Transplantation and Mechanical Circulatory Support in Adult Congenital Heart Disease-Related Advanced Heart Failure

  • Jonathan N. Menachem
  • Luke J. BurchillEmail author
Part of the Congenital Heart Disease in Adolescents and Adults book series (CHDAA)


  • Heart transplantation has become an increasingly common and effective therapy for adults with end-stage congenital heart disease (ACHD) because of advances in patient selection and surgical technique. Indications for transplantation in ACHD are similar to other forms of heart failure. Timing of referral, pretransplant assessment of ACHD patients, wait-list outcomes and opportunities for decreasing wait-list mortality as well as outcome, alternatives to transplantation, and peri- and postoperative management are discussed in this chapter.


Transplantation waiting list Sensitization Assist devices Extracorporeal membrane oxygenation Transplantation outcomes 


  1. 1.
    Burchill LJ. Heart transplantation in adult congenital heart disease. Heart. 2016;102:1871–7.CrossRefPubMedGoogle Scholar
  2. 2.
    Baggen VJ, van den Bosch AE, Eindhoven JA, Schut AW, Cuypers JA, Witsenburg M, de Waart M, van Schaik RH, Zijlstra F, Boersma E, Roos-Hesselink JW. Prognostic value of N-terminal Pro-B-type natriuretic peptide, troponin-T, and growth-differentiation factor 15 in adult congenital heart disease. Circulation. 2017;135:264–79.CrossRefPubMedGoogle Scholar
  3. 3.
    Giannakoulas G, Dimopoulos K, Bolger AP, Tay EL, Inuzuka R, Bedard E, Davos C, Swan L, Gatzoulis MA. Usefulness of natriuretic peptide levels to predict mortality in adults with congenital heart disease. Am J Cardiol. 2010;105:869–73.CrossRefPubMedGoogle Scholar
  4. 4.
    Brida M, Dimopoulos K, Kempny A, Liodakis E, Alonso-Gonzalez R, Swan L, Uebing A, Baumgartner H, Gatzoulis MA, Diller GP. Body mass index in adult congenital heart disease. Heart. 2017;103:1250–7.CrossRefPubMedGoogle Scholar
  5. 5.
    Dimopoulos K, Diller GP, Petraco R, Koltsida E, Giannakoulas G, Tay EL, Best N, Piepoli MF, Francis DP, Poole-Wilson PA, Gatzoulis MA. Hyponatraemia: a strong predictor of mortality in adults with congenital heart disease. Eur Heart J. 2010;31:595–601.CrossRefPubMedGoogle Scholar
  6. 6.
    Dimopoulos K, Diller GP, Giannakoulas G, Petraco R, Chamaidi A, Karaoli E, Mullen M, Swan L, Piepoli MF, Poole-Wilson PA, Francis DP, Gatzoulis MA. Anemia in adults with congenital heart disease relates to adverse outcome. J Am Coll Cardiol. 2009;54:2093–100.CrossRefPubMedGoogle Scholar
  7. 7.
    Dimopoulos K, Diller GP, Koltsida E, Pijuan-Domenech A, Papadopoulou SA, Babu-Narayan SV, Salukhe TV, Piepoli MF, Poole-Wilson PA, Best N, Francis DP, Gatzoulis MA. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation. 2008;117:2320–8.CrossRefPubMedGoogle Scholar
  8. 8.
    Schuuring MJ, van Riel AC, Vis JC, Duffels MG, van Straalen JP, Boekholdt SM, Tijssen JG, Mulder BJ, Bouma BJ. High-sensitivity troponin T is associated with poor outcome in adults with pulmonary arterial hypertension due to congenital heart disease. Congenit Heart Dis. 2013;8:520–6.CrossRefPubMedGoogle Scholar
  9. 9.
    Rodriguez FH 3rd, Moodie DS, Parekh DR, Franklin WJ, Morales DL, Zafar F, Adams GJ, Friedman RA, Rossano JW. Outcomes of heart failure-related hospitalization in adults with congenital heart disease in the United States. Congenit Heart Dis. 2013;8:513–9.CrossRefPubMedGoogle Scholar
  10. 10.
    John AS, Johnson JA, Khan M, Driscoll DJ, Warnes CA, Cetta F. Clinical outcomes and improved survival in patients with protein-losing enteropathy after the Fontan operation. J Am Coll Cardiol. 2014;64:54–62.CrossRefPubMedGoogle Scholar
  11. 11.
    Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg. 1998;115:1063–73.CrossRefPubMedGoogle Scholar
  12. 12.
    Tobler D, Greutmann M, Colman JM, Greutmann-Yantiri M, Librach LS, Kovacs AH. End-of-life care in hospitalized adults with complex congenital heart disease: care delayed, care denied. Palliat Med. 2012;26:72–9.CrossRefPubMedGoogle Scholar
  13. 13.
    Ross HJ, Law Y, Book WM, Broberg CS, Burchill L, Cecchin F, Chen JM, Delgado D, Dimopoulos K, Everitt MD, Gatzoulis M, Harris L, Hsu DT, Kuvin JT, Martin CM, Murphy AM, Singh G, Spray TL, Stout KK. American Heart Association Adults With Congenital Heart Disease Committee of the Council on Clinical C, Council on Cardiovascular Disease in the Young tCoCR, Intervention, the Council on Functional G and Translational B. Transplantation and mechanical circulatory support in congenital heart disease: a scientific statement From the American Heart Association. Circulation. 2016;133:802–20.PubMedPubMedCentralGoogle Scholar
  14. 14.
    Mehra MR, Canter CE, Hannan MM, Semigran MJ, Uber PA, Baran DA, Danziger-Isakov L, Kirklin JK, Kirk R, Kushwaha SS, Lund LH, Potena L, Ross HJ, Taylor DO, Verschuuren EA, Zuckermann A. International Society for Heart Lung Transplantation Infectious Diseases P, Heart F and Transplantation C. 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.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Bradley EA, Pinyoluksana KO, Moore-Clingenpeel M, Miao Y, Daniels C. Isolated heart transplant and combined heart-liver transplant in adult congenital heart disease patients: insights from the united network of organ sharing. Int J Cardiol. 2017;228:790–5.CrossRefPubMedGoogle Scholar
  16. 16.
    Wang A, Book WM, McConnell M, Lyle T, Rodby K, Mahle WT. Prevalence of hepatitis C infection in adult patients who underwent congenital heart surgery prior to screening in 1992. Am J Cardiol. 2007;100:1307–9.CrossRefPubMedGoogle Scholar
  17. 17.
    Josephus Jitta D, Wagenaar LJ, Mulder BJ, Guichelaar M, Bouman D, van Melle JP. Three cases of hepatocellular carcinoma in Fontan patients: review of the literature and suggestions for hepatic screening. Int J Cardiol. 2016;206:21–6.CrossRefPubMedGoogle Scholar
  18. 18.
    Menachem JN, Golbus JR, Molina M, Mazurek JA, Hornsby N, Atluri P, Fuller S, Birati EY, Kim YY, Goldberg LR, Wald JW. Successful cardiac transplantation outcomes in patients with adult congenital heart disease. Heart. 2017.
  19. 19.
    Greenway SC, Crossland DS, Hudson M, Martin SR, Myers RP, Prieur T, Hasan A, Kirk R. Fontan-associated liver disease: implications for heart transplantation. J Heart Lung Transplant. 2016;35:26–33.CrossRefPubMedGoogle Scholar
  20. 20.
    Reich HJ, Awad M, Ruzza A, De Robertis MA, Ramzy D, Nissen N, Colquhoun S, Esmailian F, Trento A, Kobashigawa J, Czer LS. Combined heart and liver transplantation: the Cedars-Sinai experience. Transplant Proc. 2015;47:2722–6.CrossRefPubMedGoogle Scholar
  21. 21.
    Padegimas A, Molina M, Korwin A, Birati E, Kamoun M. Successful long-term outcomes in combined heart-liver transplants across pre-formed high levels of donor-specific antibodies in highly sensitized patients. J Heart Lung Transplant. 2016;35:1382–4.CrossRefPubMedGoogle Scholar
  22. 22.
    Lewis M, Ginns J, Schulze C, Lippel M, Chai P, Bacha E, Mancini D, Rosenbaum M, Farr M. 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. 2015;22:578–82.CrossRefPubMedGoogle Scholar
  23. 23.
    Berg CJ, Bauer BS, Hageman A, Aboulhosn JA, Reardon LC. Mortality Risk Stratification in Fontan Patients Who Underwent Heart Transplantation. Am J Cardiol. 2017;119:1675–9.CrossRefPubMedGoogle Scholar
  24. 24.
    Drakos SG, Kfoury AG, Gilbert EM, Horne BD, Long JW, Stringham JC, Campbell BA, Renlund DG. Effect of reversible pulmonary hypertension on outcomes after heart transplantation. J Heart Lung Transplant. 2007;26:319–23.CrossRefPubMedGoogle Scholar
  25. 25.
    Goland S, Czer LS, Kass RM, De Robertis MA, Mirocha J, Coleman B, Capelli C, Raissi S, Cheng W, Fontana G, Trento A. Pre-existing pulmonary hypertension in patients with end-stage heart failure: impact on clinical outcome and hemodynamic follow-up after orthotopic heart transplantation. J Heart Lung Transplant. 2007;26:312–8.CrossRefPubMedGoogle Scholar
  26. 26.
    Klotz S, Wenzelburger F, Stypmann J, Welp H, Drees G, Schmid C, Scheld HH. Reversible pulmonary hypertension in heart transplant candidates: to transplant or not to transplant. Ann Thorac Surg. 2006;82:1770–3.CrossRefPubMedGoogle Scholar
  27. 27.
    Tedford RJ, Beaty CA, Mathai SC, Kolb TM, Damico R, Hassoun PM, Leary PJ, Kass DA, Shah AS. Prognostic value of the pre-transplant diastolic pulmonary artery pressure-to-pulmonary capillary wedge pressure gradient in cardiac transplant recipients with pulmonary hypertension. J Heart Lung Transplant. 2014;33:289–97.CrossRefPubMedGoogle Scholar
  28. 28.
    Costard-Jackle A, Fowler MB. Influence of preoperative pulmonary artery pressure on mortality after heart transplantation: testing of potential reversibility of pulmonary hypertension with nitroprusside is useful in defining a high risk group. J Am Coll Cardiol. 1992;19:48–54.CrossRefPubMedGoogle Scholar
  29. 29.
    Geft D, Kobashigawa J. Current concepts for sensitized patients before transplantation. Curr Opin Organ Transplant. 2017;22:236–41.CrossRefPubMedGoogle Scholar
  30. 30.
    Patel ND, Weiss ES, Allen JG, Russell SD, Shah AS, Vricella LA, Conte JV. Heart transplantation for adults with congenital heart disease: analysis of the United network for organ sharing database. Ann Thorac Surg. 2009;88:814–21. discussion 821–2CrossRefPubMedGoogle Scholar
  31. 31.
    Kaczmarek I, Deutsch MA, Kauke T, Beiras-Fernandez A, Schmoeckel M, Vicol C, Sodian R, Reichart B, Spannagl M, Ueberfuhr P, Donor-specific HLA. alloantibodies: long-term impact on cardiac allograft vasculopathy and mortality after heart transplant. Exp Clin Transplant. 2008;6:229–35.PubMedGoogle Scholar
  32. 32.
    Kobashigawa J, Mehra M, West L, Kerman R, George J, Rose M, Zeevi A, Reinsmoen N, Patel J, Reed EF, Consensus Conference P. Report from a consensus conference on the sensitized patient awaiting heart transplantation. J Heart Lung Transplant. 2009;28:213–25.CrossRefPubMedGoogle Scholar
  33. 33.
    Nwakanma LU, Williams JA, Weiss ES, Russell SD, Baumgartner WA, Conte JV. Influence of pretransplant panel-reactive antibody on outcomes in 8,160 heart transplant recipients in recent era. Ann Thorac Surg. 2007;84:1556–62. discussion 1562–3CrossRefPubMedGoogle Scholar
  34. 34.
    Kobashigawa J, Crespo-Leiro MG, Ensminger SM, Reichenspurner H, Angelini A, Berry G, Burke M, Czer L, Hiemann N, Kfoury AG, Mancini D, Mohacsi P, Patel J, Pereira N, Platt JL, Reed EF, Reinsmoen N, Rodriguez ER, Rose ML, Russell SD, Starling R, Suciu-Foca N, Tallaj J, Taylor DO, Van Bakel A, West L, Zeevi A, Zuckermann A, Consensus Conference P. Report from a consensus conference on antibody-mediated rejection in heart transplantation. J Heart Lung Transplant. 2011;30:252–69.CrossRefPubMedGoogle Scholar
  35. 35.
    Boucek D, Yetman AT, Yeung E, Miyamoto SD, Stehlik J, Kfoury AG, Kaza AK, Weng C, Everitt MD. Survival based on patient selection for heart transplant in adults with congenital heart disease: a multi-institutional study. Int J Cardiol. 2014;172:e89–90.CrossRefPubMedGoogle Scholar
  36. 36.
    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.CrossRefPubMedGoogle Scholar
  37. 37.
    Everitt MD, Donaldson AE, Stehlik J, Kaza AK, Budge D, Alharethi R, Bullock EA, Kfoury AG, Yetman AT. 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.CrossRefPubMedGoogle Scholar
  38. 38.
    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.CrossRefPubMedPubMedCentralGoogle Scholar
  39. 39.
    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.CrossRefPubMedGoogle Scholar
  40. 40.
    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.CrossRefPubMedGoogle Scholar
  41. 41.
    Chen JM, Davies RR, Mital SR, Mercando ML, Addonizio LJ, Pinney SP, Hsu DT, Lamour JM, Quaegebeur JM, Mosca RS. Trends and outcomes in transplantation for complex congenital heart disease: 1984 to 2004. Ann Thorac Surg. 2004;78:1352–61. discussion 1352–61CrossRefGoogle Scholar
  42. 42.
    Coskun O, Coskun T, El-Arousy M, Parsa MA, Reiss N, Blanz U, Von Knyphausen E, Sandica E, Schulz U, Knobl H, Tenderich G, Bairaktaris A, Kececioglu D, Korfer R. Heart transplantation in adults with congenital heart disease: experience with 15 patients. ASAIO J. 2007;53:103–6.CrossRefPubMedGoogle Scholar
  43. 43.
    Greutmann M, Pretre R, Furrer L, Bauersfeld U, Turina M, Noll G, Luescher TF, Trindade PT. Heart transplantation in adolescent and adult patients with congenital heart disease: a case-control study. Transplant Proc. 2009;41:3821–6.CrossRefPubMedGoogle Scholar
  44. 44.
    Irving C, Parry G, O’Sullivan J, Dark JH, Kirk R, Crossland DS, Chaudhari M, Griselli M, Hamilton JR, Hasan A. Cardiac transplantation in adults with congenital heart disease. Heart. 2010;96:1217–22.CrossRefPubMedGoogle Scholar
  45. 45.
    Izquierdo MT, Almenar L, Martinez-Dolz L, Moro J, Aguero J, Sanchez-Lazaro I, Cano O, Ortiz V, Sanchez R, Salvador A. Mortality after heart transplantation in adults with congenital heart disease: a single-center experience. Transplant Proc. 2007;39:2357–9.CrossRefPubMedGoogle Scholar
  46. 46.
    Jayakumar KA, Addonizio LJ, Kichuk-Chrisant MR, Galantowicz ME, Lamour JM, Quaegebeur JM, Hsu DT. Cardiac transplantation after the Fontan or Glenn procedure. J Am Coll Cardiol. 2004;44:2065–72.CrossRefPubMedGoogle Scholar
  47. 47.
    Seddio F, Gorislavets N, Iacovoni A, Cugola D, Fontana A, Galletti L, Terzi A, Ferrazzi P. Is heart transplantation for complex congenital heart disease a good option? A 25-year single centre experience. Eur J Cardiothoracic Surg. 2013;43:605–11. discussion 611CrossRefGoogle Scholar
  48. 48.
    Shah DK, Deo SV, Althouse AD, Teuteberg JJ, Park SJ, Kormos RL, Burkhart HM, Morell VO. Perioperative mortality is the Achilles heel for cardiac transplantation in adults with congenital heart disease: evidence from analysis of the UNOS registry. J Card Surg. 2016;31:755–64.CrossRefPubMedGoogle Scholar
  49. 49.
    Lamour JM, Addonizio LJ, Galantowicz ME, Quaegebeur JM, Mancini DM, Kichuk MR, Beniaminovitz A, Michler RE, Weinberg A, Hsu DT. Outcome after orthotopic cardiac transplantation in adults with congenital heart disease. Circulation. 1999;100:II200–5.CrossRefPubMedGoogle Scholar
  50. 50.
    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.CrossRefPubMedGoogle Scholar
  51. 51.
    VanderPluym CJ, Cedars A, Eghtesady P, Maxwell BG, Gelow JM, Burchill LJ, Maltais S, Koehl DA, Cantor RS, Blume ED. Outcomes following implantation of mechanical circulatory support in adults with congenital heart disease: an analysis of the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS). J Heart Lung Transplant. 2017;37:88–99.Google Scholar
  52. 52.
    Schweiger M, Falk V, Biry M, Hubler M, Wilhelm MJ. Biventricular failure in dextro-transposition of the great arteries corrected with the Mustard procedure: VAD support of the systemic ventricle is enough. Int J Artif Organs. 2015;38:233–5.CrossRefPubMedGoogle Scholar
  53. 53.
    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.CrossRefPubMedGoogle Scholar
  54. 54.
    Peng E, O'Sullivan JJ, Griselli M, Roysam C, Crossland D, Chaudhari M, Wrightson N, Butt T, Parry G, MacGowan GA, Schueler S, Hasan A. Durable ventricular assist device support for failing systemic morphologic right ventricle: early results. Ann Thorac Surg. 2014;98:2122–9.CrossRefPubMedGoogle Scholar
  55. 55.
    Poh CL, Chiletti R, Zannino D, Brizard C, Konstantinov IE, Horton S, Millar J, d’Udekem Y. Ventricular assist device support in patients with single ventricles: the Melbourne experience dagger. Interact Cardiovasc Thorac Surg. 2017;25:310–6.CrossRefPubMedGoogle Scholar
  56. 56.
    Stokes MB, Saxena P, McGiffin DC, Marasco S, Leet AS, Bergin P. Successful bridge to orthotopic cardiac transplantation with implantation of a HeartWare HVAD in management of systemic right ventricular failure in a patient with transposition of the great arteries and previous atrial switch procedure. Heart Lung Circ. 2016;25:e69–71.CrossRefPubMedGoogle Scholar
  57. 57.
    Awad M, Czer LS, De Robertis MA, Mirocha J, Ruzza A, Rafiei M, Reich H, Trento A, Moriguchi J, Kobashigawa J, Esmailian F, Arabia F, Ramzy D. Adult heart transplantation following ventricular assist device implantation: early and late outcomes. Transplant Proc. 2016;48:158–66.CrossRefPubMedGoogle Scholar
  58. 58.
    Ko BS, Drakos S, Kfoury AG, Hurst D, Stoddard GJ, Willis CA, Delgado JC, Hammond EH, Gilbert EM, Alharethi R, Revelo MP, Nativi-Nicolau J, Reid BB, McKellar SH, Wever-Pinzon O, Miller DV, Eckels DD, Fang JC, Selzman CH, Stehlik J, Utah Transplant Affiliated Hospitals Cardiac Transplant P. Immunologic effects of continuous-flow left ventricular assist devices before and after heart transplant. J Heart Lung Transplant. 2016;35:1024–30.CrossRefPubMedGoogle Scholar
  59. 59.
    Menachem JN, Swaminathan AC, Bashore TM, Ward CC, Rogers JG, Milano CA, Patel CB. Initial experience of left ventricular assist device support for adult patients with transposition of the great vessels. Congenit Heart Dis. 2015;10:382–6.CrossRefPubMedGoogle Scholar
  60. 60.
    Clark JB, Pauliks LB, Myers JL, Undar A. Mechanical circulatory support for end-stage heart failure in repaired and palliated congenital heart disease. Curr Cardiol Rev. 2011;7:102–9.CrossRefPubMedPubMedCentralGoogle Scholar
  61. 61.
    Shi WY, Saxena P, Yong MS, Marasco SF, McGiffin DC, Shipp A, Weintraub RG, d'Udekem Y, Brizard CP, Konstantinov IE. Increasing complexity of heart transplantation in patients with congenital heart disease. Semin Thorac Cardiovasc Surg. 2016;28:487–97.CrossRefPubMedGoogle Scholar
  62. 62.
    Rogers JG, Patel CB, Mentz RJ, Granger BB, Steinhauser KE, Fiuzat M, Adams PA, Speck A, Johnson KS, Krishnamoorthy A, Yang H, Anstrom KJ, Dodson GC, Taylor DH Jr, Kirchner JL, Mark DB, O'Connor CM, Tulsky JA. Palliative care in heart failure: the PAL-HF randomized, controlled clinical trial. J Am Coll Cardiol. 2017;70:331–41.CrossRefPubMedPubMedCentralGoogle Scholar
  63. 63.
    Brunner N, de Jesus Perez VA, Richter A, Haddad F, Denault A, Rojas V, Yuan K, Orcholski M, Liao X. Perioperative pharmacological management of pulmonary hypertensive crisis during congenital heart surgery. Pulm Circ. 2014;4:10–24.CrossRefPubMedPubMedCentralGoogle Scholar
  64. 64.
    Menachem JN. Advanced heart failure in the ACHD population: finding the fellows’ role in a growing field. J Am Coll Cardiol. 2017;69:1986–9.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Heart Failure and Transplantation ProgramVanderbilt University Medical CenterNashvilleUSA
  2. 2.Adult Congenital Heart Disease Program, UHN 62, Knight Cardiovascular InstituteOregon Health & Science UniversityPortlandUSA

Personalised recommendations