PREVALENCE, CLINICAL COURSE AND PATHOLOGICAL FINDINGS OF SYSTOLIC IMPAIRMENT IN HYPERTROPHIC CARDIOMYOPATHY

FERNANDEZ A; VIGLIANO C; CASABE JH; DIEZ M; FAVALORO LE; GUEVARA E; FAVALORO RR; LAGUENS R

Paris

Congreso; European Society of Cardiology Congress 2011; 2011

European Society of Cardiology

Aims: Impaired left ventricular systolic function (ILVSF) in Hypertrophic Cardiomyopathy (HCM) is a risk factor for sudden death and a determinant of high mortality. We determined its prevalence, clinical parameters, long term outcome and pathological findings of explanted hearts. Methods and results: We analyzed 382 HCM patients retrospectively; ILVSF was characterized by a left ventricular ejection fraction (EF) <50% at rest and was identified in 24 (6.3%) patients. ILVSF patients were younger than patients with normal systolic function (NSF); 43.5±14.1 vs. 55.3±20.4 years, (P=0.001), had larger left ventricular end-diastolic cavity diameter: 53.2±12.2 vs. 43.8±6.2 mm, (P=0.001); larger left atrial size 51.2±6.5 vs. 44.3±8 mm, (P<0.001) and lower fractional shortening 30.7±11.1 vs. 45.5±10.3%, (P<0.001). A combined endpoint (heart failure death or heart transplant) was considered. Median follow-up: 3 (1.2-6.3) years. Fourteen ILVSF patients (58.3%) had the endpoint compared with 3 (0.8%) NSF patients, (P<0.001). In explanted hearts, fibrosis represented 30.5±12.5% of the left ventricle; we observed a direct correlation between fibrosis and ventricular dilation (r = 0.794; P=0.001) and an inverse correlation between fibrosis and EF (r = - 0.623; P=0.023). The number and length density of small arterioles (below 50 ìm in diameter) were significantly decreased. Systolic impairment in HCM Conclusions: ILVSF in HCM has a poor prognosis and is associated with both fibrosis and a selective decreased development of small arterioles.