Abstract 14750: Mechanical Circulatory Support Devices Among Patients With Familial Dilated Cardiomyopathy

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Introduction: With increasing disease recognition and widespread availability of genetic testing, it is important to characterize the clinical outcomes and prognosis among familial dilated cardiomyopathy (DCM) patients with stage D heart failure requiring a mechanical circulatory support device (MCSD). INTERMACS (INTEragency Registry for Mechanical Assisted Circulatory Devices) was examined to assess the clinical characteristics and outcomes of familial DCM patients who received an MCSD. Methods: DCM patients who received an MCSD between June 2005-December 2017 were classified according to their etiology. The primary outcome was death (with heart transplant as a competing risk) and the secondary outcome was heart transplant (with death as a competing risk). Multivariable-adjusted Cox regression analyses were used to assess the risk of study outcomes across DCM etiologies. Results: Among 19,928 DCM patients, 8,622 (43.3%), 7,091 (35.6%), 568 (2.9%), and 3,647 (18.3%) had ischemic DCM, idiopathic DCM, familial DCM, and DCM due to other causes respectively. Familial DCM patients had the lowest median age at implantation [46 (34, 56) years] compared with other DCM etiologies. Bridge to transplant was the most common device strategy (78.4%) in the familial DCM group (45.6% were listed and an additional 32.8% were eligible for a heart transplant). Familial DCM patients had the lowest adverse events of bleeding, device thrombosis, infection, and respiratory failure post-MCSD implantation. Over a median follow-up of 1.0 (0.4, 2.0) years, familial DCM patients had the lowest risk of death [HR adj : 0.68 (95% CI: 0.55-0.83)] and the highest likelihood of heart transplantation [HR adj : 1.47 (95% CI: 1.28-1.69)] compared with the other DCM groups. Conclusions: Among DCM patients receiving MCSDs, familial DCM patients had the lowest adverse events, the lowest risk of death, and the highest likelihood of heart transplantation, compared with other DCM etiologies.