardiac Magnetic Resonance.

Anwar S, Harris MA, Whitehead KK, Keller MS, Goldmuntz E, Fogel MA, Mercer-Rosa L.

Pediatr Cardiol. 2017 Mar;38(3):617-623. doi: 10.1007/s00246-016-1558-5. Epub 2017 Jan 31.

PMID:

 

28144689

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  1. Transposition of Great Arteries with Complex Coronary Artery Variants: Time-Related Events Following Arterial Switch Operation.

Al Anani S, Fughhi I, Taqatqa A, Elzein C, Ilbawi MN, Polimenakos AC.

Pediatr Cardiol. 2017 Mar;38(3):513-524. doi: 10.1007/s00246-016-1543-z. Epub 2016 Dec 19.

PMID:

 

27995290

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  1. Tricuspid Atresia with Non-compaction: An Early Experience with Implications for Surgical Palliation.

Nguyen HH, Khan R, Silverman NH, Singh GK.

Pediatr Cardiol. 2017 Mar;38(3):495-505. doi: 10.1007/s00246-016-1541-1. Epub 2016 Dec 10.

PMID:

 

27942762

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  1. Relationship Between Habitual Exercise and Performance on Cardiopulmonary Exercise Testing Differs Between Children With Single and Biventricular Circulations.

O’Byrne ML, Desai S, Lane M, McBride M, Paridon S, Goldmuntz E.

Pediatr Cardiol. 2017 Mar;38(3):472-483. doi: 10.1007/s00246-016-1537-x. Epub 2016 Nov 23.

PMID:

 

27878634

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  1. Persistent Left Superior Vena Cava.

Nair V, Yusuf K, Yu W, AlAwad H, Paul K, Al Awad E.

Pediatr Dev Pathol. 2017 Mar-Apr;20(2):182-185. doi: 10.1177/1093526616686008. Epub 2017 Jan 25.

PMID:

 

28326958

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  1. Congenital heart diseaseat Laos Children’s Hospital: Two year experience.

Hwang IC, Sisavanh M, Billamay S, Phangmanixay S, Oudavong B, Kang J, Kwon BS, Kim GB, Bae EJ, Noh CI, Choi JY.

Pediatr Int. 2017 Mar;59(3):271-279. doi: 10.1111/ped.13156. Epub 2017 Jan 26.

PMID:

 

27589674

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Select item 28035425 320.

 

  1. Myocardial stress perfusion magnetic resonance: initial experience in a pediatric and young adult population using regadenoson.

Noel CV, Krishnamurthy R, Moffett B, Krishnamurthy R.

Pediatr Radiol. 2017 Mar;47(3):280-289. doi: 10.1007/s00247-016-3762-0. Epub 2016 Dec 29.

PMID:

 

28035425

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  1. Prevalence of non-cardiovascular findings on CT angiography in children withcongenital heart disease.

Malik A, Hellinger JC, Servaes S, Schwartz MC, Keller MS, Epelman M.

Pediatr Radiol. 2017 Mar;47(3):267-279. doi: 10.1007/s00247-016-3742-4. Epub 2016 Dec 6.

PMID:

 

27924361

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  1. Psychiatric Disorders in Adolescents With Single VentricleCongenital Heart Disease.

DeMaso DR, Calderon J, Taylor GA, Holland JE, Stopp C, White MT, Bellinger DC, Rivkin MJ, Wypij D, Newburger JW.

Pediatrics. 2017 Mar;139(3). pii: e20162241. doi: 10.1542/peds.2016-2241. Epub 2017 Feb 1.

PMID:

 

28148729

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  1. Nuclear Medicine in Pediatric Cardiology.

Milanesi O, Stellin G, Zucchetta P.

Semin Nucl Med. 2017 Mar;47(2):158-169. doi: 10.1053/j.semnuclmed.2016.10.008. Epub 2017 Jan 10. Review.

PMID:

 

28237004

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  1. Type IV Total Anomalous Pulmonary Venous Connection.

St Louis JD, Turk EM, Jacobs JP, O’Brien JE Jr.

World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):142-147. doi: 10.1177/2150135116682456.

PMID:

 

28329458

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  1. Public Views on Pulse Oximetry Screening for CriticalCongenital Heart Disease.

Kumar P, Iyengar H, Kumar P.

World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):130-134. doi: 10.1177/2150135116674468.

PMID:

 

28329450

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  1. Clinical Genetic Testing in PediatricCardiomyopathy: Is Bigger Better?

Ouellette AC, Mathew J, Manickaraj AK, Manase G, Zahavich L, Wilson J, George K, Benson L, Bowdin S, Mital S.

Clin Genet. 2017 Mar 30. doi: 10.1111/cge.13024. [Epub ahead of print]

PMID:

 

28369760

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  1. Application of the PediatricAppropriate Use Criteria for Chest Pain.

Nguyen T, Fundora MP, Welch E, Douglas PS, Eidem BW, Campbell RM, Weiner RB, Stern KW, Benavidez OJ, Lai WW, Sachdeva R, Lopez L.

J Pediatr. 2017 Mar 30. pii: S0022-3476(17)30339-6. doi: 10.1016/j.jpeds.2017.02.073. [Epub ahead of print]

PMID:

 

28366354

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  1. Effects of L-thyroxine treatment on heart functions in infants with congenital hypothyroidism.

Arslan A, Baş VN, Uytun S, Poyrazoğlu HG.

J Pediatr Endocrinol Metab. 2017 Mar 30. pii: /j/jpem.ahead-of-print/jpem-2016-0393/jpem-2016-0393.xml. doi: 10.1515/jpem-2016-0393. [Epub ahead of print]

PMID:

 

28358714

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  1. Serum Biomarkers of Myocardial Remodeling and Coronary Dysfunction in Early Stages of Hypertrophic Cardiomyopathy in the Young.

Fernlund E, Gyllenhammar T, Jablonowski R, Carlsson M, Larsson A, Ärnlöv J, Liuba P.

Pediatr Cardiol. 2017 Mar 30. doi: 10.1007/s00246-017-1593-x. [Epub ahead of print]

PMID:

 

28361263

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  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 Mar 30. doi: 10.1111/petr.12925. [Epub ahead of print]

PMID:

 

28370952

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  1. Statin-Associated Myopathy in a PediatricPreventive CardiologyPractice.

Johnson PK, Mendelson MM, Baker A, Ryan HH, Warren S, Graham D, Griggs SS, Desai NK, Yellen E, Buckley L, Zachariah JP, de Ferranti SD.

J Pediatr. 2017 Mar 29. pii: S0022-3476(17)30297-4. doi: 10.1016/j.jpeds.2017.02.047. [Epub ahead of print]

PMID:

 

28365026

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  1. Peripheral Endothelial Function After Arterial Switch Operation for D-looped Transposition of the Great Arteries.

Sun HY, Stauffer KJ, Nourse SE, Vu C, Selamet Tierney ES.

Pediatr Cardiol. 2017 Mar 27. doi: 10.1007/s00246-017-1609-6. [Epub ahead of print]

PMID:

 

28345114

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  1. Differentiation of Impaired From Preserved Hemodynamics in Patients With Fontan Circulation Using Real-time Phase-velocity Cardiovascular Magnetic Resonance.

Körperich H, Müller K, Barth P, Gieseke J, Haas N, Schulze-Neick I, Burchert W, Kececioglu D, Laser KT.

J Thorac Imaging. 2017 Mar 24. doi: 10.1097/RTI.0000000000000261. [Epub ahead of print]

PMID:

 

28346330

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  1. Adult Chest Pain in the PediatricEmergency Department: Treatment and Timeliness From Door In To Door Out.

Sacks JH, Flueckiger PB, Spandorfer PR, Mahle WT, Costello BE.

Pediatr Emerg Care. 2017 Mar 21. doi: 10.1097/PEC.0000000000001081. [Epub ahead of print]

PMID:

 

28328689

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  1. School-related adjustment in children and adolescents with CHD.

Im YM, Lee S, Yun TJ, Choi JY.

Cardiol Young. 2017 Mar 20:1-7. doi: 10.1017/S1047951117000324. [Epub ahead of print]

PMID:

 

28318457

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  1. Sets of serum exosomal microRNAs as candidate diagnostic biomarkers for Kawasaki disease.

Jia HL, Liu CW, Zhang L, Xu WJ, Gao XJ, Bai J, Xu YF, Xu MG, Zhang G.

Sci Rep. 2017 Mar 20;7:44706. doi: 10.1038/srep44706.

PMID:

 

28317854

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  1. Pharmacogenomic screening for anthracycline-induced cardiotoxicity in childhood cancer.

Aminkeng F, Ross CJ, Rassekh SR, Rieder MJ, Bhavsar AP, Sanatani S, Bernstein D, Hayden MR, Amstutz U, Carleton BC.

Br J Clin Pharmacol. 2017 Mar 19. doi: 10.1111/bcp.13218. [Epub ahead of print] No abstract available.

PMID:

 

28317142

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  1. Hyperoxia Reduces Oxygen Consumption in Children with Pulmonary Hypertension.

Guo L, Bobhate P, Kumar S, Vadlamudi K, Kaddoura T, Elgendi M, Holinski P, Coe JY, Rutledge J, Adatia I.

Pediatr Cardiol. 2017 Mar 18. doi: 10.1007/s00246-017-1602-0. [Epub ahead of print]

PMID:

 

28315943

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  1. Forgotten Right Ventricle in PediatricDilated Cardiomyopathy.

Agha HM, Ibrahim H, El Satar IA, El Rahman NA, El Aziz DA, Salah Z, El Saeidi S, Mostafa F, Attia W, El Rahman MA, El Mohsen GA.

Pediatr Cardiol. 2017 Mar 18. doi: 10.1007/s00246-017-1588-7. [Epub ahead of print]

PMID:

 

28315942

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  1. Infective endocarditis in children: an update.

Dixon G, Christov G.

Curr Opin Infect Dis. 2017 Mar 17. doi: 10.1097/QCO.0000000000000370. [Epub ahead of print]

PMID:

 

28319472

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  1. Altered aortic 3D hemodynamics and geometry in pediatricMarfan syndrome patients.

van der Palen RL, Barker AJ, Bollache E, Garcia J, Rose MJ, van Ooij P, Young LT, Roest AA, Markl M, Robinson JD, Rigsby CK.

J Cardiovasc Magn Reson. 2017 Mar 17;19(1):30. doi: 10.1186/s12968-017-0345-7.

PMID:

 

28302143

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  1. A Pilot Study: Cardiac Parameters in Children Receiving New-Generation Antidepressants.

Uchida M, Spencer AE, Kenworthy T, Chan J, Fitzgerald M, Rosales AM, Kagan E, Saunders A, Biederman J.

J Clin Psychopharmacol. 2017 Mar 15. doi: 10.1097/JCP.0000000000000683. [Epub ahead of print]

PMID:

 

28301398

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  1. Parent reports of health-related quality of life and heart failure severity score independently predict outcome in children with dilated cardiomyopathy.

den Boer SL, Baart SJ, van der Meulen MH, van Iperen GG, Backx AP, Ten Harkel AD, Rammeloo LA, du Marchie Sarvaas GJ, Tanke RB, Helbing WA, Utens EM, Dalinghaus M.

Cardiol Young. 2017 Mar 14:1-9. doi: 10.1017/S1047951116002833. [Epub ahead of print]

PMID:

 

28290258

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  1. Mutations in TMEM260 Cause a PediatricNeurodevelopmental, Cardiac, and Renal Syndrome.

Ta-Shma A, Khan TN, Vivante A, Willer JR, Matak P, Jalas C, Pode-Shakked B, Salem Y, Anikster Y, Hildebrandt F, Katsanis N, Elpeleg O, Davis EE.

Am J Hum Genet. 2017 Mar 11. pii: S0002-9297(17)30074-5. doi: 10.1016/j.ajhg.2017.02.007. [Epub ahead of print]

PMID:

 

28318500

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  1. Elucidation of MRAS-mediated Noonan syndrome with cardiac hypertrophy.

Higgins EM, Bos JM, Mason-Suares H, Tester DJ, Ackerman JP, MacRae CA, Sol-Church K, Gripp KW, Urrutia R, Ackerman MJ.

JCI Insight. 2017 Mar 9;2(5):e91225. doi: 10.1172/jci.insight.91225.

PMID:

 

28289718

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  1. Low Iron Stores in Otherwise Healthy Children Affect Electrocardiographic Markers of Important Cardiac Events.

Karadeniz C, Özdemir R, Demirol M, Katipoğlu N, Yozgat Y, Meşe T, Ünal N.

Pediatr Cardiol. 2017 Mar 7. doi: 10.1007/s00246-017-1596-7. [Epub ahead of print]

PMID:

 

28271153

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  1. Texas Pulse Oximetry Project: A Multicenter Educational and Quality Improvement Project for Implementation of Critical Congenital Heart Disease Screening Using Pulse Oximetry.

Guillory C, Gong A, Livingston J, Creel L, Ocampo E, McKee-Garrett T.

Am J Perinatol. 2017 Mar 6. doi: 10.1055/s-0037-1599214. [Epub ahead of print]

PMID:

 

28264208

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  1. Health-related quality of life in children and adolescents living in the north-east of China before and after cardiac catheter interventional treatment.

Li L, Lin P, Gao X.

Cardiol Young. 2017 Mar 6:1-5. doi: 10.1017/S104795111600247X. [Epub ahead of print]

PMID:

 

28260544

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  1. Impaired Single Right Ventricular Function Compared to Single Left Ventricles during the Early Stages of Palliation: A Longitudinal Study.

Suntratonpipat S, Khoo NS, Colen T, Alhabdan M, Troung D, Zahari N, Kutty S, Smallhorn JF, Tham EB.

J Am Soc Echocardiogr. 2017 Mar 3. pii: S0894-7317(17)30001-9. doi: 10.1016/j.echo.2017.01.001. [Epub ahead of print]

PMID:

 

28268110

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  1. Estimating Effective Dose of Radiation From PediatricCardiac CT Angiography Using a 64-MDCT Scanner: New Conversion Factors Relating Dose-Length Product to Effective Dose.

Trattner S, Chelliah A, Prinsen P, Ruzal-Shapiro CB, Xu Y, Jambawalikar S, Amurao M, Einstein AJ.

AJR Am J Roentgenol. 2017 Mar;208(3):585-594. doi: 10.2214/AJR.15.15908. Epub 2017 Jan 17.

PMID:

 

28095022

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  1. Translating Research into Improved Patient Care in Pulmonary Arterial Hypertension.

Bonnet S, Provencher S, Guignabert C, Perros F, Boucherat O, Schermuly RT, Hassoun PM, Rabinovitch M, Nicolls MR, Humbert M.

Am J Respir Crit Care Med. 2017 Mar 1;195(5):583-595. doi: 10.1164/rccm.201607-1515PP. No abstract available.

PMID:

 

27649290

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  1. Comparison of echocardiography with tissue Doppler imaging and magnetic resonance imaging with delayed enhancement in the assessment of children with hypertrophic cardiomyopathy.

Ziółkowska L, Petryka J, Boruc A, Kawalec W.

Arch Med Sci. 2017 Mar 1;13(2):328-336. doi: 10.5114/aoms.2016.60404. Epub 2016 Jun 7.

PMID:

 

28261285

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  1. The role of inflammatory biomarkers in CHD-associated pulmonary hypertension in children.

Oz-Tuncer G, Olgunturk R, Pektas A, Cilsal E, Kula S, Oguz DA, Tunaoglu SF, Pasaoglu OT, Pasaoglu H.

Cardiol Young. 2017 Mar;27(2):255-260. doi: 10.1017/S1047951116000408.

PMID:

 

28366184

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  1. Altering management decisions with gained anatomical insight from a 3D printed model of a complex ventricular septal defect.

Bhatla P, Mosca RS, Tretter JT.

Cardiol Young. 2017 Mar;27(2):377-380. doi: 10.1017/S104795111600202X. Epub 2016 Nov 21.

PMID:

 

27869052

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Select item 27751200 223.

 

  1. Do toxic metals and trace elements have a role in the pathogenesis of conotruncal heart malformations?

Kundak AA, Pektas A, Zenciroglu A, Ozdemir S, Barutcu UB, Orun UA, Okumus N.

Cardiol Young. 2017 Mar;27(2):312-317. doi: 10.1017/S1047951116000536. Epub 2016 Oct 18.

PMID:

 

27751200

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Select item 27573031 224.

 

  1. Pulmonary haemodynamics in Fontan physiology after lobectomy in a patient with a single ventricle associated with pulmonary sequestration.

Mii S, Yasuda K, Murayama H.

Cardiol Young. 2017 Mar;27(2):381-384. doi: 10.1017/S1047951116001153. Epub 2016 Aug 30.

PMID:

 

27572898

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Select item 27225489 226.

 

  1. The comparative relationships between locations of the papillary muscles and electrophysiologic QRS axis in patients with atrioventricular septal defect and common as opposed to separate orifices in the valve guarding the common atrioventricular junction.

Low L, Idriss SF, Anderson RH, Maynard C, Wagner G, Hakacova N.

Cardiol Young. 2017 Mar;27(2):261-266. doi: 10.1017/S104795111600041X. Epub 2016 Apr 18.

PMID:

 

27087659

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Select item 27064196 230.

 

  1. Prevalence of rheumatic valvular heart disease in Rwandan school children: echocardiographic evaluation using the World Heart Federation criteria.

Mucumbitsi J, Bulwer B, Mutesa L, Ndahindwa V, Semakula M, Rusingiza E, Arya P, Breakey S, Patton-Bolman C, Kaplan EL.

Cardiovasc J Afr. 2017 Mar 1;28:1-8. doi: 10.5830/CVJA-2017-007. [Epub ahead of print]

PMID:

 

28252675

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  1. Assessment of Diffuse Ventricular Myocardial Fibrosis Using Native T1 in Children With Repaired Tetralogy of Fallot.

Yim D, Riesenkampff E, Caro-Dominguez P, Yoo SJ, Seed M, Grosse-Wortmann L.

Circ Cardiovasc Imaging. 2017 Mar;10(3). pii: e005695. doi: 10.1161/CIRCIMAGING.116.005695.

PMID:

 

28292861

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Select item 28273405 238.

 

  1. Appropriate Use of Genetic Testing in Congenital Heart Disease Patients.

Ito S, Chapman KA, Kisling M, John AS.

Curr Cardiol Rep. 2017 Mar;19(3):24. doi: 10.1007/s11886-017-0834-1. Review.

PMID:

 

28224467

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  1. Application of pediatricappropriate use criteria for initial outpatient evaluation of syncope.

Phelps HM, Kelleman MS, McCracken CE, Benavidez OJ, Campbell RM, Douglas PS, Eidem BW, Lai WW, Lopez L, Stern KW, Welch E, Sachdeva R.

Echocardiography. 2017 Mar;34(3):441-445. doi: 10.1111/echo.13475. Epub 2017 Feb 8.

PMID:

 

28177138

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Select item 28168740 244.

 

  1. Longitudinal changes in adolescents with TOF: implications for care.

Bhat M, Mercer-Rosa L, Fogel MA, Harris MA, Paridon SM, McBride MG, Shults J, Zhang X, Goldmuntz E.

Eur Heart J Cardiovasc Imaging. 2017 Mar 1;18(3):356-363. doi: 10.1093/ehjci/jew272.

PMID:

 

28363199

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  1. Temporal Trends in Utilization of Transthoracic Echocardiography for Common Outpatient Pediatric CardiologyDiagnoses over the Past 15 Years.

Sachdeva R, Travers CD, McCracken CE, Samai C, Campbell RM, Slesnick TC, Border WL.

J Am Soc Echocardiogr. 2017 Mar;30(3):201-208. doi: 10.1016/j.echo.2016.12.008.

PMID:

 

28259302

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  1. Right-Dominant Unbalanced Atrioventricular Septal Defect: Echocardiography in Surgical Decision Making.

Arunamata A, Balasubramanian S, Mainwaring R, Maeda K, Selamet Tierney ES.

J Am Soc Echocardiogr. 2017 Mar;30(3):216-226. doi: 10.1016/j.echo.2016.10.017. Epub 2016 Dec 6.

PMID:

 

27939051

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  1. ECG-derived spatial QRS-T angle is strongly associated with hypertrophic cardiomyopathy.

Cortez D, Schlegel TT, Ackerman MJ, Bos JM.

J Electrocardiol. 2017 Mar – Apr;50(2):195-202. doi: 10.1016/j.jelectrocard.2016.10.001. Epub 2016 Oct 14.

PMID:

 

27839835

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  1. Mortality after hematopoietic stem cell transplantation for severe mucopolysaccharidosis type I: the 30-year University of Minnesota experience.

Rodgers NJ, Kaizer AM, Miller WP, Rudser KD, Orchard PJ, Braunlin EA.

J Inherit Metab Dis. 2017 Mar;40(2):271-280. doi: 10.1007/s10545-016-0006-2. Epub 2017 Jan 4.

PMID:

 

28054207

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  1. Advances in Pediatric CardiologyBoot Camp: Boot Camp Training Promotes Fellowship Readiness and Enables Retention of Knowledge.

Ceresnak SR, Axelrod DM, Sacks LD, Motonaga KS, Johnson ER, Krawczeski CD.

Pediatr Cardiol. 2017 Mar;38(3):631-640. doi: 10.1007/s00246-016-1560-y. Epub 2017 Feb 4.

PMID:

 

28161811

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Select item 28161810 300.

 

  1. PocketECG: A New Noninvasive Method for Continuous and Real-Time ECG Monitoring-Initial Results in Children and Adolescents.

Bieganowska K, Kaszuba A, Bieganowski M, Kaczmarek K.

Pediatr Cardiol. 2017 Mar;38(3):448-455. doi: 10.1007/s00246-016-1534-0. Epub 2017 Jan 18.

PMID:

 

28101660

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Select item 28078382 304.

 

  1. Assessing Myocardial Function in Infants with Pulmonary Hypertension: The Role of Tissue Doppler Imaging and Tricuspid Annular Plane Systolic Excursion.

Richardson C, Amirtharaj C, Gruber D, Hayes DA.

Pediatr Cardiol. 2017 Mar;38(3):558-565. doi: 10.1007/s00246-016-1548-7. Epub 2016 Dec 19.

PMID:

 

27995291

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Select item 27995290 310.

 

  1. HAND1 Loss-of-Function Mutation Causes Tetralogy of Fallot.

Wang J, Hu XQ, Guo YH, Gu JY, Xu JH, Li YJ, Li N, Yang XX, Yang YQ.

Pediatr Cardiol. 2017 Mar;38(3):547-557. doi: 10.1007/s00246-016-1547-8. Epub 2016 Dec 10.

PMID:

 

27942761

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Select item 27909753 315.

 

  1. Six-Minute Walk Test as a Predictor for Outcome in Children with Dilated Cardiomyopathy and Chronic Stable Heart Failure.

den Boer SL, Flipse DH, van der Meulen MH, Backx AP, du Marchie Sarvaas GJ, Ten Harkel AD, van Iperen GG, Rammeloo LA, Tanke RB, Helbing WA, Takken T, Dalinghaus M.

Pediatr Cardiol. 2017 Mar;38(3):465-471. doi: 10.1007/s00246-016-1536-y. Epub 2016 Dec 1.

PMID:

 

27909753

Free PMC Article

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Select item 27900408 316.

 

  1. N-terminal pro-B-type natriuretic peptide diagnostic algorithm versus American Heart Association algorithm for Kawasaki disease.

Dionne A, Meloche-Dumas L, Desjardins L, Turgeon J, Saint-Cyr C, Autmizguine J, Spigelblatt L, Fournier A, Dahdah N.

Pediatr Int. 2017 Mar;59(3):265-270. doi: 10.1111/ped.13154. Epub 2016 Dec 2.

PMID:

 

27589358

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Select item 27740725 326.

 

  1. Neonatal dilated cardiomyopathy.

Soares P, Rocha G, Pissarra S, Soares H, Flôr-de-Lima F, Costa S, Moura C, Dória S, Guimarães H.

Rev Port Cardiol. 2017 Mar;36(3):201-214. doi: 10.1016/j.repc.2016.10.007. Epub 2017 Feb 28. English, Portuguese.

PMID:

 

28256370

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Select item 28214152 338.

 

  1. Clinical and genetic diagnosis of familial hypertrophic cardiomyopathy: Results in pediatric cardiology.

Cardoso B, Gomes I, Loureiro P, Trigo C, Ferreira Pinto F.

Rev Port Cardiol. 2017 Mar;36(3):155-165. doi: 10.1016/j.repc.2016.09.009. Epub 2017 Feb 15. English, Portuguese.

PMID:

 

28214152

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Select item 28237004 339.

 

  1. Cardiac Function After Tetralogy of Fallot/Complete Atrioventricular Canal Repair.

Stephens EH, Tingo J, Najjar M, Yilmaz B, Levasseur S, Dayton JD, Mosca RS, Chai P, Quaegebeur JM, Bacha EA.

World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):189-195. doi: 10.1177/2150135116682719.

PMID:

 

28329461

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Select item 28329452 346

 

  1. Passive range of motion exercise to enhance growth in infants following the Norwood procedure: a safety and feasibility trial.

Lambert LM, Trachtenberg FL, Pemberton VL, Wood J, Andreas S, Schlosser R, Barnard T, Daniels K, Harrington AT, Dagincourt N, Miller TA; Pediatric Heart Network Investigators..

Cardiol Young. 2017 Mar 23:1-8. doi: 10.1017/S1047951117000427. [Epub ahead of print]

PMID:

 

28330522

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Select item 28330516 4.

 

  1. Response to Commentary: “How badly do we need instruction in paediatric cardiac anatomy?”

Rogers LS, Klein M, James J, FitzGerald M.

Cardiol Young. 2017 Mar 22:1-2. doi: 10.1017/S1047951117000336. [Epub ahead of print] No abstract available.

PMID:

 

28327207

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Select item 28322179 6.

 

  1. How badly do we need instruction in paediatric cardiac anatomy?

Anderson RH.

Cardiol Young. 2017 Mar 21:1-4. doi: 10.1017/S1047951117000117. [Epub ahead of print] No abstract available.

PMID:

 

28322175

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Select item 28318481 8.

 

  1. Bilateral coronary artery to left ventricular fistula: CT demonstration of drainage via a single, common channel.

Lee HN, Kim JI, Cho JM.

Cardiol Young. 2017 Mar 10:1-2. doi: 10.1017/S1047951117000294. [Epub ahead of print]

PMID:

 

28279232

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Select item 28270260 18.

 

  1. What is the diagnostic value of the paediatric exercise tolerance test? Results from a UK centre.

Sajnach-Menke MA, Walpole SC.

Cardiol Young. 2017 Mar 8:1-5. doi: 10.1017/S1047951117000233. [Epub ahead of print]

PMID:

 

28270260

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Select item 28270245 19.

 

  1. Extremely refractory Kawasaki disease with disseminated intravascular coagulation.

Koh YK, Lee JH, Park YB.

Cardiol Young. 2017 Mar 7:1-4. doi: 10.1017/S1047951116002742. [Epub ahead of print]

PMID:

 

28264746

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Select item 28260555 21.

 

  1. Cor triatriatum sinister with an intact interatrial septum and a decompressing vein in a toddler.

Binsalamah ZM, De León LE, Heinle JS.

Cardiol Young. 2017 Mar 6:1-4. doi: 10.1017/S1047951117000130. [Epub ahead of print]

PMID:

 

28260553

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Select item 28260552 23.

 

  1. Indomethacin induced and prostaglandin relieved coarctation of the aorta in right aortic arch with left arterial duct: a case report.

Golden-Plotnik S, Rosenberg HC, Altamirano-Diaz LA.

Cardiol Young. 2017 Mar 6:1-4. doi: 10.1017/S1047951117000075. [Epub ahead of print]

PMID:

 

28260552

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Select item 28260551 24.

 

  1. Quality of life of Malaysian children with CHD.

Ong LC, Teh CS, Darshinee J, Omar A, Ang HL.

Cardiol Young. 2017 Mar 6:1-8. doi: 10.1017/S1047951117000166. [Epub ahead of print]

PMID:

 

28260550

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Select item 28260549 26.

 

  1. Reply on “Constriction of the ductus arteriosus, severe right ventricular hypertension, and a right ventricular aneurysm in a fetus after maternal use of a topical treatment for striae gravidarum”.

Rawlings AV, Dweck A, Dugmore P.

Cardiol Young. 2017 Mar 6:1-2. doi: 10.1017/S1047951117000129. [Epub ahead of print] No abstract available.

PMID:

 

28260549

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Select item 28260546 27.

 

  1. Clinical guidelines for the management of patients with transposition of the great arteries with intact ventricular septum.
[No authors listed]

Cardiol Young. 2017 Mar 2:1-40. doi: 10.1017/S1047951117000014. [Epub ahead of print] No abstract available.

PMID:

 

28249633

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Select item 28366186 33.

 

  1. MRI evaluation of mitral-aortic intervalvular fibrosa aneurysm in a boy.

Caro-Dominguez P, Gill N, Yoo SJ.

Cardiol Young. 2017 Mar;27(2):402-403. doi: 10.1017/S1047951116001323.

PMID:

 

28366186

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Select item 28366185 34.

 

  1. Functional near-tricuspid atresia in a patient with absent pulmonary valve and an intact ventricular septum.

Miki Y, Tanaka T, Oshima Y.

Cardiol Young. 2017 Mar;27(2):391-393. doi: 10.1017/S104795111600127X. Epub 2016 Aug 30.

PMID:

 

28300528

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Select item 28181900 37.

 

  1. Extensive coronary and systemic arterial aneurysm development in severe refractory Kawasaki disease.

Johnston N, Coleman D, McMahon CJ.

Cardiol Young. 2017 Mar;27(2):375-376. doi: 10.1017/S1047951116001967. Epub 2016 Nov 10.

PMID:

 

27829480

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Select item 27821206 42.

 

  1. Advances in molecular genetics for pulmonary atresia.

Gao M, He X, Zheng J.

Cardiol Young. 2017 Mar;27(2):207-216. doi: 10.1017/S1047951116001487. Epub 2016 Sep 22.

PMID:

 

27655138

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Select item 27646301 45.

 

  1. Understanding the morphogenesis of the left-sided arterial duct in the setting of a right-sided aortic arch.

Bamforth SD, Anderson RH.

Cardiol Young. 2017 Mar;27(2):369-372. doi: 10.1017/S1047951116001463. Epub 2016 Sep 20. No abstract available.

PMID:

 

27646301

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Select item 27641901 46.

 

  1. Neonatal management and outcomes of prenatally diagnosed CHDs.

Bensemlali M, Bajolle F, Laux D, Parisot P, Ladouceur M, Fermont L, Lévy M, Le Bidois J, Raimondi F, Ville Y, Salomon LJ, Boudjemline Y, Bonnet D.

Cardiol Young. 2017 Mar;27(2):344-353. doi: 10.1017/S1047951116000639. Epub 2016 May 26.

PMID:

 

27225605

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Select item 27225489 52.

 

  1. Current attitudes and clinical practice towards the care of pregnant women with underlying CHD: a paediatric cardiology perspective.

Cribbs MG, Briston DA, Zaidi AN.

Cardiol Young. 2017 Mar;27(2):236-242. doi: 10.1017/S104795111600038X. Epub 2016 Apr 11.

PMID:

 

27064196

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Select item 27056663 63.

 

  1. Pathogenesis of solitary right aortic arch: a mass effect hypothesis based on observations of serial human embryonic sections.

Jin ZW, Yamada T, Kim JH, Rodríguez-Vázquez JF, Murakami G, Arakawa K.

Cardiol Young. 2017 Mar;27(2):359-368. doi: 10.1017/S1047951115002152. Epub 2015 Oct 5.

PMID:

 

26435328

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  1. Type IV Total Anomalous Pulmonary Venous Connection.

St Louis JD, Turk EM, Jacobs JP, O’Brien JE Jr.

World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):142-147. doi: 10.1177/2150135116682456.

PMID:

 

28329458

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ACHD Featured Articles of March 2017

ACHD Featured Reviews of March 2017 Publications

 

Contraceptive Practices of Women with Complex Congenital Heart Disease.

Miner PD, Canobbio MM, Pearson DD, Schlater M, Balon Y, Junge KJ, Bhatt A, Barber D, Nickolaus MJ, Kovacs AH, Moons P, Shaw K, Fernandes SM.

Am J Cardiol. 2017 Mar 15;119(6):911-915. doi: 10.1016/j.amjcard.2016.11.047. Epub 2016 Dec 18.

PMID:28087052

Similar articles

Select item 28318514 142.

Take Home Points:

 

  • A quarter of patients with ACHD had unplanned pregnancies.
  • Contraception counseling provided by the ACHD service only occurred in 43%.
  • Many patients with complex CHD who are at high risk for thromboembolic events are still using estrogen-based oral contraception with a documented risk of events of 4% in the total cohort (28% in the Fontan cohort).


Blanche CupidoCommentary from Dr Blanche Cupido (Cape Town, South Africa), section editor of ACHD Journal Watch:
Women with complex congenital heart disease (CHD) have a greater risk of adverse complications during pregnancy. Contraception and pre-conception counseling plays an important role in trying to reduce the risk of unplanned pregnancies. This study aimed to describe contraceptive practices of women with CHD (aged 18 years or older). It was a cross-sectional survey conducted from 2011-2014 in 9 ACHD centers across North America. The median age of the 505 women who participated was 33 years.  80% of patients were still menstruating.

achd media 1

 

Most patients used barrier methods (87%), with oral contraception (OC) being the next most utilized form of contraception (84%). The use of emergency contraception was 19% and 16% had been sterilized. More patients in the complex CHD group had sterilization (22% vs 12%, p=0.047).

 

The rates of progesterone-only OC use was greater in those patients with more complex CHD (19%, 27% and 39% respectively in those with simple, moderate and complex CHD, p=0.034). Despite current guideline recommendations for the use of progesterone only-based contraception in those patients at high risk of thromboembolic events (mechanical prosthetic valves, cyanotic patients, Fontan circulation patients), many patients with complex CHD (>60%) are receiving an estrogen containing contraception pill. Four percent of the total cohort on OC experienced a thrombotic event. In a high-risk subset of Fontan patients (n=31), 28% had an event.  The prevalence of thrombotic events in those patients with previous OC use was 39% compared to 17% in those with no previous OC use.

 

Although 84% of the total cohort had received some sort of contraception counseling, only 43% received it from their ACHD provider. Patients with greater complexity CHD were more likely to receive counseling (56 vs 45%, p=0.036). Regardless of the complexity of their underlying disease, 25% of patients had an unplanned pregnancy.

 

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

Similar articles

Take Home Point: 

  • The authors propose a risk score to help predict mortality in Fontan patients undergoing cardiac transplantation. This score includes: Age < 18 at time of transplant, Fontan-transplantation interval < 10 years, MELD XI score ≥ 19, systemic ventricle ejection fraction < 20%, moderate to severe atrioventricular valve regurgitation, and pre-transplant advanced mechanical support.

 

jokhadar-maanCommentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch:   The Fontan procedure and its various modifications have improved the survival of single ventricle patients.  However, Fontan circulation failure and decompensation is a common consequence and referral for cardiac transplantation is increasing in frequency though post-transplant outcomes appear to be worse in this population.  Predicting outcomes in Fontan patients referred for cardiac transplantation would be helpful in candidate selection and potentially in organ allocation.

 

Dr. Berg and colleagues from University of California in Los Angeles conducted a single center, retrospective cohort analysis of all single ventricle patients who underwent cardiac transplantation for failing Fontan between 1991 and 2014.  There was a total of 36 patients with a mean age of 21 years (7 to 48) and a mean follow-up of about 3.5 years.  Two thirds had systemic left ventricle and 2 patients had concurrent heart and liver transplantation. There were a total of 17 deaths during the follow-up that included 8 who died before hospital discharge from heart transplant, 2 with allograft failure, 2 with pulmonary hemorrhage, 2 with septic shock, 1 with superior vena cava syndrome, and 1 with coagulopathy/hemorrhage.  Late causes of death included 3 patients with cardiac allograft vasculopathy, 2 with rejection, and 1 with post-transplant lymphoproliferative disease.  There were 3 patients with unknown cause of death.

 

The patients with the highest risk of death or those under the age of 18 (HR 3.88) with less than a 10-year interval between the Fontan operation and heart transplant (HR 4.38).  Also, transplantation prior to 2004 was associated with increased mortality (HR 4.18).  Interestingly, the number of prior heart surgeries and systemic left ventricle was not associated with increased mortality.  Univariate analysis between preoperative factors and postoperative mortality showed a significant association between severely reduced systemic ventricle ejection fraction < 20% (HR 3.40), moderate to severe atrioventricular valve regurgitation (HR 8.12), advanced mechanical support (renal replacement therapy (HR 72.29) and extracorporeal membrane oxygenation (HR 6.25)), as well as MELD XI score ≥ 19 (HR 4.91).  Fontan pressure > 16.5 mmHg was actually associated with reduced perioperative mortality.  Also of note, elevated trans-pulmonary gradient, chronic renal insufficiency, cirrhosis, and ascites, were not associated with increased postoperative mortality.

 

Though protein-losing enteropathy is generally associated with increased morbidity and mortality, this was not a predictor of post-transplant mortality in this study, which is consistent with prior reports.  It is interesting to note that protein-losing enteropathy is associated with lower bilirubin due to gastrointestinal loss of proteins that include albumin-bound bilirubin.  Thus, lower bilirubin levels result and a lower MELD XI score.

 

Based on these results, the investigators created a scoring system to help predict post-transplant mortality:

 

Age < 18 at time of transplant (1 point)
Fontan-transplantation interval < 10 years (2 points)
MELD XI score ≥ 19 (2 points)
Systemic ventricle ejection fraction < 20% (1 point)
Moderate to severe atrioventricular valve regurgitation (3 points)
Pre-transplant advanced mechanical support (3 points)

Maximum total score 12 points

 

In this study, the scoring system performance was as follows:

 

Score 0-1: No mortality during follow-up period.
Scores of 2-5 points: 50% mortality during follow-up period.
Scores greater than 5: 88% mortality during follow-up period.

 

This scoring system needs prospective validation before it is adopted as part of the evaluation of Fontan patients being considered for transplantation. This study could help answer the question of which Fontan patients are too sick for transplant. However, it should be noted that perioperative and postoperative mortality differs widely from one center to another (4% to 35% with an average of about 22%). The perioperative mortality after heart transplant in this study was about 22%. Transplanted organs are a scarce resource and patients who are listed for transplantation should have a realistic chance of benefit, while reducing morbidity and mortality. Clearly, sicker patients have worse outcomes and are less likely to benefit from cardiac transplantation.

 

Though this study may help answer the question of which Fontan patients may be too sick for transplant, this study does not answer the question of which Fontan patients should be listed and when. There is a fine line between too well for transplant and too sick for transplant, which appears to be more elusive in Fontan patients. Fontan failure patients can remain stable with medical therapy for years without transplant while some have precipitous decline that is often difficult to predict.  Further study is needed to help identify the Fontan patients who are at highest risk of 12 to 24-month mortality and identifying these patients may help refine specific and appropriate transplant criteria, listing status, and organ allocation for this precious gift of life.

 

Adult Congenital Cardiac Care.

Kogon BE, Miller K, Miller P, Alsoufi B, Rosenblum JM.

World J Pediatr Congenit Heart Surg. 2017 Mar;8(2):242-247. doi: 10.1177/2150135117690126.

PMID: 28329454

Similar articles

Take Home Points:

 The Adult Congenital Heart Association has created an online clinic directory of ACHD centers.

  • From 2006 to 2015, there has been an increase in programme access and availability over time in the USA, with more programmes in more states.
  • Access to diagnostic and therapeutic services, as well as ancillary services have expanded in these ACHD programmes.

 

 

Commentary from Dr Leong Ming Chern (Kuala Lumpur, Malaysia), Section editor of ACHD Journal Watch:  The Adult Congenital Heart Association (ACHA) has created an online clinic directory of ACHD centers. This data is self-reported and it incorporates all self-identified ACHD programmes in and out of USA. The authors analyzed the directory in 2006 and 2015, evaluating the growth of the directory in the USA as a whole and the growth of these programme individually.

achd media 2

By the end of 2015, compared to 2006, there were more ACHD programmes reporting to the directory in more states (107 programmes across 42 states vs 57 programmes across 33 states), with higher overall clinical volume (591 vs 164 half-day clinics per week, 96611 vs 34446 patient visits). On average, each programme was busier (5 vs 2 half-day clinics per week per programme). Over the time, the number of reported annual operations performed nearly doubled (4346 operations by 210 surgeons vs 2461 operations by 125 surgeons). There was an increase in per-programme volume for ACHD specialized clinics.

achd media 3

 

Access to diagnostic and therapeutic services such as echocardiography, diagnostic and interventional electrophysiology, cardiac computed tomography and cardiac magnetic resonance were reportedly available in 96%-100% of programmes. Other ancillary services such as high-risk obstetrics, pulmonary hypertension, genetic counseling etc., were reported available in 77%-96% of programmes.

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In view of the increased demand and workload in these programmes, efforts have been made to verify the information reported in the clinic directory, streamlining the training of cardiologists and surgeons in the care of patients with ACHD and systemic data collection and quality assessment of certain diagnostic and therapeutic services.

In conclusion, between 2006 and 2015, the clinical directory and the individual programmes have grown in the USA.

 

Am J Cardiol. 2017 Mar 1;119(5):785-789. doi: 10.1016/j.amjcard.2016.11.021. Epub 2016 Dec 2.

Chronobiology of Acute Aortic Dissection in the Marfan Syndrome (from the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions and the International Registry of Acute Aortic Dissection).

Siddiqi HK1Luminais SN2Montgomery D3Bossone E4Dietz H5Evangelista A6Isselbacher E7LeMaire S8Manfredini R9Milewicz D10Nienaber CA11Roman M12Sechtem U13Silberbach M14Eagle KA3Pyeritz RE2GenTAC and IRAD investigators.

Take Home Points:

 Marfan Syndrome is associated with acute aortic dissection (AAD).

  • AAD is more likely in winter/spring season (November to April) compared to other half of the year.
  • It is more likely to occur during daytime hours (6am to 6pm) with men more likely to follow such temporal variation than women.

 

Commentary from Dr Leong Ming Chern (Kuala Lumpur, Malaysia), Section editor of ACHD Journal Watch:   Marfan syndrome is associated with acute aortic dissection (AAD). The authors analyzed 2 large registries i.e. the International Registry of Acute Aortic Dissection (IRAD) and the Genetically Trigger Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) registries, to extract data on 257 patients with Marfan Syndrome who had suffered from an acute aortic dissection from 1980 to 2012, with the aim to study the possible trends in the chronobiology of AAD in this group of patients.

 

Mean age at time of AAD was 38 years and 156 (61%) of subjects were men. 167 (65%) of patients suffered a type A dissection. AAD was more likely in winter/spring season (November to April) than other half of the year (57% vs 43%, p=0.05). No effect of gender, type of dissection or hypertension on the seasonal distribution of AAD.

 

Dissections were significantly more likely to occur during daytime hours, with 66% of dissection occurring from 6am to 6pm (p=0.001). Men were more likely to dissect during the daytime hours than women (74%vs51%, p=0.01).

achd media 6 achd media 7 achd media 8

 

Various studies have described higher incidence of acute coronary events in the mornings and its seasonal pattern, although the exact pathophysiology is not clearly understood. Findings in this study supports the association between AAD in Marfan Syndrome and its temporal and seasonal variation, which can be used to aid treatment and resource planning as well as risk factor modification.

 

 

Outcome of adults with Eisenmenger syndrome treated with drugs specific to pulmonary arterial hypertension: A French multicentre study.

Hascoet S, Fournier E, Jaïs X, Le Gloan L, Dauphin C, Houeijeh A, Godart F, Iriart X, Richard A, Radojevic J, Amedro P, Bosser G, Souletie N, Bernard Y, Moceri P, Bouvaist H, Mauran P, Barre E, Basquin A, Karsenty C, Bonnet D, Iserin L, Sitbon O, Petit J, Fadel E, Humbert M, Ladouceur M.

Arch Cardiovasc Dis. 2017 Mar 9. pii: S1875-2136(17)30045-1. doi: 10.1016/j.acvd.2017.01.006. [Epub ahead of print]

PMID: 28286190

Similar articles

Select item 28281093 174.

 Take Home Points:

 In tertiary care centers in France, over 80% of patients with Eisenmenger syndrome (ES) are on at least one PAH-specific drug, ranging between 41.7% to 100% at various centers

  • ES Patients who take either 1 or 2 PAH-specific drugs appear to have better clinical outcomes than patients on no PAH-specific drugs or who are on triple therapy
  • Although the onset of PAH in pretricuspid defects tends to occur later than for posttricuspid defects, this study indicates that prognosis of patients with PAH due to pretricuspid defects is fairly poor
  • In patients with ES, use of PAH-specific drugs appears to lower risk of transplantation and mortality; however, drug therapy is still palliative, and overall outcome of ES is still poor.

 

Kay_William_MD.16.CVaCommentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch:  This study very nicely demonstrates the “real world” treatment of Eisenmenger syndrome (ES) at a variety of tertiary care centers in France.  Overall, 340 patients from 20 separate centers were included.  Patients were analyzed according to defect type (pretricuspid versus posttricuspid shunt), age at time of analysis, and duration of time from PAH diagnosis to time of analysis.  The overall proportion of ES patients on PAH-specific drugs was 80.9%, varying between ~40% and 100% between centers.  The percentage of patients on PAH-specific drugs was much high for those with pretricuspid shunts than posttricuspid shunts.  In this study, ES was defined as PAH (determined by invasive hemodynamics) including a mean pulmonary artery pressure of > 25 mmHg, a normal pulmonary capillary wedge pressure of < 15 mmHg, and a pulmonary vascular resistance > 3 Woods units, in the presence of a large, non-restrictive intracardiac and/or extracardiac shunt.  Other types of PAH associated with ACHD were not included (i.e. patients with persistent Blalock-Taussig shunts or complex pulmonary artery anatomy).

Endpoints for the study included clinical outcomes, with the primary outcomes being death or the need for lung or heart-lung transplantation.  Other clinical events recorded included heart failure, severe infection, arrhythmias, stroke, hemoptysis and other bleeding events.  Events were analyzed with Kaplan-Meier curves based on two models; one model using patient age as the time scale, and a second model using time since PAH diagnosis the time scale.  Genetic syndrome was also included as a variable, with trisomy 21 by far being the most common syndrome.  Clinical data were available for the clear majority of patients, with 93.8% available at the time of initiation of PAH-specific drug therapy.  Median follow-up after drug initiation was 5.5 years (range 3.0-9.1 years).  During follow-up, 19.7% died, with median age at death 41.8 years.  Of note, age at death was not statistically different between pretricuspid and posttricuspid defects (46.2 vs 39.5 years; P = 0.1).  Nearly all deaths were related to ES.  Overall prognosis was poor; half of patients with posttricuspid defects died or were transplanted prior to 50 years of age; half of patients with pretricuspid defects died or underwent transplantation within 20 years after initial PAH diagnosis.

 

The authors demonstrate that patients have a better prognosis on one or two PAH-specific drugs than if they are on no therapy or triple therapy.  The study was not designed to determine a difference between being on 1 versus 2 drugs, and patients who had a 2nd drug started after initial inclusion were analyzed based on number of drugs they were taking at their most recent clinical evaluation.  Patients on triple therapy (ERA, PDE5 and IV prostanoid) tended to be sickest and have the highest risk of transplantation or death.  A total of 28/340 patients underwent transplantation in this study; 24/28 with heart-lung transplantation, and the remaining 4 with double-lung transplantation combined with percutaneous atrial septal defect closure.

 

The median age of patients was 41.0 years in the pretricuspid group and 34.4 years in the posttricuspid/combined group.  NYHA/WHO functional class was III or IV in 62.7% of patients upon drug initiation, whereas only 2.9% were NYHA/WHO class I.  Arterial oxygen saturation did not change significantly in any group.  However, the 6 minute walk distance increased significantly with PAH-specific drug therapy (from 330 meters to 364 meters), and a higher percentage of patients had improvement in NYHA/WHO FC versus those on no therapy.

 

The most common regimen was monotherapy (46.7%), predominantly with bosentan (64.3% of monotherapy patients), which has been approved for clinical use in ES in France for several years.  Dual therapy (40.9%) was provided mostly with the combination of bosentan and sildenafil.  Median time from PAH diagnosis to initiation of PAH-specific drug therapy was shorter in the pretricuspid group (9.1 months) versus the posttricuspid group (67.0 months).

 

Of note, patients on triple-therapy had a longer duration on medical therapy, a higher proportion of Pretricuspid defects, and a lower proportion of genetic syndromes, as well as a worse initial NYHA/WHO FC at time of drug initiation.  This subset also had the highest incidence of ES-related complications, and these patients were responsible for most transplantations.

achd media 9

Figure 2. Kaplan—Meier curves showing survival without major clinical events (death or transplantation) according to age at follow-up, overall and in subgroups defined by defect location, presence or absence of genetic syndrome and pulmonary arterial hypertension-specific drug therapy (PAH-SDT). Event-free survival. D. Event-free survival in patients with post-tricuspid (post-tric.) or combined defects. PAH < 40 yo: pulmonary arterial hypertension diagnosis before 40 years of age; pre-tric.: pre-tricuspid.

 

 

The Cost Differential Between Warfarin Versus Aspirin Treatment After a Fontan Procedure. Schilling C, Dalziel K, Iyengar AJ, d’Udekem Y.

Heart Lung Circ. 2017 Mar 6. pii: S1443-9506(17)30086-0. doi: 10.1016/j.hlc.2017.02.003. [Epub ahead of print] PMID: 28372885

 

Take Home Points:

 Warfarin patients have an additional annual cost vs. those on aspirin.

  • Aspirin is clinically as effective as warfarin and Fontan patients would benefit from a less expensive and less time-consuming treatment.
  • Clinical consensus regarding this point will be difficult to achieve, but there are overarching socio-economic costs if we as an ACHD community fail to do so.

 

Moe_Tabitha-WEBCommentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch:Management of post-Fontan patients with anti-coagulant vs. anti-platelet management of thrombotic risk is an ongoing debate among ACHD care teams.  This article represents the Australian perspective on this management paradigm.  This paper analyzes the societal costs of placing a patient on long-term warfarin including the patient’s travel, time away from work, and the costs to the health system.  These costs are often underappreciated and are exclusive of the costs associated with adverse events, and bleeding complications.  A randomized trial was unable to prove noninferiority of either aspirin or warfarin secondary to lack of power.  In a review of data from the ESC the model of care varies widely, and there is not consensus regarding the clinical effectiveness of aspirin over warfarin and is left to the cardiologist’s preference. This study is valuable in that they evaluated 475 patients who are status post Fontan, and so by congenital standards, this is a fairly large cohort, consistent with other studies that have come out of the NAZ collaborative. If the data from this one-year retrospective data analysis is extrapolated over a 15-year period then $5.8 million dollars could be saved, and is extrapolated over 30 years $11.6 million dollars could be saved. This type of health economics data is valuable in guiding management decision making.

 

Assessment of the systemic right ventricular function in overweight and obese patients with congenitally corrected transposition of the great arteries. Kowalik E, Klisiewicz A, Biernacka EK, Hoffman P. Kardiol Pol. 2017 Mar 10. doi: 10.5603/KP.a2017.0036. [Epub ahead of print] PMID: 28281729

Take Home Points:

 

  • Increasing weight in the overweight and obese range is associated with impaired systemic right ventricular systolic function in patient with CCTGA.
  • The chronic volume overload secondary to obesity may contribute to premature systemic systolic dysfunction.

  

Commentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch: CCTGA is a rare defect of atrioventricular and ventriculo-arterial discordant connections that may go unidentified even in to late adult hood.  This small cohort paper from Poland evaluates a small cohort of 56 adults with CCTGA and only 22 of whom are considered overweight or obese (BMI >25).  Obesity results in hemodynamic changes that lead to increases in the cardiac output and systemic ventricle enlargement. Obesity may be associated with left ventricular hypertrophy, in patients with structurally normal hearts. Little is known about the influence of obesity on cardiac performance in ACHD, and there isn’t any data on patient with a systemic RV.  Their study excluded patients with hypertension, NYHA Class III/IV functional status, arrhythmia, or single ventricle palliation.  This may exclude a number of the patients about whom this data would be the most valuable, however, it makes for a less complex dataset for analysis. Interestingly, there was no observed correlation between the NT-pro BNP levels and BMI. However, the evidence in noncongenital patients is markedly significant in that obesity is associated with decreased NT-pro BNP levels compared to associated decreased systemic ventricular systolic function.  There was a significant reduction in the systemic RV longitudinal strain in the overweight/obese group.  There is also an inverse correlation between BMI and the e”/a” ratio, and a positive correlation between body mass index and left atrial area. One of the limitations of the study is the inherent limitation of imaging of the RV by echo.  As a rule, the RV remains difficult to adequately assess by echo secondary to complex geometry and proximity to the sternum, as well as increased body habitus and poor echo windows in an obese cohort. What remains unknown with this short-term study is whether or not the amount of time spent within the obese range is associated with premature development of RV dysfunction. Since body weight is a largely modifiable risk factor it may be reasonable to be more aggressive with weight management in this cohort as well as others with complex congenital heart disease.

 

 

ACHD March 2017

 

  1. Anomalous Aortic Origin of a Coronary Artery From the Inappropriate Sinus of Valsalva.

Cheezum MK, Liberthson RR, Shah NR, Villines TC, O’Gara PT, Landzberg MJ, Blankstein R.

J Am Coll Cardiol. 2017 Mar 28;69(12):1592-1608. doi: 10.1016/j.jacc.2017.01.031. Review.

PMID:

 

28335843

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  1. Reply: A Distinct Cardiomyopathy: HCN4 Syndrome Comprising Myocardial Noncompaction, Bradycardia, Mitral Valve Defects, and Aortic Dilation.

Arbustini E, Favalli V, Narula N, Serio A, Grasso M.

J Am Coll Cardiol. 2017 Mar 7;69(9):1210-1211. doi: 10.1016/j.jacc.2016.11.073. No abstract available.

PMID:

 

28254189

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  1. A Distinct Cardiomyopathy: HCN4 Syndrome Comprising Myocardial Noncompaction, Bradycardia, Mitral Valve Defects, and Aortic Dilation.

Schweizer PA, Koenen M, Katus HA, Thomas D.

J Am Coll Cardiol. 2017 Mar 7;69(9):1209-1210. doi: 10.1016/j.jacc.2016.10.085. No abstract available.

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28254188

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  1. The power in being together for young adults who haveheartdisease – the photoshoot experience.

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

Heart Lung. 2017 Mar 30. pii: S0147-9563(17)30073-0. doi: 10.1016/j.hrtlng.2017.02.004. [Epub ahead of print]

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  1. The Changing Landscape of Pulmonary Arterial Hypertension in the Adult withCongenital Heart Disease.

van Dissel AC, Mulder BJ, Bouma BJ.

J Clin Med. 2017 Mar 30;6(4). pii: E40. doi: 10.3390/jcm6040040. Review.

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28358329

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  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 Mar 29. doi: 10.1111/echo.13526. [Epub ahead of print]

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  1. Association Between Cardiovascular Risk Factors and Aortic Stenosis: The CANHEART Aortic Stenosis Study.

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J Am Coll Cardiol. 2017 Mar 28;69(12):1523-1532. doi: 10.1016/j.jacc.2017.01.025.

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  1. Red Flags for Maltese Adults withCongenital 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 Mar 24. doi: 10.1007/s00246-017-1604-y. [Epub ahead of print]

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28341902

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  1. Educational needs of adolescents withcongenital heart disease: Impact of a transition intervention programme.

Ladouceur M, Calderon J, Traore M, Cheurfi R, Pagnon C, Khraiche D, Bajolle F, Bonnet D.

Arch Cardiovasc Dis. 2017 Mar 22. pii: S1875-2136(17)30057-8. doi: 10.1016/j.acvd.2017.02.001. [Epub ahead of print]

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28342658

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  1. Longitudinal Evaluation of Aortic Hemodynamics in Marfan Syndrome: New Insights from a 4D Flow Cardiovascular Magnetic Resonance Multi-Year Follow-Up Study.

Geiger J, Hirtler D, Gottfried K, Rahman O, Bollache E, Barker AJ, Markl M, Stiller B.

J Cardiovasc Magn Reson. 2017 Mar 22;19(1):33. doi: 10.1186/s12968-017-0347-5.

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28327193

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  1. Executive Summary: Trends in U.S. Cardiovascular Care: 2016 Report From 4 ACC National Cardiovascular Data Registries.

Masoudi FA, Ponirakis A, de Lemos JA, Jollis JG, Kremers M, Messenger JC, Moore JW, Moussa I, Oetgen WJ, Varosy PD, Vincent RN, Wei J, Curtis JP, Roe MT, Spertus JA.

J Am Coll Cardiol. 2017 Mar 21;69(11):1424-1426. doi: 10.1016/j.jacc.2016.12.004. Epub 2016 Dec 23. No abstract available.

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28025066

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  1. Trends in U.S. Cardiovascular Care: 2016 Report From 4 ACC National Cardiovascular Data Registries.

Masoudi FA, Ponirakis A, de Lemos JA, Jollis JG, Kremers M, Messenger JC, Moore JW, Moussa I, Oetgen WJ, Varosy PD, Vincent RN, Wei J, Curtis JP, Roe MT, Spertus JA.

J Am Coll Cardiol. 2017 Mar 21;69(11):1427-1450. doi: 10.1016/j.jacc.2016.12.005. Epub 2016 Dec 23. Review.

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  1. Noninvasive Imaging in AdultCongenital Heart Disease.

Burchill LJ, Huang J, Tretter JT, Khan AM, Crean AM, Veldtman GR, Kaul S, Broberg CS.

Circ Res. 2017 Mar 17;120(6):995-1014. doi: 10.1161/CIRCRESAHA.116.308983.

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28302744

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  1. Changing Landscape ofCongenital Heart Disease.

Bouma BJ, Mulder BJ.

Circ Res. 2017 Mar 17;120(6):908-922. doi: 10.1161/CIRCRESAHA.116.309302.

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28302739

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  1. Congenital Heart Disease: The Remarkable Journey From the “Post-Mortem Room” to Adult Clinics.

Marian AJ.

Circ Res. 2017 Mar 17;120(6):895-897. doi: 10.1161/CIRCRESAHA.117.310830. No abstract available.

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28302735

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  1. Systolic ejection click versus split firstheartsound: Are our ears deceiving us?

Hoeting NM, McCracken CE, McConnell M, Sallee D, Iannucci GJ, Oster ME.

Congenit Heart Dis. 2017 Mar 16. doi: 10.1111/chd.12460. [Epub ahead of print]

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28299882

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  1. Contraceptive Practices of Women With ComplexCongenital Heart Disease.

Miner PD, Canobbio MM, Pearson DD, Schlater M, Balon Y, Junge KJ, Bhatt A, Barber D, Nickolaus MJ, Kovacs AH, Moons P, Shaw K, Fernandes SM.

Am J Cardiol. 2017 Mar 15;119(6):911-915. doi: 10.1016/j.amjcard.2016.11.047. Epub 2016 Dec 18.

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28087052

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  1. Three decades later: The fate of the population of patients who underwent the Atriopulmonary Fontan procedure.

Poh CL, Zannino D, Weintraub RG, Winlaw DS, Grigg LE, Cordina R, Hornung T, Bullock A, Justo RN, Gentles TL, Verrall C, du Plessis K, Celermajer DS, d’Udekem Y.

Int J Cardiol. 2017 Mar 15;231:99-104. doi: 10.1016/j.ijcard.2017.01.057. Epub 2017 Jan 7.

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28100430

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Deng LX, Gleason LP, Khan AM, Drajpuch D, Fuller S, Goldberg LA, Mascio CE, Partington SL, Tobin L, Kim YY, Kovacs AH.

Int J Cardiol. 2017 Mar 15;231:105-109. doi: 10.1016/j.ijcard.2016.12.185. Epub 2017 Jan 4.

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28096041

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28082094

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Moussa NB, Karsenty C, Pontnau F, Malekzadeh-Milani S, Boudjemline Y, Legendre A, Bonnet D, Iserin L, Ladouceur M.

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Ladouceur M, Pontnau F, Iserin L.

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Kowalik E, Klisiewicz A, Rybicka J, Biernacka EK, Hoffman P.

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28283918

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Kowalik E, Klisiewicz A, Biernacka EK, Hoffman P.

Kardiol Pol. 2017 Mar 10. doi: 10.5603/KP.a2017.0036. [Epub ahead of print]

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28281729

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Hascoet S, Fournier E, Jaïs X, Le Gloan L, Dauphin C, Houeijeh A, Godart F, Iriart X, Richard A, Radojevic J, Amedro P, Bosser G, Souletie N, Bernard Y, Moceri P, Bouvaist H, Mauran P, Barre E, Basquin A, Karsenty C, Bonnet D, Iserin L, Sitbon O, Petit J, Fadel E, Humbert M, Ladouceur M.

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Ereminienė E, Kinderytė M, Miliauskas S.

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Biffi B, Bruse JL, Zuluaga MA, Ntsinjana HN, Taylor AM, Schievano S.

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

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Krexi D, Sheppard MN.

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Hellenic J Cardiol. 2017 Mar 7. pii: S1109-9666(17)30033-7. doi: 10.1016/j.hjc.2017.01.016. [Epub ahead of print] No abstract available.

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  1. The Cost Differential Between Warfarin Versus Aspirin Treatment After a Fontan Procedure.

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

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Velasco O, Beckett MQ, James AW, Loehr MN, Lewis TG, Hassan T, Janardhanan R.

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28171828

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Lüscher TF.

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Congenital EP Featured Articles of March 2017

Congenital and Pediatric Cardiac EP Reviews of March 2017 Publications

 

Mechanisms and predictors of recurrent tachycardia after catheter ablation for d-transposition of the great arteries after the Mustard or Senning operation.

Gallotti RG, Madnawat H, Shannon KM, Aboulhosn JA, Nik-Ahd F, Moore JP.

Heart Rhythm. 2017 Mar;14(3):350-356. doi: 10.1016/j.hrthm.2016.11.031. Epub 2016 Nov 28.

PMID: 27908766

 

Take Home Points:

 

  • Intra-atrial reentrant remains the most common form of supraventricular tachyarrhythmia seen in D-transposition of the great arteries after Senning or Mustard operation
  • Recurrence after ablation occurs in nearly 1/3 of patients within less than 2 years
  • Of importance, recurrence of arrhythmias is often is due to an alternative arrhythmia mechanism rather than the prior ablated substrate
  • Clearly, the risk of arrhythmia recurrence differs between surgical technique with higher recurrent in patients who have undergone Senning operation.
  • Overall success rates after repeated ablation with short-term follow-up are very high (96%).

 

A PatelCommentary from Dr. Akash Patel (San Francisco), section editor of Congenital Electrophysiology Journal Watch:  This large single center study from UCLA aimed to improve our understanding of supraventricular tachyarrhythmia mechanisms after Senning and Mustard operations in patients with D-transposition of the great arteries and identify predictors for recurrence. Previous studies have looked at identifying arrhythmia mechanisms and ablation outcomes for arrhythmias after Mustard or Senning operations.  However, there is limited data regarding the difference between these 2 surgical techniques on mechanisms of arrhythmias and ablation outcomes.

 

This study included 28 patients who underwent 38 catheter ablations from 2004 – 2016. The majority underwent Mustard operation (64%).  89% were treated with antiarrhythmic drugs prior to ablation and 89% achieved initial ablation success.

 

At the time of initial ablation, the majority of arrhythmias were due to intra-atrial reentrant (70%). See Table and Figure Below.

EP media 1 ep media 2

Diagram depicting the sites of acutely successful catheter ablation for the initial catheter ablation procedures in a modified left anterior oblique view. For intra-atrial reentrant tachycardia (IART) that was successfully ablated at the cavotricuspid isthmus (CTI), the number of patients after either the Senning or the Mustard operation is totaled in parentheses. The systemic venous atrium is depicted in blue and the pulmonary venous atrium in pink. M = Mustard; S = Senning. IART, Focal atrial tachycardia, Atrioventricular nodal reentrant tachycardia.

 

Recurrence was seen in 9 patients (32%) with 7 (78%) achieving procedural success. Most recurrences were seen after Senning operation (60% vs. 17%, p =0.34).  In addition, no other risk factor including patients characteristics, arrhythmia mechanism, procedural characteristics were found to be predictive of recurrence.

 

At the time of repeat ablation, most arrhythmias mechanisms where due to intra-atrial reentrant (54%) but compromised a smaller portion compared to the initial procedure. See Table and Figure Below.

ep media 3

ep media 4
Of significant importance was that 10 of 13 (77%) arrhythmias seen during repeat procedure for recurrence were not seen in initial procedure. In particular, Senning operation conferred a higher risk of procedural recurrence with the location of the arrhythmia occurring in the pulmonary venous atrium in 4 out of 6 patients. Of the 3 cases identified at the prior procedure, 2 required ablation in the pulmonary venous atrium for the same mechanism approached only via the systemic venous atrium to achieve procedural success. Overall, 96% of patients remained arrhythmia-free after their final procedure with a median follow-up of 1.3 years.
Prior studies and this continue to demonstrate the significance of supraventricular tachyarrhythmia associated with adult with congenital heart disease.  This study highlights the importance of variations in surgical technique (Senning vs Mustard Operation) on arrhythmia mechanism.  In addition, previously perceived “failed ablations” more often are due to additional arrhythmia mechanisms.  These findings may warrant a more comprehensive approach at the initial procedure.

 

Genotypic and phenotypic predictors of complete heart block and recovery of conduction after surgical repair of congenital heart disease.

Murray LE, Smith AH, Flack EC, Crum K, Owen J, Kannankeril PJ.

Heart Rhythm. 2017 Mar;14(3):402-409. doi: 10.1016/j.hrthm.2016.11.010. Epub 2016 Nov 5.

PMID:27826129

 

Take Home Points:

 

  • Incidence of permanent post-operative complete heart block after congenital heart surgery has remained constant over the last decades around 1-3%.
  • In addition to direct trauma, inflammation, or edema to the AV node there may potential intrinsic factors that may place patients at risk for complete heart block.
  • A gap junction protein, connexin-40, has been implicated in a familial form of complete heart block when malformed due to genetic mutations.
  • A common missense mutation polymorphism for GJA5 (genotype TT) that encodes connexin-40 was associated with a 2-fold increase in the risk of post-operative complete heart block that is similar to the risk that VSD closure confers.
  • Junctional arrhythmia and intermittent conduction were predictive of AV node recovery.
  • Early recovery of AV node function can still result in episodes of higher degree AV block during the post-operative period that necessitates pacemaker therapy.
  • Permanent AV block can rarely have late return of AV conduction.

 

Commentary from Dr. Akash Patel (San Francisco), section editor of Congenital Electrophysiology Journal Watch:  This large single center study from Vanderbilt aimed to improve our understanding of post-operative complete heart block with the assessment of a genetic polymorphism in the gap junction protein, connexin-40, in addition to typical risk factors including diagnosis, preoperative and operative characteristics.

 

This study included 1199 patients who underwent one or more congenital heart surgeries with cardiopulmonary bypass at Vanderbilt from 2007 to 2015.  The case mix was standard for congenital heart surgical program with the 5 most common diagnoses were tetralogy of Fallot (11.3%), ventricular septal defect (10.4%), hypoplastic left heart (9.2%), atrial septal defect (8.3%) and complete AV canal defect (7.3%). The primary surgical procedure involved a ventricular septal defect closure in 41% of the cases.  There were 56 patients (4.7%) who had complete heart block in the operating room or within the first 48 hours.

 

Perioperative risk factors associated with complete heart block included younger age, small size (weight), pre-operative digoxin use, and earlier surgical era. See Table below.

ep media 5Intraoperative risk factors associated with complete heart block included longer cardiopulmonary bypass time, longer aortic cross clamp time, and procedures with ventricular septal defect closure based on univariate analysis. See Table below.

 

 

ep media 6
Post-operative risk factors associated with complete heart block included use of dopamine, use of milrinone, lower pO2, higher lactate on admission to CICU, higher ionized calcium on admission to CICU, need for post-operative ecmo, longer duration of mechanical ventilation, longer CU and hospital length of stay, and increased mortality based on univariate analysis. See Table below.

ep media 7
Genetic risk factors of GJA5 rs10465885 TT genotype polymorphism was associated with increased risk of complete heart block based on univariate analysis.  There were 3 genotype polymorphisms seen in the total cohort C/C (n = 245, 20%), T/C (n=607, 51%), and T/T (n=347, 29%). Overall risk for complete heart block by genotype was 2.8% for C/C, 3.9% for C/T, and 7.2% for T/T. See table below.

ep media 8
On multivariate analysis, use of pre-op digoxin, procedure with VSD closure, and prolonged aortic cross clamp time conferred increased risk. In addition, the presence of the GJA5 polymorphism with TT genotype carried a 2.1-fold risk comparable to the 2.2-fold risk with VSD closure.  See table below.

ep media 9

Recovery of AV node function was seen in 35 of the 56 patients (63%) and pacemaker free recovery was seen in 27 patients (48%) with a median recovery time of 3 days.  Based on assessment of the same pre-operative, intraoperative, post-operative, and genetic risk factors; only pre-operative ACE inhibitor use, intermittent AV conduction, and junctional acceleration (junctional ectopic tachycardia and accelerated junctional rhythm) were associated with recovery of AV node function. Multivariate analysis demonstrated only intermittent AV conduction (Adjusted OR of 9.1) and junctional acceleration (Adjusted OR of 4) were associate with recovery with positive predictive value of 89%.  Of interest were 8 patients with recovery had a pacemaker implanted but 6 (75%) had evidence of 2nd degree or higher AV block. Three had evidence of transient high grade AV block and 3 had evidence of residual conduction disease with first degree and periods of second degree AV block.  All patients received their devices between 7 and 21 days post-operatively.

 

Permanent heart block was seen in 1.4% of the total group. Of interest was late recurrent of AV conduction was seen in 1 patient noted at 101 days post-operative who had pacemaker implanted after nearly 2 weeks of complete heart block.

 

This study raises the intriguing possibility of genetic risks factors for post-operative AV block in addition the conventional wisdom of trauma from surgery.  Further investigation looking at other intrinsic factors may provide for insights into patient specific vulnerability for AV block.

 

 

 

Additional Articles.

 

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Shen WK, Sheldon RS, Benditt DG, Cohen MI, Forman DE, Goldberger ZD, Grubb BP, Hamdan MH, Krahn AD, Link MS, Olshansky B, Raj SR, Sandhu RK, Sorajja D, Sun BC, Yancy CW.

Heart Rhythm. 2017 Mar 9. pii: S1547-5271(17)30297-7. doi: 10.1016/j.hrthm.2017.03.004. [Epub ahead of print] No abstract available.

PMID: 28286247

 

  1. Genotype-Phenotype Correlation of SCN5AMutation for the Clinical and Electrocardiographic Characteristics of Probands with Brugada Syndrome: A Japanese Multicenter Registry.

Yamagata K, Horie M, Aiba T, Ogawa S, Aizawa Y, Ohe T, Yamagishi M, Makita N, Sakurada H, Tanaka T, Shimizu A, Hagiwara N, Kishi R, Nakano Y, Takagi M, Makiyama T, Ohno S, Fukuda K, Watanabe H, Morita H, Hayashi K, Kusano K, Kamakura S, Yasuda S, Ogawa H, Miyamoto Y, Kapplinger JD, Ackerman MJ, Shimizu W.

Circulation. 2017 Mar 24. pii: CIRCULATIONAHA.117.027983. doi: 10.1161/CIRCULATIONAHA.117.027983. [Epub ahead of print]

PMID:28341781

 

CHD EP March 2017

 

  1. Sudden Cardiac Death in Pre-Excitation and Wolff-Parkinson-White: Demographic and Clinical Features.

Finocchiaro G, Papadakis M, Behr ER, Sharma S, Sheppard M.

J Am Coll Cardiol. 2017 Mar 28;69(12):1644-1645. doi: 10.1016/j.jacc.2017.01.023. No abstract available.

PMID:

 

28335848

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J Am Coll Cardiol. 2017 Mar 28;69(12):1642-1643. doi: 10.1016/j.jacc.2017.01.030. No abstract available.

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28335847

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Arch Cardiovasc Dis. 2017 Mar 27. pii: S1875-2136(17)30046-3. doi: 10.1016/j.acvd.2017.01.007. [Epub ahead of print] Review.

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28359691

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Shen WK, Sheldon RS, Benditt DG, Cohen MI, Forman DE, Goldberger ZD, Grubb BP, Hamdan MH, Krahn AD, Link MS, Olshansky B, Raj SR, Sandhu RK, Sorajja D, Sun BC, Yancy CW.

Heart Rhythm. 2017 Mar 9. pii: S1547-5271(17)30297-7. doi: 10.1016/j.hrthm.2017.03.004. [Epub ahead of print] No abstract available.

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Shen WK, Sheldon RS, Benditt DG, Cohen MI, Forman DE, Goldberger ZD, Grubb BP, Hamdan MH, Krahn AD, Link MS, Olshansky B, Raj SR, Sandhu RK, Sorajja D, Sun BC, Yancy CW.

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Varosy PD, Chen LY, Miller AL, Noseworthy PA, Slotwiner DJ, Thiruganasambandamoorthy V.

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  1. Arrhythmia risk and β-blocker therapy in pregnant women with long QT syndrome.

Ishibashi K, Aiba T, Kamiya C, Miyazaki A, Sakaguchi H, Wada M, Nakajima I, Miyamoto K, Okamura H, Noda T, Yamauchi T, Itoh H, Ohno S, Motomura H, Ogawa Y, Goto H, Minami T, Yagihara N, Watanabe H, Hasegawa K, Terasawa A, Mikami H, Ogino K, Nakano Y, Imashiro S, Fukushima Y, Tsuzuki Y, Asakura K, Yoshimatsu J, Shiraishi I, Kamakura S, Miyamoto Y, Yasuda S, Akasaka T, Horie M, Shimizu W, Kusano K.

Heart. 2017 Mar 14. pii: heartjnl-2016-310617. doi: 10.1136/heartjnl-2016-310617. [Epub ahead of print]

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28292826

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  1. Arrhythmias After Fontan Operation with Intra-atrial Lateral Tunnel Versus Extra-cardiac Conduit: A Systematic Review and Meta-analysis.

Li D, Fan Q, Hirata Y, Ono M, An Q.

Pediatr Cardiol. 2017 Mar 7. doi: 10.1007/s00246-017-1595-8. [Epub ahead of print]

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28271152

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  1. Vectrocardiography Identifies Patients with Electrocardiographically Concealed Long QT Syndrome (ecLQTS).

Cortez D, Bos JM, Ackerman MJ.

Heart Rhythm. 2017 Mar 6. pii: S1547-5271(17)30296-5. doi: 10.1016/j.hrthm.2017.03.003. [Epub ahead of print]

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28279743

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  1. KCNQ1 p.L353L affects splicing and modifies the phenotype in a founder population with long QT syndrome type 1.

Kapplinger JD, Erickson A, Asuri S, Tester DJ, McIntosh S, Kerr CR, Morrison J, Tang A, Sanatani S, Arbour L, Ackerman MJ.

J Med Genet. 2017 Mar 6. pii: jmedgenet-2016-104153. doi: 10.1136/jmedgenet-2016-104153. [Epub ahead of print]

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28264985

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  1. Postprocedural Outcomes and Risk Factors for Arrhythmias Following Transcatheter Closure of Congenital Perimembranous Ventricular Septal Defect: A Single-center Retrospective Study.

Zhao LJ, Han B, Zhang JJ, Yi YC, Jiang DD, Lyu JL.

Chin Med J (Engl). 2017 Mar 5;130(5):516-521. doi: 10.4103/0366-6999.200551.

PMID:

 

28229981

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  1. Limited fluoroscopy catheter ablation of accessory pathways in children.

Swissa M, Birk E, Dagan T, Abby Naimer S, Fogelman M, Einbinder T, Bruckheimer E, Fogelman R.

J Cardiol. 2017 Mar 5. pii: S0914-5087(17)30024-2. doi: 10.1016/j.jjcc.2016.12.018. [Epub ahead of print]

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28274505

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  1. Propranolol Versus Digoxin in the Neonate for Supraventricular Tachycardia (from the PediatricHealth Information System).

Bolin EH, Lang SM, Tang X, Collins RT.

Am J Cardiol. 2017 Mar 1. pii: S0002-9149(17)30203-5. doi: 10.1016/j.amjcard.2017.02.017. [Epub ahead of print]

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  1. Incorrect ventricular lead placement into the systemic right ventricle of a patient with D-transposition of the great vessels after Mustard procedure.

Powell AW, Kimball TR.

Cardiol Young. 2017 Mar;27(2):394-397. doi: 10.1017/S1047951116001293. Epub 2016 Aug 30.

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27981916

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  1. Thyroid hormone intoxication as a not yet described cause of J-wave syndrome in a pediatricpatient.

Flores-González JC, Grujic B, Lechuga-Sancho AM.

Endocrine. 2017 Mar;55(3):989-991. doi: 10.1007/s12020-017-1228-2. Epub 2017 Jan 25. No abstract available.

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28124260

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  1. Understanding sudden death risk in tetralogy of Fallot: from bedside to bench.

Seslar S, Robinson M.

Heart. 2017 Mar;103(5):333-334. doi: 10.1136/heartjnl-2016-310338. Epub 2016 Oct 14. No abstract available.

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27742798

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  1. Cardiac Arrest in PediatricPatients Receiving Azithromycin.

Valdés SO, Kim JJ, Niu MC, de la Uz CM, Miyake CY, Moffett BS.

J Pediatr. 2017 Mar;182:311-314.e1. doi: 10.1016/j.jpeds.2016.11.012. Epub 2016 Nov 29.

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27912925

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  1. Development and impact of arrhythmias after the Norwood procedure: A report from the PediatricHeart Network.

Oster ME, Chen S, Dagincourt N, Bar-Cohen Y, Brothers M, Cain N, Colan SD, Czosek RJ, Decker JA, Gamboa DG, Idriss SF, Kirsh JA, LaPage MJ, Ohye RG, Radojewski E, Shah M, Silver ES, Singh AK, Temple JD, Triedman J, Kaltman JR; Pediatric Heart Network Investigators..

J Thorac Cardiovasc Surg. 2017 Mar;153(3):638-645.e2. doi: 10.1016/j.jtcvs.2016.10.078. Epub 2016 Nov 16.

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  1. Nadolol for Treatment of Supraventricular Tachycardia in Infants and Young Children.

von Alvensleben JC, LaPage MJ, Caruthers R, Bradley DJ.

Pediatr Cardiol. 2017 Mar;38(3):525-530. doi: 10.1007/s00246-016-1544-y. Epub 2016 Dec 19.

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  1. The Sudden Death in the Young Case Registry: Collaborating to Understand and Reduce Mortality.

Burns KM, Bienemann L, Camperlengo L, Cottengim C, Covington TM, Dykstra H, Faulkner M, Kobau R, Erck Lambert AB, MacLeod H, Parks SE, Rosenberg E, Russell MW, Shapiro-Mendoza CK, Shaw E, Tian N, Whittemore V, Kaltman JR; Sudden Death in the Young Case Registry Steering Committee..

Pediatrics. 2017 Mar;139(3). pii: e20162757. doi: 10.1542/peds.2016-2757.

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  1. Preoperative right ventricle voltage mapping in Ebstein’s anomaly: can we make the late arrhythmogenic mortality following the cone procedure zero?

Weber LA, May JW, Needleman M.

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  1. Traumatic Gerbode ventricular septal defect and third-degree heart block.

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Fetal Cardiology Featured Articles of March 2017

Fetal Cardiology Featured Reviews of March 2017 Publications

 

Does First-Trimester Screening Modify the Natural History of Congenital Heart Disease? Analysis of Outcome of Regional Cardiac Screening at 2 Different Time Periods.

Jicinska H, Vlasin P, Jicinsky M, Grochova I, Tomek V, Volaufova J, Skovranek J, Marek J.

Circulation. 2017 Mar 14;135(11):1045-1055. doi: 10.1161/CIRCULATIONAHA.115.020864. Epub 2017 Jan 31.

PMID: 28143885

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Take Home Points:

  • Implementation of first trimester screening has changed the spectrum of CHD later in pregnancy and the outcome of fetuses diagnosed with CHD
  • Associated comorbidities (including chromosomal abnormalities) were more frequently found in fetuses diagnosed with CHD in the first trimester

 

Voges IngaCommentary from Dr. Inga Voges (London UK), section editor of Congenital Heart Surgery Journal Watch: This is a large retrospective study, which took place in Southern Moravia (Czech Republic). The authors analyzed the impact of first trimester screening on the spectrum of congenital heart defects (CHDs) later in pregnancy and outcomes of fetuses with CHDs.

 

Two major comparisons were performed (see figure 1):

1) Fetuses diagnosed with CHD in the first trimester were compared with fetuses who were diagnosed with CHD in the second trimester in the period between 2007-2013

2) Fetuses diagnosed with CHD in the second trimester before 2001 (first trimester screening was not routinely performed at that time) were compared with fetuses screened and diagnosed with CHDs only in the second trimester after 2007

 

127 fetuses in the first and 344 fetuses in the second trimester were diagnosed with CHD in the time from 2007 to 2013 (comparison 1).  The diagnoses of hypoplastic left heart syndrome, pulmonary atresia, and tricuspid atresia were significantly higher in the first trimester. The frequency of double outlet right ventricle transposition of great arteries, and pulmonary stenosis was higher in the second trimester. In the second trimester, more fetuses were diagnosed with aortic stenosis and vascular ring. Comorbidities, chromosomal abnormalities, noncardiac abnormalities, univentricular CHDs, intrauterine deaths, and terminations of pregnancy were more frequent in fetuses diagnosed with CHD in the first trimester (see figure 2).

 

Comparison 2 showed that before 2001 significantly more fetuses were diagnosed with hypoplastic left heart syndrome and pulmonary atresia compared with after 2007. Detection of coarctation of aorta, tetralogy of Fallot, vascular ring, and pulmonary stenosis was significantly higher in the second trimester after 2007. A significantly higher frequency of univentricular CHDs, intrauterine deaths and terminations of pregnancy were found in the second trimester from 1996-2001 compared with the second trimester from 2007-2013.

 

This study shows that the implementation of first trimester screening has moved the detection of cardiac abnormalities to early stages of gestation. The authors conclude furthermore that first trimester screening has an important impact on the spectrum of CHD later in pregnancy and on the outcome of fetuses with a CHD.

 fetal media 1

 fetal media 2

 

 Prenatal Diagnosis of the Criss-Cross Heart.

Ravi P, Fruitman D, Mills L, Colen T, Hornberger LK.

Am J Cardiol. 2017 Mar 15;119(6):916-922. doi: 10.1016/j.amjcard.2016.11.046. Epub 2016 Dec 18.

PMID:  28215417

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Select item 28087052 141.

 

Take Home Points

 

  • This is a 5 patient case series from Alberta, Canada detailing the fetal diagnosis of a criss-cross heart.
  • Authors give very useful “pointers” for making the diagnosis of criss-cross heart in the fetus.
  • Authors also provide an excellent review of the literature regarding this rare heart defect.

 

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Fetal Cardiology Journal Watch:   Criss-cross heart is a rare congenital heart defect (CHD).  This defect occurs when the ventricles twist along the ventricular long axis without twisting of the atria.  This causes the inflow tracts to cross each other from hence the name criss-cross heart originates.  Criss-cross heart is a complex CHD which can be difficult to understand and image.  This article from Alberta reviews 5 cases of criss-cross heart diagnosed by fetal echocardiography as well as an excellent review of the literature.   In this article researchers state that criss-cross heart can be diagnosed with “slow cross-sectional sweeps from the upper abdomen demonstrating the relation of the visceral, atrial, ventricular, and great arterial connections and relations”.  Some keys points to fetal diagnosis of criss-cross heart as outlined in this article are as follows:

 

  1. “Inability to obtain a normal 4-chamber view and parallel AV connections by 2D and color Doppler” was seen in all cases of criss-cross heart.
  2. Sagittal sweeps of the fetal heart help determine spatial relation of the ventricle, ventricular septum and great arteries.
  3. A horizontal (parallel) position of the ventricular septum was a key finding in 4 of the 5 cases
  4. Authors of this article caution – “The leftward and superior location of the morphologic right ventricle can be confusing and may be misinterpreted as an L-looped (left sided) right ventricle. To overcome this, the concept introduced by Van Praagh, that the internal spatial organization of the ventricles (handedness) is independent of their location, is helpful.”

 

As the authors discuss the most common type of criss-cross heart is D-loop of the ventricle with discordant ventriculoarterial connection which was seen in 3 of the 5 cases in this case series.  This occurs when there is clockwise rotation of the ventricles relative to the atria which leads to the right-sided right atrium connecting to the leftward superior right ventricle and the left atrium connecting with an inferior left ventricle (superior-inferior orientation of ventricles).  Authors also discuss that the majority of patients with criss-cross heart often have a hypoplastic right ventricle and tricuspid valve, a ventricular septal defect, straddling of the AV valves with pulmonary stenosis and most require single ventricle palliation.  This article also includes useful fetal echocardiographic images to better understand this complex heart lesion.

fetal media 3

fetal media 4

 

Counseling Practices for Fetal Hypoplastic Left Heart Syndrome.

Walsh MJ, Verghese GR, Ferguson ME, Fino NF, Goldberg DJ, Owens ST, Pinto N, Zyblewski SC, Quartermain MD.

Pediatr Cardiol. 2017 Mar 27. doi: 10.1007/s00246-017-1601-1. [Epub ahead of print]

PMID:28345115

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Select item 28345114 86.

 

Take Home Points

 

  • This survey of pediatric cardiologists found that long term complications were commonly not discussed when counselling expectant families after a fetal diagnosis of hypoplastic left heart syndrome.
  • The survey also found significant variability in the setting and content of fetal counselling.
  • Standardization of current counselling practices and training which includes both the short and long term effects of surgical palliation of hypoplastic left heart syndrome is needed and would most likely help optimize a family’s understanding.

 

Abarbanell picture smallCommentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Fetal Cardiology Journal Watch:  Often times the first discussion with a family after a fetal diagnosis of significant congenital heart disease can be challenging and heart wrenching.  Most fetal cardiologists struggle with not overwhelming the family but still conveying the options available including cardiac surgery, as well as comfort care or termination of the pregnancy in some cases.  This article reports the results of a web-based survey of pediatric cardiologists that perform fetal echocardiograms regarding their fetal counselling of expectant families with a fetus with hypoplastic left heart syndrome.  There were 201 physicians that responded.  The results of this survey found that most pediatric cardiologists discuss what families can expect during the first few years of life but the long term complications and outcomes are not as commonly discussed.  See figure. Many of the responders (74%) answered that they did not often counsel families of the long-term complications because “The information would be too overwhelming for the family”.  This study found significant variability in the setting and content of fetal counseling for hypoplastic left heart syndrome.  As the authors conclude “opportunities exist to broaden and standardize current counseling practices and training experiences in an effort to optimize a family’s understanding of the common short- and long-term effects of palliated HLHS physiology.

fetal media 5

 

 

Fetal Cardiology and genetics March 2017

 

  1. Exome sequencing reveals novel IRXI mutation incongenital heart disease.

Guo C, Wang Q, Wang Y, Yang L, Luo H, Cao XF, An L, Qiu Y, Du M, Ma X, Li H, Lu C.

Mol Med Rep. 2017 Mar 30. doi: 10.3892/mmr.2017.6410. [Epub ahead of print]

PMID:

 

28358424

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  1. TCF21 rs12190287 Polymorphisms Are Associated with Ventricular Septal Defects in a Chinese Population.

Yang L, Gao X, Luo H, Huang Q, Su D, Tan X, Lu C.

Genet Test Mol Biomarkers. 2017 Mar 27. doi: 10.1089/gtmb.2016.0324. [Epub ahead of print]

PMID:

 

28346832

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  1. Electronic STIC improves four-dimensional fetal echocardiography.

Guasina F, Bellussi F, Morganelli G, Salsi G, Pilu G, Simonazzi G.

Ultrasound Obstet Gynecol. 2017 Mar 24. doi: 10.1002/uog.17474. [Epub ahead of print]

PMID:

 

28337810

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  1. Point mutations in murine <i>Nkx2-5</i> phenocopy humancongenital heart diseaseand induce pathogenic Wnt signaling.

Furtado MB, Wilmanns JC, Chandran A, Perera J, Hon O, Biben C, Willow TJ, Nim HT, Kaur G, Simonds S, Wu Q, Willians D, Salimova E, Plachta N, Denegre JM, Murray SA, Fatkin D, Cowley M, Pearson JT, Kaye D, Ramialison M, Harvey RP, Rosenthal NA, Costa MW.

JCI Insight. 2017 Mar 23;2(6):e88271. doi: 10.1172/jci.insight.88271.

PMID:

 

28352650

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  1. Cardiac Regeneration: Lessons From Development.

Galdos FX, Guo Y, Paige SL, VanDusen NJ, Wu SM, Pu WT.

Circ Res. 2017 Mar 17;120(6):941-959. doi: 10.1161/CIRCRESAHA.116.309040.

PMID:

 

28302741

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  1. Genetics and Genomics ofCongenital Heart Disease.

Zaidi S, Brueckner M.

Circ Res. 2017 Mar 17;120(6):923-940. doi: 10.1161/CIRCRESAHA.116.309140.

PMID:

 

28302740

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Select item 28302739 124.

 

  1. Prenatal Diagnosis of the Criss-CrossHeart.

Ravi P, Fruitman D, Mills L, Colen T, Hornberger LK.

Am J Cardiol. 2017 Mar 15;119(6):916-922. doi: 10.1016/j.amjcard.2016.11.046. Epub 2016 Dec 18.

PMID:

 

28215417

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  1. Does First-Trimester Screening Modify the Natural History ofCongenital Heart Disease? Analysis of Outcome of Regional Cardiac Screening at 2 Different Time Periods.

Jicinska H, Vlasin P, Jicinsky M, Grochova I, Tomek V, Volaufova J, Skovranek J, Marek J.

Circulation. 2017 Mar 14;135(11):1045-1055. doi: 10.1161/CIRCULATIONAHA.115.020864. Epub 2017 Jan 31.

PMID:

 

28143885

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  1. Concordance of fetal echocardiography in the diagnosis ofcongenitalcardiac diseaseutilizing updated guidelines.

Aguilera M, Dummer K.

J Matern Fetal Neonatal Med. 2017 Mar 12:1-6. doi: 10.1080/14767058.2017.1297791. [Epub ahead of print]

PMID:

 

28277918

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  1. Role of HDLive in Imaging the FetalHeart.

Lakshmy SR, Jain B, Rose N.

J Ultrasound Med. 2017 Mar 11. doi: 10.7863/ultra.16.05071. [Epub ahead of print]

PMID:

 

28295440

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  1. Placental Characteristics of Fetuses WithCongenital Heart Disease.

Albalawi A, Brancusi F, Askin F, Ehsanipoor R, Wang J, Burd I, Sekar P.

J Ultrasound Med. 2017 Mar 4. doi: 10.7863/ultra.16.04023. [Epub ahead of print]

PMID:

 

28258617

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  1. Prenatal Diagnosis of a Retroesophageal Left Brachiocephalic Vein: Two Case Reports.

Cheng YK, Law KM, Chak PK, To KF, Chan YM, Leung TY.

J Ultrasound Med. 2017 Mar 4. doi: 10.7863/ultra.16.05003. [Epub ahead of print]

PMID:

 

28258609

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  1. A case of prenatally detected left isomerism and hemiazygos continuation of inferior vena cava.

Moradi B, Moosavi NS, Kazemi MA, Tahmasebpour AR, Fattahi Masrour F.

J Clin Ultrasound. 2017 Mar 3. doi: 10.1002/jcu.22464. [Epub ahead of print]

PMID:

 

28256002

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  1. MicroRNA-1 upregulation promotes myocardiocyte proliferation and suppresses apoptosis duringheartdevelopment.

Liu L, Yuan Y, He X, Xia X, Mo X.

Mol Med Rep. 2017 Mar 3. doi: 10.3892/mmr.2017.6282. [Epub ahead of print]

PMID:

 

28260051

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  1. Blood flow patterns underlie developmentalheartdefects.

Midgett M, Thornburg K, Rugonyi S.

Am J Physiol Heart Circ Physiol. 2017 Mar 1;312(3):H632-H642. doi: 10.1152/ajpheart.00641.2016. Epub 2017 Jan 6.

PMID:

 

28062416

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  1. Notch-Tnf signalling is required for development and homeostasis of arterial valves.

Wang Y, Wu B, Farrar E, Lui W, Lu P, Zhang D, Alfieri CM, Mao K, Chu M, Yang D, Xu D, Rauchman M, Taylor V, Conway SJ, Yutzey KE, Butcher JT, Zhou B.

Eur Heart J. 2017 Mar 1;38(9):675-686. doi: 10.1093/eurheartj/ehv520.

PMID:

 

26491108

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Select item 28363204 261.

 

  1. Fetal echocardiography for early detection ofcongenitalheart diseases.

Chitra N, Vijayalakshmi IB.

J Echocardiogr. 2017 Mar;15(1):13-17. doi: 10.1007/s12574-016-0308-2. Epub 2016 Aug 16.

PMID:

 

27530200

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  1. The Fetus with Ectopia Cordis: Experience and Expectations from Two Centers.

Escobar-Diaz MC, Sunderji S, Tworetzky W, Moon-Grady AJ.

Pediatr Cardiol. 2017 Mar;38(3):531-538. doi: 10.1007/s00246-016-1545-x. Epub 2016 Dec 19.

PMID:

 

27995289

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Select item 27942762 312.

 

  1. HAND1 Loss-of-Function Mutation Causes Tetralogy of Fallot.

Wang J, Hu XQ, Guo YH, Gu JY, Xu JH, Li YJ, Li N, Yang XX, Yang YQ.

Pediatr Cardiol. 2017 Mar;38(3):547-557. doi: 10.1007/s00246-016-1547-8. Epub 2016 Dec 10.

PMID:

 

27942761

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Select item 27900408 314.

 

  1. Longitudinal changes in fetal biometry and cerebroplacental hemodynamics in fetuses withcongenital heart disease.

Ruiz A, Cruz-Lemini M, Masoller N, Sanz-Cortés M, Ferrer Q, Ribera I, Martínez JM, Crispi F, Arévalo S, Gómez O, Pérez-Hoyos S, Carreras E, Gratacós E, Llurba E.

Ultrasound Obstet Gynecol. 2017 Mar;49(3):379-386. doi: 10.1002/uog.15970.

PMID:

 

27214694

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  1. Fetal left-sided cardiac structural dimensions in left-sided congenital diaphragmatic hernia – association with severity and impact on postnatal outcomes.

Kailin J, Dhillon G, Maskatia S, Cass D, Shamshirsaz A, Mehollin-Ray A, Cassady C, Ayres N, Wang Y, Belfort M, Olutoye O, Ruano R.

Prenat Diagn. 2017 Mar 31. doi: 10.1002/pd.5045. [Epub ahead of print]

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28370263

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  1. Counseling Practices for Fetal Hypoplastic Left Heart Syndrome.

Walsh MJ, Verghese GR, Ferguson ME, Fino NF, Goldberg DJ, Owens ST, Pinto N, Zyblewski SC, Quartermain MD.

Pediatr Cardiol. 2017 Mar 27. doi: 10.1007/s00246-017-1601-1. [Epub ahead of print]

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28345115

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  1. Assessment of Cardiac Function in Fetuses of Gestational Diabetic Mothers During the Second Trimester.

Atiq M, Ikram A, Hussain BM, Saleem B.

Pediatr Cardiol. 2017 Mar 24. doi: 10.1007/s00246-017-1600-2. [Epub ahead of print]

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28337515

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  1. Microarray Analysis of Differential Gene Expression Profile Between Human Fetal and Adult Heart.

Geng Z, Wang J, Pan L, Li M, Zhang J, Cai X, Chu M.

Pediatr Cardiol. 2017 Mar 22. doi: 10.1007/s00246-017-1569-x. [Epub ahead of print]

PMID:

 

28331934

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  1. Motion compensated cine CMR of the fetal heart using radial undersampling and compressed sensing.

Roy CW, Seed M, Kingdom JC, Macgowan CK.

J Cardiovasc Magn Reson. 2017 Mar 20;19(1):29. doi: 10.1186/s12968-017-0346-6.

PMID:

 

28316282

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  1. Prenatal diagnosis of aortopulmonary window associated with aberrant subclavian artery.

Louis-Jacques AF, Obican S, Nguyen T, Odibo A.

Cardiol Young. 2017 Mar 16:1-3. doi: 10.1017/S104795111700035X. [Epub ahead of print]

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28300009

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Select item 28299882 131.

 

  1. Concordance of fetal echocardiography in the diagnosis of congenital cardiac disease utilizing updated guidelines.

Aguilera M, Dummer K.

J Matern Fetal Neonatal Med. 2017 Mar 12:1-6. doi: 10.1080/14767058.2017.1297791. [Epub ahead of print]

PMID:

 

28277918

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  1. Developmental considerations with regard to so-called absence of the leaflets of the arterial valves.

Tretter JT, Steffensen T, Westover T, Anderson RH, Spicer DE.

Cardiol Young. 2017 Mar;27(2):302-311. doi: 10.1017/S1047951116000524. Epub 2016 Apr 29.

PMID:

 

27125520

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  1. Development of the atrial septum in relation to postnatal anatomy and interatrial communications.

Jensen B, Spicer DE, Sheppard MN, Anderson RH.

Heart. 2017 Mar;103(6):456-462. doi: 10.1136/heartjnl-2016-310660. Epub 2016 Dec 21. Review.

PMID:

 

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  1. Symptomatic Fetal-Type Cardiac Rhabdomyoma.

Kayali S, Dogan V, Arda NL, Koç M, Ertugrul I, Özgür S, Örün UA, Karademir S.

J Coll Physicians Surg Pak. 2017 Mar;27(3):S53-S55.

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28302247

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  1. Uhl’s anomaly: perspective of fetal echocardiography and histopathological correlation.

Dixon DB, Mackey-Bojack SM, Sivanandam S.

Cardiol Young. 2017 Mar;27(2):388-390. doi: 10.1017/S1047951116001232.

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  1. The neurodevelopmental implications of hypoplastic left heart syndrome in the fetus.

Lloyd DF, Rutherford MA, Simpson JM, Razavi R.

Cardiol Young. 2017 Mar;27(2):217-223. doi: 10.1017/S1047951116001645. Epub 2016 Nov 8.

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