Primary Prevention of Sudden Death in Patients with Lamin A/C Gene Mutations

Meune, Christophe; Berlo, Jop H. van; Anselme, F.; Bonne, Gisèle; Pinto, Yigal M.; Duboc, Denis

doi:10.1056/nejmc052632

Cited by 334 publications

(230 citation statements)

References 5 publications

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“…In some of these patients, electrical instability, which facilitates AF, might be present with decreased cardiac tissue excitability. The mechanism and grade of atrial failure influences the electrophysiological properties observed during the procedure and affects the steps of the procedure [10,15].…”

Section: Discussionmentioning

confidence: 99%

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Cardiac pacing in 21 patients with Emery-Dreifuss muscular dystrophy: a single-centre study with a 39-year follow-up

Steckiewicz¹,

Stolarz²,

Świętoń³

et al. 2016

Kardiol Pol

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A b s t r a c tBackground: Emery-Dreifuss muscular dystrophy (EDMD) is a genetic condition associated with cardiac arrhythmias. The patients typically develop early, asymptomatic bradyarrhythmia, which may lead to sudden death, preventable with a cardiac implantable electronic device (CIED). EDMD may be characterised by atrial electrical silence. Intra-operative electrophysiological evaluation of the myocardium helps ultimately determine the true nature of the disorder and select an appropriate CIED. Aim:To analyse permanent electrotherapy procedures in EDMD patients: atrial pacing limitations that stem from the electrophysiological properties of the myocardium and long-term follow-up of implanted devices.Methods: A total of 21 EDMD patients (mean age 29 ± 9 years) with a CIED implanted due to bradyarrhythmia were included in the study. The implantation procedures and factors determining the CIED type selection were analysed.Results: CIEDs were implanted in five women and in 16 men with EDMD types 1 and 2 (mean follow-up: 11 ± 8 years). Intra-operatively assessed atrial electrophysiology resulted in changing the planned CIED type during the procedure in three men with EDMD type 1. Eventually, we implanted: eight DDD, one VDD, 11 VVI, and one CD-DR device, with four of the patients' devices switched later from DDD to VVI mode in response to electrophysiological changes in the atria. Conclusions:Intra-operative assessment of atrial electrophysiological properties resulted in changing the planned DDD mode for VVI in 19% of patients with EDMD type 1. Progression of the underlying disease over a 39-year follow-up resulted in a later change of the initially selected pacing mode from DDD to VVI in 40% of cases.

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Section: Discussionmentioning

confidence: 99%

“…Ventricular arrhythmias are the main cause of SCD [9]. An implantable cardioverter-defibrillator (ICD) should be implanted for primary prevention of these arrhythmias [10][11][12].…”

Section: Introductionmentioning

confidence: 99%

Cardiac pacing in 21 patients with Emery-Dreifuss muscular dystrophy: a single-centre study with a 39-year follow-up

Steckiewicz¹,

Stolarz²,

Świętoń³

et al. 2016

Kardiol Pol

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show abstract

“…Possible exceptions are LMNA gene mutation carriers, who may benefit from early implantable cardioverter defibrillator (ICD) therapy if they develop conduction disease. 20 Moreover, a recent study showed that male patients and patients with either nonsustained ventricular tachycardias or left ventricular ejection fraction of o45% carrying a non-missense mutation in LMNA have a higher risk for developing malignant ventricular arrhythmias. 21 …”

Section: 13mentioning

confidence: 99%

Clinical utility gene card for: Dilated Cardiomyopathy (CMD)

et al. 2012

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“…LMNA dilated cardiomyopathy has a more aggressive course than other inherited dilated cardiomyopathies. While sudden death from arrhythmias may be prevented by implantation of a pacemaker and/or implantable defibrillator, the progressive heart failure eventually becomes resistant to treatment (Golzio et al 2007, van Berlo et al 2005, Meune et al, 2006. No drugs are curative and heart transplantation is frequently necessary.…”

Section: Lmna Dilated Cardiomyopathymentioning

confidence: 99%

MEK1/2 Inhibitors to Treat Dilated Cardiomyopathy Caused by LMNA Mutations

Muchir¹

2012

Advances in Protein Kinases

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Primary Prevention of Sudden Death in Patients with Lamin A/C Gene Mutations

Cited by 334 publications

References 5 publications

Cardiac pacing in 21 patients with Emery-Dreifuss muscular dystrophy: a single-centre study with a 39-year follow-up

Cardiac pacing in 21 patients with Emery-Dreifuss muscular dystrophy: a single-centre study with a 39-year follow-up

Clinical utility gene card for: Dilated Cardiomyopathy (CMD)

MEK1/2 Inhibitors to Treat Dilated Cardiomyopathy Caused by LMNA Mutations

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