Format

Send to

Choose Destination
See comment in PubMed Commons below
Clin Cardiol. 2010 Jul;33(7):430-8. doi: 10.1002/clc.20795.

Long-term outcome of 4 Korean families with hypertrophic cardiomyopathy caused by 4 different mutations.

Author information

1
Division of Cardiology, Department of Medicine, Cardiac and Vascular Centre, Samsung Medical Centre, Sungkyunkwan University School of Medicine, Seoul, South Korea.

Abstract

BACKGROUND:

We sought to describe the long-term outcome of individuals in 4 Korean families with hypertrophic cardiomyopathy (HCM) with known mutations.

HYPOTHESIS:

Long-term clinical features of familial HCM might be characterized according to the mutation causing HCM.

METHODS:

We performed long-term (mean, 13.1 y) clinical evaluations on 46 subjects from 4 Korean families with different mutations.

RESULTS:

Myosin light chain 3 gene (MYL3) mutation was associated with late-onset HCM with relatively poor prognosis; 1 sudden cardiac death and 2 cases of heart failure with atrial fibrillation occurred among 12 subjects with this mutation. Myosin binding protein C gene (MYBPC3) mutation was associated with 2 cases of sudden cardiac death and 3 cases of heart failure among 7 affected members. Cardiac troponin I type 3 gene (TNNI3) mutation was associated with 5 deaths related to atrial fibrillation and stroke among 12 mutation-positive members. Myosin heavy chain 7 gene (MYH7) mutation was associated with 11 deaths in 15 affected members.

CONCLUSIONS:

The clinical course was quite different for different HCM mutations. Even within the same family, individuals carrying the same mutation differed in disease expression and prognosis.

PMID:
20641121
DOI:
10.1002/clc.20795
[Indexed for MEDLINE]
Free full text
PubMed Commons home

PubMed Commons

0 comments

    Supplemental Content

    Full text links

    Icon for Wiley
    Loading ...
    Support Center