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

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Abstract

Increased survival rates after corrective or palliative surgery for complex congenital heart disease (CHD) in infancy and childhood are now being coupled with increased numbers of patients who survive to adulthood with various residual lesions or sequelae. These patients are likely to deteriorate in cardiac function or end-organ function, eventually requiring lifesaving treatment including heart transplantation. Although early and late outcomes of heart transplantation have been improving for adult survivors of CHD, outcomes and pretransplant management could still be improved. Survivors of Fontan procedures are a vulnerable cohort, particularly when single ventricle physiology fails, mostly with protein-losing enteropathy and hepatic dysfunction. Therefore, we reviewed single-institution and larger database analyses of adults who underwent heart transplantation for CHD, to enable risk stratification by identifying the indications and outcomes. As the results, despite relatively high early mortality, long-term results were encouraging after heart transplantation. However, further investigations are needed to improve the indication criteria for complex CHD, especially for failed Fontan. In addition, the current system of status criteria and donor heart allocation system in heart transplantation should be arranged as suitable for adults with complex CHD. Furthermore, there is a strong need to develop ventricular assist devices as a bridge to transplantation or destination therapy, especially where right-sided circulatory support is needed.

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References

  1. Jacobs JP, Pasquali SK, Jeffries H, Jones SB, Cooper DS, Vincent R. Outcomes analysis and quality improvement for the treatment of patients with pediatric and congenital cardiac disease. World J Pediatr Congenit Heart Surg. 2011;2:620–33.

    Article  PubMed  Google Scholar 

  2. Diller GP, Kempny A, Alonso-Gonzalez R, Swan L, Uebing A, Li W, et al. Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre. Circulation. 2015;132:2118–25.

    Article  PubMed  Google Scholar 

  3. Sommerville J. Management of adults with congenital heart disease: an increasing problem. Ann Rev Med. 1997;40:283–93.

    Article  Google Scholar 

  4. Hsu DT, Lamour JM. Changing indications for pediatric heart transplantation. Complex congenital heart disease. Circulation. 2015;31:91–9.

    Article  Google Scholar 

  5. Hosseinpour AR, Cullen S, Tsang VT. Transplantation for adults with congenital heart disease. Eur Cardiothorac Surg. 2006;30:508–14.

    Article  Google Scholar 

  6. Burchill LJ. Heart transplantation in adult congenital heart disease. Heart. 2016;102:1871–7.

    Article  PubMed  Google Scholar 

  7. Stout KK, Broberg CS, Book WM, Cecchin F, Chen JM, Dimopoulos K, et al. Chronic heart failure in congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2016;133:770–801.

    PubMed  Google Scholar 

  8. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26:140–8.

    Article  Google Scholar 

  9. Engelfriet P, Boersma E, Oechslin E, Tijssen J, Gatzoulis MA, Thilén U, et al. The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period. Eur Heart J. 2005;26:2325–33.

    Article  PubMed  Google Scholar 

  10. Khairy P, Femandes SM, Mayer JE Jr, Triedman JK, Walsh EP, Lock JE, Landzberg MJ. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation. 2008;117:85–92.

    Article  PubMed  Google Scholar 

  11. Rossano JW, Dipchand AI, Edwards LB, Goldfarb S, Kucheryavaya AY, Levvey BJ, et al. The Registry of the International Society for Heart and Lung Transplantation: nineteenth pediatric heart transplantation report-2016; focus theme: primary diagnostic indications for transplant. J Heart Lung Transplant. 2016;35:1185–206.

    Article  PubMed  Google Scholar 

  12. Lund LH, Edwards LB, Dipchand AI, Goldfarb S, Kucheryavaya AY, Levvey BJ, et al. The Registry of the International Society for Heart and Lung Transplantation: thirty-third adult heart transplantation report-2016; focus theme: primary diagnostic indications for transplant. J Heart Lung Transplant. 2016;35:1159–69.

    Google Scholar 

  13. Bhama JK, Shulman J, Bermudez CA, Bansal A, Ramani R. Heart transplantation for adults with congenital heart disease: results in the modern era. J Heart Lung Transplant. 2013;32:499–504.

    Article  PubMed  Google Scholar 

  14. Speziali G, Driscoll DJ, Danielson GK, Julsrud PR, Porter CJ, Dearani JA, et al. Cardiac transplantation for end-stage congenital heart defects: the Mayo Clinic experience. Mayo Cardiothoracic Transplant Team. Mayo Clin Proc. 1998;73:923–8.

    Article  CAS  PubMed  Google Scholar 

  15. Lamour JM, Addonizio LJ, Galantowicz ME, Quaegebeur JM, Mancini DM, Kichuk MR, et al. Outcome after orthotopic heart transplantation in adults with congenital heart disease. Circulation. 1999;100(SupplII):II200–5.

    CAS  PubMed  Google Scholar 

  16. Chen JM, Davies RR, Mital SR, Mercando ML, Addonizio LJ, Pinney SP, et al. Trends and outcomes in transplantation for complex congenital heart disease: 1984–2004. Ann Thorac Surg. 2004;78:1352–61.

    Article  PubMed  Google Scholar 

  17. Coskun O, Coskun T, El-Arousy M, Parsa MA, Reiss N, Blanz U, Knyphalisen V, et al. Heart transplantation in adults with congenital heart disease. Experience with 15 patients. ASAIO J. 2007;53:103–6.

    Article  PubMed  Google Scholar 

  18. Greutmann M, Pretre R, Furrer L, Bauersfeld U, Turina M, Noll G, et al. Heart transplantation in adolescence and adult with congenital heart disease: a case–control study. Transplant Proc. 2009;41:3821–6.

    Article  CAS  PubMed  Google Scholar 

  19. Irving C, Parry G, O’Sullivan J, Dark JH, Kirk R, Crossland DS, et al. Cardiac transplantation in adults with congenital heart disease. Heart. 2010;96:1217–22.

    Article  CAS  PubMed  Google Scholar 

  20. Cheng RK, Depasquale M, Allaredy M, Cadeiras M, KhuuT Baas A, et al. Post-heart transplant survival for adult congenital heart disease. J Hear Lung Transplant. 2013;31:S206.

    Article  Google Scholar 

  21. Mori M, Vega D, Book W, Kogon BE. Heart transplantation in adults with congenital heart disease: 100% survival with operations performed by a surgeon specializing in congenital heart disease in an adult hospital. Ann Thorac Surg. 2015;99:2173–8.

    Article  PubMed  Google Scholar 

  22. Besik J, Szarszoi O, Hegarova M, Konarik M, Smetana M, Netuka I, et al. Non-Fontan adult congenital heart disease transplantation survival is equivalent to acquired heart disease. Ann Thorac Surg. 2016;101:1768–73.

    Article  PubMed  Google Scholar 

  23. Patel ND, Weiss ES, Allen JG, Russell SD, Shah AS, Vricella LA, et al. Heart transplantation for adults with congenital heart disease: analysis of the United Network for organ sharing database. Ann Thorac Surg. 2009;88:814–21.

    Article  PubMed  Google Scholar 

  24. Karamlou T, Hirsch J, Welke K, Ohye RG, Bove EL, Devaney EJ, et al. A United Network for Organ Sharing analysis of heart transplantation in adults with congenital heart disease: outcomes and factors associated with mortality and retransplantation. J Thorac Cardiovasc Surg. 2010;140:161–8.

    Article  PubMed  Google Scholar 

  25. Davies RR, Russo MJ, Yang J, Quaegebeur JM, Mosca RS, Chen JM. Listing and transplanting adults with congenital heart disease. Circulation. 2011;123:759–67.

    Article  PubMed  Google Scholar 

  26. Lamour JM, Kanter KR, Naftel DC, Chrisant MR, Morrow WR, Clemson BS, et al. The effect of age, diagnosis, and previous surgery in children and adults undergoing heart transplantation for congenital heart disease. J Am Coll Cardiol. 2009;54:160–5.

    Article  PubMed  Google Scholar 

  27. Burchill LJ, Edwards LB, Dipchand AI, Stehlik J, Ross HJ. Impact of adult congenital heart disease on survival and mortality after heart transplantation. J Heart Lung Transplant. 2014;33:1157–63.

    Article  PubMed  Google Scholar 

  28. Ross HJ, Law Y, Book WM, Broberg CS, Burchill L, Cecchin F, et al. Transplantation and mechanical circulatory support in congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2016;133:802–20.

    PubMed  Google Scholar 

  29. Everitt MD, Donaldson AE, Stehlik J, Kaza AK, Budge D, Alharethi R, et al. Would access to device therapies improve transplant outcomes for adults with congenital heart disease? Analysis of the United Network for Organ Sharing (UNOS). J Heart Lung Transplant. 2011;30:395–401.

    Article  PubMed  Google Scholar 

  30. Alshawabkeh LI, Hu N, Carter KD, Opotowsky AR, Light-McGroary K, Cavanaugh JE, et al. Wait-list outcomes for adults with congenital heart disease listed for heart transplantation in the US. J Am Coll Cardiol. 2016;68:908–17.

    Article  PubMed  Google Scholar 

  31. Cohen S, Houyel L, Guillemain R, Varnous S, Jannot AS, Ladouceur M, et al. Temporal trends and changing profile of adults with congenital heart disease undergoing heart transplantation. Eur Heart J. 2016;37:783–9.

    Article  PubMed  Google Scholar 

  32. Karamlou T, Diggs BS, Welke K, Tibayan F, Gelow J, Guyton SW, et al. Impact of single-ventricle physiology on death after heart transplantation in adults with congenital heart disease. Ann Thorac Surg. 2012;94:1281–7.

    Article  PubMed  Google Scholar 

  33. Lewis M, Ginns J, Schulze C, Lippel M, Chai P, Bacha E, et al. Outcomes of adult patients with congenital heart disease after heart transplantation: impact of disease type, previous thoracic surgeries, and Bystander organ dysfunction. J Card Fail. 2016;22:578–82.

    Article  PubMed  Google Scholar 

  34. Goldberg SW, Fisher SA, Wehman B, Mehra MR. Adults with congenital heart disease and heart transplantation: optimizing outcomes. J Heart Lung Transplant. 2014;33:873–7.

    Article  PubMed  Google Scholar 

  35. Mehra MR, Canter CE, Hannan MM, Semigran MJ, Uber PA, Baran DA, et al. The 2016 International Society for Heart Lung Transplantation listing criteria for heart transplantation: a 10-year update. J Heart Lung Transplant. 2016;35:1–23.

    Article  PubMed  Google Scholar 

  36. Davies RR, Sorabella RA, Quaegebeur JM. Outcomes after transplantation for failed Fontan: a single institutional experience. J Thorac Cardiovasc Surg. 2012;143:1183–92.

    Article  PubMed  Google Scholar 

  37. Simpson E, Cibulka N, Lee CK, Huddleston CH, Canter CE. Failed Fontan heart transplantation candidates with preserved ventricular function: 2 distinct patient populations. J Heart Lung Transplant. 2012;31:545–7.

    Article  PubMed  Google Scholar 

  38. Miller J, Simson KE, Epstein DJ, Lancaster TS, Henn MC, Schuessler RB, et al. Improved survival after heart transplant for failed Fontan patients with preserved ventricular function. J Heart Lung Transplant. 2016;35:877–83.

    Article  PubMed  Google Scholar 

  39. Krishnamurthy Y, Cooper LB, Lu D, Schroder JN, Daneshmand MA, Rogers JG, et al. Trends and outcomes of patients with adult congenital heart disease and pulmonary hypertension listed for orthotopic heart transplantation in the US. J Heart Lung Transplant. 2016;35:619–24.

    Article  PubMed  Google Scholar 

  40. Kavarana MN, Savage A, O’Connell R, Rubinstein CS, Flynn-Reeves J, Joshi K, et al. Composite risk factors predict survival after transplantation for congenital heart disease. J Thorac Cardiovasc Surg. 2013;146:888–93.

    Article  PubMed  Google Scholar 

  41. Doumouras BS, Alba AC, Foroutan F, Burchill LJ, Dipchand AI, Ross HJ. Outcomes in adult congenital heart disease patients undergoing heart transplantation: a systematic review and meta-analysis. J Heart Lung Transplant. 2016;35:1337–47.

    Article  PubMed  Google Scholar 

  42. González-López MT, Pérez-Caballero-Martínez R, Amoros-Rivera C, Zamorano-Serrano J, Pita-Fernández AM, Gil-Jaurena JM. Orthotopic heart transplantation in an adult patient with heterotaxy syndrome: surgical implications. J Card Surg. 2015;30:910–2.

    Article  PubMed  Google Scholar 

  43. Matsuda H, Fukushima N, Ichikawa H, Sawa Y. Orthotropic heart transplantation for adult congenital heart disease: a case with heterotaxy and dextrocardia. Gen Thorac Cardiovasc Surg. 2017;65:47–51.

    Article  PubMed  Google Scholar 

  44. Marcelletti CF, Hanley FL, Mavroudis C, McElhinney DB, Abella RF, Marianeschi SM, et al. Revision of previous Fontan connection to total extracardiac cavopulmonary anastomosis: a multicenter experience. J Thorac Cardiovasc Surg. 2000;119:340–6.

    Article  CAS  PubMed  Google Scholar 

  45. Khambadkone S. The Fontan pathway: what’s down the road? Ann Pediatr Cardiol. 2008;1:83–92.

    Article  PubMed  PubMed Central  Google Scholar 

  46. Rychik J. Protein-losing enteropathy after Fontan operation. Congenit Heart Dis. 2007;2:288–300.

    Article  PubMed  Google Scholar 

  47. Michielon G, van Melle JP, Wolff D, Di Carlo D, Jacobs JP, Mattila IP, et al. Favourable mid-term outcome after heart transplantation for late Fontan failure. Eur J Cardiothorac Surg. 2015;47:665–71.

    Article  PubMed  Google Scholar 

  48. Pundi K, Pundi KN, Kamath PS, Cetta F, Li Z, Poterucha JT, et al. Liver disease in patients after the Fontan operation. Am J Cardiol. 2016;117:456–60.

    Article  PubMed  Google Scholar 

  49. Greenway SC, Crossland DS, Hudson M, Martin SR, Myers RP, Prieur T, et al. Fontan-associated liver disease: implication for heart transplantation. J Heart Lung Transplant. 2016;35:26–33.

    Article  PubMed  Google Scholar 

  50. Backer CL, Russell HM, Pahl E, Mongé MC, Gambetta K, Kindel SJ, et al. Heart transplantation for the failing Fontan. Ann Thorac Surg. 2013;96:1413–9.

    Article  PubMed  Google Scholar 

  51. Gamba A, Merlo M, Fiocchi R, Terzi A, Mammana C, Sebastiani R, et al. Heart transplantation in patients with previous Fontan operations. J Thorac Cardiovasc Surg. 2004;127:555–62.

    Article  PubMed  Google Scholar 

  52. Jayakumar KA, Addonizio LJ, Kichuk-Chrisant MR, Galantowicz ME, Lamour JM, Quaegebeur JM, et al. Cardiac transplantation after the Fontan or Glenn procedure. J Am Coll Cardiol. 2004;44:2065–72.

    Article  PubMed  Google Scholar 

  53. Griffiths ER, Kaza AK, Wyler von Ballmoos MC, Loyola H, Valente AM, Blume ED, et al. Evaluating failing Fontan for heart transplantation: predictors of death. Ann Thorac Surg. 2009;88:558–63.

    Article  PubMed  PubMed Central  Google Scholar 

  54. Pundi KN, Pundi K, Driscoll DJ, Dearani JA, Li Z, Dahl SH, et al. Heart transplantation after Fontan: results from a surgical Fontan cohort. Pediatr Transplant. 2016;20:1087–92.

    Article  PubMed  Google Scholar 

  55. Matsuda H, Covino E, Hirose H, Nakano S, Kishimoto H, Miyamoto Y, et al. Acute liver dysfunction after modified Fontan operation for complex cardiac lesions. Analysis of the contributing factors and its relation to the early prognosis. J Thorac Cardiovasc Surg. 1988;96:219–26.

    CAS  PubMed  Google Scholar 

  56. Deo SV, Al-Kindi SG, Altarabsheh SE, Hang D, Kumar S, Ginwalla MB, et al. Model for end-stage liver disease excluding international normalized ratio (MELD-XI) score predicts heart transplant outcomes: evidence from the registry of the United Network for Organ Sharing. Heart Lung Transplant. 2016;35:222–7.

    Article  Google Scholar 

  57. Murtuza B, Hermuzi A, Crossland DS, Parry G, Lord S, Hudson M, et al. Impact of mode of failure and end-organ dysfunction on the survival of adult Fontan patients undergoing cardiac transplantation. Eur J Cardiothorac Surg. 2017;51:135–41.

    Article  PubMed  Google Scholar 

  58. Elder RW, McCabe NM, Hebson C, Veledar E, Romero R, Ford RM, et al. Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study. Int J Cardiol. 2013;168:3764–9.

    Article  PubMed  PubMed Central  Google Scholar 

  59. Davies RR, Russo MJ, Hong KN, O’Byrne ML, Cork DP, Moskowitz AJ, et al. The use of mechanical circulatory support as a bridge to transplantation in pediatric patients: an analysis of the United Network for Organ Sharing database. J Thoracic Cardiovasc Surg. 2008;135:421–7.

    Article  Google Scholar 

  60. De Rita F, Hasan A, Haynes S, Crossland D, Kirk R, Ferguson L, et al. Mechanical cardiac support in children with congenital heart disease with intention to bridge to heart transplantation. Eur J Cardiothorac Surg. 2014;46:656–62.

    Article  PubMed  Google Scholar 

  61. Shah NR, Lam WW, Rodriguez FH, Ermis PR, Simpson L, Frazier OH, et al. Clinical outcomes after ventricular assist device implantation in adults with congenital heart disease. J Heart Lung Transplant. 2013;32:615–20.

    Article  PubMed  Google Scholar 

  62. Maxwell BG, Wong JK, Sheikh AY, Lee PH, Lobato RL. Heart transplantation with or without prior mechanical circulatory support in adults with congenital heart disease. Eur J Cardiothorac Surg. 2014;45:842–6.

    Article  PubMed  Google Scholar 

  63. Joyce DL, Crow SS, John R, St Louis JD, Braunlin EA, Pyles LA, et al. Mechanical circulatory support in patients with heart failure secondary to transposition of the great arteries. J Heart Lung Transplant. 2010;29:1302–5.

    Article  PubMed  Google Scholar 

  64. Maly J, Netuka I, Besik J, Dorazilova Z, Pirk J, Szarszoi O. Bridge to transplantation with long-term mechanical assist device in adults after the Mustard procedure. J Heart Lung Transplant. 2015;34:1177–81.

    Article  PubMed  Google Scholar 

  65. Morales DLS, Adachi I, Heinle JS, Fraser CD Jr. A new era: use of an intracorporeal systemic ventricular assist device to support a patient with a failing Fontan circulation. J Thorac Cardiovasc Surg. 2011;142:e138–40.

    Article  PubMed  Google Scholar 

  66. Prêtre R, Häussler A, Bettex D, Genoni M. Right-sided univentricular cardiac assistance in a failing Fontan circulation. Ann Thorac Surg. 2008;86:1018–20.

    Article  PubMed  Google Scholar 

  67. Derk G, Laks H, Biniwale R, Patel S, De LaCruz K, Mazor E, et al. Novel techniques of mechanical circulatory support for the right heart and Fontan circulation. Int J Cardiol. 2014;176:828–33.

    Article  PubMed  Google Scholar 

  68. Riemer RK, Amir G, Reichenbach SH, Reinhartz O. Mechanical support of total cavopulmonary connection with axial flow pump. J Thorac Cardiovasc Surg. 2005;130:351–4.

    Article  PubMed  Google Scholar 

  69. Horne D, Conway J, Rebeyka IM, Bouchholz. Mechanical circulatory support in univentricular hearts: current management. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2015;18:17–24.

    Article  PubMed  Google Scholar 

  70. Gondolfo F, Brancaccio G, Donatiello S, Filippelli S, Perri G, Iannace E, et al. Mechanically assisted to total cavopulmonary connection with an axial flow pump: computational and in Vivo study. Artif Organs. 2016;40:43–9.

    Article  Google Scholar 

  71. Haggerty CM, Fynn-Thompson F, McElinney DB, Valente AM, Saikrishnan, Del Nido PJ, et al. Experimental and numeric investigation of Impella pumps as cavopulmonary assistance for failing Fontan. J Thorac Cardiovasc Surg. 2012;144:563–9.

    Article  PubMed  Google Scholar 

  72. Shimizu S, Kawada T, Une D, Fukumitsu M, Turner MJ, Kamiya A, et al. Partial cavopulmonary assist from the inferior vena cava to the pulmonary artery improves hemodynamics in failing Fontan circulation: a theoretical analysis. J Physiol Sci. 2016;66:249–55.

    Article  PubMed  Google Scholar 

  73. Adachi I, Burki S, Zafar F, Morales DL. Pediatric ventricular assist devices. J Thorac Dis. 2015;7:2194–202.

    PubMed  PubMed Central  Google Scholar 

  74. Gelow JM, Song HK, Weiss JB, Mudd JO, Broberg CS. Organ allocation in adults with congenital heart disease listed for heart transplant: impact of ventricular assist devices. J Heart Lung Transplant. 2013;32:1059–64.

    Article  PubMed  PubMed Central  Google Scholar 

  75. Meyer DM, Rogers JG, Edwaards LB, Callahan ER, Webber SA, Johnson MR, et al. The furure direction of the adult heart allocation system in the US. Am J Transplant. 2015;15:45–54.

    Article  Google Scholar 

  76. Rogers JG. Changes in US heart allocation: a community energized to improve policy. J Thorac Cardiovasc Surg 2016;1484–1486.

  77. Matsuda H, Fukushima N, Sawa Y, Nishimura M, Matsumiya G, Shirakura R. First brain dead donor heart transplantation under new legislation in Japan. Jpn J Thorac Cardiovasc Surg. 1999;47:499–505.

    Article  CAS  PubMed  Google Scholar 

  78. Nakatani T, Fukushima N, Ono M, Saiki Y, Matsuda H, Nunoda S, et al. The Registry Report of Heart Transplantation in Japan (1999–2014). Cir J. 2016;80:44–50.

    Article  Google Scholar 

  79. Tanoue Y, Jinzai Y, Tominaga R. Jarvik 2000 axial-flow ventricular assist device placement to a systemic morphologic right ventricle in congenitally corrected transposition of the great arteries. J Artif Organs. 2016;19:97–9.

    Article  PubMed  Google Scholar 

  80. Ohuchi H, Kagisaki K, Miyazaki A, Kitano M, Yazaki S, Sakaguchi H, et al. Impact of the evolution of the Fontan operation on early and late mortality: a single-center experience of 405 patients over 3 decades. Ann Thorac Surg. 2011;92:1457–66.

    Article  PubMed  Google Scholar 

  81. Nakano T, Kado H, Tatewaki H, Hinokiyama K, Oda S, Ushinohama H, et al. Results of extracardiac conduit total cavopulmonary connection in 500 patients. Eur J Cardiothorac Surg. 2015;48:825–32.

    Article  PubMed  Google Scholar 

  82. Hoashi T, Ichikawa H, Fukushima N, Ueno T, Kogaki S, Sawa Y. Long-term clinical outcome of atrial isomerism after univentricular repair. J Cardiac Surg. 2009;24:19–23.

    Article  Google Scholar 

  83. Sakamoto T, Nagashima M, Hiramatsu T, Matsumura G, Park IS, Yamazaki K. Fontan circulation over 30 years. What should we learn from those patients? Asian Cardiovasc Thorac Ann. 2016;24:765–71.

    Article  PubMed  Google Scholar 

  84. Ozawa H, Ueno T, Iwai S, Kawata H, Nishigaki K, Kishimoto H, et al. Contractility-afterload mismatch in patients with protein-losing enteropathy after the Fontan operation. Pediatr Cadiol. 2014;35:1225–31.

    Article  Google Scholar 

  85. Gurvitz M, Burns KM, Brindis R, Broberg CS, Daniels CJ, Fuller SM, et al. Emerging research directions in adult congenital heart disease. A report from an NHLBI/ACHA Working Group. J Am Coll Cardiol. 2016;67:1956–64.

    Article  PubMed  PubMed Central  Google Scholar 

  86. d’Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton GR, et al. Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation. 2014;130(Suppl 1):S32–8.

    Article  PubMed  Google Scholar 

  87. Schilling C, Dalziel K, Nunn R, Du Plessis K, Shi WY, Celermajer D, et al. The Fontan epidemic: population projections from the Australia and New Zealand Fontan Registry. Int J Cardiol. 2016;219:14–9.

    Article  PubMed  Google Scholar 

  88. Jaquiss H, Aziz H. Is four stage management the future of univentricular hearts? Destination therapy in the young. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2016;19:50–4.

    Article  PubMed  Google Scholar 

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Matsuda, H., Ichikawa, H., Ueno, T. et al. Heart transplantation for adults with congenital heart disease: current status and future prospects. Gen Thorac Cardiovasc Surg 65, 309–320 (2017). https://doi.org/10.1007/s11748-017-0777-x

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