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

Ingrid A. W. van Rijsingen; 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; Andrea Pilotto; Michele Pasotti; Sharon Jenkins; Camilla Rowland; Uzma Aslam; Arthur A. M. Wilde; Andreas Perrot; Sabine Pankuweit; Aeilko H. Zwinderman; Philippe Charron; Yigal M. Pinto

doi:10.1016/j.jacc.2011.08.078

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

Ingrid A. W. van Rijsingen
, 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
, Andrea Pilotto
, Michele Pasotti
, Sharon Jenkins
, Camilla Rowland
, Uzma Aslam
, Arthur A. M. Wilde
, Andreas Perrot
, Sabine Pankuweit
, Aeilko H. Zwinderman
, Philippe Charron
, Yigal M. Pinto^*

^*Corresponding author for this work

Cardiovascular Centre (CVC)

Research output: Contribution to journal › Article › Academic › peer-review

468 Citations (Scopus)

Abstract

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

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

Original language	English
Pages (from-to)	493-500
Number of pages	8
Journal	Journal of the American College of Cardiology
Volume	59
Issue number	5
DOIs	https://doi.org/10.1016/j.jacc.2011.08.078
Publication status	Published - 31-Jan-2012

Keywords

cardiomyopathy
implantable cardioverter-defibrillator
lamin A/C
risk factors
sudden cardiac death
FAMILIAL DILATED CARDIOMYOPATHY
DREIFUSS MUSCULAR-DYSTROPHY
CONDUCTION-SYSTEM DISEASE
GENE-MUTATIONS
SUDDEN-DEATH
LAMINOPATHIES

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van Rijsingen, I. A. W., Arbustini, E., Elliott, P. M., Mogensen, J., Hermans-van Ast, J. F., van der Kooi, A. J., van Tintelen, J. P., van den Berg, M. P., Pilotto, A., Pasotti, M., Jenkins, S., Rowland, C., Aslam, U., Wilde, A. A. M., Perrot, A., Pankuweit, S., Zwinderman, A. H., Charron, P., & Pinto, Y. M. (2012). Risk Factors for Malignant Ventricular Arrhythmias in Lamin A/C Mutation Carriers A European Cohort Study. Journal of the American College of Cardiology, 59(5), 493-500. https://doi.org/10.1016/j.jacc.2011.08.078

@article{642b10e347ea4cabb6a8c71239093077,

title = "Risk Factors for Malignant Ventricular Arrhythmias in Lamin A/C Mutation Carriers A European Cohort Study",

abstract = "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 fractionConclusions 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",

keywords = "cardiomyopathy, implantable cardioverter-defibrillator, lamin A/C, risk factors, sudden cardiac death, FAMILIAL DILATED CARDIOMYOPATHY, DREIFUSS MUSCULAR-DYSTROPHY, CONDUCTION-SYSTEM DISEASE, GENE-MUTATIONS, SUDDEN-DEATH, LAMINOPATHIES",

author = "\{van Rijsingen\}, \{Ingrid A. W.\} and Eloisa Arbustini and Elliott, \{Perry M.\} and Jens Mogensen and \{Hermans-van Ast\}, \{Johanna F.\} and \{van der Kooi\}, \{Anneke J.\} and \{van Tintelen\}, \{J. Peter\} and \{van den Berg\}, \{Maarten P.\} and Andrea Pilotto and Michele Pasotti and Sharon Jenkins and Camilla Rowland and Uzma Aslam and Wilde, \{Arthur A. M.\} and Andreas Perrot and Sabine Pankuweit and Zwinderman, \{Aeilko H.\} and Philippe Charron and Pinto, \{Yigal M.\}",

year = "2012",

month = jan,

day = "31",

doi = "10.1016/j.jacc.2011.08.078",

language = "English",

volume = "59",

pages = "493--500",

journal = "Journal of the American College of Cardiology",

issn = "0735-1097",

publisher = "ELSEVIER SCIENCE INC",

number = "5",

}

van Rijsingen, IAW, Arbustini, E, Elliott, PM, Mogensen, J, Hermans-van Ast, JF, van der Kooi, AJ, van Tintelen, JP, van den Berg, MP, Pilotto, A, Pasotti, M, Jenkins, S, Rowland, C, Aslam, U, Wilde, AAM, Perrot, A, Pankuweit, S, Zwinderman, AH, Charron, P & Pinto, YM 2012, 'Risk Factors for Malignant Ventricular Arrhythmias in Lamin A/C Mutation Carriers A European Cohort Study', Journal of the American College of Cardiology, vol. 59, no. 5, pp. 493-500. https://doi.org/10.1016/j.jacc.2011.08.078

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 - 2012/1/31

Y1 - 2012/1/31

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 fractionConclusions 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 fractionConclusions 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

KW - cardiomyopathy

KW - implantable cardioverter-defibrillator

KW - lamin A/C

KW - risk factors

KW - sudden cardiac death

KW - FAMILIAL DILATED CARDIOMYOPATHY

KW - DREIFUSS MUSCULAR-DYSTROPHY

KW - CONDUCTION-SYSTEM DISEASE

KW - GENE-MUTATIONS

KW - SUDDEN-DEATH

KW - LAMINOPATHIES

U2 - 10.1016/j.jacc.2011.08.078

DO - 10.1016/j.jacc.2011.08.078

M3 - Article

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 -

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

Abstract

Keywords

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