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Published on: July 18, 2014
Survival of patients with familial dilated cardiomyopathy on optimal heart failure therapy
Elisabete Martins1, José Silva Cardoso, Manuel Campelo
1Serviço de Cardiologia, Hospital de São João, Porto, Portugal. elisabetemartins09@gmail.com
Insights
Familial dilated cardiomyopathy (FDCM) patients on optimal medical therapy show a five-year survival rate of 68%. This prognosis is comparable to other non-ischemic dilated cardiomyopathy forms.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Familial dilated cardiomyopathy (FDCM) presents significant clinical and genetic variability.
- Limited survival data exists for FDCM patient subgroups.
Purpose of the Study:
- To assess the long-term prognosis of FDCM patients receiving optimal medical therapy.
- To evaluate cardiovascular morbidity and mortality in a cohort managed in a heart failure clinic.
Main Methods:
- A prospective study was conducted on 36 FDCM patients.
- Patient data was collected according to European Society of Cardiology guidelines.
- Cardiovascular events and mortality were tracked over a mean follow-up of 3.8 years.
Main Results:
- The cohort had a mean age of 42 years, with 67% in NYHA class II.
- Baseline ejection fraction was 28%, with significant LV dilation.
- Five-year survival was 68%, with 5 deaths and 4 heart transplants during follow-up.
Conclusions:
- Optimal medical therapy in FDCM patients yields a five-year survival rate of 68%.
- This survival mirrors outcomes seen in other non-ischemic dilated cardiomyopathy types.
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.
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