Multi-Organ Transplantation in Adult Congenital Heart Disease: Navigating the Unique Challenges of a Distinct Patient Population

Valeria E. Duarte; Marcus A. Urey; Eric D. Adler; Brenda Merkelz; Mark J. Hobeika; Erik E. Suarez; Andrea G. Quarti; Rayan Yousefzai

doi:10.14797/mdcvj.1576

Multi-Organ Transplantation in Adult Congenital Heart Disease: Navigating the Unique Challenges of a Distinct Patient Population

Methodist DeBakey Cardiovascular Journal

Volume 21 (2025): Issue 3

By: Valeria E. Duarte , Marcus A. Urey , Eric D. Adler , Brenda Merkelz, Mark J. Hobeika , Erik E. Suarez, Andrea G. Quarti and Rayan Yousefzai

Open Access

|May 2025

Stefanescu A, DeFaria Yeh D, Dudzinski DM. Heart failure in adult congenital heart disease. Curr Treat Options Cardiovasc Med. 2014 Sep;16(9):337. doi: 10.1007/s11936-014-0337-y
Open DOI Search in Google Scholar Back to article
Bolger AP, Coats AJ, Gatzoulis MA. Congenital heart disease: the original heart failure syndrome. Eur Heart J. 2003 May;24(10):970-6. doi: 10.1016/s0195-668x(03)00005-8
Open DOI Search in Google Scholar Back to article
Van Der Bom T, Zomer AC, Zwinderman AH, et al. The prevalence of adult congenital heart disease, results from a systematic review and evidence based calculation. Am Heart J. 2012 Oct;164(4):568-75. doi: 10.1016/j.ahj.2012.07.023
Open DOI Search in Google Scholar Back to article
Gilboa SM, Devine OJ, Kucik JE, et al. Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010. Circulation. 2016 Jul 12;134(2):101-9. doi: 10.1161/CIRCULATIONAHA.115.019307
Open DOI Search in Google Scholar Back to article
Oechslin EN, Harrison DA, Connelly MS, Webb GD, Siu SC. Mode of death in adults with congenital heart disease. Am J Cardiol. 2000 Nov 15;86(10):1111-6. doi: 10.1016/s0002-9149(00)01169-3
Open DOI Search in Google Scholar Back to article
Pillutla P, Shetty KD, Foster E. Mortality associated with adult congenital heart disease: Trends in the US population from 1979 to 2005. Am Heart J. 2009 Nov;158(5):874-9. doi: 10.1016/j.ahj.2009.08.014
Open DOI Search in Google Scholar Back to article
OPTN [Internet]. Richmond, VA: Organ Procurement & Transplantation Network; c2025. UNOS Data and trends; 2025 [cited 2025 Apr 22]. Available from: https://optn.transplant.hrsa.gov/data/view-data-reports/national-data
Search in Google Scholar Back to article
Alshawabkeh LI, Hu N, Carter KD, et al. Wait-list outcomes for adults with congenital heart disease listed for heart transplantation in the U.S. J Am Coll Cardiol. 2016 Aug 30;68(9):908-17. doi: 10.1016/j.jacc.2016.05.082
Open DOI Search in Google Scholar Back to article
Lewis M, Ginns J, Schulze C, 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 Jul;22(7):578-82. doi: 10.1016/j.cardfail.2015.09.002
Open DOI Search in Google Scholar Back to article
Reiter FP, Hadjamu NJ, Nagdyman N, et al. Congenital heart disease-associated liver disease: A narrative review. Cardiovasc Diagn Ther. 2021 Apr;11(2):577-590. doi: 10.21037/cdt-20-595
Open DOI Search in Google Scholar Back to article
Emamaullee J, Zaidi AN, Schiano T, et al. Fontan-associated liver disease: Screening, management, and transplant considerations. Circulation. 2020 Aug 11;142(6):591-604. doi: 10.1161/CIRCULATIONAHA.120.045597
Open DOI Search in Google Scholar Back to article
Gordon-Walker TT, Bove K, Veldtman G. Fontan-associated liver disease: A review. J Cardiol. 2019 Sep;74(3):223-232. doi: 10.1016/j.jjc.2019.02.016
Open DOI Search in Google Scholar Back to article
Lui GK, Saidi A, Bhatt AB, et al. Diagnosis and management of noncardiac complications in adults with congenital heart disease: A scientific statement from the American Heart Association. Circulation. 2017 Nov 14;136(20):e348-e392. doi: 10.1161/CIR.0000000000000535
Open DOI Search in Google Scholar Back to article
Weisberg IS, Jacobsob IM. Cardiovascular diseases and the liver. Clin Liver Dis. 2011 Feb;15(1):1-20. doi: 10.1016/j.cld.2010.09.010
Open DOI Search in Google Scholar Back to article
Morgan C, Al-Aklabi M, Garcia Guerra G. Chronic kidney disease in congenital heart disease patients: a narrative review of evidence. Can J Kidney Health Dis. 2015 Aug 11;2:27. doi: 10.1186/s40697-015-0063-8
Open DOI Search in Google Scholar Back to article
Nashat FS, Portal RW. The effects of changes in hematocrit on renal function. J Physiol. 1967 Dec;1933(3):513-22. doi: 10.1113/jphysiol.1967.sp008375
Open DOI Search in Google Scholar Back to article
Spear GS. Glomerular alterations in cyanotic congenital heart disease. Bull Johns Hopkins Hosp. 1960 Jun;106:347-67. PMID: 13833192
Search in Google Scholar Back to article
Le Jemtel TH, Rajapreyar I, Selby MG, et al. Direct evidence of podocyte damage in cardiorenal syndrome type 2: Preliminary evidence. Cardiorenal Med. 2015 Apr;5(2):125-34. doi: 10.1159/000375130
Open DOI Search in Google Scholar Back to article
Pradhan SK, Adnani H, Safadi R, et al. Cardiorenal syndrome in the pediatric population: A systemic review. Ann Pediatr Cardiol. 2023;15(5-6):493-510. doi: 10.4103/apc.apc_50_22
Open DOI Search in Google Scholar Back to article
Mirzai S, Eck BL, Chen PH, et al. Current approach to the diagnosis of sarcopenia in heart failure: A narrative review on the role of clinical and imaging assessments. Circ Heart Fail. 2022 Oct;15(10):e009322. doi: 10.1161/CIRCHEARTFAILURE.121.009322
Open DOI Search in Google Scholar Back to article
Moreno-Gonzalez R, Cruzado JM, Corsonello A, et al. Kidney function and other associated factors of sarcopenia in community-dwelling older adults: The SCOPE study. Eur J Intern Med. 2024 May;123:81-93. doi: 10.1016/j.ejim.2023.12.002
Open DOI Search in Google Scholar Back to article
Ojo AO, Held PJ, Port FK, et al. Chronic Renal Failure after Transplantation of a Nonrenal Organ. N Engl J Med. 2003 Sep 4;349(10):931-40. doi: 10.1056/NEJMoa021744
Open DOI Search in Google Scholar Back to article
Jone PN, Ivy DD, Hauck A, et al. Pulmonary hypertension in congenital heart disease: A scientific statement from the American Heart Association. Circ Heart Fail. 2023 Jul;16(7):e00080. doi: 10.1161/HHF.0000000000000080
Open DOI Search in Google Scholar Back to article
Nakamura Y, Yagihara T, Kagisaki K, et al. Pulmonary arteriovenous malformations after a Fontan operation in the left isomerism and absent vena cava. Eur J Cardiothorac Surg. 2009 Jul;36(1):69-76. doi: 10.1016/j.ejcts.2009.02.046
Open DOI Search in Google Scholar Back to article
Alonso-Gonzalez R, Borgia F, Diller GP, et al. Abnormal lung function in adults with congenital heart disease: Prevalence, relation to cardiac anatomy, and association with survival. Circ Cardiovasc Imaging. 2013 Feb 26;127(8):882-90. doi: 10.1161/CIRCULATIONAHA.112.126755
Open DOI Search in Google Scholar Back to article
Shah MJ, Rychick J, Fogel MA, et al. Pulmonary arteriovenous malformations after superior cavopulmonary connection: Resolution after inclusion of hepatic veins in the pulmonary circulation. Ann Thorac Surg. 1997 Apr;63(4):960-3. doi: 10.1016/s0003-4975(96)00961-7
Open DOI Search in Google Scholar Back to article
McElhinney DB, Kreutzer J, Lang P, et al. Incorporation of the hepatic veins into the cavopulmonary circulation in patients with heterotaxy and pulmonary arteriovenous malformations after Kawashima procedure. Ann Thorac Surg. 2005 Nov;80(5):1597-603. doi: 10.1016/j.athoracsur.2005.05.101
Open DOI Search in Google Scholar Back to article
Singampalli KL, Jui E, Shani K, et al. Congenital Heart Disease: An Immunologic Perspective. Front Cardiovasc Med. 2021 Aug 9;8:701375. doi: 10.3389/fcvm.2021.701375
Open DOI Search in Google Scholar Back to article
Parikh S, Bharucha B, Kamdar S, Kshirsagar N. Polymorphonuclear leukocyte functions in children with cyanotic and acyanotic congenital heart disease. Indian Pediatr. 1993 Jul:30(7):883-90. PMID: 8132280
Search in Google Scholar Back to article
Khalil A, Trehan R, Tiwari A, Malik R, Arora R. Immunological profile in congenital heart disease. Indian Pediatr. 1994 Mar:31(3):295-300. PMID: 7896364
Search in Google Scholar Back to article
Rhoden DK, Leatherbury L, Helman S, Gaffney M, Strong WB, Guill MF. Abnormalities in lymphocyte populations in infants with neural crest cardiovascular defects. Pediatr Cardiol. 1996 May-Jun:17(3):143-9. doi: 10.1007/BF02505203
Open DOI Search in Google Scholar Back to article
Sharma R, Bolger AP, Li W, et al. Elevated circulating levels of inflammatory cytokines and bacterial endotoxins in adults with congenital heart disease. Am J Cardiol. 2003 Jul 15:92(2):188-93. doi: 10.1016/S0002-9149(03)00536-8
Open DOI Search in Google Scholar Back to article
Bearly S, Gentle TA, Baynham MID, Roberts KD, Abrams LD, Thompson RA. Immunodeficiency Following Neonatal Thymectomy in Man. Clin Exp Immunol. 1987 Nov:70(2):322-7. PMID: 3427824
Search in Google Scholar Back to article
Silva SL, Albuquerque A, Amaral AJ, et al. Autoimmunity and Allergy Control in Adults Submitted to Complete Thymectomy Early in Infancy. PLoS One. 2017 Jul 7:12(7):e0180385. doi: 10.1371/journal.pone.0180385
Open DOI Search in Google Scholar Back to article
van den Broek T, Madi A, Delemarre EM, et al. Human Neonatal Thymectomy Induces Altered B-Cell Responses and Autoreactivity. Eur J Immunol. 2017 Nov:47(11):1970-81. doi: 10.1098/rspb.eji.201746971
Open DOI Search in Google Scholar Back to article
Gudmundsdottir J, Oskardsdottir S, Skogberg G, et al. Early Thymectomy Leads to Premature Immunologic Ageing: An 18-Year Follow-Up. J Allergy Clin Immunol. 2016 Nov:138(5):1439-43.e10. doi: 10.1016/j.jaci.2016.05.14
Open DOI Search in Google Scholar Back to article
van den Broek T, Broek T, Delemarre E, et al. Neonatal Thymectomy Reveals Differentiation and Plasticity Within Human Naïve T Cells. J Clin Invest. 2016 Mar 1:126(3):1126-36. doi: 10.1172/JCI84997
Open DOI Search in Google Scholar Back to article
Cavalcanti NV, Palmeira P, Jatene MB, de Barros Dorna M, Carneiro-Sampaio M. Early Thymectomy Is Associated With Long-Term Impairment of the Immune System: A Systematic Review. Front Immunol. 2021 Nov 25:12:774780. doi: 10.3389/fimmu.2021.774780
Open DOI Search in Google Scholar Back to article
Morsheimer MM, Rychik J, Forbes L, Dodds K, Goldberg DJ, Sullivan K, Heimall JR. Risk Factors and Clinical Significance of Lymphopenia in Survivors of the Fontan Procedure for Single-Ventricle Congenital Cardiac Disease. J Allergy Clin Immunol. 2016 May-Jun:4(3):491-6. doi: 10.1016/j.jaip.2015.11.034
Open DOI Search in Google Scholar Back to article
Ohuchi H. Adult patients with Fontan circulation: What we know and how to manage adults with Fontan circulation? J Cardiol. 2016 Sep:68(3):181-9. doi: 10.1016/j.jjcc.2016.04.001
Open DOI Search in Google Scholar Back to article
Kaufman BD, Shaddy RE. Immunologic Considerations in Heart Transplantation for Congenital Heart Disease. Curr Cardiol Rev. 2011 May:7(2):67-71. doi: 10.2174/157340311797484204
Open DOI Search in Google Scholar Back to article
Habal M. Current Desensitization Strategies in Heart Transplantation. Front Immunol. 2021 Aug 24:12:702186. doi: 10.3389/fimmu.2021.702186
Open DOI Search in Google Scholar Back to article
Ross HJ, Law Y, Book WM, Broberg CS, Burchill L, Cecchin F. Transplantation and Mechanical Circulatory Support in Congenital Heart Disease: A Scientific Statement From the American Heart Association. Circulation. 2016 Feb 23:133(8):802-20. doi: 10.1161/CIR0000000000000353
Open DOI Search in Google Scholar Back to article
Alshawabkeh L, Herrick NL, Opotowsky AR, et al. The role of sensitization in post-transplant outcomes in adults with congenital heart disease sensitization in adults with congenital heart disease. Inter J Cardiol Congenital Heart Dis. 2022 Apr 25:8:100384. doi: 10.1016/j.ijcchd.2022.100384
Open DOI Search in Google Scholar Back to article
Margolin EA, Adler ED, Alshawabkeh L, Riedl MA, Urey MA. Hypogammaglobinemia in Patients Palliated with the Fontan Circulation: Implications for Cardiac Transplantation. J Heart Lung Transplant. 2022 Apr:41(4 Sup):S436. doi: 10.1016/j.healun.2022.01.1101
Open DOI Search in Google Scholar Back to article
Daly RC, Rosenbaum AN, Dearani JA, et al. Heart-After-Liver Transplantation Attenuates Rejection of Cardiac Allografts in Sensitized Patients. J Am Coll Cardiol. 2021 Mar 16;77(10):1331-1340. doi: 10.1016/j.jacc.2021.01.013
Open DOI Search in Google Scholar Back to article
Chou AS, Habertheuer A, Chin AL, Sultan I, Vallabhajosyula P. Heart-Kidney and Heart-Liver Transplantation Provide Immunoprotection to the Cardiac Allograft. Ann Thorac Surg. 2019 Aug;108(2):458-66. doi: 10.1016/j.athoracsur.2019.02.012
Open DOI Search in Google Scholar Back to article
Lewis MJ, Reardon LC, Aboulhosn J, et al. Clinical Outcomes of Adult Fontan-Associated Liver Disease and Combined Heart-Liver Transplantation. J Am Coll Cardiol. 2023 Jun 6;81(22):2149-60. doi: 10.1016/j.jacc.2023.03.421
Open DOI Search in Google Scholar Back to article
Duarte VE, Rajpal S. The Role of Multimodality Imaging in the Evaluation of Heart Failure and Surgical Transplant Planning of Patients with Adult Congenital Heart Disease. Heart Fail Clin 2024 Apr;20(2):189-98. doi: 10.1016/j.hfc.2024.01.002
Open DOI Search in Google Scholar Back to article
Cohen S, Houyel L, Guillemain R, et al. Temporal trends and changing profile of adults with congenital heart disease undergoing heart transplantation. Eur Heart J. 2016 Mar 1;37(9):783-9. doi: 10.1093/eurheartj/ehv62
Open DOI Search in Google Scholar Back to article
Heid CA, Chandra R, Liu C, et al. Cardiac transplantation in adults with congenital heart disease: A single center case series. Clin Transplant. 2021 Oct;35(10):e14430. doi: 10.1111/ctr.14430
Open DOI Search in Google Scholar Back to article
Shah DK, Deo SV, Althouse AD, et al. 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 Dec;31(12):755-64. doi: 10.1111/jocs.12857
Open DOI Search in Google Scholar Back to article
Reardon LC, Lin JP, VanArsdell GS, et al. Orthotopic Heart and Combined Heart Liver Transplantation: the Ultimate Treatment Option for Failing Fontan Physiology. Curr Transplant Rep. 2021;8(1):9-20. doi: 10.1007/s40472-021-00315-4
Open DOI Search in Google Scholar Back to article
Riggs KW, Zafar F, Radzi Y, et al. Adult Congenital Heart Disease: Current Early Expectations After Cardiac Transplantation. Ann Thorac Surg. 2020 Feb;109(2):480-6. doi: 10.1016/j.athoracsur.2019.06.067
Open DOI Search in Google Scholar Back to article
Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971 May;26(3):240-8. doi: 10.1136/thx.26.3.240
Open DOI Search in Google Scholar Back to article
Agnoletti G, Ferraro G, Bordese R, et al. Fontan circulation causes early severe liver damage. Should we offer patients a tailored strategy? Int J Cardiol. 2016 Apr;209:60-5. doi: 10.1016/j.ijcard.2016.02.041
Open DOI Search in Google Scholar Back to article
Keung CY, Zentner D, Gibson RN, et al. Fontan-associated liver disease: pathophysiology, investigations, predictors of severity and management. Eur J Gastroenterol. 2020 Aug;32(8):907-915. doi: 10.1097/MEG.0000000000001641
Open DOI Search in Google Scholar Back to article
DiPaola FW, Schumacher KR, Goldberg CS, et al. Fontan operation on liver stiffness in children with single ventricle physiology. Eur Radiol. 2017 Oct 17;27(6):2434-2442. doi: 10.1007/s00330-016-4614-x
Open DOI Search in Google Scholar Back to article
Chavhan GB, Farras Roca L, Coblentz AC. Liver magnetic resonance imaging: how we do it. Pediatr Radiol. 2022 Feb;52(2):167-176. doi: 10.1007/s00247-021-05053-4
Open DOI Search in Google Scholar Back to article
Sganga D, Hollander SA, Vaikunth S, et al. Comparison of combined heart-liver vs heart-only transplantation in pediatric and young adult Fontan recipients. J Heart Lung Transplant. 2021 Apr:40(4):298-306. doi: 10.1016/j.healun.2020.12.008
Open DOI Search in Google Scholar Back to article
Starzl T, Bahnson H, Hardesty R, et al. Heart-liver transplantation in a patient with familial hypercholesterolemia. Lancet. 1984 Jun 23;1(8391):1382-1383. doi: 10.1016/s0140-6736(84)91876-2
Open DOI Search in Google Scholar Back to article
Margreiter R. History of Lung and Heart-Lung Transplantation, With Special Emphasis on German-Speaking Countries. Transplant Proc. 2016;48(8):2779-2781. PMID: 27788817. doi: 10.1016/j.transproceed.2016.07.015
Open DOI Search in Google Scholar Back to article
OPTN [Internet]. Richmond, VA: Organ Procurement & Transplantation Network; c2025. National Data; 2025 [cited 2025 Apr 29]. Available from: https://optn.transplant.hrsa.gov/data/view-data-reports/national-data/
Search in Google Scholar Back to article
Lutz AJ, Nagaraju S, Sharfuddin AA, et al. Simultaneous Heart-kidney Transplant With Planned Delayed Implantation of the Kidney Graft After Ex Vivo Perfusion. Transplantation. 2023 Sep 1;107(9):2043-2046. Epub 2023 Jun 5. PMID: 37271866. doi: 10.1097/TP.0000000000004661
Open DOI Search in Google Scholar Back to article
Feizpour CA, Hoffman J, Patel MS, et al. Simultaneous ex vivo normothermic preservation of liver and heart grafts from a donation after circulatory death donor. J Card Surg. 2022 Apr;37(4):1076-1079. Epub 2022 Jan 28. PMID: 35092068. doi: 10.1111/jocs.16269
Open DOI Search in Google Scholar Back to article
Das T, Rampersad P, Ghobrial J. Caring for the critically ill adult congenital heart disease patient. Curr Cardiol Rep. 2024;26(5):283-291. doi: 10.1007/s11886-024-02034-5
Open DOI Search in Google Scholar Back to article
Kovacs AH, Brouillette J, Ibeziako P, et al. Psychological outcomes and interventions for individuals with congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2022;15(8):e000110. doi: 10.1161/HCQ.0000000000000110
Open DOI Search in Google Scholar Back to article
Shonkoff JP, Garner AS. Committee on psychosocial aspects of child and family health; committee on early childhood, adoption, and dependent care; section on developmental and behavioral pediatrics. The lifelong effects of early childhood adversity and toxic stress. Pediatrics. 2012 Jan;129(1):e232-e246. doi: 10.1542/peds.2011-2663
Open DOI Search in Google Scholar Back to article
Peled Y, Ducharme A, Kittleson M, et al. International Society for Heart and Lung Transplantation Guidelines for the evaluation and care of cardiac transplant candidates—2024. J Heart Lung Transplant. 2024 Oct;43(10):1529-1628. 10.1016/j.healun.2024.05.010
Open DOI Search in Google Scholar Back to article
Kim MY, Johnson JL, Sawatzky R. Relationship between types of social support, coping strategies, and psychological distress in individuals living with congenital heart disease. J Cardiovasc Nurs. 2019 Jan 2;34(1):76-84. doi: 10.1097/JCN.0000000000000531
Open DOI Search in Google Scholar Back to article
Stout KK, Broberg CS, Book WM, et al. Chronic heart failure in congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2016;133:770-801. 10.1161/CIR.0000000000000352
Open DOI Search in Google Scholar Back to article
de la Rosa AL, Singer-Englar T, Tompkins RO, et al. Advanced heart failure and heart transplantation in adult congenital heart disease in the current era. Clin Transplant. 2021 Aug 8;35(11):e14451. 10.1111/ctr.14451
Open DOI Search in Google Scholar Back to article
Bhama JK, Shulman J, Bermudez CA, et al. Heart transplantation for adults with congenital heart disease: results in the modern era. J Heart Lung Transplant. 2013 May;32(5):499-504. 10.1016/j.healun.2013.01.1047
Open DOI Search in Google Scholar Back to article
Nguyen VP, Dolgner SJ, Dardas TF, et al. Improved outcomes of heart transplantation in adults with congenital heart disease receiving regionalized care. J Am Coll Cardiol. 2019 Dec 10;74(23):2908-18. 10.1016/j.jacc.2019.09.062
Open DOI Search in Google Scholar Back to article

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DOI: https://doi.org/10.14797/mdcvj.1576 | Journal eISSN: 1947-6108

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Language: English

Page range: 72 - 82

Submitted on: Jan 31, 2025

Accepted on: Apr 9, 2025

Published on: May 15, 2025

Published by: Houston Methodist DeBakey Heart & Vascular Center

In partnership with: Paradigm Publishing Services

Keywords:

adult congenital heart disease,

multi-organ transplant,

heart transplant,

complication associated with adult congenital heart disease

© 2025 Valeria E. Duarte, Marcus A. Urey, Eric D. Adler, Brenda Merkelz, Mark J. Hobeika, Erik E. Suarez, Andrea G. Quarti, Rayan Yousefzai, published by Houston Methodist DeBakey Heart & Vascular Center
This work is licensed under the Creative Commons Attribution-NonCommercial 4.0 License.

Volume 21 (2025): Issue 3