Key result
Malignant ventricular arrhythmias affect ~18% of LMNA carriers, occurring exclusively with two or more risk factors.
Cohort (n=269)
Yes
Answers are grounded in this paper and cite their sources.
In LMNA mutation carriers, the presence of at least two risk factors (NSVT, LVEF <45%, male sex, non-missense mutations) identifies patients at high risk for malignant ventricular arrhythmias who may benefit from an ICD.
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 of clinical phenotypes, including dilated cardiomyopathy and conduction disease. Many LMNA mutation carriers have a poor prognosis, because of a high frequency of MVA and progression to end-stage heart failure. However, it is unclear how to identify mutation carriers that are at risk for MVA. METHODS: In this multicenter cohort of 269 LMNA mutation carriers, we evaluated risk factors for MVA, defined as sudden cardiac death, resuscitation, and appropriate implantable cardioverter-defibrillator (ICD) treatment. RESULTS: In a median follow-up period of 43 months (interquartile range: 17 to 101 months), 48 (18%) persons experienced a first episode of MVA: 11 persons received successful cardiopulmonary resuscitation, 25 received appropriate ICD treatment, and 12 persons died suddenly. Independent risk factors for MVA were nonsustained ventricular tachycardia, left ventricular ejection fraction <45% at the first clinical contact, male sex, and non-missense mutations (ins-del/truncating or mutations affecting splicing). MVA occurred only in persons with at least 2 of these risk factors. There was a cumulative risk for MVA per additional risk factor. CONCLUSIONS: Carriers of LMNA mutations with a high risk of MVA can be identified using these risk factors. This facilitates selection of LMNA mutation carriers who are most likely to benefit from an ICD.
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Rijsingen et al. (2012) conducted a cohort in Lamin A/C (LMNA) mutation carriers (n=269). Risk factors (NSVT, LVEF <45%, male sex, non-missense mutations) vs. Fewer than 2 risk factors was evaluated on Malignant ventricular arrhythmias (sudden cardiac death, resuscitation, and appropriate ICD treatment). Malignant ventricular arrhythmias occurred in 18% of LMNA mutation carriers, exclusively among those with at least 2 risk factors (NSVT, LVEF <45%, male sex, or non-missense mutations).
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