作者
Ingrid AW van Rijsingen, Eline A Nannenberg, Eloisa Arbustini, Perry M Elliott, Jens Mogensen, Johanna F Hermans‐van Ast, Anneke J van der Kooi, J Peter van Tintelen, Maarten P van den Berg, Maurizia Grasso, Alessandra Serio, Sharon Jenkins, Camilla Rowland, Pascale Richard, Arthur AM Wilde, Andreas Perrot, Sabine Pankuweit, Aeilko H Zwinderman, Philippe Charron, Imke Christiaans, Yigal M Pinto
发表日期
2013/4
期刊
European journal of heart failure
卷号
15
期号
4
页码范围
376-384
简介
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 …
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IAW van Rijsingen, EA Nannenberg, E Arbustini… - European journal of heart failure, 2013