Diffuse left ventricular interstitial fibrosis is associated with sub-clinical myocardial dysfunction in Alstrom Syndrome: an observational study.

ABSTRACT:

Background

Alström syndrome is a rare inherited ciliopathy with progressive multisystem involvement. Dilated cardiomyopathy is common in infancy and recurs or presents de novo in adults with high rates of premature cardiovascular death. Although Alström syndrome is characterised by fibrosis in solid organs such as the liver, the pathogenesis of related cardiomyopathy are not clear. To date it is not known whether diffuse interstitial myocardial fibrosis is present before the onset of heart failure symptoms or changes in conventional parameters of left ventricular function.

Methods

In this observational study, 26 patients with Alström syndrome (mean age 27 ± 9 years, 65 % male, 24 h ABPM 130 ± 14 / 77 ± 9 mmHg) without symptomatic cardiovascular disease were recruited from a

SUBMITTER: Edwards NC 

PROVIDER: S-EPMC4483224 | biostudies-literature | 2015 Jun

REPOSITORIES: biostudies-literature