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Gender-specific differences in major cardiac events and mortality in lamin A/C mutation carriers.

Abstract：

AIMS:Mutations in the lamin A/C gene (LMNA) cause a variety of clinical phenotypes, including dilated cardiomyopathy. LMNA is one of the most prevalent mutated genes in dilated cardiomyopathy, and is associated with a high risk of arrhythmias, sudden cardiac death, and heart failure. There are few data on the impact of age and gender on cardiac disease penetrance and mortality. METHODS AND RESULTS:In a multicentre cohort of 269 LMNA mutation carriers, we evaluated gender-specific penetrance of cardiac involvement and major cardiac events. All-cause mortality of mutation carriers [standardized mortality ratio (SMR)] was determined. Cardiac disease penetrance was age dependent and almost complete at the age of 70 years. The presence of an LVEF ≤45% was significantly higher in men (P < 0.001). However, there was no difference between genders in the prevalence of atrioventricular block, atrial tachyarrhythmias, and non-sustained ventricular tachycardia. Malignant ventricular arrhythmias (26% vs. 8%) and end-stage heart failure (28% vs. 14%) were more common in men than in women (P < 0.001 and P = 0.006, respectively). All-cause mortality of mutation carriers was significantly increased [SMR 4.0, 95% confidence interval (CI) 2.8-5.2] between the ages of 15 and 75 years. Mortality in men was higher than in women (hazard ratio 2.2, 95% CI 1.2-4.3). CONCLUSIONS:This large cohort of LMNA mutation carriers demonstrates a high cardiac disease penetrance and a high mortality in mutation carriers. Male mutation carriers have a worse prognosis due to a higher prevalence of malignant ventricular arrhythmias and end-stage heart failure.

journal_name

Eur J Heart Fail

journal_title

European journal of heart failure

authors

van Rijsingen IA,Nannenberg EA,Arbustini E,Elliott PM,Mogensen J,Hermans-van Ast JF,van der Kooi AJ,van Tintelen JP,van den Berg MP,Grasso M,Serio A,Jenkins S,Rowland C,Richard P,Wilde AA,Perrot A,Pankuweit S,Zwinderman

doi

10.1093/eurjhf/hfs191

subject

Has Abstract

pub_date

2013-04-01 00:00:00

pages

376-84

issue

eissn

1388-9842

issn

1879-0844

pii

hfs191

journal_volume

pub_type

杂志文章,多中心研究

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Gender-specific differences in major cardiac events and mortality in lamin A/C mutation carriers.