TY - JOUR
T1 - Risk Factors for Malignant Ventricular Arrhythmias in Lamin A/C Mutation Carriers A European Cohort Study
AU - van Rijsingen, Ingrid A. W.
AU - Arbustini, Eloisa
AU - Elliott, Perry M.
AU - Mogensen, Jens
AU - Hermans-van Ast, Johanna F.
AU - van der Kooi, Anneke J.
AU - van Tintelen, J. Peter
AU - van den Berg, Maarten P.
AU - Pilotto, Andrea
AU - Pasotti, Michele
AU - Jenkins, Sharon
AU - Rowland, Camilla
AU - Aslam, Uzma
AU - Wilde, Arthur A. M.
AU - Perrot, Andreas
AU - Pankuweit, Sabine
AU - Zwinderman, Aeilko H.
AU - Charron, Philippe
AU - Pinto, Yigal M.
PY - 2012
Y1 - 2012
N2 - 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 (insdel/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. (J Am Coll Cardiol 2012; 59: 493-500) (C) 2012 by the American College of Cardiology Foundation
AB - 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 (insdel/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. (J Am Coll Cardiol 2012; 59: 493-500) (C) 2012 by the American College of Cardiology Foundation
U2 - https://doi.org/10.1016/j.jacc.2011.08.078
DO - https://doi.org/10.1016/j.jacc.2011.08.078
M3 - Article
C2 - 22281253
SN - 0735-1097
VL - 59
SP - 493
EP - 500
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
IS - 5
ER -