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Rhythm disorders in isolated left ventricular noncompaction


Steffel, J; Duru, F (2012). Rhythm disorders in isolated left ventricular noncompaction. Annals of Medicine, 44(2):101-108.

Abstract

Both supraventricular and ventricular rhythm disorders are frequently observed in patients with isolated left ventricular noncompaction (IVNC). Most importantly, these patients are prone to develop life-threatening ventricular arrhythmias, which are amongst their most frequent causes of death. Data regarding risk stratification of ventricular arrhythmias, however, are scarce due to the rareness of the disease. Indeed, even invasive electrophysiological studies may be of limited value in this regard in the majority of patients. Implantable cardioverter defibrillators (ICDs) have been demonstrated to be highly effective for the prevention of sudden arrhythmic death in IVNC and should be considered in patients who are clinically judged to be at high risk for ventricular tachyarrhythmias. These include patients with a severely reduced ejection fraction as well as those with a prior history of sustained ventricular tachycardia or fibrillation, recurrent syncope of unknown etiology, or a family history of ventricular tachyarrhythmias or sudden cardiac death. This review summarizes the electrocardiographic and electrophysiological findings in patients with IVNC and discusses possibilities for risk stratification as well as the rationale for ICD implantation for the prevention of sudden cardiac death.

Both supraventricular and ventricular rhythm disorders are frequently observed in patients with isolated left ventricular noncompaction (IVNC). Most importantly, these patients are prone to develop life-threatening ventricular arrhythmias, which are amongst their most frequent causes of death. Data regarding risk stratification of ventricular arrhythmias, however, are scarce due to the rareness of the disease. Indeed, even invasive electrophysiological studies may be of limited value in this regard in the majority of patients. Implantable cardioverter defibrillators (ICDs) have been demonstrated to be highly effective for the prevention of sudden arrhythmic death in IVNC and should be considered in patients who are clinically judged to be at high risk for ventricular tachyarrhythmias. These include patients with a severely reduced ejection fraction as well as those with a prior history of sustained ventricular tachycardia or fibrillation, recurrent syncope of unknown etiology, or a family history of ventricular tachyarrhythmias or sudden cardiac death. This review summarizes the electrocardiographic and electrophysiological findings in patients with IVNC and discusses possibilities for risk stratification as well as the rationale for ICD implantation for the prevention of sudden cardiac death.

Citations

5 citations in Web of Science®
10 citations in Scopus®
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Additional indexing

Item Type:Journal Article, refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Cardiology
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2012
Deposited On:06 Mar 2012 17:03
Last Modified:05 Apr 2016 15:41
Publisher:Informa Healthcare
ISSN:0785-3890
Publisher DOI:10.3109/07853890.2011.554427
PubMed ID:21639721

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