Cardiac and Multiorgan Transplantation for End-Stage Congenital Heart Disease

• Published in: Mayo Clinic proceedings Vol. 89; no. 4; pp. 478 - 483
• Main Authors: Robinson, Jeffrey A., MD, Driscoll, David J., MD, O’Leary, Patrick W., MD, Burkhart, Harold M., MD, Dearani, Joseph A., MD, Daly, Richard C., MD, Edwards, Brooks S., MD, Dahl, Sonja H., RN, CNP, Johnson, Jonathan N., MD
• Format: Journal Article
• Language: English
• Published: England Elsevier Inc 01.04.2014; Elsevier, Inc; Elsevier Limited
• Subjects: Academic Medical Centers; Adolescent; Adult; Child; Child, Preschool; Cohort Studies; Confidence Intervals; Congenital heart disease; Critical Illness; Female; Follow-Up Studies; Graft Rejection; Graft Survival; Heart; Heart Defects, Congenital - diagnosis; Heart Defects, Congenital - mortality; Heart Defects, Congenital - surgery; Heart Failure - diagnosis; Heart Failure - mortality; Heart Failure - surgery; Heart Transplantation - methods; Heart Transplantation - mortality; Humans; Infant; Internal Medicine; Kidney Transplantation - methods; Kidney Transplantation - mortality; Liver Transplantation - methods; Liver Transplantation - mortality; Male; Minnesota; Retrospective Studies; Risk Assessment; Risk factors; Survival Rate; Transplantation; Treatment Outcome; Young Adult; Minnesota; congenital heart disease; UNOS; United Network for Organ Sharing; CHD
• ISSN: 0025-6196; 1942-5546
• DOI: 10.1016/j.mayocp.2013.11.019

Abstract Objective To report our single-center experience with patients who had cardiac and multiorgan transplantation for end-stage congenital heart disease (CHD). Patients and Methods We reviewed records for all patients with CHD who had undergone heart transplantation at Mayo Clinic, Rochester, Minnesota, from November 1, 1990, through June 30, 2012. Patients with cardiomyopathy were excluded, unless CHD was present. Results Overall, 45 patients had cardiac transplantation for end-stage CHD (mean age, 26.1±18.4 years; range, 1 month to 65 years). Two patients (4%) had combined heart/liver transplantation; 1 (2%) had heart/kidney transplantation. Six patients (13%) had no previous cardiac operation; the remaining 39 patients had a mean of 3 (range, 1-8) previous cardiac operations. Patient survival (95% CI) at 1, 5, and 10 years was 89% (80%-98%), 89% (80%-98%), and 72% (56%-87%), respectively, while graft survival at 1, 5, and 10 years was 89% (80%-98%), 89% (80%-98%), and 61% (44%-78%), respectively. During the same era, the International Society for Heart & Lung Transplantation reported that survival in patients undergoing transplant for non-congenital diagnoses was 85%, 72%, and 56%, respectively. Over a mean follow-up of 8.7±6.2 years, rejection requiring treatment was documented in 35 patients (78%). Eleven patients (24%) have been diagnosed with neoplasia (8 skin, 1 blood, 1 lymph, and 1 other), and 3 patients (7%) have required retransplantation. Four patients (9%) have developed significant coronary vasculopathy; 1 successfully underwent retransplantation, and 3 died 6, 8, and 14 years after transplantation. Conclusion With appropriate patient selection and posttransplant monitoring, survival has improved for patients with complex end-stage CHD. Multiorgan transplantation is an option for selected patients with CHD.