Background: Familial dilated cardiomyopathy (FDCM) is characterized by clinical and genetic heterogeneity. There are still few survival studies concerning this subgroup of patients.
Aim: To determine the prognosis of patients with FDCM on optimal medical therapy and attending a heart failure clinic.
Methods: This is a prospective study including patients with FDCM, defined according to the guidelines of the European Society of Cardiology. Cardiovascular morbidity and all-cause mortality were evaluated.
Results: Thirty-six patients, 23 (64%) men, were followed for 3.8 +/- 2.5 years. Age at baseline was 42 +/- 14 years and 67% were in NYHA class II. In 22% heart failure symptoms first occurred after a respiratory infection, and in 6%, after pregnancy/delivery. Most patients were in sinus rhythm (89%) and 33% had left bundle branch block (LBBB). Baseline left ventricular (LV) ejection fraction was 28 +/- 9%, LV end-diastolic diameter was 68 +/- 8 mm and left atrial dimension was 46 +/- 9 mm. Baseline serum sodium was 140 +/- 3 mEq/l. All patients were taking angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin-II receptor blockers (ARBs), 81% beta-blockers and 47% spironolactone. During follow-up, 5 patients died, 4 underwent heart transplantation and one received an implantable cardioverter-defibrillator. Five-year survival was 68%.
Conclusions: Five-year survival of our patients with FDCM, under optimal medical therapy, was similar to that of other forms of nonischemic DCM reported in the literature.