*Purpose: Marfan syndrome (MS) is an autosomal dominant connective tissue disorder due to a defect in the fibrillin-1 gene. MS patients can develop a discrete cardiomyopathy specific to their MS diagnosis. Due to known vascular complications and unknown effect of chronic immunosuppression in MS, we sought to investigate long-term heart transplant outcomes in MS patients.

*Methods: The UNOS registry was used to compare patient characteristics and transplant outcomes between MS and non-MS patients. Patients were excluded were age <10y, lost-to-follow-up, and multi-organ transplant recipients. Multivariate Cox proportional hazard regression model adjusting for age, sex, race, diabetes, ischemic time, need for dialysis/life support, recipient wait-time, and HLA mismatch. Kaplan Meier survival was censored at 10 years.

*Results: Between 1987 and 2019, 22 patients with MS received heart transplants with a mean age of 35.0 ± 16.3 years of which the majority were male (73%) and Caucasian (73%). Patients with MS were more likely to have had prior non-transplant cardiac surgery (54.5% vs. 18.3%, p < 0.001) and require ventilator support (13.6% vs 4.2%, p=0.029). Mean waitlist times were similar between the two groups (MS vs other: 94.0 days vs 83.0; p=0.54). There was no significant difference in ischemic time. There was no difference in survival post heart transplant (log rank, p=0.69).

*Conclusions: Despite increased prior cardiac surgery and ventilator use, post heart transplant survival for patients with Marfan syndrome was excellent with no difference compared to non-MS patients. While further study is warranted, selected patients with Marfan syndrome should not be excluded from heart transplantation.

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