Fetal Cardiology Featured Articles of December 2016

Risk Factors for Coarctation of the Aorta on Prenatal Ultrasound: A Systematic Review and Meta-Analysis.

Familiari A, Morlando M, Khalil AA, Sven-Erik S, Scala C, Rizzo G, Del Sordo G, Vassallo C, Flacco ME, Manzoli L, Lanzone A, Scambia G, Acharya G, D’Antonio F.

Circulation. 2016 Dec 29. pii: CIRCULATIONAHA.116.024068. doi: 10.1161/CIRCULATIONAHA.116.024068. [Epub ahead of print]

Take Home Points:

  • Coarctation of the aorta can be difficult to diagnose prenatally.
  • This systematic review found there were several significant differences between fetuses diagnosed with coarctation postnatally compared to those without coarctation.
  • The only prenatal ultrasound parameter found to be independently associated with postnatal coarctation was a hypoplastic aortic arch. All other parameters in isolation had poor diagnostic accuracy.

Abarbanell picture smallComment from Dr. Ginnie Abarbanell (Atlanta), section editor of Fetal Cardiology Journal Watch: This systematic review evaluated the published literature to determine prenatal ultrasound risk factors associated with coarctation of the aorta.  Coarctation of the aorta can be difficult to diagnose prenatally and a number of prenatal centers have published different parameters that could be predictive of coarctation.  This review included 12 articles with a total of 922 fetuses at risk for coarctation of the aorta.  The following parameters were significantly different between fetuses postnatally diagnosed with coarctation compared to those without coarctation.

  1. Lower mean mitral valve diameter z-score
  2. Higher mean tricuspid valve diameter z-score
  3. Lower mean aortic valve diameter z-score
  4. No difference in ascending aorta diameter
  5. Lower mean aortic isthmus diameter z-scores
  6. Higher mean pulmonary artery diameter z-score
  7. Higher right/left ventricle ratio
  8. Higher pulmonary artery/ascending aorta diameter ratio
  9. Lower aortic isthmus/arterial duct diameter ratio
  10. Presence of persistent left superior vena cava, ventricular septal defect or bicuspid aortic valve were not significantly different between fetuses with coarctation compared to those without coarctation.

Hypoplastic aortic arch was the only independently associated prenatal ultrasound risk factor found for postnatal diagnosis of coarctation (sensitivity of 90.0% and specificity of 87.1%).  A coarctation shelf was found to have a high specificity (97.7%) but had a low sensitivity of 48%.  The findings from this study support that a multiple criteria prediction model may improve the detection rate of prenatal coarctation of the aorta.

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Fetal Cardiology and Bench Genetics Articles – December 2016

 

  1. The influence of maternal exposure history to virus and medicine during pregnancy on congenital heart defects of fetus.

Liang Q, Gong W, Zheng D, Zhong R, Wen Y, Wang X.

Environ Sci Pollut Res Int. 2016 Dec 30. doi: 10.1007/s11356-016-8198-4. [Epub ahead of print]

  1. Risk Factors for Coarctation of the Aorta on Prenatal Ultrasound: A Systematic Review and Meta-Analysis.

Familiari A, Morlando M, Khalil AA, Sven-Erik S, Scala C, Rizzo G, Del Sordo G, Vassallo C, Flacco ME, Manzoli L, Lanzone A, Scambia G, Acharya G, D’Antonio F.

Circulation. 2016 Dec 29. pii: CIRCULATIONAHA.116.024068. doi: 10.1161/CIRCULATIONAHA.116.024068. [Epub ahead of print]

  1. Prenatal diagnosis of congenital heart diseases by fetal echocardiography in second trimester: A Chinese multicenter study.

Chu C, Yan Y, Ren Y, Li X, Gui Y.

Acta Obstet Gynecol Scand. 2016 Dec 28. doi: 10.1111/aogs.13085. [Epub ahead of print]

  1. CASZ1 loss-of-function mutation associated with congenital heart disease.

Huang RT, Xue S, Wang J, Gu JY, Xu JH, Li YJ, Li N, Yang XX, Liu H, Zhang XD, Qu XK, Xu YJ, Qiu XB, Li RG, Yang YQ.

Gene. 2016 Dec 20;595(1):62-68. doi: 10.1016/j.gene.2016.09.044.

  1. Myocardial VHL-HIF Signaling Controls an Embryonic Metabolic Switch Essential for Cardiac Maturation.

Menendez-Montes I, Escobar B, Palacios B, Gómez MJ, Izquierdo-Garcia JL, Flores L, Jiménez-Borreguero LJ, Aragones J, Ruiz-Cabello J, Torres M, Martin-Puig S.

Dev Cell. 2016 Dec 19;39(6):724-739. doi: 10.1016/j.devcel.2016.11.012.

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

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

Pediatr Cardiol. 2016 Dec 19. [Epub ahead of print]

  1. Maternal exosomes in diabetes contribute to the cardiac development deficiency.

Shi R, Zhao L, Cai W, Wei M, Zhou X, Yang G, Yuan L.

Biochem Biophys Res Commun. 2016 Dec 18. pii: S0006-291X(16)32142-8. doi: 10.1016/j.bbrc.2016.12.097. [Epub ahead of print]

  1. A Breakdown in Cooperativity Leads to Cardiac Identity Crisis.

Vujic A, Mahmoud AI, Lee RT.

Cell. 2016 Dec 15;167(7):1674-1676. doi: 10.1016/j.cell.2016.11.056.

  1. Cardiovascular profile score as a predictor of acute intrapartum non-reassuring fetal status in infants with congenital heart defects.

Miyoshi T, Katsuragi S, Neki R, Kurosaki KI, Shiraishi I, Nakai M, Nishimura K, Yoshimatsu J, Ikeda T.

J Matern Fetal Neonatal Med. 2016 Dec 14:1-7. [Epub ahead of print]

  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. 2016 Dec 10. [Epub ahead of print]

  1. [Molecular cytogenetic analysis of a case with ring chromosome 3 syndrome].

Zhang K, Song F, Zhang D, Zhang H, Wang Y, Dong R, Zhang Y, Liu Y, Gai Z.

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2016 Dec 10;33(6):816-819. Chinese.

  1. Developmental Mechanisms of Aortic Valve Malformation and Disease.

Wu B, Wang Y, Xiao F, Butcher JT, Yutzey KE, Zhou B.

Annu Rev Physiol. 2016 Dec 9. [Epub ahead of print]

  1. Prenatal diagnosis of idiopathic infantile arterial calcification without fetal hydrops.

Yi Y, Tong T, Liu T, Lin Q, Xiong Y, Xu J.

Echocardiography. 2016 Dec 8. doi: 10.1111/echo.13420. [Epub ahead of print]

  1. Association of maternal chronic disease with risk of congenital heart disease in offspring.

Chou HH, Chiou MJ, Liang FW, Chen LH, Lu TH, Li CY.

CMAJ. 2016 Dec 6;188(17-18):E438-E446.

 

  1. Paternal uniparental disomy with segmental loss of heterozygosity of chromosome 11 are hallmark characteristics of syndromic and sporadic embryonal rhabdomyosarcoma.

Robbins KM, Stabley DL, Holbrook J, Sahraoui R, Sadreameli A, Conard K, Baker L, Gripp KW, Sol-Church K.

Am J Med Genet A. 2016 Dec;170(12):3197-3206. doi: 10.1002/ajmg.a.37949.

 

  1. Fetal Situs, Isomerism, Heterotaxy Syndrome: Diagnostic Evaluation and Implication for Postnatal Management.

Degenhardt K, Rychik J.

Curr Treat Options Cardiovasc Med. 2016 Dec;18(12):77. Review.

  1. Rare novel variants in the ZIC3 gene cause X-linked heterotaxy.

Paulussen AD, Steyls A, Vanoevelen J, van Tienen FH, Krapels IP, Claes GR, Chocron S, Velter C, Tan-Sindhunata GM, Lundin C, Valenzuela I, Nagy B, Bache I, Maroun LL, Avela K, Brunner HG, Smeets HJ, Bakkers J, van den Wijngaard A.

Eur J Hum Genet. 2016 Dec;24(12):1783-1791. doi: 10.1038/ejhg.2016.91.

  1. Notch signalling in ventricular chamber development and cardiomyopathy.

D’Amato G, Luxán G, de la Pompa JL.

FEBS J. 2016 Dec;283(23):4223-4237. doi: 10.1111/febs.13773. Review.

  1. De novo missense variants in PPP1CB are associated with intellectual disability and congenital heart disease.

Ma L, Bayram Y, McLaughlin HM, Cho MT, Krokosky A, Turner CE, Lindstrom K, Bupp CP, Mayberry K, Mu W, Bodurtha J, Weinstein V, Zadeh N, Alcaraz W, Powis Z, Shao Y, Scott DA, Lewis AM, White JJ, Jhangiani SN, Gulec EY, Lalani SR, Lupski JR, Retterer K, Schnur RE, Wentzensen IM, Bale S, Chung WK.

Hum Genet. 2016 Dec;135(12):1399-1409.

  1. Human Cardiomyocytes Prior to Birth by Integration-Free Reprogramming of Amniotic Fluid Cells.

Jiang G, Herron TJ, Di Bernardo J, Walker KA, O’Shea KS, Kunisaki SM.

Stem Cells Transl Med. 2016 Dec;5(12):1595-1606.

  1. Systematic review and meta-analysis of persistent left superior vena cava on prenatal ultrasound: associated anomalies, diagnostic accuracy and postnatal outcome.

Gustapane S, Leombroni M, Khalil A, Giacci F, Marrone L, Bascietto F, Rizzo G, Acharya G, Liberati M, D’Antonio F.

Ultrasound Obstet Gynecol. 2016 Dec;48(6):701-708. doi: 10.1002/uog.15914. Review.

  1. Area of the Fetal Heart’s Four-Chamber View: A Practical Screening Tool to Improve Detection of Cardiac Abnormalities in a Low-Risk Population.

DeVore GR, Satou G, Sklansky M.

Prenat Diagn. 2016 Dec 11. doi: 10.1002/pd.4980. [Epub ahead of print]

  1. Parameters of fetal pulmonary vascular health: baseline trends and response to maternal hyperoxia in the 2nd and 3rd trimesters.

Yamamoto Y, Hirose A, Howley L, Savard W, Jain V, Hornberger LK.

Ultrasound Obstet Gynecol. 2016 Dec 10. doi: 10.1002/uog.17383. [Epub ahead of print]

  1. Fluid mechanics of human fetal right ventricles from image-based computational fluid dynamics using 4D clinical ultrasound scans.

Wiputra H, Lai CQ, Lim GL, Heng JJ, Guo L, Soomar SM, Leo HL, Biwas A, Mattar CN, Yap CH.

Am J Physiol Heart Circ Physiol. 2016 Dec 1;311(6):H1498-H1508. doi: 10.1152/ajpheart.00400.2016.

  1. Fetal Heart Size: A Comparison Between the Point-to-Point Trace and Automated Ellipse Methods Between 20 and 40 Weeks’ Gestation.

DeVore GR, Tabsh K, Polanco B, Satou G, Sklansky M.

J Ultrasound Med. 2016 Dec;35(12):2543-2562.

  1. Aorto-pulmonary calcification in twin-twin transfusion: Successful heart surgery.

Inamura N, Hira S, Iwai S.

Pediatr Int. 2016 Dec;58(12):1375-1376. doi: 10.1111/ped.13106. No abstract available.

  1. Prediction of neonatal outcome of TTTS by fetal heart and Doppler ultrasound parameters before and after laser treatment.

Delabaere A, Leduc F, Reboul Q, Fuchs F, Wavrant S, Fouron JC, Audibert F.

Prenat Diagn. 2016 Dec;36(13):1199-1205. doi: 10.1002/pd.4956.

  1. Anatomically corrected malposed great arteries misdiagnosed as transposition of great arteries: Diagnosis on fetal echocardiography.

Kumar V, Shah S.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):263-4. doi: 10.4103/0974-2069.18912

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CHD Intervention Featured Articles of December 2016

 


CHD Intervention Articles – December 2016

 

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. 2016 Dec 31. pii: ivw366. doi: 10.1093/icvts/ivw366. [Epub ahead of print]

2. Pulmonary and tricuspid valvuloplasty in carcinoid heart disease.

Karimi A, Pourafshar N, Fudge JC.

Catheter Cardiovasc Interv. 2016 Dec 28. doi: 10.1002/ccd.26615. [Epub ahead of print]

3. [Interventional cardiac catheterization in congenital heart disease].

Godart F, Houeijeh A.

Presse Med. 2016 Dec 27. pii: S0755-4982(16)30386-4. doi: 10.1016/j.lpm.2016.11.013. [Epub ahead of print] French.

4. 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. 2016 Dec 26. doi: 10.1007/s11547-016-0714-6. [Epub ahead of print]

5. ‘End-stage’ heart failure therapy: potential lessons from congenital heart disease: from pulmonary artery banding and interatrial communication to parallel circulation.

Schranz D, Akintuerk H, Voelkel NF.

Heart. 2016 Dec 23. pii: heartjnl-2015-309110. doi: 10.1136/heartjnl-2015-309110. [Epub ahead of print] Review.

6. 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. 2016 Dec 22. pii: ezw378. doi: 10.1093/ejcts/ezw378. [Epub ahead of print]

7. Pressure-volume loop-derived cardiac indices during dobutamine stress: a step towards understanding limitations in cardiac output in children with hypoplastic left heart syndrome.

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

Int J Cardiol. 2016 Dec 22. pii: S0167-5273(16)34577-6. doi: 10.1016/j.ijcard.2016.12.087. [Epub ahead of print]

8. Severely regurgitant left ventricle to ascending aorta conduit in a failing fontan patient treated with a vascular endograft and melody transcatheter pulmonary valve via hybrid approach.

Boe BA, Rectenwald JE, Bocks ML.

Catheter Cardiovasc Interv. 2016 Dec;88(7):1113-1117. doi: 10.1002/ccd.26578.

9. Expansion Characteristics of Stents Used in Congenital Heart Disease: Serial Dilation Offers Improved Expansion Potential Compared to Direct Dilation: Results from a Pediatric Interventional Cardiology Early Career Society (PICES) Investigation.

Danon S, Gray RG, Crystal MA, Morgan G, Gruenstein DH, Goldstein BH, Gordon BM.

Congenit Heart Dis. 2016 Dec;11(6):741-750. doi: 10.1111/chd.12399.

10. Distal Superficial Femoral Vein Cannulation for Peripherally Inserted Central Catheter Placement in Infants with Cardiac Disease.

Richter RP, Law MA, Borasino S, Surd JA, Alten JA.

Congenit Heart Dis. 2016 Dec;11(6):733-740. doi: 10.1111/chd.12398.

11. Characteristics of Congenital Coronary Artery Fistulas Complicated with Infective Endocarditis: Analysis of 25 Reported Cases.

Said SA.

Congenit Heart Dis. 2016 Dec;11(6):756-765. doi: 10.1111/chd.12392. Review.

12. First-in-Human Percutaneous Balloon Pulmonary Valvuloplasty Under Echocardiographic Guidance Only.

Wang SZ, Ou-Yang WB, Hu SS, Pang KJ, Liu Y, Zhang FW, Zhang DW, Pan XB.

Congenit Heart Dis. 2016 Dec;11(6):716-720. doi: 10.1111/chd.12380.

13. Transcatheter Patent Ductus Arteriosus Occlusion in Small Infants.

Schwartz MC, Nykanen D, Winner LH, Perez J, McMahan M, Munro HM, Suguna Narasimhulu S.

Congenit Heart Dis. 2016 Dec;11(6):647-655. doi: 10.1111/chd.12360.

14. Fenestrated Transcatheter ASD Closure in Adults with Diastolic Dysfunction and/or Pulmonary Hypertension: Case Series and Review of the Literature.

Abdelkarim A, Levi DS, Tran B, Ghobrial J, Aboulhosn J.

Congenit Heart Dis. 2016 Dec;11(6):663-671. doi: 10.1111/chd.12367.

15. Preprocedural Transthoracic Echocardiography Can Predict Amplatzer Septal Occluder Device Size for Transcatheter Atrial Septal Defect Closure.

Sah SP, Bartakian S, El-Said H, Molkara DP, Printz B, Moore JW.

Congenit Heart Dis. 2016 Dec;11(6):656-662. doi: 10.1111/chd.12365.

16. Radiation Protocol for Three-Dimensional Rotational Angiography to Limit Procedural Radiation Exposure in the Pediatric Cardiac Catheterization Lab.

Haddad L, Waller BR, Johnson J, Choudhri A, McGhee V, Zurakowski D, Kuhls-Gilcrist A, Sathanandam S.

Congenit Heart Dis. 2016 Dec;11(6):637-646. doi: 10.1111/chd.12356.

17. The Impact of Rapid Left Ventricular Pacing during Pediatric Aortic Valvuloplasty on Postprocedural Aortic Insufficiency.

Ertugrul I, Karagoz T, Celiker A, Alehan D, Ozer S, Ozkutlu S.

Congenit Heart Dis. 2016 Dec;11(6):584-588. doi: 10.1111/chd.12340.

18. Catheter-measured Hemodynamics of Adult Fontan Circulation: Associations with Adverse Event and End-organ Dysfunctions.

Mori M, Hebson C, Shioda K, Elder RW, Kogon BE, Rodriguez FH, Jokhadar M, Book WM.

Congenit Heart Dis. 2016 Dec;11(6):589-597. doi: 10.1111/chd.12345.

19. Complications after a Bidirectional Cavopulmonary Anastomosis with Accessory Sources of Pulmonary Blood Flow.

Martínez-Quintana E, Rodríguez-González F.

Int J Angiol. 2016 Dec;25(5):e51-e53. doi: 10.1055/s-0034-1374809.

20. Cardiac index assessment by the pressure recording analytical method in infants after paediatric cardiac surgery: a pilot retrospective study.

Favia I, Rizza A, Garisto C, Haiberger R, Di Chiara L, Romagnoli S, Ricci Z.

Interact Cardiovasc Thorac Surg. 2016 Dec;23(6):919-923.

21. Transthoracic ultrasound guided balloon dilation of cor triatriatum dexter in 2 Rottweiler puppies.

Birettoni F, Caivano D, Bufalari A, Giorgi ME, Miglio A, Paradies P, Porciello F.

J Vet Cardiol. 2016 Dec;18(4):385-390. doi: 10.1016/j.jvc.2016.06.006.

22. The Use and Outcomes of Small, Medium and Large Premounted Stents in Pediatric and Congenital Heart Disease.

Boe BA, Zampi JD, Schumacher KR, Yu S, Armstrong AK.

Pediatr Cardiol. 2016 Dec;37(8):1525-1533.

23. Implementation of Methodology for Quality Improvement in Pediatric Cardiac Catheterization: A Multi-center Initiative by the Congenital Cardiac Catheterization Project on Outcomes-Quality Improvement (C3PO-QI).

Cevallos PC, Rose MJ, Armsby LB, Armstrong AK, El-Said H, Foerster SR, Glatz AC, Goldstein BH, Hainstock MR, Kreutzer J, Latson LA, Leahy RA, Petit CJ, Torres A, Shahanavaz S, Zampi JD, Bergersen L.

Pediatr Cardiol. 2016 Dec;37(8):1436-1445.

24. Radiation Exposure by Three-Dimensional Rotational Angiography (3DRA) During Trans-catheter Melody Pulmonary Valve Procedures (TMPV) in a Pediatric Cardiac Catheterization Laboratory.

Nguyen HH, Balzer DT, Murphy JJ, Nicolas R, Shahanavaz S.

Pediatr Cardiol. 2016 Dec;37(8):1429-1435.

25. Tolerance to ischemia reperfusion injury in a congenital heart disease model.

Asada D, Itoi T, Nakamura A, Hamaoka K.

Pediatr Int. 2016 Dec;58(12):1266-1273. doi: 10.1111/ped.13022.

26. Fontan Fenestration Closure Prior to Posterior Spinal Fusion in Patients With Single-Ventricle Heart Disease.

Schwartz MC, Nykanen D, Fleishman C, Munro HM, Phillips J, Knapp RD, Felix D.

Spine (Phila Pa 1976). 2016 Dec 1;41(23):E1425-E1428.

27. 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. 2016 Dec 28. doi: 10.1001/jamacardio.2016.4954. [Epub ahead of print]

28. Sheath placement in femoral artery during cardiac catheterization in children can influence pressure waveform.

Shiraishi M, Murakami T, Nawa T, Higashi K, Nakajima H, Aotsuka H.

Int J Cardiol. 2016 Dec 22. pii: S0167-5273(16)34609-5. doi: 10.1016/j.ijcard.2016.12.118. [Epub ahead of print]

29. Institution of Veno-arterial Extracorporeal Membrane Oxygenation Does Not Lead to Increased Wall Stress in Patients with Impaired Myocardial Function.

Koth AM, Axelrod DM, Reddy S, Roth SJ, Tacy TA, Punn R.

Pediatr Cardiol. 2016 Dec 22. doi: 10.1007/s00246-016-1546-9. [Epub ahead of print]

30. Initial Experience with Elective Perventricular Melody Valve Placement in Small Patients.

Gupta A, Kenny D, Caputo M, Amin Z.

Pediatr Cardiol. 2016 Dec 20. [Epub ahead of print]

31. An in vivo pilot study of a microporous thin film nitinol-covered stent to assess the effect of porosity and pore geometry on device interaction with the vessel wall.

Chun Y, Kealey CP, Levi DS, Rigberg DA, Chen Y, Tillman BW, Mohanchandra KP, Shayan M, Carman GP.

J Biomater Appl. 2016 Dec 8. pii: 0885328216682691. [Epub ahead of print]

32. Ultrasound-guided femoral arterial access in pediatric cardiac catheterizations: A prospective evaluation of the prevalence, risk factors, and mechanism for acute loss of arterial pulse.

Alexander J, Yohannan T, Abutineh I, Agrawal V, Lloyd H, Zurakowski D, Waller BR 3rd, Sathanandam S.

Catheter Cardiovasc Interv. 2016 Dec;88(7):1098-1107. doi: 10.1002/ccd.26702.

33. Long sheath use in femoral artery catheterizations in infants <15 kg is associated with a higher thrombosis rate: Proposed Protocol for Detection and Management.

Ding L, Pockett C, Moore J, El-Said H.

Catheter Cardiovasc Interv. 2016 Dec;88(7):1108-1112. doi: 10.1002/ccd.26690.

34. A graph-based approach for spatio-temporal segmentation of coronary arteries in X-ray angiographic sequences.

M’hiri F, Duong L, Desrosiers C, Leye M, Miró J, Cheriet M.

Comput Biol Med. 2016 Dec 1;79:45-58. doi: 10.1016/j.compbiomed.2016.10.001.

35. Interpreting measurements of cardiac function using vendor-independent speckle tracking echocardiography in children: a prospective, blinded comparison with catheter-derived measurements.

Goudar SP, Baker GH, Chowdhury SM, Reid KJ, Shirali G, Scheurer MA.

Echocardiography. 2016 Dec;33(12):1903-1910. doi: 10.1111/echo.13347.

36. Percutaneous valve implantation in “tricuspid” position after a Fontan-Björk operation.

Mendes IC, Maymone-Martins F, Anjos R.

J Card Surg. 2016 Dec;31(12):750-754. doi: 10.1111/jocs.12853.

37. Percutaneous PDA Closure in Extremely Low Birth Weight Babies.

Narin N, Pamukcu O, Baykan A, Sunkak S, Ulgey A, Uzum K.

J Interv Cardiol. 2016 Dec;29(6):654-660. doi: 10.1111/joic.12352.

38. Impact of Transcatheter Intervention on Myocardial Deformation in Patients with Coarctation of the Aorta.

Kheiwa A, Aggarwal S, Forbes TJ, Turner DR, Kobayashi D.

Pediatr Cardiol. 2016 Dec;37(8):1590-1597.

39. Evaluation of Impedance Cardiography for Measurement of Stroke Volume in Congenital Heart Disease.

Ebrahim M, Hegde S, Printz B, Abcede M, Proudfoot JA, Davis C.

Pediatr Cardiol. 2016 Dec;37(8):1453-1457.

40. Right Ventricular Pressure Overload and Pathophysiology of Growing Porcine Biomodel.

Kobr J, Slavik Z, Uemura H, Saeed I, Furck A, Pizingerová K, Fremuth J, Tonar Z.

Pediatr Cardiol. 2016 Dec;37(8):1498-1506.

41. Variable Myocardial Response to Load Stresses in Infants with Single Left Ventricular Anatomy: Influence of Initial Physiology and Surgical Palliative Strategy.

Horriat NL, Deatsman SL, Stelter J, Frommelt PC, Hill GD.

Pediatr Cardiol. 2016 Dec;37(8):1569-1574.

42. Fetal balloon aortic valvotomy: Celebrating first birthday.

Koneti NR, Saileela R, Singh JR.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):270-2. doi: 10.4103/0974-2069.189111. No abstract available.

43. Balloon valvotomy for rheumatic valvar stenosis in a patient with complex congenital heart disease.

Ananthakrishna R, Rao DP, Moorthy N, Nanjappa MC.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):265-8. doi: 10.4103/0974-2069.189114. No abstract available.

44. Unusual method of creation of a transcatheter fenestration in an extracardiac conduit Fontan circulation.

Singhi AK, Kothandum S.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):258-62. doi: 10.4103/0974-2069.189112.

45. Modified Stumper technique for acute postoperative bifurcation stenosis causing right ventricular failure after Ross procedure.

Divekar AA.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):248-50. doi: 10.4103/0974-2069.189108.

46. Cardiovascular collapse with attempted pericardial drain withdrawal.

Kraus MB, Spitznagel RA, Kugler JA.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):241-3. doi: 10.4103/0974-2069.189118.

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CHD Surgery Featured Articles of December 2016

Center Variability in Timing of Stage 2 Palliation and Association with Interstage Mortality: A Report from the National Pediatric Cardiology Quality Improvement Collaborative.

Hill GD, Rudd NA, Ghanayem NS, Hehir DA, Bartz PJ.

Pediatr Cardiol. 2016 Dec;37(8):1516-1524.

Take Home Points:

  • There is wide variation in the timing of S2P both between and within centers.
  • Earlier performance of S2P may be associated with improved interstage survival.

jeremy-herrmannCommentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  Timing of stage 2 palliation (S2P) for single ventricle management may vary between institutions, and the optimal timing can be difficult to determine. The authors utilized the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) to evaluation of patterns of timing of S2P among the 55 participating institutions and possible relationship with interstage mortality. Excluded were patients who underwent a hybrid palliation procedure; patients who underwent transplant prior to S2P; patients deemed to candidates for S2P; and patients who were lost to follow-up. Centers with <10 eligible patients were also excluded. Overall, 789 patients from 31 centers were included. The authors also assigned an age cutoff of 153 days to divide centers into 2 groups, early or late. This was followed by a multivariate and univariate analysis of factors possibly relating to interstate mortality.

The median patient age at S2P for all centers was 155 days (109-214 days; Figure 2). As shown in Figure 2, there was considerable variation between centers as well as within centers, as indicated by the wide interquartile ranges. Comparison of late versus early centers revealed that patients at the late centers had a higher prevalence of ventilation prior to stage I palliation (S1P) (34.7 vs. 26.9 %, p = 0.02), longer average post-S1P duration of intubation (14.4 ± 19.7 vs. 10.2 ± 11.4 days, p<0.001), and longer S1P hospital length of stay (48.5 ± 30.4 vs. 38.5 ± 22.3 days, p<0.0001). Patients in the late group also had a significantly longer interstage duration and older age at S2P.

There were 57 deaths in the interstage period giving a total interstage mortality rate of 7.2%. However, the interstage mortality rate was higher in the late group (9.9%) compared to the early group (5.7%; p=0.03). Multivariate analysis demonstrated factors associated with higher interstage mortality included a modified Blalock-Taussig shunt as well late performing centers. Notably, factors not found to significantly correlate with higher interstage mortality included gestational age, need for ECMO support, and a restrictive/intact atrial septum. The authors reasoned these factors may not have been significant in the interstage period as those patients likely had higher mortality around the S1P procedure, were more likely to remain hospitalized prior to the S2P, and may have been more likely to receive a heart transplant. Though not specifically analyzed, higher volume centers tended to perform S2P earlier than lower volume centers. Other important potentially contributing factors not collected and analyzed from the database include genetic abnormalities, need for reintervention, and presence of home monitoring programs.

This study has important limitations largely due to inherent limitations of the database, and it is difficult to draw many concrete conclusions. However, this study’s broad inclusion of centers and large number of patients provide a key glimpse into the issue of determining the precise, optimal timing of S2P. Whether center volume and earlier timing of S2P may contribute to greater interstage survival will be best answered by prospective studies evaluating patient- and center-specific factors.

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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. 2016 Dec 6. pii: S0894-7317(16)30644-7. doi: 10.1016/j.echo.2016.10.017. [Epub ahead of print]

Take Home Point:

  • Preoperative determination of RV/LV inflow angle in the setting of unbalanced atrioventricular septal defect may aid in determining whether to pursue a single- or biventricular surgical approach.

Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  Moderate forms of unbalanced atrioventricular septal defects (AVSD) can present management challenges in terms of deciding whether to proceed down a single ventricle or biventricular pathway or even transplantation. An incorrect decision for initial management can result in worse outcomes for either pathway, and developing predictive models continues to be of significant interest.

In this report, the authors provide an updated analysis of echocardiographic parameters both previously described and novel in this study. They included patients with unbalanced AVSD with adequate echocardiographic assessments at their institution. They excluded patients with other forms of AVSD, mitral or aortic atresia, genetic anomalies (except trisomy 21), and older than 1 year at time of surgery. Groups consisted of single-ventricular palliation and biventricular repair (subsequently analyzed by survivors and those who died or underwent transplantation). Preoperative echocardiograms were blindly reviewed for multiple functional and anatomic features including atrioventricular valve index (AVVI, defined as the ratio of the LAVV area to the RAVV area) and RV/LV inflow angle. Of 181 patients who underwent AVSD repair, 46 met inclusion criteria. Twenty-eight patients underwent single-ventricle palliation (6 with an initial Norwood procedure), and 18 patients underwent biventricular repair.

In comparing echocardiographic measurements between groups, the single ventricle group had smaller LV size, AVVI, and RV/LV inflow angle in systole. Their receiver operative characteristics curve showed that an LVEDV Z score ≤2.5, AVVI ≤0.70, and RV/LV inflow angle (in systole) ≤114 degrees had moderate associations with single-ventricle palliation (Table 4).  The overall difference in seven-year survival between the biventricular group (89%) and the single ventricle group (68%) was not significant. The others attributed the relatively high mortality rate in the single ventricle group to associated need for repair of total anomalous pulmonary venous return as well as frequent need for a systemic-to-PA shunt. There was also no difference in survival between groups by early or late era. After excluding patients with trisomy 21 and/or total anomalous pulmonary venous return, only RV/LV inflow angle in systole remained as a significant difference between the two groups. Interestingly, the presence of atrioventricular valve insufficiency was not associated with adverse outcomes in the biventricular group. Finally, the RV/LV inflow angle and the VSD size (indexed to surface area) showed strong negative correlations. That the RV/LV inflow angle in systole but not diastole was a significant association contrasts with the original report from the Congenital Heart Surgeons’ Society study that observed the opposite to be true. In the current report, no patient with an RV/LV inflow angle of less than 94 degrees underwent biventricular repair.

While the authors note that the small sample size may have reduced their ability to observe differences in other parameters, their findings add to the growing body of literature supporting the use of relative measurements of LAVV and RAVV sizes as a predictive tool for single- or biventricular surgical management in patients with unbalanced AVSD. The need for larger, multi-institutional, prospective data collection to further define precise cutoffs to assist clinical decision-making is apparent.

References

  • Cohen MS, Jegatheeswaran A, Baffa JM, et al. Echocardiographic features defining right dominant unbalanced atrioventricular septal defect: a multi-institutional Congenital Heart Surgeons’ Society study. Circ Cardiovasc Imaging 2013;6:508-13.

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CHD Surgery Articles – December 2016

 

  1. Glial fibrillary acidic protein plasma levels are correlated with degree of hypothermia during cardiopulmonary bypass in congenital heart disease surgery.

Vedovelli L, Padalino M, D’Aronco S, Stellin G, Ori C, Carnielli VP, Simonato M, Cogo P.

Interact Cardiovasc Thorac Surg. 2016 Dec 31. pii: ivw395. doi: 10.1093/icvts/ivw395. [Epub ahead of print]

  1. Lung ultrasound profile after cardiopulmonary bypass in paediatric cardiac surgery: first experience in a simple cohort†.

Vitale V, Ricci Z, Gaddi S, Testa G, Toma P, Cogo P.

Interact Cardiovasc Thorac Surg. 2016 Dec 31. pii: ivw357. doi: 10.1093/icvts/ivw357. [Epub ahead of print]

  1. The problematic of polytetrafluoroethylene membranes for valvular repair.

Taşoğlu İ, Avcı T, Collaku A, Paç M.

Eur J Cardiothorac Surg. 2016 Dec 30. pii: ezw396. doi: 10.1093/ejcts/ezw396. [Epub ahead of print] No abstract available.

  1. Recoarctation After Norwood I Procedure for Hypoplastic Left Heart Syndrome: Impact of Patch Material.

Vitanova K, Cleuziou J, Pabst von Ohain J, Burri M, Eicken A, Lange R.

Ann Thorac Surg. 2016 Dec 23. pii: S0003-4975(16)31479-5. doi: 10.1016/j.athoracsur.2016.10.030. [Epub ahead of print]

  1. Systemic right ventricular morphology in the early postoperative course after extracardiac Fontan operation: is there still a need for special care?

Nordmeyer S, Rohder M, Nordmeyer J, Miera O, Peters B, Cho MY, Photiadis J, Berger F, Ovroutski S.

Eur J Cardiothorac Surg. 2016 Dec 22. pii: ezw374. doi: 10.1093/ejcts/ezw374. [Epub ahead of print]

  1. Natural History of Asymptomatic and Unrepaired Vascular Rings: Is Watchful Waiting a Viable Option? A New Case and Review of Previously Reported Cases.

Loomba RS.

Children (Basel). 2016 Dec 21;3(4). pii: E44. doi: 10.3390/children3040044.

  1. [Risk factors for acute respiratory distress syndrome following surgeries for pediatric critical and complex congenital heart disease].

Gong SR, Zhang YR, Yu RG.

Nan Fang Yi Ke Da Xue Xue Bao. 2016 Dec 20;36(12):1660-1666. Chinese.

  1. Hydrodynamic Assessment of Aortic Valves Prepared from Porcine Small Intestinal Submucosa.

Ramaswamy S, Lordeus M, Mankame OV, Valdes-Cruz L, Bibevski S, Bell SM, Baez I, Scholl F.

Cardiovasc Eng Technol. 2016 Dec 19. [Epub ahead of print]

  1. Readmissions after adult congenital heart surgery: Frequency and risk factors.

Kim YY, He W, MacGillivray TE, Benavidez OJ.

Congenit Heart Dis. 2016 Dec 19. doi: 10.1111/chd.12433. [Epub ahead of print]

  1. Bicuspid aortic valve disease: systematic review and meta-analysis of surgical aortic valve repair.

Salcher M, Naci H, Pender S, Kuehne T; CARDIOPROOF Consortium., Kelm M.

Open Heart. 2016 Dec 16;3(2):e000502. doi: 10.1136/openhrt-2016-000502.

  1. Serum TNF-α levels in children with congenital heart disease undergoing cardiopulmonary bypass: A cohort study in China and a meta-analysis of the published literature.

Song ST, Bai CM, Zhou JW.

J Clin Lab Anal. 2016 Dec 13. doi: 10.1002/jcla.22112. [Epub ahead of print]

  1. Delirium in Children After Cardiac Bypass Surgery.

Patel AK, Biagas KV, Clarke EC, Gerber LM, Mauer E, Silver G, Chai P, Corda R, Traube C.

Pediatr Crit Care Med. 2016 Dec 13. [Epub ahead of print]

  1. Surgical Repair of Congenital Left Atrial Aneurysm and Mitral Valve Insufficiency in a Four-Year-Old Child.

Sarioglu CT, Turkekul Y, Arnaz A, Sisli E, Yalcinbas YK, Sarioglu A.

World J Pediatr Congenit Heart Surg. 2016 Dec 12. pii: 2150135116678416. [Epub ahead of print]

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

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

Pediatr Cardiol. 2016 Dec 10. [Epub ahead of print]

  1. Anesthetic Management of a Tracheoesophageal Fistula in a Patient With a Large Uncorrected Aortopulmonary Window.

Adler AC, Steven JM, Jolley MA.

A A Case Rep. 2016 Dec 9. [Epub ahead of print]

  1. [Postsurgical morbidity in pediatric patients undergoing surgery for congenital heart disease in UMAE from Yucatan].

Castillo-Espínola A, Velázquez-Ibarra A, Zetina-Solórzano A, Bolado-García P, Gamboa-López G.

Arch Cardiol Mex. 2016 Dec 9. pii: S1405-9940(16)30101-X. doi: 10.1016/j.acmx.2016.10.004. [Epub ahead of print] Spanish.

  1. Kaolin-activated thromboelastography and standard coagulation assays in cyanotic and acyanotic infants undergoing complex cardiac surgery: a prospective cohort study.

Rizza A, Ricci Z, Pezzella C, Favia I, Di Felice G, Ranucci M, Cogo P.

Paediatr Anaesth. 2016 Dec 9. doi: 10.1111/pan.13038. [Epub ahead of print]

  1. Systemic inflammatory response syndrome after pediatric congenital heart surgery: Incidence, risk factors, and clinical outcome.

Boehne M, Sasse M, Karch A, Dziuba F, Horke A, Kaussen T, Mikolajczyk R, Beerbaum P, Jack T.

J Card Surg. 2016 Dec 7. doi: 10.1111/jocs.12879. [Epub ahead of print]

  1. Superior Vena Cava Banding to Facilitate Unilateral Bidirectional Glenn Operation in Patients With Single Ventricle Heart Disease and Bilateral Superior Vena Cavae.

Schwartz MC, Nykanen D, DeCampli W, Pourmoghadam K.

World J Pediatr Congenit Heart Surg. 2016 Dec 7. pii: 2150135116679824. [Epub ahead of print]

  1. Contegra 12 mm: How Long Can It Last?

Falchetti A, Pellegrin MA, Sanoussi A, Demanet H, Wauthy P.

World J Pediatr Congenit Heart Surg. 2016 Dec 7. pii: 2150135116675171. [Epub ahead of print]

  1. Hybrid Nuss Procedure for Pectus Excavatum With Severe Retrosternal Adhesions After Sternotomy.

Li S, Yang D, Ma Y, Tang ST, Yang L, Li S, Cao G, Li K, Zhang X, Hu X.

Ann Thorac Surg. 2016 Dec 6. pii: S0003-4975(16)31379-0. doi: 10.1016/j.athoracsur.2016.10.001. [Epub ahead of print]

  1. Two Rare Vascular Rings With Ductal Origin of the Left Pulmonary Artery: A Previously Unrecognized Syndrome?

Thankavel PP, Guleserian KJ, Anderson RH.

World J Pediatr Congenit Heart Surg. 2016 Dec 6. pii: 2150135116675166. [Epub ahead of print]

  1. Perioperative management and outcomes of esophageal atresia and tracheoesophageal fistula.

Lal DR, Gadepalli SK, Downard CD, Ostlie DJ, Minneci PC, Swedler RM, Chelius T, Cassidy L, Rapp CT, Deans KJ, Fallat ME, Finnell SM, Helmrath MA, Hirschl RB, Kabre RS, Leys CM, Mak G, Raque J, Rescorla FJ, Saito JM, St Peter SD, von Allmen D, Warner BW, Sato TT; Midwest Pediatric Surgery Consortium..

J Pediatr Surg. 2016 Dec 5. pii: S0022-3468(16)30597-8. doi: 10.1016/j.jpedsurg.2016.11.046. [Epub ahead of print]

  1. Relationship Between Preoperative Anemia and In-Hospital Mortality in Children Undergoing Noncardiac Surgery.

Faraoni D, DiNardo JA, Goobie SM.

Anesth Analg. 2016 Dec;123(6):1582-1587.

  1. Association of 24/7 In-House Intensive Care Unit Attending Physician Coverage With Outcomes in Children Undergoing Heart Operations.

Gupta P, Rettiganti M, Jeffries HE, Brundage N, Markovitz BP, Scanlon MC, Simsic JM.

Ann Thorac Surg. 2016 Dec;102(6):2052-2061. doi: 10.1016/j.athoracsur.2016.04.042.

  1. Outcomes of ventricular assist device implantation in children and young adults: the Melbourne experience.

Shi WY, Marasco SF, Saxena P, d’Udekem Y, Yong MS, Mitnovetski S, Brizard CP, McGiffin DC, Weintraub RG, Konstantinov IE.

ANZ J Surg. 2016 Dec;86(12):996-1001. doi: 10.1111/ans.13368.

  1. Long-term Outcomes after Truncus Arteriosus Repair: A Single-center Experience for More than 40 Years.

Asagai S, Inai K, Shinohara T, Tomimatsu H, Ishii T, Sugiyama H, Park IS, Nagashima M, Nakanishi T.

Congenit Heart Dis. 2016 Dec;11(6):672-677. doi: 10.1111/chd.12359.

  1. Postcardiotomy ECMO Support after High-risk Operations in Adult Congenital Heart Disease.

Acheampong B, Johnson JN, Stulak JM, Dearani JA, Kushwaha SS, Daly RC, Haile DT, Schears GJ.

Congenit Heart Dis. 2016 Dec;11(6):751-755. doi: 10.1111/chd.12396.

  1. Use of Oxandrolone to Promote Growth in Neonates following Surgery for Complex Congenital Heart Disease: An Open-Label Pilot Trial.

Burch PT, Spigarelli MG, Lambert LM, Loftus PD, Sherwin CM, Linakis MW, Sheng X, LuAnn Minich L, Williams RV.

Congenit Heart Dis. 2016 Dec;11(6):693-699. doi: 10.1111/chd.12376.

  1. Surgical results for pulmonary atresia with intact ventricular septum: a single-centre 15-year experience and medium-term follow-up.

Zheng J, Gao B, Zhu Z, Shi G, Xu Z, Liu J, He X.

Eur J Cardiothorac Surg. 2016 Dec;50(6):1083-1088. doi: 10.1093/ejcts/ezw226.

  1. Heart transplantation in adult congenital heart disease.

Burchill LJ.

Heart. 2016 Dec 1;102(23):1871-1877. doi: 10.1136/heartjnl-2015-309074. Review.

  1. Anaesthetic management of parturients with univentricular congenital heart disease and the Fontan operation.

Monteiro RS, Dob DP, Cauldwell MR, Gatzoulis MA.

Int J Obstet Anesth. 2016 Dec;28:83-91. doi: 10.1016/j.ijoa.2016.08.004.

  1. Performance of stented biological valves for right ventricular outflow tract reconstruction.

Buchholz C, Mayr A, Purbojo A, Glöckler M, Toka O, Cesnjevar RA, Rüffer A.

Interact Cardiovasc Thorac Surg. 2016 Dec;23(6):933-939.

  1. Endoscopic atrioventricular valve surgery in adults with difficult-to-access uncorrected congenital chest wall deformities.

van der Merwe J, Casselman F, Stockman B, Vermeulen Y, Degrieck I, Van Praet F.

Interact Cardiovasc Thorac Surg. 2016 Dec;23(6):851-855.

  1. Aberrant origin of left subclavian artery from the pulmonary artery and right aortic arch in an aortopulmonary window.

Zhu C, Wang T, Zhu Z, Liu K.

Interact Cardiovasc Thorac Surg. 2016 Dec;23(6):991-992.

  1. Perioperative mortality is the Achilles heel for cardiac transplantation in adults with congenital heart disease: Evidence from analysis of the UNOS registry.

Shah DK, Deo SV, Althouse AD, Teuteberg JJ, Park SJ, Kormos RL, Burkhart HM, Morell VO.

J Card Surg. 2016 Dec;31(12):755-764. doi: 10.1111/jocs.12857.

  1. Tissue-engineered cardiac patch seeded with human induced pluripotent stem cell derived cardiomyocytes promoted the regeneration of host cardiomyocytes in a rat model.

Sugiura T, Hibino N, Breuer CK, Shinoka T.

J Cardiothorac Surg. 2016 Dec 1;11(1):163.

  1. Assessment of Risk Factors for a Sustainable “On-Table Extubation” Program in Pediatric Congenital Cardiac Surgery: 5-Year Experience.

Joshi RK, Aggarwal N, Agarwal M, Dinand V, Joshi R.

J Cardiothorac Vasc Anesth. 2016 Dec;30(6):1530-1538. doi: 10.1053/j.jvca.2016.06.017.

  1. 3-Factor Prothrombin Complex Concentrates in Infants With Refractory Bleeding After Cardiac Surgery.

Jooste EH, Machovec KA, Einhorn LM, Ames WA, Homi HM, Jaquiss RD, Lodge AJ, Levy JH, Welsby IJ.

J Cardiothorac Vasc Anesth. 2016 Dec;30(6):1627-1631. doi: 10.1053/j.jvca.2016.01.029. No abstract available.

  1. Children with heart disease: Risk stratification for non-cardiac surgery.

Saettele AK, Christensen JL, Chilson KL, Murray DJ.

J Clin Anesth. 2016 Dec;35:479-484. doi: 10.1016/j.jclinane.2016.09.016.

  1. Outcome of aortic arch reconstruction in infants with coarctation: Importance of operative approach.

Tulzer A, Mair R, Kreuzer M, Tulzer G.

J Thorac Cardiovasc Surg. 2016 Dec;152(6):1506-1513.e1. doi: 10.1016/j.jtcvs.2016.08.029.

  1. Minimally Invasive Cardiac Surgery versus Conventional Median Sternotomy for Atrial Septal Defect Closure.

Jung JC, Kim KH.

Korean J Thorac Cardiovasc Surg. 2016 Dec;49(6):421-426.

  1. Hypoplastic Left Heart Syndrome: Exploring a Paradigm Shift in Favor of Surgery.

Paul EA, Orfali K, Starc TJ.

Pediatr Cardiol. 2016 Dec;37(8):1446-1452.

  1. A case of surgery for congenital esophagobronchial fistula accompanied by a destroyed lung.

Ikeda M, Murata Y, Ohnishi R, Kato T, Hara A, Fujinaga T.

Surg Case Rep. 2016 Dec;2(1):93. doi: 10.1186/s40792-016-0221-y.

  1. Late Clinical Presentation of Inferior Vena Cava Deviation into the Left Atrium Following Atrial Septal Defect Repair in the Childhood.

El-Sayed Ahmad A, Kleine P, Lehnert T, Moritz A.

Thorac Cardiovasc Surg Rep. 2016 Dec;5(1):50-53. doi: 10.1055/s-0035-1566265.

  1. Concurrent use of continuous and pulsatile flow Ventricular Assist Device on a fontan patient: A simulation study.

Di Molfetta A, Ferrari G, Iacobelli R, Filippelli S, Amodeo A.

Artif Organs. 2016 Dec 26. doi: 10.1111/aor.12859. [Epub ahead of print]

  1. Outcomes and Trends of Ventricular Assist Device Selection in Children with End-Stage Heart Failure.

Miller JR, Lancaster TS, Epstein DJ, DuPont NC, Simpson KE, Castleberry C, Canter CE, Eghtesady P, Boston US.

ASAIO J. 2016 Dec 20. doi: 10.1097/MAT.0000000000000506. [Epub ahead of print]

  1. Rehospitalization Patterns in Pediatric Outpatients with Continuous Flow VADs.

Hollander SA, Chen S, Murray JM, Lin A, McBrearty E, Almond CS, Rosenthal DN.

ASAIO J. 2016 Dec 20. doi: 10.1097/MAT.0000000000000505. [Epub ahead of print]

  1. Pericardial effusion requiring surgical intervention after stem cell transplantation: a case series.

Pfeiffer TM, Rotz SJ, Ryan TD, Hirsch R, Taylor M, Chima R, Pate A, Hlavaty J, Grimley M, Myers K, El-Bietar J, Davies SM, Jodele S, Dandoy C.

Bone Marrow Transplant. 2016 Dec 19. doi: 10.1038/bmt.2016.331. [Epub ahead of print] No abstract available.

  1. Factors determining outcomes in grown up patients operated for congenital heart diseases.

Talwar S, Kumar MV, Sreenivas V, Choudhary SK, Sahu M, Airan B.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):222-8. doi: 10.4103/0974-2069.189113.

  1. Heart transplantation in children with intellectual disability: An analysis of the UNOS database.

Goel AN, Iyengar A, Schowengerdt K, Fiore AC, Huddleston CB.

Pediatr Transplant. 2016 Dec 9. doi: 10.1111/petr.12858. [Epub ahead of print]

  1. The Influence of Race and Common Genetic Variations on Outcomes After Pediatric Heart Transplantation.

Green DJ, Brooks MM, Burckart GJ, Chinnock RE, Canter C, Addonizio LJ, Bernstein D, Kirklin JK, Naftel DC, Girnita DM, Zeevi A, Webber SA.

Am J Transplant. 2016 Dec 8. doi: 10.1111/ajt.14153. [Epub ahead of print]

  1. Cardiac failure in very long chain acyl-CoA dehydrogenase deficiency requiring extracorporeal membrane oxygenation (ECMO) treatment: A case report and review of the literature.

Katz S, Landau Y, Pode-Shakked B, Pessach IM, Rubinshtein M, Anikster Y, Salem Y, Paret G.

Mol Genet Metab Rep. 2016 Dec 8;10:5-7.

  1. Hemodiafiltration-A Technique for Physiological Correction of Priming Solution in Pediatric Cardiac Surgery: An In Vitro Study.

Garg P, Bishnoi AK, Patel K, Wadhawa V, Surti J, Solanki A, Shah K, Patel S.

Artif Organs. 2016 Dec 7. doi: 10.1111/aor.12830. [Epub ahead of print]

  1. Systemic inflammatory response syndrome after pediatric congenital heart surgery: Incidence, risk factors, and clinical outcome.

Boehne M, Sasse M, Karch A, Dziuba F, Horke A, Kaussen T, Mikolajczyk R, Beerbaum P, Jack T.

J Card Surg. 2016 Dec 7. doi: 10.1111/jocs.12879. [Epub ahead of print]

  1. Transient severe tricuspid regurgitation after transplantation of an extremely oversized donor heart in a child-Does size matter? A case report.

Birnbaum J, Ulrich SM, Schramm R, Hagl C, Lehner A, Fischer M, Haas NA, Heineking B.

Pediatr Transplant. 2016 Dec 7. doi: 10.1111/petr.12863. [Epub ahead of print]

  1. The Use of Berlin Heart EXCOR VAD in Children Less than 10 kg: A Single Center Experience.

Di Molfetta A, Gandolfo F, Filippelli S, Perri G, Di Chiara L, Iacobelli R, Adorisio R, Favia I, Rizza A, Testa G, Di Nardo M, Amodeo A.

Front Physiol. 2016 Dec 6;7:614. doi: 10.3389/fphys.2016.00614.

  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. 2016 Dec 6. pii: S0894-7317(16)30644-7. doi: 10.1016/j.echo.2016.10.017. [Epub ahead of print]

  1. Membrane Oxygenator Use With Biventricular Assist Device: Facilitation of Support and Lung Recovery.

Nelson-McMillan K, Ravekes WJ, Thompson WR, Brown KM, Wolff L, Wadia RS, McNamara LM, Shaffner DH, Berkowitz ID, Jacobs ML, Vricella LA.

World J Pediatr Congenit Heart Surg. 2016 Dec 6. pii: 2150135116668832. [Epub ahead of print]

  1. Perioperative hypothermia in neonatal intensive care unit patients: effectiveness of a thermoregulation intervention and associated risk factors.

Engorn BM, Kahntroff SL, Frank KM, Singh S, Harvey HA, Barkulis CT, Barnett AM, Olambiwonnu OO, Heitmiller ES, Greenberg RS.

Paediatr Anaesth. 2016 Dec 5. doi: 10.1111/pan.13047. [Epub ahead of print]

  1. Combined Nuss Procedure and Cardiac Procedure Through a Left Anterolateral Thoracotomy.

Roubertie F, Ramanan S, Lavrand F, Thambo JB.

Ann Thorac Surg. 2016 Dec;102(6):e537-e539. doi: 10.1016/j.athoracsur.2016.05.044.

  1. Biomechanics of Failed Pulmonary Autografts Compared With Normal Pulmonary Roots.

Mookhoek A, Krishnan K, Chitsaz S, Kuang H, Ge L, Schoof PH, Bogers AJ, Takkenberg JJ, Tseng EE.

Ann Thorac Surg. 2016 Dec;102(6):1996-2002. doi: 10.1016/j.athoracsur.2016.05.010.

  1. Scimitar Syndrome Repair in Adults: Intermediate-Term Results Using an Extracardiac Conduit.

Guerra NC, Pernot M, Nesseris G, Al-Yamani M, Roques X, Thambo JB, Kreitmann B, Roubertie F.

Ann Thorac Surg. 2016 Dec;102(6):2070-2076. doi: 10.1016/j.athoracsur.2016.05.011.

  1. Recurrent Coarctation After Neonatal Univentricular and Biventricular Norwood-Type Arch Reconstruction.

Whiteside W, Hancock HS, Pasquali SK, Yu S, Armstrong AK, Menchaca A, Hadley A, Hirsch-Romano J.

Ann Thorac Surg. 2016 Dec;102(6):2087-2094. doi: 10.1016/j.athoracsur.2016.04.099.

  1. Nanofibrous bioengineered heart valve-Application in paediatric medicine.

Namdari M, Eatemadi A.

Biomed Pharmacother. 2016 Dec;84:1179-1188. doi: 10.1016/j.biopha.2016.10.058. Review.

  1. Bailout shunt/banding for backward left heart failure after adequate neonatal coarctectomy in borderline left hearts.

Brown SC, Eyskens B, Boshoff D, Cools B, Heying R, Rega F, Meyns B, Gewillig M.

Interact Cardiovasc Thorac Surg. 2016 Dec;23(6):929-932.

  1. The Significance of the Interleaflet Triangles in Determining the Morphology of Congenitally Abnormal Aortic Valves: Implications for Noninvasive Imaging and Surgical Management.

Tretter JT, Spicer DE, Mori S, Chikkabyrappa S, Redington AN, Anderson RH.

J Am Soc Echocardiogr. 2016 Dec;29(12):1131-1143. doi: 10.1016/j.echo.2016.08.017. Review.

  1. Erratum to: Impact of varied center volume categories on volume-outcome relationship in children receiving ECMO for heart operations.

Rettiganti M, Seib PM, Robertson MJ, Wilcox A, Gupta P.

J Artif Organs. 2016 Dec 1. [Epub ahead of print] No abstract available.

  1. Early clinical outcomes of right ventricular outflow tract reconstruction with small caliber bovine jugular vein conduit (Contegra®) in small children.

Kido T, Hoashi T, Kagisaki K, Fujiyoshi T, Kitano M, Kurosaki K, Shiraishi I, Yagihara T, Sawa Y, Ichikawa H.

J Artif Organs. 2016 Dec;19(4):364-371.

  1. A multi-institutional evaluation of antibody-mediated rejection utilizing the Pediatric Heart Transplant Study database: Incidence, therapies and outcomes.

Thrush PT, Pahl E, Naftel DC, Pruitt E, Everitt MD, Missler H, Zangwill S, Burch M, Hoffman TM, Butts R, Mahle WT.

J Heart Lung Transplant. 2016 Dec;35(12):1497-1504. doi: 10.1016/j.healun.2016.06.014.

  1. Outcome of aortic arch reconstruction in infants with coarctation: Importance of operative approach.

Tulzer A, Mair R, Kreuzer M, Tulzer G.

J Thorac Cardiovasc Surg. 2016 Dec;152(6):1506-1513.e1. doi: 10.1016/j.jtcvs.2016.08.029.

  1. Concept of an expandable cardiac valve for surgical implantation in infants and children.

Emani SM, Piekarski BL, Zurakowski D, Baird CA, Marshall AC, Lock JE, Del Nido PJ.

J Thorac Cardiovasc Surg. 2016 Dec;152(6):1514-1523. doi: 10.1016/j.jtcvs.2016.08.040.

  1. A hidden culprit for ventricular dysfunction in aortopulmonary window repair: Anomalous origin of left coronary artery. Case report and review of literature.

Alhadlaq A, Dhillon S, Hancock-Friesen CL, Hussain A.

J Thorac Cardiovasc Surg. 2016 Dec;152(6):e123-e126. doi: 10.1016/j.jtcvs.2016.07.055. No abstract available.

  1. Center Variability in Timing of Stage 2 Palliation and Association with Interstage Mortality: A Report from the National Pediatric Cardiology Quality Improvement Collaborative.

Hill GD, Rudd NA, Ghanayem NS, Hehir DA, Bartz PJ.

Pediatr Cardiol. 2016 Dec;37(8):1516-1524.

  1. Decline in ventricular function as a result of general anesthesia in pediatric heart transplant recipients.

Elhoff JJ, Chowdhury SM, Taylor CL, Hassid M, Savage AJ, Atz AM, Butts RJ.

Pediatr Transplant. 2016 Dec;20(8):1106-1110. doi: 10.1111/petr.12825.

  1. Expanding the donor pool: regional variation in pediatric organ donation rates.

Godown J, McKane M, Wujcik K, Mettler BA, Dodd DA.

Pediatr Transplant. 2016 Dec;20(8):1093-1097. doi: 10.1111/petr.12779.

  1. Heart transplantation after Fontan: Results from a surgical Fontan cohort.

Pundi KN, Pundi K, Driscoll DJ, Dearani JA, Li Z, Dahl SH, Mora BN, O’Leary PW, Daly RC, Cetta F, Johnson JN.

Pediatr Transplant. 2016 Dec;20(8):1087-1092. doi: 10.1111/petr.12753.

  1. Functional, quality of life, and neurodevelopmental outcomes after congenital cardiac surgery.

Ringle ML, Wernovsky G.

Semin Perinatol. 2016 Dec;40(8):556-570. doi: 10.1053/j.semperi.2016.09.008. Review.

  1. Outcomes of Continuous Renal Replacement Therapy With Regional Citrate Anticoagulation in Small Children After Cardiac Surgery: Experience and Protocol From a Single Center.

Musielak A, Warzywoda A, Wojtalik M, Kociński B, Kroll P, Ostalska-Nowicka D, Zachwieja J.

Ther Apher Dial. 2016 Dec;20(6):639-644. doi: 10.1111/1744-9987.12456.

  1. Donor predictors of allograft utilization for pediatric heart transplantation.

Khan AM, Green RS, Lytrivi ID, Sahulee R.

Transpl Int. 2016 Dec;29(12):1269-1275. doi: 10.1111/tri.12835.

  1. Hepatoazygos venous shunt for Fontan completion after Kawashima operation.

Baruah SD, Mishra S, Marwah A, Sharma R.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):254-7. doi: 10.4103/0974-2069.189121.

  1. Modified pediatric Bentall procedure: A novel technique in a rare case.

Salve GG, Javali SR, Dalvi BV, Krishnanaik S.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):244-7. doi: 10.4103/0974-2069.189124.

  1. The “excluding” suture technique for surgical closure of ventricular septal defects: A retrospective study comparing the standard technique.

Varghese R, Saheed S, Ravi AK, Sherrif EA, Agarwal R, Kothandam S.

Ann Pediatr Cardiol. 2016 Sep-Dec;9(3):229-35. doi: 10.4103/0974-2069.189116.

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Pediatric Cardiology Featured Articles of November 2016

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.

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

J Am Coll Cardiol. 2016 Nov 21. pii: S0735-1097(16)36323-9. doi: 10.1016/j.jacc.2016.09.921. [Epub ahead of print] No abstract available.

Take Home Points:

  1. The number of pediatric cardiology fellowship openings is projected to outnumber the available jobs over the next 2 years.
  2. The current job market for finishing pediatric cardiology fellows is perceived as “somewhat difficult”.
  3. The most difficult fields within pediatric cardiology to find a job are cardiac catheterization, electrophysiology and general cardiology. The least challenging fields to find jobs are critical care cardiology, heart failure/transplant and adult congenital cardiology.

Abarbanell picture smallCommentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: How competitive is the current job market for pediatric cardiologists in the US?  Are we training too many pediatric cardiology fellows?

The American College of Cardiology (ACC), American Heart Association (AHA), American Academy of Pediatrics (AAP) and the Society for Pediatric Cardiology Training Program Directors conducted a survey to try to answer these questions.  The survey response rate was 28% with 823 completed surveys.  The response rate was higher among pediatric cardiology fellowship directors (87%) and division chiefs (71%).  Of the program directors that responded to the survey it was reported that no current or previous third or fourth year pediatric cardiology graduates chose or were forced to take a job outside of pediatric cardiology. Over 50% of fellows after completing a 3-year core fellowship and 89% of those who completed a fourth year of subspecialty training entered into academic practice. Program directors were asked to rank the ease of which the pediatric cardiology fellows were obtaining jobs and ranked the job market as “somewhat difficult” (Figure 1). This survey found that the most difficult fields to find jobs were cardiac catheterization, electrophysiology and general cardiology while the least challenging fields to find jobs were critical care cardiology, heart failure/transplant and adult congenital cardiology (Figure 2).  Division chiefs reported that there have been 142 new hires/year with 53% of the positions being filled by fellows with 4 or more years of training, followed by 26% being filled by faculty from another institution and only 21% from 3-year core fellowship graduates.  Using the division chief’s survey responses there is projected to be 135 job openings/year over the next two years.  In comparison there are currently 141 fellowship match openings in pediatric cardiology per year and the number of match opening has nearly doubled over the last decade (Figure 3).  In response to the questions posed above: “How competitive is the current job market for pediatric cardiologists in the US and are we training too many pediatric cardiology fellows?”  The authors conclude “there is concern that those graduating in the next few years may have more trouble finding jobs in their area of interest.”

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Prevalence and pattern of executive dysfunction in school age children with congenital heart disease.

Sanz JH, Berl MM, Armour AC, Wang J, Cheng YI, Donofrio MT.

Congenit Heart Dis. 2016 Nov 11. doi: 10.1111/chd.12427. [Epub ahead of print]

Take Home Points:

  1. Parents of children with congenital heart disease report higher executive dysfunction, in particular for working memory and flexibility.
  2. Male gender, prematurity (< 37 weeks) and CHD with aortic obstruction were predictive of greater impairments in executive function in this study.
  3. A clinically elevated score on the BRIEF (Behavior Rating Inventory of Executive Function) did not increase the odds of receiving special education services.
  4. Findings from this study suggest that children with CHD who may qualify are not receiving special education services.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch: Many recent studies have documented that children with congenital heart disease (CHD) have neurodevelopmental deficits.  This study focuses on executive function (executive function is the ability for a person to complete purposeful and goal directed activity) in children with CHD who underwent cardiac surgery within the first year of life.  91 children (mean age 9 years) were recruited via social media, in-hospital advertisements and at cardiology or neuropsychology clinic visits.  These selection methods may well have biased the results. Their parents completed the Behavior Rating Inventory of Executive Function (BRIEF) and a medical history questionnaire.  The results of the BRIEF were compared to a control sample from a normative database.  Parents reported higher executive dysfunction, in particular for working memory and flexibility compared to the control sample.  Male gender, prematurity (< 37 weeks) and CHD with aortic obstruction were predictive of greater impairments in executive function.  The type of surgical repair (single ventricle vs. biventricular repair) did not predict a higher degree of executive dysfunction.

A third of children with CHD received support at school with either an IEP, 504 or similar student support plan if in private school.  However, a clinically elevated score on the BRIEF did not increase the odds of receiving special school support (See Table 4).  There was a trend for children who were more impulsive (i.e. had inhibitory control problems) to received special school services.  As the authors highlight in the discussion “this makes intuitive sense, since impulsive children can be disruptive in a classroom setting.” But only 22% of the children in this study were noted to have issues with inhibitory control. Additionally, “This study also suggests that many children who may qualify for services and supports in the school setting are not receiving them.”  This study supports that neurodevelopmental assessment in children who have undergone cardiac surgical repair is needed especially to identify children who would benefit from special school services and support.

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White Matter Volume Predicts Language Development in Congenital Heart Disease.

Rollins CK, Asaro LA, Akhondi-Asl A, Kussman BD, Rivkin MJ, Bellinger DC, Warfield SK, Wypij D, Newburger JW, Soul JS.

J Pediatr. 2016 Nov 9. pii: S0022-3476(16)31054-X. doi: 10.1016/j.jpeds.2016.09.070. [Epub ahead of print]

Take Home Points:

  1. Children following biventricular repair of congenital heart disease (CHD) have neurodevelopmental delays at 1 year of age.
  2. Brain MRI at 1 year of age in infants with CHD demonstrated decreased total brain volume, cerebral white matter and brainstem volumes in comparison to healthy controls.
  3. These significant reductions in brain volumes, especially reduction in cerebral white matter correlated to deficits in language development in particular the development of phrases and gestures as well as vocabulary comprehension.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch:  This study from Boston, MA correlated brain MRI findings at 1 year of age in patients with congenital heart disease (CHD) with neurodevelopmental testing.  48 infants with biventricular CHD (transposition of the great arteries, tetralogy of Fallot, Truncus arteriosus, ventricular septal defect or atrioventricular septal defect) who underwent cardiac surgery prior to 9 months of age were included in this study.  Infants underwent a brain MRI and neurodevelopmental testing consisting of Bayley Scales of Infant Development-II V(BSID-II) and the MacArthur-Bates Communicative Development Inventories (CDI) at 1 year of life.  BSID-II is a used to evaluate global neurodevelopment while CDI specifically evaluates language development.  On brain MRI, infants with CHD had significant reductions in total brain, cerebral white matter and brainstem volumes compared to a control group of 13 healthy infants of comparable ages.  Additionally, neurologic evaluation at 1 year was abnormal in over 50% of infants with CHD.  Infants with CHD scored significantly lower on the BSID-II (both psychomotor and mental developmental indices) compared to the control group and significantly below age and sex norms on the CDI.  The MRI findings of reduced brain volumes did not correlate to the findings on the BSID-II but did correlated to lower results on the CDI.  Specifically, cerebral white matter and brainstem volume reductions significantly correlated to development of phrases and gestures as well as vocabulary comprehension.  See Table V and Figure.  The authors concluded:  “Following reparative cardiac surgery, head growth normalized yet cerebral white matter and brainstem volumes remained small. Our findings suggest that persistent reduction in brain volumes in biventricular CHD months after surgery may be driven by early injury to and/or altered development of white matter, and that these observed structural differences are associated with language development.”

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Adiposity and Cardiovascular Risk Factor Variables in Childhood are Associated With Premature Death From Coronary Heart Disease in Adults: The Bogalusa Heart Study.

Berenson GS, Srinivasan SR, Xu JH, Chen W.

Am J Med Sci. 2016 Nov;352(5):448-454. doi: 10.1016/j.amjms.2016.08.006.

Take Home Points:

  1. The Bogalusa study has spanned a period of 40 years and this recent publication examines risk factors that present in childhood that could be associated with coronary artery disease (CAD) or myocardial infarction (MI) related deaths as an adult.
  2. This study found that non HDL cholesterol, body mass index (BMI) and blood pressure increased over time from childhood to adulthood in subjects that had died from CAD and/or MI compared to the living cohort.
  3. Additionally, logistic regression found a higher risk for CAD or MI deaths for subjects of male sex that had increased BMI and mean arterial blood pressure over time.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch:  The Bogalusa study began over 40 years ago in 1973 to identify risk factors for heart disease.  Several cross-sectional surveys have been performed on 5991 subjects from 1973-2010.  Deaths within this cohort have been tracked and over 600 deaths have been documented over the study duration.  This publication specifically evaluated the deaths secondary to cardiovascular causes.  There were 97 cardiovascular deaths of which 46 deaths were considered secondary to coronary artery disease (CAD) or myocardial infarction (MI).   The mean age of death within those 46 deaths was 44.7 years.  Non HDL cholesterol, body mass index (BMI) and blood pressure increased over time in those subjects that had died from CAD and/or MI compared to the living cohort.  See Figure.  Logistic regression found a higher risk for CAD or MI deaths for subjects of male sex, larger BMI and higher mean arterial blood pressure.  This study would suggest that increased BMI as well as increased blood pressure in childhood and adolescence are contributors to increased risk of early CAD/MI deaths.  The study does not address whether therapy could later these outcomes.

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The importance of social media for patients and families affected by congenital anomalies: A Facebook cross-sectional analysis and user survey.

Jacobs R, Boyd L, Brennan K, Sinha CK, Giuliani S.

J Pediatr Surg. 2016 Nov;51(11):1766-1771. doi: 10.1016/j.jpedsurg.2016.07.008.

Take Home Points:

  1. Our patients are turning to social media and the Internet for medical information.
  2. This survey of Facebook users who identified themselves or a member of their family as affected by either anorectal malformation, congenital diaphragmatic hernia, congenital disease (CHD) and hypospadias/epispadias found that 84% would like to have healthcare professionals take an active role in social media. (63% of respondents identified themselves or a member of their family as affected by CHD)
  3. A third of the survey responders felt they did not receive adequate support from their healthcare system/providers.
  4. These results demonstrate that our patients and their families feel that the healthcare system and providers need to develop an appropriate avenue for communicating with our patients via social media.

Commentary from Dr. Ginnie Abarbanell (Atlanta), section editor of Pediatric Cardiology Journal Watch:  We are all aware in this era our patients are turning to the Internet and social media for information and advice.  This study from the United Kingdom sought to evaluate the demographics and needs of individuals using Facebook for information and support related to four congenital anomalies associated with long term morbidity:  anorectal malformation, congenital diaphragmatic hernia, congenital disease (CHD) and hypospadias/epispadias.  Researchers used a keyword search in Facebook to identify relevant Groups/Pages and posted an anonymous survey on Facebook related to the group/page demographics and healthcare needs.  54 groups and 24 pages in Facebook were found.  Specifically, there were 9 groups and 7 pages identified which related to CHD.  Among the 4 groups noted above there were 16,191 group members and 48,766 page likes.  1133 individuals responded to the survey within 2 months of initially posting the survey on Facebook.  Of the respondents 63% identified themselves or a member of their family as affected by CHD. The survey responders were predominantly women (mothers of affected individuals) 26-40 years of age.  The common reasons for joining the Facebook groups/pages were: seeking support, education, making friends, and providing support to others.    84% of respondents would like to have healthcare professionals take an active role in the Facebooks groups and 97% stated they would join a group linked to their primary hospital.  A third (31%) felt they did not receive adequate support from their healthcare system/providers.  The results of this study would support a more active role for healthcare providers in social media.  However, as the authors discuss there is concern regarding the legality of providing advice online as well as issues with patient confidentiality and professional boundaries. Hence, any online forum would need to be “tightly regulated” to ensure appropriate content and confidentiality.  The final sentence of this study is a quote from a survey respondent, which sums up the status of healthcare involvement in social media. “Healthcare must move to the next level and providers must begin to actively engage on a broader scope.”

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Pediatric Cardiology Articles – November 2016

  1. Defining pediatric inpatient cardiology care delivery models: A survey of pediatric cardiology programs in the USA and Canada.

Mott AR, Neish SR, Challman M, Feltes TF.

Congenit Heart Dis. 2016 Nov 25. doi: 10.1111/chd.12438. [Epub ahead of print]

  1. Trends, microbiology, and outcomes of infective endocarditis in children during 2000-2010 in the United States.

Gupta S, Sakhuja A, McGrath E, Asmar B.

Congenit Heart Dis. 2016 Nov 25. doi: 10.1111/chd.12425. [Epub ahead of print]

  1. Limited Ventricular Preload is the Main Reason for Reduced Stress Reserve After Atrial Baffle Repair.

Eicken A, Michel J, Hager A, Tanase D, Kaemmerer H, Cleuziou J, Hess J, Ewert P.

Pediatr Cardiol. 2016 Nov 24. [Epub ahead of print]

  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. 2016 Nov 23. [Epub ahead of print]

  1. Cardiopulmonary Exercise Testing in Fontan Patients With and Without Isomerism (Heterotaxy) as Compared to Patients With Primary Ciliary Dyskinesia and Subjects With Structurally Normal Hearts.

Loomba RS, Danduran M, Nielsen KG, Ring AM, Kovach J, Anderson RH.

Pediatr Cardiol. 2016 Nov 23. [Epub ahead of print]

  1. Global Deformation Parameters Response to Exercise in Adolescents with Repaired Tetralogy of Fallot.

Mese T, Guven B, Yilmazer MM, Demirol M, Çoban Ş, Karadeniz C.

Pediatr Cardiol. 2016 Nov 23. [Epub ahead of print]

  1. Accelerated Cardiac Rhabdomyoma Regression with Everolimus in Infants with Tuberous Sclerosis Complex.

Aw F, Goyer I, Raboisson MJ, Boutin C, Major P, Dahdah N.

Pediatr Cardiol. 2016 Nov 23. [Epub ahead of print]

  1. Elevated serum levels of ghrelin and TNF-α in patients with cyanotic and acyanotic congenital heart disease.

Zhang S, Guo GL, Yang LL, Sun LQ.

World J Pediatr. 2016 Nov 23. [Epub ahead of print]

  1. Jellyfish-Like Accessory Mitral Valve Tissue Causing Near-Collapse in a Young Child.

Kato H, Escudero C, Sherwin E, Hosking M, Gandhi SK.

World J Pediatr Congenit Heart Surg. 2016 Nov 23. pii: 2150135116668335. [Epub ahead of print]

  1. Pediatric cardiac readmissions: An opportunity for quality improvement?

Sacks JH, Kelleman M, McCracken C, Glanville M, Oster M.

Congenit Heart Dis. 2016 Nov 22. doi: 10.1111/chd.12436. [Epub ahead of print]

  1. Coarctation repair normalizes left ventricular function and aorto-septal angle in neonates.

Jashari H, Lannering K, Mellander M, Ibrahimi P, Rydberg A, Henein MY.

Congenit Heart Dis. 2016 Nov 22. doi: 10.1111/chd.12430. [Epub ahead of print]

  1. Intra- and inter-reader reproducibility of blood flow measurements on the ascending aorta and pulmonary artery using cardiac magnetic resonance.

Di Leo G, D’Angelo ID, Alì M, Cannaò PM, Mauri G, Secchi F, Sardanelli F.

Radiol Med. 2016 Nov 22. [Epub ahead of print]

  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.

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

J Am Coll Cardiol. 2016 Nov 21. pii: S0735-1097(16)36323-9. doi: 10.1016/j.jacc.2016.09.921. [Epub ahead of print] No abstract available.

  1. NT-proBNP as Marker of Ventricular Dilatation and Pulmonary Regurgitation After Surgical Correction of Tetralogy of Fallot: A MRI Validation Study.

Paolino A, Hussain T, Pavon A, Velasco MN, Uribe S, Ordoñez A, Valverde I.

Pediatr Cardiol. 2016 Nov 21. [Epub ahead of print]

  1. Cardiac rehabilitation in an adolescent with Digeorge syndrome: a case report.

Kim DJ, Lee KY, Choe Y, Han JY, Choi IS.

Eur J Phys Rehabil Med. 2016 Nov 18. [Epub ahead of print]

  1. Congenital Variants and Anomalies of the Aortic Arch.

Hanneman K, Newman B, Chan F.

Radiographics. 2016 Nov 18:160033. [Epub ahead of print]

  1. Reducing variation in feeding newborns with congenital heart disease.

Simsic JM, Carpenito KR, Kirchner K, Peters S, Miller-Tate H, Joy B, Galantowicz M.

Congenit Heart Dis. 2016 Nov 16. doi: 10.1111/chd.12435. [Epub ahead of print]

  1. The not so innocent heart murmur: A five year experience.

Kueh SH, Pasley T, Wheeler M, Pemberton J.

Intern Med J. 2016 Nov 16. doi: 10.1111/imj.13331. [Epub ahead of print]

  1. The Use of Nesiritide in Children With Congenital Heart Disease.

Bronicki RA, Domico M, Checchia PA, Kennedy CE, Akcan-Arikan A.

Pediatr Crit Care Med. 2016 Nov 15. [Epub ahead of print]

  1. Analysis of Serum Metabolites to Diagnose Bicuspid Aortic Valve.

Wang W, Maimaiti A, Zhao Y, Zhang L, Tao H, Nian H, Xia L, Kong B, Wang C, Liu M, Wei L.

Sci Rep. 2016 Nov 15;6:37023. doi: 10.1038/srep37023.

  1. Home Exercise Training in Children and Adolescents with Pulmonary Arterial Hypertension: A Pilot Study.

Zöller D, Siaplaouras J, Apitz A, Bride P, Kaestner M, Latus H, Schranz D, Apitz C.

Pediatr Cardiol. 2016 Nov 14. [Epub ahead of print]

  1. Prevalence and pattern of executive dysfunction in school age children with congenital heart disease.

Sanz JH, Berl MM, Armour AC, Wang J, Cheng YI, Donofrio MT.

Congenit Heart Dis. 2016 Nov 11. doi: 10.1111/chd.12427. [Epub ahead of print]

  1. Accelerated ferumoxytol-enhanced 4D multiphase, steady-state imaging with contrast enhancement (MUSIC) cardiovascular MRI: validation in pediatric congenital heart disease.

Zhou Z, Han F, Rapacchi S, Nguyen KL, Brunengraber DZ, Kim GJ, Finn JP, Hu P.

NMR Biomed. 2016 Nov 11. doi: 10.1002/nbm.3663. [Epub ahead of print]

  1. Circulating microRNA as a Novel Biomarker for Pulmonary Arterial Hypertension Due to Congenital Heart Disease.

Chen W, Li S.

Pediatr Cardiol. 2016 Nov 11. [Epub ahead of print]

  1. Utility and Scope of Rapid Prototyping in Patients with Complex Muscular Ventricular Septal Defects or Double-Outlet Right Ventricle: Does it Alter Management Decisions?

Bhatla P, Tretter JT, Ludomirsky A, Argilla M, Latson LA Jr, Chakravarti S, Barker PC, Yoo SJ, McElhinney DB, Wake N, Mosca RS.

Pediatr Cardiol. 2016 Nov 11. [Epub ahead of print]

  1. Pentraxin 3 in neonates with and without diagnosis of pulmonary hypertension.

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

Clin Biochem. 2016 Nov 10. pii: S0009-9120(16)30528-8. doi: 10.1016/j.clinbiochem.2016.11.009. [Epub ahead of print]

  1. Anomalous left brachiocephalic vein: important vascular anomaly concomitant with congenital anomalies and heart diseases.

Kahkouee S, Sadr M, Pedarzadeh E, Fardin S, Borhani A, Gholami S, Amjad G.

Folia Morphol (Warsz). 2016 Nov 10. doi: 10.5603/FM.a2016.0031. [Epub ahead of print]

  1. A novel, more efficient, staged approach for critical congenital heart disease screening.

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

J Perinatol. 2016 Nov 10. doi: 10.1038/jp.2016.204. [Epub ahead of print]

  1. Early pulmonary arterial hypertension immediately after closure of a ventricular or complete atrioventricular septal defect beyond 6months of age.

Bambul Heck P, Eicken A, Kasnar-Samprec J, Ewert P, Hager A.

Int J Cardiol. 2016 Nov 9;228:313-318. doi: 10.1016/j.ijcard.2016.11.056. [Epub ahead of print]

  1. Three-dimensional Echocardiography in Congenital Heart Disease: An Expert Consensus Document from the European Association of Cardiovascular Imaging and the American Society of Echocardiography.

Simpson J, Lopez L, Acar P, Friedberg MK, Khoo NS, Helen Ko H, Marek J, Marx G, McGhie JS, Meijboom F, Roberson D, Van den Bosch A, Miller O, Shirali G.

J Am Soc Echocardiogr. 2016 Nov 9. pii: S0894-7317(16)30431-X. doi: 10.1016/j.echo.2016.08.022. [Epub ahead of print]

  1. White Matter Volume Predicts Language Development in Congenital Heart Disease.

Rollins CK, Asaro LA, Akhondi-Asl A, Kussman BD, Rivkin MJ, Bellinger DC, Warfield SK, Wypij D, Newburger JW, Soul JS.

J Pediatr. 2016 Nov 9. pii: S0022-3476(16)31054-X. doi: 10.1016/j.jpeds.2016.09.070. [Epub ahead of print]

  1. Appropriateness and diagnostic yield of inpatient pediatric echocardiograms.

Lang SM, Bolin E, Daily JA, Tang X, Thomas Collins R 2nd.

Congenit Heart Dis. 2016 Nov 8. doi: 10.1111/chd.12428. [Epub ahead of print]

  1. Expanding the clinical spectrum of chromosome 15q26 terminal deletions associated with IGF-1 resistance.

O’Riordan AM, McGrath N, Sharif F, Murphy NP, Franklin O, Lynch SA, O’Grady MJ.

Eur J Pediatr. 2016 Nov 8. [Epub ahead of print]

  1. African-American:White Disparity in Infant Mortality due to Congenital Heart Disease.

Collins JW Jr, Soskolne G, Rankin KM, Ibrahim A, Matoba N.

J Pediatr. 2016 Nov 8. pii: S0022-3476(16)31091-5. doi: 10.1016/j.jpeds.2016.10.023. [Epub ahead of print]

  1. Feasibility and Accuracy of Cardiac Right-to-Left-Shunt Detection in Children by New Transpulmonary Ultrasound Dilution Method.

Boehne M, Baustert M, Paetzel V, Boethig D, Köditz H, Dennhardt N, Beerbaum P, Bertram H.

Pediatr Cardiol. 2016 Nov 8. [Epub ahead of print]

  1. Evaluation of predictive models for six minute walk test among children with pulmonary hypertension.

Patel SS, Fernie JC, Taylor AL, Ivy DD, Rausch CM.

Int J Cardiol. 2016 Nov 7. pii: S0167-5273(16)33488-X. doi: 10.1016/j.ijcard.2016.11.042. [Epub ahead of print]

  1. Congenital Heart Disease in Premature Infants 25-32 Weeks’ Gestational Age.

Chu PY, Li JS, Kosinski AS, Hornik CP, Hill KD.

J Pediatr. 2016 Nov 3. pii: S0022-3476(16)31101-5. doi: 10.1016/j.jpeds.2016.10.033. [Epub ahead of print]

  1. Biomarkers of Myocardial Injury in Congenital Heart Disease: More Questions than Answers.

Chen CA.

Pediatr Neonatol. 2016 Nov 3. pii: S1875-9572(16)30248-0. doi: 10.1016/j.pedneo.2016.10.001. [Epub ahead of print] No abstract available.

  1. A Very Rare Case of Co-Existence of Cor Triatriatum Sinister and Left Pulmonary Vein Atresia.

Aparci M, Yalcin M, Isilak Z, Dogan M, Kardesoglu E.

Acta Cardiol Sin. 2016 Nov;32(6):758-761.

  1. Pulse oximetry could significantly enhance the early detection of critical congenital heart disease in neonatal intensive care units.

Hu XJ, Zhao QM, Ma XJ, Yan WL, Ge XL, Jia B, Liu F, Wu L, Ye M, Huang GY.

Acta Paediatr. 2016 Nov;105(11):e499-e505. doi: 10.1111/apa.13553.

  1. Atypical presentation of infantile-onset farber disease with novel ASAH1 mutations.

Kim SY, Choi SA, Lee S, Lee JS, Hong CR, Lim BC, Kang HJ, Kim KJ, Park SH, Choi M, Chae JH.

Am J Med Genet A. 2016 Nov;170(11):3023-3027. doi: 10.1002/ajmg.a.37846.

  1. Adiposity and Cardiovascular Risk Factor Variables in Childhood Are Associated With Premature Death From Coronary Heart Disease in Adults: The Bogalusa Heart Study.

Berenson GS, Srinivasan SR, Xu JH, Chen W.

Am J Med Sci. 2016 Nov;352(5):448-454. doi: 10.1016/j.amjms.2016.08.006.

  1. Genealogical and molecular analysis of a family-based cohort of congenital heart disease patients from the São Miguel Island (Azores, Portugal).

Cabral R, Pires R, Anjos R, Branco CC, Maciel P, Mota-Vieira L.

Ann Hum Biol. 2016 Nov;43(6):547-553.

  1. Geographic distribution of live births with tetralogy of Fallot in North Carolina 2003 to 2012.

Nelson JS, Stebbins RC, Strassle PD, Meyer RE.

Birth Defects Res A Clin Mol Teratol. 2016 Nov;106(11):881-887. doi: 10.1002/bdra.23566.

  1. Surveillance of ventricular septal defects in Delaware.

Acheson A, Vaidy A, Stomieroski K, Thompson DR, Maiden KM, Ehrenthal DB, Yezdani S, Bhat AM, Locke R, Bartoshesky LE.

Birth Defects Res A Clin Mol Teratol. 2016 Nov;106(11):888-893. doi: 10.1002/bdra.23574.

  1. Current Clinical Applications of Three-Dimensional Echocardiography: When the Technique Makes the Difference.

Surkova E, Muraru D, Aruta P, Romeo G, Bidviene J, Cherata D, Badano LP.

Curr Cardiol Rep. 2016 Nov;18(11):109. doi: 10.1007/s11886-016-0787-9. Review.

  1. Prospective ECG-gated high-pitch dual-source cardiac CT angiography in the diagnosis of congenital cardiovascular abnormalities: Radiation dose and diagnostic efficacy in a pediatric population.

Koplay M, Kizilca O, Cimen D, Sivri M, Erdogan H, Guvenc O, Oc M, Oran B.

Diagn Interv Imaging. 2016 Nov;97(11):1141-1150. doi: 10.1016/j.diii.2016.03.014.

  1. Best practice critical cardiac care in the neonatal unit.

Rigby ML.

Early Hum Dev. 2016 Nov;102:5-11. doi: 10.1016/j.earlhumdev.2016.09.003. Review.

  1. Sildenafil, pulmonary hypertension and bronchopulmonary dysplasia.

Herbert S, Tulloh R.

Early Hum Dev. 2016 Nov;102:21-24. doi: 10.1016/j.earlhumdev.2016.09.009.

  1. ANKS3 is mutated in a family with autosomal recessive laterality defect.

Shamseldin HE, Yakulov TA, Hashem A, Walz G, Alkuraya FS.

Hum Genet. 2016 Nov;135(11):1233-1239.

  1. Rare congenital mitral valve malformations assessed by real-time three-dimensional echocardiography.

Sun F, Chen Y, Huang L, Ren W, Yu X, Ni C.

Int J Cardiol. 2016 Nov 1;222:1027-30. doi: 10.1016/j.ijcard.2016.08.078. No abstract available.

  1. Giant dissecting ventricular septal haematoma associated with critical congenital heart disease.

Suteu CC, Muntean I, Benedek T, Togănel R.

Interact Cardiovasc Thorac Surg. 2016 Nov;23(5):837-838.

  1. Pediatric Echocardiography by Work Relative Value Units: Is Study Complexity Adequately Captured?

Balasubramanian S, Kipps AK, Smith SN, Tacy TA, Selamet Tierney ES.

J Am Soc Echocardiogr. 2016 Nov;29(11):1084-1091. doi: 10.1016/j.echo.2016.05.015.

  1. Infantile (Preductal) Coarctation of Aorta Presenting as Cerebellar Infarct – A Rare Presentation.

Sivaraman A, Kumhar M, Sahu UK, Mali MK.

J Assoc Physicians India. 2016 Nov;64(11):93-94.

  1. Challenges to success in heart failure: Cardiac cell therapies in patients with heart diseases.

Oh H, Ito H, Sano S.

J Cardiol. 2016 Nov;68(5):361-367. doi: 10.1016/j.jjcc.2016.04.010. Review.

  1. Birth defects data from surveillance hospitals in Dalian city, China, 2006-2010.

Liu QG, Sun J, Xiao XW, Song GR.

J Matern Fetal Neonatal Med. 2016 Nov;29(22):3615-21. doi: 10.3109/14767058.2016.1140136.

  1. Brain Dysplasia Associated with Ciliary Dysfunction in Infants with Congenital Heart Disease.

Panigrahy A, Lee V, Ceschin R, Zuccoli G, Beluk N, Khalifa O, Votava-Smith JK, DeBrunner M, Munoz R, Domnina Y, Morell V, Wearden P, Sanchez De Toledo J, Devine W, Zahid M, Lo CW.

J Pediatr. 2016 Nov;178:141-148.e1. doi: 10.1016/j.jpeds.2016.07.041.

  1. Supporting Vulnerable Children after Life-Threatening Neonatal Illness: Opportunities for Improving Outcomes.

Sobotka SA, Msall ME.

J Pediatr. 2016 Nov;178:12-14. doi: 10.1016/j.jpeds.2016.07.037. No abstract available.

  1. School-Age Test Proficiency and Special Education After Congenital Heart Disease Surgery in Infancy.

Mulkey SB, Bai S, Luo C, Cleavenger JE, Gibson N, Holland G, Mosley BS, Kaiser JR, Bhutta AT.

J Pediatr. 2016 Nov;178:47-54.e1. doi: 10.1016/j.jpeds.2016.06.063.

  1. Postoperative Amplitude-Integrated Electroencephalography Predicts Four-Year Neurodevelopmental Outcome in Children with Complex Congenital Heart Disease.

Latal B, Wohlrab G, Brotschi B, Beck I, Knirsch W, Bernet V.

J Pediatr. 2016 Nov;178:55-60.e1. doi: 10.1016/j.jpeds.2016.06.050.

  1. Creating Opportunities for Optimal Nutritional Experiences for Infants With Complex Congenital Heart Disease.

Steltzer MM, Sussman-Karten K, Kuzdeba HB, Mott S, Connor JA.

J Pediatr Health Care. 2016 Nov – Dec;30(6):599-605. doi: 10.1016/j.pedhc.2016.08.002.

  1. The importance of social media for patients and families affected by congenital anomalies: A Facebook cross-sectional analysis and user survey.

Jacobs R, Boyd L, Brennan K, Sinha CK, Giuliani S.

J Pediatr Surg. 2016 Nov;51(11):1766-1771. doi: 10.1016/j.jpedsurg.2016.07.008.

  1. Coronary-Pulmonary Artery Fistulas: A Systematic Review.

Verdini D, Vargas D, Kuo A, Ghoshhajra B, Kim P, Murillo H, Kirsch J, Lane M, Restrepo C.

J Thorac Imaging. 2016 Nov;31(6):380-390.

  1. Impact of postoperative duration of Aspirin use on longevity of bioprosthetic pulmonary valve in patients who underwent congenital heart disease repair.

Hwang TW, Kim SO, Lee SY, Kim SH, Choi EY, Jang SI, Park SJ, Kwon HW, Lim HB, Lee CH, Choi ES.

Korean J Pediatr. 2016 Nov;59(11):446-450.

  1. KinCor, a national registry for paediatric patients with congenital and other types of heart disease in the Netherlands: aims, design and interim results.

Silva LM, Kuipers IM, van den Heuvel F, Mendes R, Berger RM, van Beynum IM, Rozendaal L, Rammeloo LA, van Iperen GG, Schokking M, Frerich S, Blom NA, Breur JM, Helbing WA.

Neth Heart J. 2016 Nov;24(11):628-639.

  1. Improved Survival While Waiting and Risk Factors for Death in Pediatric Patients Listed for Cardiac Transplantation.

Zakaria D, Frazier E, Imamura M, Garcia X, Pye S, Knecht KR, Prodhan P, Gossett JR, Swearingen CJ, Morrow WR.

Pediatr Cardiol. 2016 Nov 1. [Epub ahead of print]

  1. The Utility of Brain Natriuretic Peptide in Pediatric Cardiology: A Review.

Neves AL, Henriques-Coelho T, Leite-Moreira A, Areias JC.

Pediatr Crit Care Med. 2016 Nov;17(11):e529-e538.

  1. Risk factors for pulmonary arterial hypertension in children and young adults.

Naumburg E, Söderström L, Huber D, Axelsson I.

Pediatr Pulmonol. 2016 Nov 1. doi: 10.1002/ppul.23633. [Epub ahead of print]

  1. Effect of the forward-projected model-based iterative reconstruction solution algorithm on image quality and radiation dose in pediatric cardiac computed tomography.

Nishiyama Y, Tada K, Nishiyama Y, Mori H, Maruyama M, Katsube T, Yamamoto N, Kanayama H, Yamamoto Y, Kitagaki H.

Pediatr Radiol. 2016 Nov;46(12):1663-1670.

  1. Delayed cortical gray matter development in neonates with severe congenital heart disease.

Claessens NH, Moeskops P, Buchmann A, Latal B, Knirsch W, Scheer I, Išgum I, de Vries LS, Benders MJ, von Rhein M.

Pediatr Res. 2016 Nov;80(5):668-674. doi: 10.1038/pr.2016.145.

  1. Assessment of cardiac function in absence of congenital and acquired heart disease in patients with Down syndrome.

Balli S, Yucel IK, Kibar AE, Ece I, Dalkiran ES, Candan S.

World J Pediatr. 2016 Nov;12(4):463-469.

  1. Intact Imaging of Human Heart Structure Using X-ray Phase-Contrast Tomography.

Kaneko Y, Shinohara G, Hoshino M, Morishita H, Morita K, Oshima Y, Takahashi M, Yagi N, Okita Y, Tsukube T.

Pediatr Cardiol. 2016 Nov 30. [Epub ahead of print]

  1. Left Ventricular Function in Healthy Term Neonates During the Transitional Period.

Jain A, El-Khuffash AF, Kuipers BC, Mohamed A, Connelly KA, McNamara PJ, Jankov RP, Mertens L.

J Pediatr. 2016 Nov 28. pii: S0022-3476(16)31231-8. doi: 10.1016/j.jpeds.2016.11.003. [Epub ahead of print]

  1. Assessment of copy number variations in 120 patients with Poland syndrome.

Vaccari CM, Tassano E, Torre M, Gimelli S, Divizia MT, Romanini MV, Bossi S, Musante I, Valle M, Senes F, Catena N, Bedeschi MF, Baban A, Calevo MG, Acquaviva M, Lerone M, Ravazzolo R, Puliti A.

BMC Med Genet. 2016 Nov 25;17(1):89.

  1. Morphological Assessment of Single-Ventricle Atrioventricular Valve Regurgitation on Dual-Source 128-Slice Multidetector Computed Tomography and 4-Dimensional Imaging.

Kodama Y, Nakamura M, Sagawa K, Ishikawa S, Nakano T, Kado H.

Circ J. 2016 Nov 25;80(12):2555-2556. No abstract available.

  1. Limited Ventricular Preload is the Main Reason for Reduced Stress Reserve After Atrial Baffle Repair.

Eicken A, Michel J, Hager A, Tanase D, Kaemmerer H, Cleuziou J, Hess J, Ewert P.

Pediatr Cardiol. 2016 Nov 24. [Epub ahead of print]

  1. Finding Harmony between Science and Art in Pediatric Cardiology: Acknowledging When Being “Objective” May Not Truly Be Objective.

Loomba RS.

Children (Basel). 2016 Nov 23;3(4). pii: E37.

  1. Oscillometric and auscultatory blood pressure measurement methods in children: a systematic review and meta-analysis.

Duncombe SL, Voss C, Harris KC.

J Hypertens. 2016 Nov 23. [Epub ahead of print]

  1. Difference in Risk Factors for Subtypes of Acute Cardiac Lesions Resulting from Kawasaki Disease.

Yamashita M, Ae R, Yashiro M, Aoyama Y, Sano T, Makino N, Nakamura Y.

Pediatr Cardiol. 2016 Nov 23. [Epub ahead of print]

  1. Global Deformation Parameters Response to Exercise in Adolescents with Repaired Tetralogy of Fallot.

Mese T, Guven B, Yilmazer MM, Demirol M, Çoban Ş, Karadeniz C.

Pediatr Cardiol. 2016 Nov 23. [Epub ahead of print]

  1. Gender dimorphism in pediatric OSA: Is it for real?

Brockmann PE, Koren D, Kheirandish-Gozal L, Gozal D.

Respir Physiol Neurobiol. 2016 Nov 23. pii: S1569-9048(16)30223-3. doi: 10.1016/j.resp.2016.11.010. [Epub ahead of print] Review.

  1. Genetic Variation in the SLC8A1 Calcium Signaling Pathway Is Associated with Susceptibility to Kawasaki Disease and Coronary Artery Abnormalities.

Shimizu C, Eleftherohorinou H, Wright VJ, Kim J, Alphonse MP, Perry JC, Cimaz R, Burgner D, Dahdah N, Hoang LT, Khor CC, Salgado A, Tremoulet AH, Davila S, Kuijpers TW, Hibberd ML, Johnson TA, Takahashi A, Tsunoda T, Kubo M, Tanaka T, Onouchi Y, Yeung RS, Coin LJ, Levin M, Burns JC.

Circ Cardiovasc Genet. 2016 Nov 21. pii: CIRCGENETICS.116.001533. [Epub ahead of print]

  1. Experimental, Systems and Computational Approaches to Understanding the MicroRNA-Mediated Reparative Potential of Cardiac Progenitor Cell-Derived Exosomes From Pediatric Patients.

Agarwal U, George A, Bhutani S, Ghosh-Choudhary S, Maxwell JT, Brown ME, Mehta Y, Platt MO, Liang Y, Sahoo S, Davis ME.

Circ Res. 2016 Nov 21. pii: CIRCRESAHA.116.309935. [Epub ahead of print]

  1. Transcriptional Analysis of Intravenous Immunoglobulin Resistance in Kawasaki Disease Using an Induced Pluripotent Stem Cell Disease Model.

Ikeda K, Mizoro Y, Ameku T, Nomiya Y, Mae SI, Matsui S, Kuchitsu Y, Suzuki C, Hamaoka-Okamoto A, Yahata T, Sone M, Okita K, Watanabe A, Osafune K, Hamaoka K.

Circ J. 2016 Nov 19. [Epub ahead of print]

  1. Intellectual Functioning in Children with Congenital Heart Defects Treated with Surgery or by Catheter Interventions.

Ryberg C, Sunnegårdh J, Thorson M, Broberg M.

Front Pediatr. 2016 Nov 17;4:113.

  1. Prevalence of congenital coronary artery anomalies as shown by multi-slice computed tomography coronary angiography: a single-centre study from Turkey.

Tongut A, Özyedek Z, Çerezci İ, Erentürk S, Hatemi AC.

J Int Med Res. 2016 Nov 17. pii: 0300060516667118. [Epub ahead of print]

  1. Effect of Release of the First Pediatric Appropriate Use Criteria on Transthoracic Echocardiogram Ordering Practice.

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

Am J Cardiol. 2016 Nov 15;118(10):1545-1551. doi: 10.1016/j.amjcard.2016.08.019.

  1. Hepatoma-derived Growth Factor Predicts Disease Severity and Survival in Pulmonary Arterial Hypertension.

Yang J, Nies MK, Fu Z, Damico R, Korley FK, Hassoun PM, Ivy DD, Austin ED, Everett AD.

Am J Respir Crit Care Med. 2016 Nov 15;194(10):1264-1272.

  1. Corrigendum to “FUTURE-2: Results from an open-label, long-term safety and tolerability extension study using the pediatric FormUlation of bosenTan in pUlmonary arterial hypeRtEnsion” [Int. J. Cardiol. 202 (2016) 52-58].

Berger RM, Haworth SG, Bonnet D, Dulac Y, Fraisse A, Galiè N, Ivy DD, Jaïs X, Miera O, Rosenzweig EB, Efficace M, Kusic-Pajic A, Beghetti M.

Int J Cardiol. 2016 Nov 15;223:1072-1073. doi: 10.1016/j.ijcard.2016.08.333. No abstract available.

  1. Ebstein’s anomaly and aortic arch lesions: When right meets left.

Geerdink LM, Ter Heide H, Kapusta L.

Int J Cardiol. 2016 Nov 15;223:568-570. doi: 10.1016/j.ijcard.2016.08.181. No abstract available.

  1. A singular case of non-obstructive supracardiac total anomalous pulmonary venous connection with two vertical veins in a 30weeks preterm neonate.

Figueras-Coll M, Sabaté-Rotés A, Cañete-Abajo N, Abella RF.

Int J Cardiol. 2016 Nov 15;223:50-51. doi: 10.1016/j.ijcard.2016.08.047. No abstract available.

  1. A rare cause of cardiomyopathy in an infant: middle aortic syndrome.

Mir A, Stam B, Sperrazza C.

Cardiol Young. 2016 Nov 14:1-3. [Epub ahead of print]

  1. Impact of sickle cell anaemia on cardiac chamber size in the paediatric population.

Adjagba PM, Habib G, Robitaille N, Pastore Y, Raboisson MJ, Curnier D, Dahdah N.

Cardiol Young. 2016 Nov 14:1-7. [Epub ahead of print]

  1. Accelerated ferumoxytol-enhanced 4D multiphase, steady-state imaging with contrast enhancement (MUSIC) cardiovascular MRI: validation in pediatric congenital heart disease.

Zhou Z, Han F, Rapacchi S, Nguyen KL, Brunengraber DZ, Kim GJ, Finn JP, Hu P.

NMR Biomed. 2016 Nov 11. doi: 10.1002/nbm.3663. [Epub ahead of print]

  1. Circulating microRNA as a Novel Biomarker for Pulmonary Arterial Hypertension Due to Congenital Heart Disease.

Chen W, Li S.

Pediatr Cardiol. 2016 Nov 11. [Epub ahead of print]

  1. Longitudinal Validation of the Diastolic to Systolic Time-Velocity Integral Ratio as a Doppler-Derived Measure of Pulmonary Regurgitation in Patients with Repaired Tetralogy of Fallot.

Bhat M, Goldmuntz E, Fogel MA, Rychik J, Mercer-Rosa L.

Pediatr Cardiol. 2016 Nov 11. [Epub ahead of print]

  1. Evaluation of Residual Coarctation in Infants with a Single Right Ventricle after Stage I Palliation.

Fundora MP, Sasaki J, Muniz JC, Rossi A, Rhodes JF Jr, Hannan RL, Burke RP, Lopez L.

Pediatr Cardiol. 2016 Nov 11. [Epub ahead of print]

  1. Galectin-3 in Children with Chronic Heart Failure with Normal and Reduced Ejection Fraction: Relationship to Disease Severity.

Kotby AA, Youssef OI, Elmaraghy MO, El Sharkawy OS.

Pediatr Cardiol. 2016 Nov 11. [Epub ahead of print]

  1. Influence of apical position on the left ventricular outflow tract obstruction in congenitally corrected transposition.

Lee ML, Chiu IS.

J Cardiol. 2016 Nov 10. pii: S0914-5087(16)30173-3. doi: 10.1016/j.jjcc.2016.07.019. [Epub ahead of print]

  1. Late Wall Thickening and Calcification in Patients After Kawasaki Disease.

Tsujii N, Tsuda E, Kanzaki S, Ishizuka J, Nakashima K, Kurosaki K.

J Pediatr. 2016 Nov 10. pii: S0022-3476(16)31094-0. doi: 10.1016/j.jpeds.2016.10.026. [Epub ahead of print]

  1. Neurologic, Neurocognitive and Functional Outcomes in Children Under 6 Years Treated with The Berlin Heart Excor Ventricular Assist Device.

VanderPluym JH, Robertson CM, Joffe AR, Conway J, Rebeyka IM, Ross DB, Guerra GG, Al Aklabi MM, Buchholz H.

ASAIO J. 2016 Nov 9. [Epub ahead of print]

  1. Usefulness of soluble urokinase plasminogen activator receptor (suPAR) as an inflammatory biomarker in obese children.

Kosecik M, Dervisoglu P, Koroglu M, Isguven P, Elmas B, Demiray T, Altindis M.

Int J Cardiol. 2016 Nov 9;228:158-161. doi: 10.1016/j.ijcard.2016.11.201. [Epub ahead of print]

  1. Appropriateness and diagnostic yield of inpatient pediatric echocardiograms.

Lang SM, Bolin E, Daily JA, Tang X, Thomas Collins R 2nd.

Congenit Heart Dis. 2016 Nov 8. doi: 10.1111/chd.12428. [Epub ahead of print]

  1. Safety of Enalapril in Infants Admitted to the Neonatal Intensive Care Unit.

Ku LC, Zimmerman K, Benjamin DK, Clark RH, Hornik CP, Smith PB; Best Pharmaceuticals for Children Act – Pediatric Trials Network Steering Committee..

Pediatr Cardiol. 2016 Nov 8. [Epub ahead of print]

  1. The Action of Smooth Muscle Cell Potassium Channels in the Pathology of Pulmonary Arterial Hypertension.

Hayabuchi Y.

Pediatr Cardiol. 2016 Nov 8. [Epub ahead of print] Review.

  1. Diagnostic Yield of Outpatient Pediatric Echocardiograms: Impact of Indications and Specialty.

Lang SM, Bolin E, Hardy S, Tang X, Collins RT 2nd.

Pediatr Cardiol. 2016 Nov 8. [Epub ahead of print]

  1. Right aortic arch with isolation of the left subclavian artery: a rare association with airway obstruction.

Yubbu P, Latiff HA, Adam Abbaker AM.

Cardiol Young. 2016 Nov 7:1-4. [Epub ahead of print]

  1. Assessment of early atherosclerosis and left ventricular dysfunction in children with 21-hydroxylase deficiency.

Özdemir R, Korkmaz HA, Küçük M, Karadeniz C, Meşe T, Özkan B.

Clin Endocrinol (Oxf). 2016 Nov 7. doi: 10.1111/cen.13275. [Epub ahead of print]

  1. Racial Differences in Aortic Stiffness in Children.

Lefferts WK, Augustine JA, Spartano NL, Atallah-Yunes NH, Heffernan KS, Gump BB.

J Pediatr. 2016 Nov 3. pii: S0022-3476(16)31055-1. doi: 10.1016/j.jpeds.2016.09.071. [Epub ahead of print]

  1. Echocardiographic Follow-Up of Patent Foramen Ovale and the Factors Affecting Spontaneous Closure.

Yildirim A, Aydin A, Demir T, Aydin F, Ucar B, Kilic Z.

Acta Cardiol Sin. 2016 Nov;32(6):731-737.

  1. Student perceptions of a video-based blended learning approach for improving pediatric physical examination skills.

Lehmann R, Seitz A, Bosse HM, Lutz T, Huwendiek S.

Ann Anat. 2016 Nov;208:179-182. doi: 10.1016/j.aanat.2016.05.009.

  1. Characteristics and outcomes of Indian children enrolled in a rheumatic heart disease registry.

Mehta A, Saxena A, Juneja R, Ramakrishnan S, Gupta S, Kothari SS.

Int J Cardiol. 2016 Nov 1;222:1136-40. doi: 10.1016/j.ijcard.2016.08.259.

  1. Inflammatory myofibroblastic tumor of the left atrium in infant.

Bao M, Zheng C, Zhang H, Ruan Y, Cao A, Wu T, Luo YI.

Int J Cardiol. 2016 Nov 1;222:965-7. doi: 10.1016/j.ijcard.2016.08.029. No abstract available.

  1. Pediatric Echocardiography by Work Relative Value Units: Is Study Complexity Adequately Captured?

Balasubramanian S, Kipps AK, Smith SN, Tacy TA, Selamet Tierney ES.

J Am Soc Echocardiogr. 2016 Nov;29(11):1084-1091. doi: 10.1016/j.echo.2016.05.015.

  1. Cardiovascular magnetic resonance is successfully feasible in many patients aged 3 to 8years without general anesthesia or sedation.

Kharabish A, Mkrtchyan N, Meierhofer C, Martinoff S, Ewert P, Stern H, Fratz S.

J Clin Anesth. 2016 Nov;34:11-4. doi: 10.1016/j.jclinane.2016.02.048.

  1. Disease control via intensified lipoprotein apheresis in three siblings with familial hypercholesterolemia.

Taylan C, Schlune A, Meissner T, Ažukaitis K, Udink Ten Cate FE, Weber LT.

J Clin Lipidol. 2016 Nov – Dec;10(6):1303-1310. doi: 10.1016/j.jacl.2016.08.006.

  1. Correlates of Achieving Statin Therapy Goals in Children and Adolescents with Dyslipidemia.

Mendelson MM, Regh T, Chan J, Baker A, Ryan HH, Palumbo N, Johnson PK, Griggs S, Boghani M, Desai NK, Yellen E, Buckley L, Gillman MW, Zachariah JP, Graham D, de Ferranti SD.

J Pediatr. 2016 Nov;178:149-155.e9. doi: 10.1016/j.jpeds.2016.08.003.

  1. Referral Patterns and Cascade Screening for Familial Hypercholesterolemia in a Pediatric Lipid Clinic.

Stempel H, Dodge A, Marriott E, Peterson AL.

J Pediatr. 2016 Nov;178:285-287. doi: 10.1016/j.jpeds.2016.08.016.

  1. Hypercalcemia in Patients with Williams-Beuren Syndrome.

Sindhar S, Lugo M, Levin MD, Danback JR, Brink BD, Yu E, Dietzen DJ, Clark AL, Purgert CA, Waxler JL, Elder RW, Pober BR, Kozel BA.

J Pediatr. 2016 Nov;178:254-260.e4. doi: 10.1016/j.jpeds.2016.08.027.

  1. Parameters indicative of persistence of valvular pathology at initial diagnosis in acute rheumatic carditis: the role of albumin and CD19 expression.

Oner T, Ozdemir R, Genc DB, Kucuk M, Karadeniz C, Demirpence S, Yilmazer MM, Mese T, Tavli V, Genel F.

J Pediatr (Rio J). 2016 Nov – Dec;92(6):581-587. doi: 10.1016/j.jped.2016.01.013.

  1. The state of point-of-care ultrasonography use and training in neonatal-perinatal medicine and pediatric critical care medicine fellowship programs.

Nguyen J, Amirnovin R, Ramanathan R, Noori S.

J Perinatol. 2016 Nov;36(11):972-976. doi: 10.1038/jp.2016.126.

  1. Decision analysis to define the optimal management of athletes with anomalous aortic origin of a coronary artery.

Mery CM, Lopez KN, Molossi S, Sexson-Tejtel SK, Krishnamurthy R, McKenzie ED, Fraser CD Jr, Cantor SB.

J Thorac Cardiovasc Surg. 2016 Nov;152(5):1366-1375.e7. doi: 10.1016/j.jtcvs.2016.07.076.

  1. Near-infrared spectroscopy for detection of a significant patent ductus arteriosus.

Chock VY, Rose LA, Mante JV, Punn R.

Pediatr Res. 2016 Nov;80(5):675-680. doi: 10.1038/pr.2016.148.

  1. New-onset diabetes mellitus after heart transplantation in children – Incidence and risk factors.

Sehgal S, Bock MJ, Louks Palac H, Brickman WJ, Gossett JG, Marino BS, Backer CL, Pahl E.

Pediatr Transplant. 2016 Nov;20(7):963-969. doi: 10.1111/petr.12759.

  1. Isolated Subclavian Artery: A Rare Entity Revisited.

Sen S, Mohanty S, Kulkarni S, Rao SG.

World J Pediatr Congenit Heart Surg. 2016 Nov;7(6):744-749.

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ACHD Featured Articles of November 2016

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

Müller J, Heck PB, Ewert P, Hager A.

Ann Thorac Surg. 2016 Nov 30. pii: S0003-4975(16)31290-5. doi: 10.1016/j.athoracsur.2016.09.038. [Epub ahead of print]

Take Home Points:

  1. CHD-PAH is very unlikely in a patient with a VO2 Max >25.2.
  2. CHD-PAH is increasingly likely in a patient with a VO2 Max <16.3 , and a breathing reserve <37.4%.
  3. CPET is neither sensitive nor specific for the new diagnosis of CHD-PAH outside of the above mentioned parameters.

Moe_Tabitha-WEBCommentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch:  Pulmonary arterial hypertension may be suspected on routine transthoracic echocardiograms as a part of regular screening and follow up for patients with palliated congenital heart disease. There are multiple reasons why screening with TTE alone is limited – including complex anatomy, multiple prior sternotomies, limited echocardiographic acoustical windows, as well as increasing body mass index associated with body habitus.   This study evaluated 683 adult patients who had a cardiac catheterization within 6 months after a cardio-pulmonary exercise study.  The CPET protocol included a symptom limited CPET on a bicycle in the upright position together with preceding spirometry.  Breathing reserve was calculated by 100 x (maximum voluntary ventilation – VE peak)/maximum voluntary ventilation with VE indicating the amount of air moved in and out of the lungs/min. The 130 patients with proven pulmonary hypertension were compared with the 563 patients with CHD but no PAH.  The most discriminating variable was the peak VO2. Interestingly, those patients with CHD-PAH had lower peak heart rates, low peak systolic blood pressures, and a poor mean ventilatory efficiency (VE/VCO2 slope) of 38.2. However, none of these variables reached statistical significance.  There were two thresholds, the highest specificity of 95% of PAH was found in patients with a Peak VO2 of <16.3 mL/min/kg or less and a breathing reserve of 37.4% or less.  In patients with a peak VO2 exceeding 16.3 there was a high specificity but a low sensitivity of 53.1%.  The cutoff of 25.2 mL/min/kg renders a sensitivity for PAH of only 10%.  The detection of PAH is patients with CHD by CPET is neither sensitive nor specific unless the VO2 was <16.3 and the breathing reserve was <37.4%.  Although a noninvasive study in addition to echo findings would be a valuable confirmatory testing modality, from this study it would appear that CPET with this protocol is not the discriminator test that we are looking for. However, this is a retrospective study of clinical data, and a randomized, protocol driven study for the identification of early indicators of the development of CHD-PAH may be warranted.

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Plasma Proteomic Study in Pulmonary Arterial Hypertension Associated with Congenital Heart Diseases.

Zhang X, Hou HT, Wang J, Liu XC, Yang Q, He GW.

Sci Rep. 2016 Nov 25;6:36541. doi: 10.1038/srep36541.

Take-Home Points:

  1. Translational medicine in PAH-CHD has two new investigational targets.
  2. Gene ontology found decreased levels of carbamoyl-phosphate synthetase I (CPSI.
  3. Complement factor H-related protein 2 (CFHR2) is also decreased in plasma.

Commentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch:  This article is from the Tianjin, China team highlights the need to understand personalized medicine in the 21st century.  They evaluated 272 young (average age in the pediatric age range, somewhat older in those patients with proven PAH for each category) CHD patients with or without PAH.  They included both patients with simple congenital heart disease with either a VSD or an ASD as well as somewhat more complicated defects with mixed disease which they defined as two or more defects including ASD, VSD, or PDA. Differential plasma proteins were detected by iTRAQ proteomic technology and those suspected to be of clinical significance were identified for further ELISA validation in a second cohort of 152 patients.  The factors identified by gene ontology included carbamoyl-phosphate synthetase I (CPSI, related to the urea cycle and endogenous nitric oxide production) and complement factor H-related protein 2 (CFHR2, related to activation of the complement system and the coagulation cascade). Both the CPSI and the CFHR2 were downregulated with decreased plasma levels. This identifies for the very first time that a large number of proteins are altered in the plasma of CHD-PAH patients, two distinct mechanisms linked to CHD-PAH patients which were not identified within the control cohort.  The decreased expression of CPSI in PAH-CHD patients may reveal a mechanism related to endogenous nitric oxide production, and the decrease of CFHR2 protein may demonstrate the deficiency of the immune system linked to the coagulation cascade.  These findings allow pilot data for translational medicine in CHD-PAH as it relates to the diagnosis and progress of the disease.

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From bosentan to macitentan for pulmonary arterial hypertension and adult congenital heart disease: Further improvement?

Blok IM, van Riel AC, van Dijk AP, Mulder BJ, Bouma BJ.

Int J Cardiol. 2016 Nov 9;227:51-52. doi: 10.1016/j.ijcard.2016.11.211. [Epub ahead of print] No abstract available.

Take-Home Points:

  1. Macitentan pharmacology with increased receptor binding, reduced dosing schedule (once-daily), and no need for routine hepatology screening labs has several benefits for a heterogenous PAH population.
  2. This small observational study suggests that several surrogate end points improve with the transition from bosentan to macitentan in a PAH-CHD cohort.
  3. The inclusion of Down syndrome and Eisenmenger patients in this study cohort allow for extrapolation of study data to the real-world practice of the care of our ACHD patients.

Commentary from Dr. Tabitha Moe (Phoenix), section editor of ACHD Journal Watch:  This study outlines the clinical outcomes of 40 patients with PAH-CHD who switched from bosentan to macitentan in the Netherlands.  Bosentan was proven through BREATHE-5 to be a reasonable options for the management of PAH-CHD, as it improves exercise capacity as well as quality of life.  Macitentan demonstrates improved receptor binding compared with bosentan as well as improved mortality and morbidity data in a heterogenous cohort of PAH.  This is a prospective observational study in an existing cohort of 43 PAH-CHD patients which included Down syndrome patients.  The median treatment duration on bosentan was 7.2 years, and 10 of the patients were on combination bosentan-sildenafil therapy.  After 6 months of follow up, the number of patients in WHO-FC III or IV decreased by 48%. Levels of NT-proBNP improved from 723 to 488.  TAPSE improved from 19 to 21.  There were fewer macitentan patients hospitalized for heart failure.  There were no adverse events in the cohort of patients who switched.  This study included patients with Eisenmenger syndrome, as well as Down syndrome patients.  Switch from bosentan to macitentan was associated with improvement of WHO-FC, NT-proBNP, and TAPSE even after several years on bosentan.  The findings suggest that PAH-CHD patients who are currently on bosentan therapy might benefit from a transition to macitentan accompanied by close and careful follow up at a congenital center.

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Survival and cardiovascular events after coarctation-repair in long-term follow-up (COAFU): Predictive value of clinical variables.

Bambul Heck P, Pabst von Ohain J, Kaemmerer H, Ewert P, Hager A.

Int J Cardiol. 2016 Nov 9;228:347-351. doi: 10.1016/j.ijcard.2016.11.164. [Epub ahead of print]

Take-Home Points:

  1. In patients with surgical repair of aortic coarctation, late mortality 31 years after aortic surgery was 5.7% at a median age of 41 years.
  2. The presence of bicuspid aortic valve increases the risk of morbidity and mortality.
  3. Additional predictors of major cardiovascular events include a postoperative brachial–ankle gradient difference greater than 20 mmHg and impaired left ventricle function.

jokhadarCommentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch:  Coarctation of the aorta is associated with a generalized arteriopathy, vasculopathy, and cerebrovascular disease and is strongly associated with hypertension and its associated complications.  Long-term outcomes in patients with repaired coarctation of the aorta appear to be influenced by the comorbid medical conditions, including bicuspid aortic valve and aortic complications.

Dr. Heck and colleagues from Germany conduct a long-term follow-up of 273 patients with isolated coarctation of the aorta who underwent surgical repair between 1974 and 2000.  These patients were initially enrolled in the COALA study (1) and were contacted for follow-up.  In the original COALA study, most patients were hypertensive and had reduced exercise capacity, which was not related to the surgical results.

The results show a 5.7% mortality during the 31 year follow-up.  Long-term survival rates are estimated at 94%, 91%, and 80% at 20, 30, and 39 years after operative repair, respectively.  Improvements in surgical and medical care, including anti-hypertensive therapy, appear to have contributed to improved survival, when compared with older studies.  Patients with repair prior to the age of 20 had better outcomes compared with those who had surgery at an older age.  The common late causes of death were coronary disease and heart failure.  Though mortality is relatively low, 18% of survivors had a major cardiovascular event during the past 14 years.  Associated bicuspid aortic valve was present and the majority of adverse events.  This is likely due to the association between bicuspid aortic valve and aortic valve disease as well as aortic root disease, including dilation and dissection.  A postoperative ankle-brachial gradient of greater than 20 mmHg was a predictor of major cardiovascular events.  One patient in this study had a stroke after cerebral aneurysm bleeding.

  1. Hager A, et al. Coarctation long-term assessment (COALA): Significant of arterial hypertension and a cord of 404 patient’s up to 27 years after surgical repair of isolated coarctation of the aorta, even in the absence of restenosis and prosthetic material. J Thorac. Cardiovasc. Surg.  134 (3) (2007) 738–745.

 

 

Aortopathy in adults with tetralogy of Fallot has a negative impact on the left ventricle.

Shiina Y, Murakami T, Kawamatsu N, Niwa K.

Int J Cardiol. 2016 Nov 14;228:380-384. doi: 10.1016/j.ijcard.2016.11.252. [Epub ahead of print]

Take-Home Point:

A dilated ascending aorta in tetralogy of Fallot is associated with reduced left ventricular function, particularly diastolic dysfunction.

Commentary from Dr. Maan Jokhadar (Atlanta), section editor of ACHD Journal Watch:  Aortic pressure wave reflection is usually physiologic and helps maintain coronary perfusion.  However, excessive aortic pressure wave reflection can increase cardiovascular events and is associated with arterial stiffness.  Aortic pressure wave reflection can be measured using a validated noninvasive method of radial augmentation index (rAI), which is obtained from the central arterial pressure waveform as the ratio of augmentation pressure to the total pulse pressure.

Dr. Shiina and colleagues from Japan enrolled 51 consecutive patients with repaired tetralogy of Fallot, excluding patients with a patent shunt or significant aortic valve regurgitation, as this would limit the ability to assess rAI.  Twenty-one patients had rAI greater or equal to 1 standard deviation (group A) and 30 patients had rAI less than 1 standard deviation (group B).  Left ventricle function was evaluated using echocardiography and cardiac MRI.

When compared to group B, group A larger adjusted ascending aorta diameters, diminished left ventricle global longitudinal strain, left ventricle global circumferential strain, left ventricle diastolic peak strain rate, lower E/A, left ventricle tissue Doppler velocities, and higher Tie index.  Left ventricle ejection fraction was similar in both groups.  Both groups were similar in age and comorbid medical conditions.  Group A had more angiotensin II receptor blocker and beta blocker use.

It is hypothesized that increased rAI and aortic stiffness can lead to further structural changes in the aortic wall and in turn, lead to aortic dilation.  Also, an interesting explanation for how aortopathy can worsen ventricular function is as the aorta increases in stiffness, afterload increases in systole, which increases ventricular hypertrophy, while at the same time coronary perfusion is decreased in diastole.

 

Prognostic value of multiple biomarkers for cardiovascular mortality in adult congenital heart disease: comparisons of single-/two-ventricle physiology, and systemic morphologically right/left ventricles.

Miyamoto K, Takeuchi D, Inai K, Shinohara T, Nakanishi T.

Heart Vessels. 2016 Nov;31(11):1834-1847.

Take-Home Points:

  1. Biomarkers, combined with NYHA functional class and cardiothoracic ratio on chest x-ray predicts heart failure related mortality in adults with congenital heart disease.
  2. The potential of biomarkers to predict mortality differs in single versus biventricular physiology and the morphology of the single ventricle.

Commentary from Dr. Leong Ming Chern (Kuala Lumpur), section editor of ACHD Journal Watch:  Inflammation has been implicated in the cause of heart failure in adults with congenital heart disease. Inflammatory biomarkers and their receptors, neurohormones and BNP may be used in prognosis of heart failure in adults with congenital heart disease (ACHD). The authors sought to investigate the relationship between biomarkers and clinical cardiovascular factors such as NYHA functional class and cardiothoracic ratio on chest xray (CTR); and to evaluate the profile of a series of clinical biomarkers for acute decompensated heart failure mortality risk in ACHD patients.

This is retrospective cohort study conducted at a single center. Between 2005 and 2009, 103 patients with ACHD who were admitted for cardiac evaluation or heart failure had their biomarkers taken and these patients were followed up from 2005 to 2013 for heart failure related mortality.

Twelve patients (11%) died of acute decompensated heart failure. Predictive biomarkers, which were related to higher NYHA class and CTR in all patients, were elevated levels of BNP, endothelin-1, soluble TNF- receptor type 1, Norepinephrine and Interleukin-6. The group demonstrated that elevated biomarkers are different in biventricular and single ventricular physiology. Similarly, single ventricle with morphological right and left ventricle demonstrated different biomarker profiles.

Although this is small study, it supports the findings of previous studies that clinical biomarkers have significant prognostic value in ACHD.

 

Serial cardiac MRIs in adult Fontan patients detect progressive hepatic enlargement and congestion.

Lewis MJ, Hecht E, Ginns J, Benton J, Prince M, Rosenbaum MS.

Congenit Heart Dis. 2016 Nov 28. doi: 10.1111/chd.12422. [Epub ahead of print]

Take-Home Points:

  1. The liver and spleen are in the field of view of routine cardiac MRI, and serial measurements of these structures can be made to assess markers of hepatic congestion after the Fontan.
  2. In a series of 27 adult Fontan patients, almost all patients with serial cardiac MRIs showed increase in size of liver and spleen across serial studies, regardless of type of Fontan or underlying anatomy.
  3. A systemic ejection fraction < 50% may be a risk factor for accelerated hepatic congestion.
  4. The only parameter to correlate with time from Fontan to cardiac MRI was the diameter of the right hepatic vein; other anatomic indices did not correlate with duration of Fontan physiology.
  5. This data, if confirmed in prospective studies, may help determine the frequency at which Fontan patients should have serial hepatic imaging. This data suggests that patients with reduced ejection fraction should have more frequent hepatic imaging.

Kay_William_MD.16.CVaCommentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch:  It is now common knowledge that frequently Fontan patients will develop significant hepatic dysfunction with aging.  What are not well established are specific risk factors for hepatic disease, the time frame at which hepatic disease progresses, or the best way to assess (or how often to assess) for liver disease.

The authors did a retrospective analysis of all adult Fontan patients who had had at least two CMRs for clinical reasons, and utilized the expertise of abdominal radiologists to review imaging parameters of the liver, spleen, inferior vena cava (IVC), and right hepatic vein (RHV), and compared these findings with several clinical variables of interest.  Additionally, when possible, they evaluated, using gadolinium enhancement, the degree of liver congestion.

A total of 27 patients were included with an average time of 5.1 years between CMR studies.  Overall, across the study, there was a significant increase in liver size, spleen size, and RHV diameter.  In fifteen out of 25 patients with serial studies using gadolinium, there was evidence of progressive hepatic congestion.

The only imaging risk factors that were found in this study to associate with progressive hepatic congestion were a single ventricle EF < 50% and/or larger indexed end-diastolic and end-systolic volumes.  Type of Fontan and morphology of ventricle were not associated.   Interestingly, hepatic congestion significantly correlated with higher MELD-XI score (9.3 with congestion versus 6.1 without congestion, p = .045).

The only variable that correlated with length of time since Fontan was RHV diameter.  Of note, biochemical variables such as LFTs, platelet count, and albumin did not correlate with length of time since Fontan.  The authors suggest that RHV diameter may serve as an overall indicator of chronicity of high venous pressure after Fontan completion.

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Prognostic Value of N-Terminal Pro-B-Type Natriuretic Peptide, Troponin-T, and Growth-Differentiation Factor 15 in Adult Congenital Heart Disease.

Baggen VJ, van den Bosch A, Eindhoven JA, Schut AR, Cuypers JA, Witsenburg M, de Waart M, van Schaik RH, Zijlstra F, Boersma E, Roos-Hesselink JW.

Circulation. 2016 Nov 3. pii: CIRCULATIONAHA.116.023255. [Epub ahead of print]

Take-Home Points:

  1. Biomarkers may help reduce the frequency of outpatient visits as well as outpatient diagnostic testing in the ACHD population.
  2. NT-proBNP levels were found to correlate well with morbidity and mortality over a median follow up of 42 months in this study of nearly 600 patients, of which 90% were NYHA class I at baseline.
  3. NT-proBNP levels in the upper quartile (> 33.3 pmol/L) are strongly associated with cardiac events (HR = 9.05), and with death or heart failure (HR = 16.0).
  4. A low NT-pro BNP level (< 15.2 pmol/L) had a very high negative predictive value for adverse outcomes, with a risk of death or heart failure of 1% in this study; thus nT-proBNP level may be a helpful screening test in the future
  5. In patients with elevated NT-proBNP, elevated hs-TnT and elevated GDF-15 identified patients at additional risk of cardiovascular events.
  6. NT-proBNP was strongly associated with cardiovascular events, independent of clinical variables, including electrocardiogram and echocardiogram data.

Commentary from Dr. W. Aaron Kay (Indianapolis), section editor of ACHD Journal Watch:  In this elegantly designed trial from the Netherlands, adult patients with moderate or complex congenital heart disease were recruited for analysis of biomarkers, including N-terminal pro B-type natriuretic peptide (NT-proBNP), high-sensitive troponin T (hs-TnT) and growth-differentiation factor 15 (GDF-15), all of which have been extensively studied in heart failure.  Using predictions from the Euro Heart Survey, it was determined that approximately 25% of patients would have events; thus an appropriate sample size was obtained in order to be able to make meaningful comparisons of biomarker data to various clinical data, including echocardiographic and electrocardiographic measurements.

Although 90% were thought to be NYHA I, only 256/595 (43%) had no biomarkers elevated; 234 had one biomarker elevated, 70 had two biomarkers elevated, and 22 had three biomarkers elevated.  Patients with all three biomarkers elevated were at highest risk of cardiac events, heart failure, and death.

NT-proBNP had the highest association with the primary and secondary endpoint.  Very rarely did a patient have an elevated hs-TnT or GDF-15 with a low level of NT-proBNP.  However, in patients with an elevated NT-proBNP, levels of the other biomarkers were found to further stratify the risk.

Interestingly, in addition to correlating with the primary and secondary endpoints (cardiac events, heart failure and death, respectively), higher NT-proBNP levels also correlated with older age, female sex, initial CHD repair at an older age, more complex type of CHD, the use of cardiac medications, lower arterial oxygen saturations, NYHA class II-III, loss of sinus rhythm, longer QRS duration, higher left atrial volume, higher left ventricular volume, and both systolic and diastolic dysfunction.

A NT-proBNP below the median (< 15.2 pmol/L) correlated with a risk of <1% of heart failure or death across the study period.  The authors propose that a low NT-proBNP level could serve to reduce the frequency of outpatient follow-up and outpatient imaging of an otherwise stable patient.

achd 5Figure 1. Cardiovascular event-free survival and heart failure-free survival, stratified according to quartiles of NT-proBNP, hs-TnT and GDF-15. NT-proBNP: First quartile <6.8 pmol/L (n=150), Second quartile 6.8-15.2 pmol/L (n=148), Third quartile 15.2-33.3 pmol/L (n=148), Fourth quartile >33.3 pmol/L (n=149). Hs-TnT: First quartile <3 ng/L (n=196), Second quartile 3-4.8 ng/L (n=131), Third quartile 4.8-7.7 ng/L (n=131), Fourth quartile >7.7 ng/L (n=131). GDF-15: First quartile <487 ng/L (n=148), Second quartile 487-618 ng/L (n=147), Third quartile 618-867 ng/L (n=147), Fourth quartile >867 ng/L (n=147).

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ACHD Articles – November 2016

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

Müller J, Heck PB, Ewert P, Hager A.

Ann Thorac Surg. 2016 Nov 30. pii: S0003-4975(16)31290-5. doi: 10.1016/j.athoracsur.2016.09.038. [Epub ahead of print]

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

Am J Cardiol. 2016 Nov 28. pii: S0002-9149(16)31674-5. doi: 10.1016/j.amjcard.2016.10.004. [Epub ahead of print] No abstract available.

  1. Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects.

Niaz T, Poterucha JT, Johnson JN, Craviari C, Nienaber T, Palfreeman J, Cetta F, Hagler DJ.

Congenit Heart Dis. 2016 Nov 28. doi: 10.1111/chd.12429. [Epub ahead of print]

  1. Serial cardiac MRIs in adult Fontan patients detect progressive hepatic enlargement and congestion.

Lewis MJ, Hecht E, Ginns J, Benton J, Prince M, Rosenbaum MS.

Congenit Heart Dis. 2016 Nov 28. doi: 10.1111/chd.12422. [Epub ahead of print]

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

Caruana M, Grech V.

Congenit Heart Dis. 2016 Nov 28. doi: 10.1111/chd.12439. [Epub ahead of print]

  1. Plasma Proteomic Study in Pulmonary Arterial Hypertension Associated with Congenital Heart Diseases.

Zhang X, Hou HT, Wang J, Liu XC, Yang Q, He GW.

Sci Rep. 2016 Nov 25;6:36541. doi: 10.1038/srep36541.

  1. Cardio-Ankle Vascular Index (CAVI) and Plasma Transforming Growth Factor-β1 (TGF-β1) Level Correlate with Aortopathy in Adults with Repaired Tetralogy of Fallot.

Shiina Y, Niwa K.

Pediatr Cardiol. 2016 Nov 24. [Epub ahead of print]

  1. The prevalence of sexual dysfunction and its association with quality of life in adults with congenital heart disease.

Neiman A, Ginde S, Earing MG, Bartz PJ, Cohen S.

Int J Cardiol. 2016 Nov 19;228:953-957. doi: 10.1016/j.ijcard.2016.11.192. [Epub ahead of print]

  1. Characteristics of Infective Endocarditis in a Tertiary Hospital in East China.

Xu H, Cai S, Dai H.

PLoS One. 2016 Nov 18;11(11):e0166764. doi: 10.1371/journal.pone.0166764.

  1. Short-Term Costs and Hospitalization Rates in Patients With Adult Congenital Heart Disease After Pulmonic Valve Replacement.

Mikhalkova D, Novak E, Cedars A.

Am J Cardiol. 2016 Nov 15;118(10):1552-1557. doi: 10.1016/j.amjcard.2016.08.018.

  1. Congenital coronary artery anomalies in adults: review of 111 cases from a single-centre experience.

Çanga Y, Güvenç TS, Karataş MB, Çalık AN, Onuk T, Tanık VO, Güngör B, Bolca O.

Cardiol Young. 2016 Nov 15:1-10. [Epub ahead of print]

  1. Travel insurance in adult congenital heart disease – Do they declare their condition?

Pickup L, Bowater S, Thorne S, Clift P, Hudsmith L.

Int J Cardiol. 2016 Nov 15;223:316-317. doi: 10.1016/j.ijcard.2016.08.098. No abstract available.

  1. Aortopathy in adults with tetralogy of Fallot has a negative impact on the left ventricle.

Shiina Y, Murakami T, Kawamatsu N, Niwa K.

Int J Cardiol. 2016 Nov 14;228:380-384. doi: 10.1016/j.ijcard.2016.11.252. [Epub ahead of print]

  1. Survival and cardiovascular events after coarctation-repair in long-term follow-up (COAFU): Predictive value of clinical variables.

Bambul Heck P, Pabst von Ohain J, Kaemmerer H, Ewert P, Hager A.

Int J Cardiol. 2016 Nov 9;228:347-351. doi: 10.1016/j.ijcard.2016.11.164. [Epub ahead of print]

  1. Feasibility of low radiation dose retrospectively-gated cardiac CT for functional analysis in adult congenital heart disease.

Groves DW, Olivieri LJ, Shanbhag SM, Bronson KC, Yu JH, Nelson EA, Rollison SF, Stagliano MS, John AS, Kuehl K, Chen MY.

Int J Cardiol. 2016 Nov 9;228:180-183. doi: 10.1016/j.ijcard.2016.11.108. [Epub ahead of print]

  1. Preoperative evaluation of coronary artery fistula using dual-source computed tomography.

Shi K, Gao HL, Yang ZG, Zhang Q, Liu X, Guo YK.

Int J Cardiol. 2016 Nov 9;228:80-85. doi: 10.1016/j.ijcard.2016.11.169. [Epub ahead of print]

  1. From bosentan to macitentan for pulmonary arterial hypertension and adult congenital heart disease: Further improvement?

Blok IM, van Riel AC, van Dijk AP, Mulder BJ, Bouma BJ.

Int J Cardiol. 2016 Nov 9;227:51-52. doi: 10.1016/j.ijcard.2016.11.211. [Epub ahead of print] No abstract available.

  1. Prognostic Value of N-Terminal Pro-B-Type Natriuretic Peptide, Troponin-T, and Growth-Differentiation Factor 15 in Adult Congenital Heart Disease.

Baggen VJ, van den Bosch A, Eindhoven JA, Schut AR, Cuypers JA, Witsenburg M, de Waart M, van Schaik RH, Zijlstra F, Boersma E, Roos-Hesselink JW.

Circulation. 2016 Nov 3. pii: CIRCULATIONAHA.116.023255. [Epub ahead of print]

  1. Hypertension in adults with repaired coarctation of the aorta.

Rinnström D, Dellborg M, Thilén U, Sörensson P, Nielsen NE, Christersson C, Johansson B.

Am Heart J. 2016 Nov;181:10-15. doi: 10.1016/j.ahj.2016.07.012.

  1. Congenital Heart Disease and the Athlete: What We Know and What We Do Not Know.

Dean PN, Battle RW.

Cardiol Clin. 2016 Nov;34(4):579-589. doi: 10.1016/j.ccl.2016.06.007. Review.

  1. Genetics of bicuspid aortic valve aortopathy.

Andreassi MG, Della Corte A.

Curr Opin Cardiol. 2016 Nov;31(6):585-592.

  1. Echocardiography vs magnetic resonance imaging in assessing ventricular function and systemic atrioventricular valve status in adults with congenitally corrected transposition of the great arteries.

Kowalik E, Mazurkiewicz Ł, Kowalski M, Klisiewicz A, Marczak M, Hoffman P.

Echocardiography. 2016 Nov;33(11):1697-1702. doi: 10.1111/echo.13339.

  1. Giant left atrial appendage aneurysm compressing the left anterior descending coronary artery.

Wagdy K, Samaan A, Romeih S, Simry W, Afifi A, Hassan M.

Echocardiography. 2016 Nov;33(11):1790-1792. doi: 10.1111/echo.13296.

  1. No difference between aspirin and warfarin after extracardiac Fontan in a propensity score analysis of 475 patients.

Iyengar AJ, Winlaw DS, Galati JC, Wheaton GR, Gentles TL, Grigg LE, Justo RN, Radford DJ, Attard C, Weintraub RG, Bullock A, Sholler GS, Celermajer DS, d’Udekem Y; Australia and New Zealand Fontan Registry..

Eur J Cardiothorac Surg. 2016 Nov;50(5):980-987.

  1. Cardiac magnetic resonance findings predicting mortality in patients with pulmonary arterial hypertension: a systematic review and meta-analysis.

Baggen VJ, Leiner T, Post MC, van Dijk AP, Roos-Hesselink JW, Boersma E, Habets J, Sieswerda GT.

Eur Radiol. 2016 Nov;26(11):3771-3780. Review.

  1. Heartbeat: Imaging complex vascular anatomy in congenital heart disease.

Otto CM.

Heart. 2016 Nov 1;102(21):1695-1696. doi: 10.1136/heartjnl-2016-310600. No abstract available.

  1. Increasing the evidence base in adult congenital heart disease.

Stefanescu Schmidt AC, Bhatt AB.

Heart. 2016 Nov 1;102(21):1701-1702. doi: 10.1136/heartjnl-2016-309904. No abstract available.

  1. Prognostic value of multiple biomarkers for cardiovascular mortality in adult congenital heart disease: comparisons of single-/two-ventricle physiology, and systemic morphologically right/left ventricles.

Miyamoto K, Takeuchi D, Inai K, Shinohara T, Nakanishi T.

Heart Vessels. 2016 Nov;31(11):1834-1847.

  1. Global area strain is a sensitive marker of subendocardial damage in adults after optimal repair of aortic coarctation: three-dimensional speckle-tracking echocardiography data.

Kowalik E, Kowalski M, Klisiewicz A, Hoffman P.

Heart Vessels. 2016 Nov;31(11):1790-1797.

  1. Prevalence and characterization of fibrosis in surveillance liver biopsies of patients with Fontan circulation.

Surrey LF, Russo P, Rychik J, Goldberg DJ, Dodds K, O’Byrne ML, Glatz AC, Rand EB, Lin HC.

Hum Pathol. 2016 Nov;57:106-115. doi: 10.1016/j.humpath.2016.07.006.

  1. Association between levels of anti-angiogenic isoform of vascular endothelial growth factor A and pulmonary hypertension.

Suzuki S, Yoshihisa A, Yokokawa T, Misaka T, Sakamoto N, Sugimoto K, Yamaki T, Kunii H, Nakazato K, Saitoh S, Takeishi Y.

Int J Cardiol. 2016 Nov 1;222:416-20. doi: 10.1016/j.ijcard.2016.07.277.

  1. Mental disorders in adults with congenital heart disease: Unmet needs and impact on quality of life.

Westhoff-Bleck M, Briest J, Fraccarollo D, Hilfiker-Kleiner D, Winter L, Maske U, Busch MA, Bleich S, Bauersachs J, Kahl KG.

J Affect Disord. 2016 Nov 1;204:180-6. doi: 10.1016/j.jad.2016.06.047.

  1. Assessment of Diastolic Function in Single-Ventricle Patients After the Fontan Procedure.

Margossian R, Sleeper LA, Pearson GD, Barker PC, Mertens L, Quartermain MD, Su JT, Shirali G, Chen S, Colan SD; Pediatric Heart Network Investigators..

J Am Soc Echocardiogr. 2016 Nov;29(11):1066-1073. doi: 10.1016/j.echo.2016.07.016.

  1. Decision analysis to define the optimal management of athletes with anomalous aortic origin of a coronary artery.

Mery CM, Lopez KN, Molossi S, Sexson-Tejtel SK, Krishnamurthy R, McKenzie ED, Fraser CD Jr, Cantor SB.

J Thorac Cardiovasc Surg. 2016 Nov;152(5):1366-1375.e7. doi: 10.1016/j.jtcvs.2016.07.076.

  1. Update on the Role of Cardiac Magnetic Resonance in Acquired Nonischemic Cardiomyopathies.

Rajiah P, Raza S, Saboo SS, Ghoshhajra B, Abbara S.

J Thorac Imaging. 2016 Nov;31(6):348-366.

  1. Transition from paediatric to adult care of adolescent patients with congenital heart disease: a pathway to optimal care.

Strijbosch AM, Zwart R, Blom NA, Bouma BJ, Groenink M, Boekholdt SM, de Winter R, Mulder BJ, Backx AP.

Neth Heart J. 2016 Nov;24(11):682-690.

  1. Mobile health in adults with congenital heart disease: current use and future needs.

Schuuring MJ, Backx AP, Zwart R, Veelenturf AH, Robbers-Visser D, Groenink M, Abu-Hanna A, Bruining N, Schijven MP, Mulder BJ, Bouma BJ.

Neth Heart J. 2016 Nov;24(11):647-652.

  1. NT-proBNP and exercise capacity in adult patients with congenital heart disease and a prosthetic valve: a multicentre PROSTAVA study.

Schoonbeek RC, Pieper PG, van Slooten YJ, Freling HG, Sieswerda GT, van Dijk AP, Jongbloed MR, Post MC, Bouma BJ, Berger RM, Ebels T, van Melle JP.

Neth Heart J. 2016 Nov;24(11):653-665.

  1. The Netherlands as frontrunner of collaborative research in adult congenital heart disease.

Robbers-Visser D, Mulder BJ.

Neth Heart J. 2016 Nov;24(11):625-627. No abstract available.

  1. Maternal Diabetes, Birth Weight, and Neonatal Risk of Congenital Heart Defects in Norway, 1994-2009.

Leirgul E, Brodwall K, Greve G, Vollset SE, Holmstrøm H, Tell GS, Øyen N.

Obstet Gynecol. 2016 Nov;128(5):1116-1125.

  1. Design and Implementation of a Prospective Adult Congenital Heart Disease Biobank.

Opotowsky AR, Loukas B, Ellervik C, Moko LE, Singh MN, Landzberg EI, Rimm EB, Landzberg MJ.

World J Pediatr Congenit Heart Surg. 2016 Nov;7(6):734-743.

  1. Isolated Double Chambered Right Ventricle in an Adult Imaged With Magnetic Resonance.

Vaikunth SS, Sepulveda JS, Shinbane JS, Chang PM.

World J Pediatr Congenit Heart Surg. 2016 Nov;7(6):758-760. No abstract available.

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

Müller J, Heck PB, Ewert P, Hager A.

Ann Thorac Surg. 2016 Nov 30. pii: S0003-4975(16)31290-5. doi: 10.1016/j.athoracsur.2016.09.038. [Epub ahead of print]

  1. Unravelling cardiovascular disease using four dimensional flow cardiovascular magnetic resonance.

Kamphuis VP, Westenberg JJ, van der Palen RL, Blom NA, de Roos A, van der Geest R, Elbaz MS, Roest AA.

Int J Cardiovasc Imaging. 2016 Nov 25. [Epub ahead of print]

  1. Fibrosis and Fibrotic Gene Expression in Pediatric and Adult Patients with Idiopathic Dilated Cardiomyopathy.

Woulfe KC, Siomos AK, Nguyen H, SooHoo M, Galambo C, Stauffer BL, Sucharov C, Miyamoto S.

J Card Fail. 2016 Nov 24. pii: S1071-9164(16)31235-0. doi: 10.1016/j.cardfail.2016.11.006. [Epub ahead of print]

  1. Cardiopulmonary Exercise Testing in Fontan Patients With and Without Isomerism (Heterotaxy) as Compared to Patients With Primary Ciliary Dyskinesia and Subjects With Structurally Normal Hearts.

Loomba RS, Danduran M, Nielsen KG, Ring AM, Kovach J, Anderson RH.

Pediatr Cardiol. 2016 Nov 23. [Epub ahead of print]

  1. Diagnostic and Prognostic Value of Plasma Levels of Cardiac Myosin Binding Protein-C as a Novel Biomarker in Heart Failure.

El Amrousy D, Hodeib H, Suliman G, Hablas N, Salama ER, Esam A.

Pediatr Cardiol. 2016 Nov 23. [Epub ahead of print]

  1. NT-proBNP as Marker of Ventricular Dilatation and Pulmonary Regurgitation After Surgical Correction of Tetralogy of Fallot: A MRI Validation Study.

Paolino A, Hussain T, Pavon A, Velasco MN, Uribe S, Ordoñez A, Valverde I.

Pediatr Cardiol. 2016 Nov 21. [Epub ahead of print]

  1. Cardiac Mechanoperception: A Life-Long Story from Early Beats to Aging and Failure.

Pesce M, Messina E, Chimenti I, Beltrami AP.

Stem Cells Dev. 2016 Nov 14. [Epub ahead of print]

  1. Cardiopulmonary exercise testing is a better outcome predictor than exercise echocardiography in asymptomatic aortic stenosis.

Domanski O, Richardson M, Coisne A, Polge AS, Mouton S, Godart F, Edmé JL, Matran R, Lancellotti P, Montaigne D.

Int J Cardiol. 2016 Nov 3. pii: S0167-5273(16)33244-2. doi: 10.1016/j.ijcard.2016.10.070. [Epub ahead of print]

  1. The role of the multidisciplinary health care team in the management of patients with Marfan syndrome.

von Kodolitsch Y, Rybczynski M, Vogler M, Mir TS, Schüler H, Kutsche K, Rosenberger G, Detter C, Bernhardt AM, Larena-Avellaneda A, Kölbel T, Debus ES, Schroeder M, Linke SJ, Fuisting B, Napp B, Kammal AL, Püschel K, Bannas P, Hoffmann BA, Gessler N, Vahle-Hinz E, Kahl-Nieke B, Thomalla G, Weiler-Normann C, Ohm G, Neumann S, Benninghoven D, Blankenberg S, Pyeritz RE.

J Multidiscip Healthc. 2016 Nov 3;9:587-614. Review.

  1. [Pulmonary Hypertension: Cologne Consensus Conference 2016].

Rosenkranz S, Ghofrani HA, Grünig E, Klose H, Olschewski H, Opitz C, Hoeper M.

Dtsch Med Wochenschr. 2016 Nov;141(24):1778-1782. German.

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