Heart Transplantation Outcomes in Patients with Marfan Syndrome: UNOS Registry Analysis

• Published in: The Journal of heart and lung transplantation Vol. 39; no. 4; p. S225
• Main Authors: Genyk, P.A., Nattiv, J., Liu, G.S., Banankhah, P., Kingsford, P.A., Li, J.P., Wolfson, A.M., Vucicevic, D., Vaidya, A.S., DePasquale, E.C.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.04.2020
• ISSN: 1053-2498; 1557-3117
• DOI: 10.1016/j.healun.2020.01.871

Marfan syndrome (MS) is an autosomal dominant connective tissue disorder that causes a defect in the gene fibrillin-1. MS patients develop advanced cardiomyopathies, in which previous studies have demonstrated the presence of a discrete cardiomyopathy associated with MS. Given the vascular complications associated with MS and unclear impact immunosuppression may have on systemic vascular disease, little evidence guides suitability of patients with MS for heart transplant. We sought to investigate long-term heart transplant outcomes of patients with MS listed in the UNOS registry. The UNOS database was queried for all cardiac transplants in the registry from 1987 to 2019 to compare characteristics and outcomes of patients with MS and all other heart transplant patients. Hazard ratios (HR) were calculated using multivariate Cox proportional hazard regression analysis. Survival curves were generated with Kaplan Meier method. Between 1987 and 2019, 22 patients with MS were listed for a heart transplant with a mean age of 35.0 ± 16.3 years. Most patients were male (73%) and Caucasian (73%). Patients with MS were more likely to have prior cardiac surgery (non-transplant) compared to all other heart transplant patients (54.5% vs. 18.3%, p < 0.001). They were as likely to be on life support, including IABP and VAD, but more likely to be on ventilator support (p=0.029). Mean waitlist time were similar, 94.0 days (MS patients) vs 83.0 days (all other patients, p=0.54). There was no significant difference in ischemic time. Transplant survival outcomes were similar at 150 months post-HT (Figure, p=0.69). Survival of Marfan patients was comparable to non-Marfan patients despite increased surgical complexity and greater association with prior cardiac surgery. While further study is warranted, heart transplant is a suitable treatment option in patients with Marfan syndrome and advanced heart failure.