RESTRICTIVE CARDIOMYOPATHY: ETIOLOGIES AND OUTCOME IN A SINGLE CENTER EXPERIENCE

GREGORIETTI V; PERADEJORDI LASTRA M; BERTOLOTTI A; DIEZ M; FAVALORO LE; TALAVERA L; VIGLIANO C; LAGUENS R; FAVALORO RR

Buenos Aires

Congreso; The 2008 World Congress of Cardiology; 2008

World Heart Federation

Objetives: To assess the etiologies and the prognosis of patients (pts.) with EMB diagnosed RCM during a 15-year follow up in only one center. Material and Methods: We analyzed retrospectively 583 EMB performed between September 1992 to September 2007, All of them were performed for cardiomyopathy diagnosis in patients referred to our Heart Failure Clinic. Fourty two (7,2%) of this 583 EBM were diagnosis as RCM. Mean follow up was 51 months. Results: The mean age of the population was 46 ± 21years; 50% were females. The etiologies were as follows: amyloidosis 42.9% (18 pts.) endomyocardial fibrosis 47.6% (20 pts) and others 9.5% (4 pts.): Pompe's Disease (1), fibroelastosis (2) and mitochondrial abnormalities with restrictive features (1). Eight pts. underwent transplantation( 7 endomyocardial fibrosis and 1 amyloidosis) and 1 patient is on waiting list for heart transplant. The mortality rate for the amyloidosis group was 72% (13 pts.) and for the fibrosis group was 40% (8 pts.) during follow up (p:0.04). A higher mortality rate was observed among male pts: 66% vs. 33%: p:0.03. The survival rate based on the Kaplan Meier curve after the diagnosis of RCM reached 89% at one year, and 50% at 5.6 years. Conclusions: In our experience amyloidosis and endomyocardial fibrosis were the most frequent etiologies in patient with RCM. After diagnosis the mean survival rate was 50% at 5.6 years, being amyloidosis related with worse prognosis.