Clinical phenotype and outcome of hypertrophic cardiomyopathy associated with thin-filament gene mutations.

ABSTRACT:

Background

Mild hypertrophy but increased arrhythmic risk characterizes the stereotypic phenotype proposed for hypertrophic cardiomyopathy (HCM) caused by thin-filament mutations. However, whether such clinical profile is different from more prevalent thick-filament-associated disease is unresolved.

Objectives

This study aimed to assess clinical features and outcomes in a large cohort of patients with HCM associated with thin-filament mutations compared with thick-filament HCM.

Methods

Adult HCM patients (age >18 years), 80 with thin-filament and 150 with thick-filament mutations, were followed for an average of 4.5 years.

Results

Compared with thick-filament HCM, patients with thin-filament mutations showed: 1) milder and atypically distributed left ventricula

SUBMITTER: Coppini R 

PROVIDER: S-EPMC4270453 | biostudies-literature | 2014 Dec

REPOSITORIES: biostudies-literature