Risk Factors for Malignant Ventricular Arrhythmias in Lamin A/C Mutation Carriers

摘要: OBJECTIVES: The purpose of this study was to determine risk factors that predict malignant ventricular arrhythmias (MVA) in Lamin A/C (LMNA) mutation carriers. BACKGROUND: LMNA mutations cause a variety clinical phenotypes, including dilated cardiomyopathy and conduction disease. Many carriers have poor prognosis, because high frequency MVA progression end-stage heart failure. However, it is unclear how identify are at for MVA. METHODS: In multicenter cohort 269 carriers, we evaluated MVA, defined as sudden cardiac death, resuscitation, appropriate implantable cardioverter-defibrillator (ICD) treatment. RESULTS: median follow-up period 43 months (interquartile range: 17 101 months), 48 (18%) persons experienced first episode MVA: 11 received successful cardiopulmonary 25 ICD treatment, 12 died suddenly. Independent were nonsustained tachycardia, left ejection fraction <45% the contact, male sex, non-missense (ins-del/truncating or affecting splicing). occurred only with least 2 these factors. There cumulative per additional factor. CONCLUSIONS: Carriers can be identified using This facilitates selection who most likely benefit from an ICD.