188CARDIOMYOPATHY IN MARFAN SYNDROME

Objectives: This report aims to evaluate the incidence of cardiomyopathy in patients with Marfan syndrome (MFS) who underwent surgical management for cardiovascular sequelae. We aim to determine whether cardiomyopathy in MFS is primary or secondary and whether the myocardium is susceptible to ischaemia regardless of myocardial protection used. Likewise, we aim to analyse the long-term outcome of different treatment modalities in Marfan cardiomyopathy. Methods: Between April 1986 and May 2012, 421 MFS patients were surgically treated for cardiovascular manifestations. Among these, 47 (mean age 39.45 ± 12.64 years) had cardiomyopathy. They were grouped into A: ischaemic cardiomyopathy with coronary revascularisation (n = 11), B: end-stage cardiomyopathy (n = 23; 21 underwent heart transplantation and 2 awaiting for donors) and C: end-stage cardiomyopathy who had mechanical circulatory support (n = 13; left ventricular assist device n = 5, biventricular assist device n = 4, total artificial heart n = 4) to support the failing heart. Results: In this series, the overall incidence of cardiomyopathy in patients without prior surgery is 27.6%: group A, 3 (27.2%), group B, 5 (38.4%) and group C, 5 (21.7%). At a mean follow-up of 9.4 ± 1.37 years, overall survival rate is 51.8%. Survival rates based on treatment modality were 54.5% in group A and 50% in both groups B and C. However, there is no significant difference (P = 0.56) in incidence of cardiomyopathy when the type of myocardial protection and duration of ischaemia times were considered. Conclusion: Our finding support the existence of primary cardiomyopathy in a subset of patients with MFS. Whether the type of myocardial protection and duration of ischaemia appear to trigger the development of cardiomyopathy remains unanswered.