ACHD Featured Articles of June 2015

1. Comparison of 3 Risk Estimation Methods for Predicting Cardiac Outcomes in Pregnant Women With Congenital Heart Disease.

Lu CW, Shih JC, Chen SY, Chiu HH, Wang JK, Chen CA, Chiu SN, Lin MT, Lee CN, Wu MH.

Circ J. 2015 Jun 25;79(7):1609-17. doi: 10.1253/circj.CJ-14-1368. Epub 2015 May 9.

PMID: 25959432 Free Article

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Moe_Tabitha-WEBMehul Patel photoComment from Drs. Tabitha Moe (Phoenix) and Mehul Patel (Grand Rapids, MI) section editors of Adult Congenital Heart Journal Watch: This article addresses an issue that all practitioners who care for our pregnant patients struggle with.  We have three excellent, well-studied risk calculators available for our use. With each pre-natal consult we carefully calculate the risk, and then attempt to translate the results of these clinical calculators into practical advice that our patients can relate to. Although this retrospective study looks primarily at women in Taiwan in a relatively homogenous population their results are encouraging. The authors studied 268 pregnancies in 190 women with CHD and18 (6.7%) had cardiac complications. It would appear that the CARPREG risk score, the ZAHARA risk score, and the modified WHO classification are all relevant to our practice and there is not wide variability in the applicability or ability to risk stratify our patients. CARPREG is a Canadian risk calculator. ZAHARA is a European collaborative study. The modified WHO is recommended by the ESC. CARPREG utilizes 4 risk predictors: prior cardiac event, NYHA functional class >II or cyanosis at baseline, impaired systemic ventricular function, or severe left sided obstruction. ZAHARA was designed specifically for women with congenital heart disease, and includes 8 risk predictors: history of arrhythmia, cardiac medications prior to pregnancy, NYHA class >II, severe left heart obstruction, severe systemic AV valve regurgitation, severe pulmonary AV valve regurgitation, mechanical valve prosthesis and cyanotic heart disease. CARPREG and WHO can be applied to all women. The study was divided into cardiac events, obstetric events, and fetal events. Their final conclusion is that the modified WHO may be able to better predict cardiac outcomes of pregnancy in patients with CHD, but all three risk scores were effective. An App that would permit calculating all three scores with just a few data points would be very useful.

Take home points:

  1. ZAHARA score was designed specifically for women with congenital heart disease.
  2. The modified WHO classification is the only method that takes into account both specific heart lesions (eg, Marfan syndrome, bicuspid aortic valve, tetralogy of Fallot, aortic coarctation, Fontan circulation or systemic right ventricle) and the clinical cardiac status (eg, systemic ventricular dysfunction).
  3. Modified WHO may be able to better predict cardiac outcomes of pregnancy in patients with CHD, but all three risk scores were effective.

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2. Long-Term Risk for Aortic Complications After Aortic Valve Replacement in Patients With Bicuspid Aortic Valve Versus Marfan syndrome.

Itagaki S, Chikwe JP, Chiang YP, Egorova NN, Adams DH.

J Am Coll Cardiol. 2015 Jun 9;65(22):2363-9. doi: 10.1016/j.jacc.2015.03.575.

PMID: 26046728

Comment from Drs. Tabitha Moe (Phoenix) and Mehul Patel (Grand Rapids, MI) section editors of Adult Congenital Heart Journal Watch: For too long we have been tending to equate bicuspid aortic valve disease and associated aortopathy with Marfan syndrome aortopathy. This paper nicely outlines the risks of continuing to treat all aortopathies with the same algorithm. This paper argues for the development, appropriately, of distinct algorithms for the varying kinds of aortopathies including Marfan syndrome, Vascular Ehlers Danlos, SMAD, and ACTA, as well as the infinitely more common, and less likely to progress bicuspid aortic valve.  Dr. Adams and his colleagues at Mt. Sinai have brought to light a gap in care between internal medicine trained cardiologists, and congenitally trained cardiologists. The notable findings include that the long-term incidence of thoracic aortic dissection is 10-fold higher in patients with Marfan syndrome compared to those with bicuspid aortic valve, while patients with bicuspid aortic valve are only at 20% greater risk of dissection than an age-matched normally structured aortic valve control group. We should not extrapolate operative management of patients with bicuspid aortic valve from Marfan data. One of the limitations of this study was that patients who underwent planned thoracic aortic procedures were excluded, and that therefore limited the inclusion of many of the initially identified Marfan patients. During the 2-year follow up period of the study half of the BAV patients were non-progressors while the majority of the Marfan patients were progressors.

Take home points:

  1. The risk of dissection is 10-fold higher in patients with Marfan syndrome compared to those with bicuspid aortic valve, while patients with bicuspid aortic valve are only at 20% greater risk of dissection than an age-matched normally structured aortic valve control group.
  2. A common algorithm should thus be avoided while managing a spectrum of aortopathies of varying etiologies.
  3. Morbidity even after aortic valve replacement in Marfan syndrome is substantially high with long-term risks for thoracic aortic dissection, new thoracic aortic aneurysms, and need for thoracic aortic surgery compared with patients with bicuspid aortic valves.

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3. Cross-sectional imaging of the Fontan circuit in adult congenital heart disease.

Lewis G, Thorne S, Clift P, Holloway B.

Clin Radiol. 2015 Jun;70(6):667-75. doi: 10.1016/j.crad.2015.02.011. Epub 2015 Mar 26. Review.

PMID: 25818807

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Comment from Drs. Tabitha Moe (Phoenix) and Mehul Patel (Grand Rapids, MI) section editors of Adult Congenital Heart Journal Watch: This single-center commentary is particularly valuable in reviewing cardiac MRI (CMR), and cardiac CT protocols in Fontan patients. For CMR the protocol should consist of planned anatomical sequences including the whole chest with black blood spin-echo or steady state free precession (SSFP) sequences in the 3 orthogonal planes and cine balanced SSFP sequences in the long and short axis through the single ventricle. This allows assessment of ventricular function.  The cine b-SSFP and phase contrast sequences through the ventricular outflow tract, Fontan circuit, and branch PA’s are essential in evaluating obstruction, stenosis, or energy sinks. Time-resolved MRA may be valuable to assess flow dynamics in experienced operators hands.  CT is useful for evaluating acute or chronic complications including: Pulmonary embolism, AV malformations, and venous collaterals. Due to Fontan hemodynamics contrast delivery should be considered pre-procedure. Opacified blood within the pulmonary arteries mixes with non-opacified blood from the IVC more quickly than with typical anatomy. This leads to heterogenous enhancement with standardized PE protocols that is easily misinterpreted at a filling defect, or pulmonary embolus. A dual arm contrast injection, with a delayed phase image acquiring may allow for homogenous contrast uptake, but there is a tradeoff of decreased contrast concentration. Additional, consideration in patients with an interrupted IVC, and Kawashima would not benefit from the same strategy.

Take home points:

  1. Regular follow-up with MRI, and CT as an adjunct in patients with Fontan circulation is now increasingly utilized.
  2. Important radiologically identifiable complications include thromboembolism, pulmonary artery stenosis and pulmonary vein compression, hepatic venous congestion, and cirrhosis and shunting.

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ACHD Articles of June 2015

  1. Maternal cardiac and obstetric performance in consecutive pregnancies in women with heart disease.

Gelson E, Curry R, Gatzoulis MA, Swan L, Lupton M, Steer PJ, Johnson MR.

BJOG. 2015 Jun 29. doi: 10.1111/1471-0528.13489. [Epub ahead of print]

PMID: 26118937

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  1. Pregnancy and Delivery in Women With Congenital Heart Disease.

Warnes CA.

Circ J. 2015 Jun 25;79(7):1416-21. doi: 10.1253/circj.CJ-15-0572. Epub 2015 Jun 4.

PMID: 26040336 Free Article

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  1. Comparison of 3 Risk Estimation Methods for Predicting Cardiac Outcomes in Pregnant Women With Congenital Heart Disease.

Lu CW, Shih JC, Chen SY, Chiu HH, Wang JK, Chen CA, Chiu SN, Lin MT, Lee CN, Wu MH.

Circ J. 2015 Jun 25;79(7):1609-17. doi: 10.1253/circj.CJ-14-1368. Epub 2015 May 9.

PMID: 25959432 Free Article

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  1. Associations among Depression Symptoms with Alcohol and Smoking Tobacco Use in Adult Patients with Congenital Heart Disease.

Khan M, Monaghan M, Klein N, Ruiz G, John AS.

Congenit Heart Dis. 2015 Jun 25. doi: 10.1111/chd.12282. [Epub ahead of print]

PMID: 26108339

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  1. Pregnancy in women with congenital heart disease.

Greutmann M, Pieper PG.

Eur Heart J. 2015 Jun 25. pii: ehv288. [Epub ahead of print] Review.

PMID: 26112887

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  1. Pregnancy in Women with a Mechanical Heart Valve: Data of the European Society of Cardiology Registry of Pregnancy and Cardiac disease (ROPAC).

van Hagen IM, Roos-Hesselink JW, Ruys TP, Merz WM, Goland S, Gabriel H, Lelonek M, Trojnarska O, Al Mahmeed WA, Balint HO, Ashour Z, Baumgartner H, Boersma E, Johnson MR, Hall R; ROPAC investigators and the EORP team.

Circulation. 2015 Jun 22. pii: CIRCULATIONAHA.115.015242. [Epub ahead of print]

PMID: 26100109

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  1. Three-Dimensional Transesophageal Echocardiography in the Anatomical Assessment of Isolated Parachute Mitral Valve in an Adult Patient.

López-Pardo F, Urbano-Moral JA, González-Calle A, Laviana-Martinez F, Esteve-Ruiz I, Lagos-Degrande O, López-Haldon JE.

Echocardiography. 2015 Jun 20. doi: 10.1111/echo.13003. [Epub ahead of print]

PMID: 26096823

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  1. Maternal and foetal outcomes in pregnant systemic lupus erythematosus patients: an incident cohort from a stable referral population followed during 1990-2010.

Jakobsen IM, Helmig RB, Stengaard-Pedersen K.

Scand J Rheumatol. 2015 Jun 19:1-8. [Epub ahead of print]

PMID: 26087812

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  1. Liver stiffness measurements for evaluation of central venous pressure in congenital heart diseases.

Jalal Z, Iriart X, De Lédinghen V, Barnetche T, Hiriart JB, Vergniol J, Foucher J, Thambo JB.

Heart. 2015 Jun 17. pii: heartjnl-2014-307385. doi: 10.1136/heartjnl-2014-307385. [Epub ahead of print]

PMID: 26085526

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  1. Longitudinal development of psychopathology and subjective health status in CHD adults: a 30- to 43-year follow-up in a unique cohort.

Opić P, Roos-Hesselink JW, Cuypers JA, Witsenburg M, van den Bosch A, van Domburg RT, Bogers AJ, Utens EM.

Cardiol Young. 2015 Jun 16:1-9. [Epub ahead of print]

PMID: 26076871

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  1. Myocardial factor revisited: The importance of myocardial fibrosis in adults with congenital heart disease.

Broberg CS, Burchill LJ.

Int J Cardiol. 2015 Jun 15;189:204-210. doi: 10.1016/j.ijcard.2015.04.064. Epub 2015 Apr 10. Review.

PMID: 25897907

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  1. Systemic to pulmonary venous collaterals in adults with single ventricle physiology after cavopulmonary palliation.

Lluri G, Levi DS, Aboulhosn J.

Int J Cardiol. 2015 Jun 15;189:159-63. doi: 10.1016/j.ijcard.2015.04.065. Epub 2015 Apr 10.

PMID: 25897896

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  1. A 42-year-old woman collapses in a park.

Trojan MK, Patel SM, Cook SC.

Heart. 2015 Jun 9. pii: heartjnl-2014-306826. doi: 10.1136/heartjnl-2014-306826. [Epub ahead of print] No abstract available.

PMID: 26060119

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  1. Risk of Systemic Hypertension and Cerebrovascular Accident in Patients With Aortic Coarctation Aged <60 Years (from a National Database Study).

Wu MH, Chen HC, Kao FY, Huang SK.

Am J Cardiol. 2015 Jun 4. pii: S0002-9149(15)01436-8. doi: 10.1016/j.amjcard.2015.05.052. [Epub ahead of print]

PMID: 26100586

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  1. Whole exome sequencing confirms the clinical diagnosis of Marfan syndrome combined with X-linked hypophosphatemia.

Sheng X, Chen X, Lei B, Chen R, Wang H, Zhang F, Rong W, Ha R, Liu Y, Zhao F, Yang P, Zhao C.

J Transl Med. 2015 Jun 4;13:179. doi: 10.1186/s12967-015-0534-9.

PMID: 26040324 Free PMC Article

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  1. Ventricular function and cardiac reserve in contemporary Fontan patients.

Bossers SS, Kapusta L, Kuipers IM, van Iperen G, Moelker A, Kroft LJ, Romeih S, de Rijke Y, Ten Harkel AD, Helbing WA.

Int J Cardiol. 2015 Jun 3;196:73-80. doi: 10.1016/j.ijcard.2015.05.181. [Epub ahead of print]

PMID: 26080280

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  1. Peak oxygen uptake, ventilatory efficiency and QRS-duration predict event free survival in patients late after surgical repair of tetralogy of Fallot.

Müller J, Hager A, Diller GP, Derrick G, Buys R, Dubowy KO, Takken T, Orwat S, Inuzuka R, Vanhees L, Gatzoulis M, Giardini A.

Int J Cardiol. 2015 Jun 2;196:158-164. doi: 10.1016/j.ijcard.2015.05.174. [Epub ahead of print]

PMID: 26114442

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  1. Epoprostenol Therapy for Pulmonary Arterial Hypertension.

Akagi S, Nakamura K, Matsubara H, Ogawa A, Sarashina T, Ejiri K, Ito H.

Acta Med Okayama. 2015 Jun;69(3):129-36.

PMID: 26101188 Free Article

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  1. Global longitudinal strain may identify preserved systolic function of the systemic right ventricle.

Lipczyńska M, Szymański P, Kumor M, Klisiewicz A, Mazurkiewicz Ł, Hoffman P.

Can J Cardiol. 2015 Jun;31(6):760-6. doi: 10.1016/j.cjca.2015.02.028. Epub 2015 Feb 26.

PMID: 25935885

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  1. Pulmonary Vascular Diseases.

Cummings KW, Bhalla S.

Clin Chest Med. 2015 Jun;36(2):235-248. doi: 10.1016/j.ccm.2015.02.007. Epub 2015 Mar 26. Review.

PMID: 26024602

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  1. Cross-sectional imaging of the Fontan circuit in adult congenital heart disease.

Lewis G, Thorne S, Clift P, Holloway B.

Clin Radiol. 2015 Jun;70(6):667-75. doi: 10.1016/j.crad.2015.02.011. Epub 2015 Mar 26. Review.

PMID: 25818807

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  1. Approaching atrial septal defects in pulmonary hypertension.

Schwerzmann M, Pfammatter JP.

Expert Rev Cardiovasc Ther. 2015 Jun;13(6):693-701. doi: 10.1586/14779072.2015.1047763. Epub 2015 May 17.

PMID: 25982879

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  1. [Noncompaction cardiomyopathy].

Shariati J, Schlosser T, Erbel R.

Herz. 2015 Jun;40(4):583-90. doi: 10.1007/s00059-015-4233-3. German.

PMID: 25963034

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  1. Normal values of right pulmonary to left pulmonary flow ratio in healthy individuals determined by cardiovascular magnetic resonance.

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

Int J Cardiol. 2015 Jun 1;188:84-5. doi: 10.1016/j.ijcard.2015.04.027. Epub 2015 Apr 4. No abstract available.

PMID: 25889334

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  1. Advanced therapies in patients with congenital heart disease-related pulmonary arterial hypertension: results from a long-term, single center, real-world follow-up.

Favilli S, Spaziani G, Ballo P, Fibbi V, Santoro G, Chiappa E, Arcangeli C.

Intern Emerg Med. 2015 Jun;10(4):445-50. doi: 10.1007/s11739-014-1185-1. Epub 2015 Jan 9.

PMID: 25573622

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  1. Left Ventricular Strain as Predictor of Chronic Aortic Regurgitation.

Park SH, Yang YA, Kim KY, Park SM, Kim HN, Kim JH, Jang SY, Bae MH, Lee JH, Yang DH.

J Cardiovasc Ultrasound. 2015 Jun;23(2):78-85. doi: 10.4250/jcu.2015.23.2.78. Epub 2015 Jun 26.

PMID: 26140149

 

Select item 26140148

 

  1. Prevalence of Congenital Heart Disease and Pulmonary Hypertension in Down’s Syndrome: An Echocardiographic Study.

Espinola-Zavaleta N, Soto ME, Romero-Gonzalez A, Gómez-Puente Ldel C, Muñoz-Castellanos L, Gopal AS, Keirns C, Lupi-Herrera E.

J Cardiovasc Ultrasound. 2015 Jun;23(2):72-7. doi: 10.4250/jcu.2015.23.2.72. Epub 2015 Jun 26.

PMID: 26140148

 

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  1. Effectiveness of structured education on knowledge and health behaviors in patients with congenital heart disease.

Goossens E, Fieuws S, Van Deyk K, Luyckx K, Gewillig M, Budts W, Moons P.

J Pediatr. 2015 Jun;166(6):1370-1376.e1. doi: 10.1016/j.jpeds.2015.02.041. Epub 2015 Apr 1.

PMID: 25841537

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  1. Ethics of preparticipation cardiovascular screening for athletes.

Maron BJ, Friedman RA, Caplan A.

Nat Rev Cardiol. 2015 Jun;12(6):375-378. doi: 10.1038/nrcardio.2015.21. Epub 2015 Feb 24. Review.

PMID: 25707388

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  1. Cardiac Disease in Pregnancy.

Gandhi M, Martin SR.

Obstet Gynecol Clin North Am. 2015 Jun;42(2):315-333. doi: 10.1016/j.ogc.2015.01.012. Review.

PMID: 26002169

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  1. Adult Congenital Heart Disease in Pregnancy.

Lindley KJ, Conner SN, Cahill AG.

Obstet Gynecol Surv. 2015 Jun;70(6):397-407. doi: 10.1097/OGX.0000000000000190.

PMID: 26115648

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  1. Asymmetric Dimethyl-L-Arginine is a Biomarker for Disease Stage and Follow-Up of Pulmonary Hypertension Associated with Congenital Heart Disease.

Fang ZF, Huang YY, Tang L, Hu XQ, Shen XQ, Tang JJ, Zhou SH.

Pediatr Cardiol. 2015 Jun;36(5):1062-9. doi: 10.1007/s00246-015-1127-3. Epub 2015 Mar 4.

PMID: 25737007

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  1. Selective pulmonary vasodilation improves ventriculovascular coupling and gas exchange in a patient with unrepaired single-ventricle physiology.

Rischard F, Vanderpool R, Jenkins I, Dalabih M, Colombo J, Lax D, Seckeler M.

Pulm Circ. 2015 Jun;5(2):407-11. doi: 10.1086/681269.

PMID: 26064468 Free PMC Article

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  1. Survival in an incident cohort of patients with pulmonary arterial hypertension in Denmark.

Korsholm K, Andersen A, Kirkfeldt RE, Hansen KN, Mellemkjær S, Nielsen-Kudsk JE.

Pulm Circ. 2015 Jun;5(2):364-9. doi: 10.1086/681270.

PMID: 26064463 Free PMC Article

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  1. Cardiac Adaptation of the Maternal Heart During Pregnancy: A Color-Coded Tissue Doppler Imaging Study – Feasibility, Reproducibility and Course during Pregnancy.

Vogt M, Müller J, Kühn A, Elmenhorst J, Mühlbauer F, Oberhoffer R.

Ultraschall Med. 2015 Jun;36(3):270-275. Epub 2014 Apr 11.

PMID: 24729437

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  1. Left ventricular non compaction with aortic valve anomalies: a recurrent feature of 22q11.2 distal deletion syndrome.

Digilio MC, Versacci P, Bernardini L, Novelli A, Marino B, Dallapiccola B.

Eur J Med Genet. 2015 Jun 30. pii: S1769-7212(15)00107-X. doi: 10.1016/j.ejmg.2015.05.005. [Epub ahead of print] No abstract available.

PMID: 26141236

 

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  1. Aortic dimensions in Turner syndrome.

Quezada E, Lapidus J, Shaughnessy R, Chen Z, Silberbach M.

Am J Med Genet A. 2015 Jun 27. doi: 10.1002/ajmg.a.37208. [Epub ahead of print]

PMID: 26118429

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  1. Quantitative Assessment of Mitral Apparatus Geometry Using Dual-Source Computed Tomography in Mitral Regurgitation.

Zhang L, Qiu J, Yu L, Chen S, Sun K, Yao L.

Int Heart J. 2015 Jun 24. [Epub ahead of print]

PMID: 26104175 Free Article

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  1. Comparison of Cine-MRI and Transthoracic Echocardiography for the Assessment of Aortic Root Diameters in Patients with Suspected Marfan Syndrome.

Bannas P, Rybczynski M, Sheikhzadeh S, von Kodolitsch Y, Derlin T, Yamamura J, Lund G, Adam G, Groth M.

Rofo. 2015 Jun 22. [Epub ahead of print]

PMID: 26098252

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  1. The Association Between Aortic Valve Leaflet Morphology and Patterns of Aortic Dilation in Patients With Bicuspid Aortic Valves.

Ruzmetov M, Shah JJ, Fortuna RS, Welke KF.

Ann Thorac Surg. 2015 Jun;99(6):2101-8. doi: 10.1016/j.athoracsur.2015.02.036. Epub 2015 Apr 25.

PMID: 25921253

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  1. BNP and haematological parameters are markers of severity of Ebstein’s anomaly: correlation with CMR and cardiopulmonary exercise testing.

Hösch O, Ngyuen TT, Lauerer P, Schuster A, Kutty S, Staab W, Unterberg-Buchwald C, Sohns JM, Paul T, Lotz J, Steinmetz M.

Eur Heart J Cardiovasc Imaging. 2015 Jun;16(6):670-5. doi: 10.1093/ehjci/jeu312. Epub 2015 Mar 2.

PMID: 25736309

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  1. Endocarditic sinus of valsalva fistulae to right ventricular outflow tract in adult ventricular septal defects.

Zamor N, Phoon C, Malhotra S, Ngai J.

J Clin Anesth. 2015 Jun;27(4):347-52. doi: 10.1016/j.jclinane.2015.02.001. Epub 2015 Apr 21.

PMID: 25910533

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  1. SDHB/SDHA immunohistochemistry in pheochromocytomas and paragangliomas: a multicenter interobserver variation analysis using virtual microscopy: a Multinational Study of the European Network for the Study of Adrenal Tumors (ENS@T).

Papathomas TG, Oudijk L, Persu A, Gill AJ, van Nederveen F, Tischler AS, Tissier F, Volante M, Matias-Guiu X, Smid M, Favier J, Rapizzi E, Libe R, Currás-Freixes M, Aydin S, Huynh T, Lichtenauer U, van Berkel A, Canu L, Domingues R, Clifton-Bligh RJ, Bialas M, Vikkula M, Baretton G, Papotti M, Nesi G, Badoual C, Pacak K, Eisenhofer G, Timmers HJ, Beuschlein F, Bertherat J, Mannelli M, Robledo M, Gimenez-Roqueplo AP, Dinjens WN, Korpershoek E, de Krijger RR.

Mod Pathol. 2015 Jun;28(6):807-21. doi: 10.1038/modpathol.2015.41. Epub 2015 Feb 27.

PMID: 25720320

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Select item 25824336

 

  1. Exercise efficiency relates with mitochondrial content and function in older adults.

Broskey NT, Boss A, Fares EJ, Greggio C, Gremion G, Schlüter L, Hans D, Kreis R, Boesch C, Amati F.

Physiol Rep. 2015 Jun;3(6). pii: e12418. doi: 10.14814/phy2.12418.

PMID: 26059033 Free Article

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  1. Cardiac Adaptation of the Maternal Heart During Pregnancy: A Color-Coded Tissue Doppler Imaging Study – Feasibility, Reproducibility and Course during Pregnancy.

Vogt M, Müller J, Kühn A, Elmenhorst J, Mühlbauer F, Oberhoffer R.

Ultraschall Med. 2015 Jun;36(3):270-275. Epub 2014 Apr 11.

PMID: 24729437

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