title: Comparison of prevalence, clinical course, and pathological findings of left ventricular systolic impairment versus normal systolic function in patients with hypertrophic cardiomyopathy.
abstract: Impaired left ventricular systolic function (ILVSF) in hypertrophic cardiomyopathy (HC) is a risk factor for sudden death and a determinant of high mortality. We determined its prevalence, clinical parameters, long-term outcome, and pathologic findings of explanted hearts. We retrospectively analyzed 382 patients with HC; ILVSF was characterized by LV ejection fraction <50% at rest and was identified in 24 patients (6.3%). Patients with ILVSF were younger than patients with normal SF (43.5 ± 14.1 vs 55.3 ± 20.4 years, p = 0.001) and had larger LV end-diastolic cavity diameter (53.2 ± 12.2 vs 43.8 ± 6.2 mm, p = 0.001), larger left atrium (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 end point (heart failure death or heart transplantation) was considered. Median follow-up was 3 years (1.2 to 6.3). Fourteen patients with ILVSF (58.3%) had the end point compared to 3 (0.8%) with normal SF (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 ejection fraction (r = -0.623, p = 0.023). Number and length density of small arterioles (<50 μm in diameter) were significantly decreased. In conclusion, ILVSF in HC has a poor prognosis and is associated with fibrosis and selective decreased development of small arterioles.
authors.affiliation: Department of Ambulatory Medicine, Favaloro Foundation, University Hospital, Buenos Aires, Argentina. afernandez@ffavaloro.org
authors.forename: Adrián
authors.initials: A
authors.lastname: Fernández
authors.forename: Carlos A
authors.initials: CA
authors.lastname: Vigliano
authors.forename: J Horacio
authors.initials: JH
authors.lastname: Casabé
authors.forename: Mirta
authors.initials: M
authors.lastname: Diez
authors.forename: Liliana E
authors.initials: LE
authors.lastname: Favaloro
authors.forename: Eduardo
authors.initials: E
authors.lastname: Guevara
authors.forename: Roberto R
authors.initials: RR
authors.lastname: Favaloro
authors.forename: Rubén P
authors.initials: RP
authors.lastname: Laguens
mesh_terms: D000328:Adult; D000368:Aged; D002312:Cardiomyopathy, Hypertrophic / Q000473:pathology* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D012307:Risk Factors; D013599:Systole; D018487:Ventricular Dysfunction, Left / Q000453:epidemiology / Q000473:pathology* / Q000503:physiopathology*