Conduction abnormalities in pediatric patients with restrictive cardiomyopathy

Mark A. Walsh, Michelle A. Grenier, John Jefferies, Jeffrey Towbin, Angela Lorts, Richard J. Czosek

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Background-Pediatric restrictive cardiomyopathy carries a poor prognosis secondary to a high risk of sudden death previously attributed to ventricular tachyarrhythmias. The extent of conduction abnormalities in this population and their relationship to life-threatening events has not been previously reported. Methods and Results-A retrospective study of pediatric patients with restrictive cardiomyopathy diagnosed between April 1994 and May 2011 was performed. Demographic, cardiac, and ECG characteristics and the mechanisms of serious arrhythmic events (death or episode of acute hemodynamic compromise thought to be secondary to arrhythmia) were evaluated. Sixteen patients (1-17 years of age) were reviewed, with 5 sudden cardiac events noted, including 4 deaths. Two deaths were caused by development of acute heart block; another patient with syncope had intermittent heart block and survived as the result of pacing features of an implanted defibrillator system. The median PR interval (222 versus 144 ms; P<0.01) and the QRS duration (111 versus 74; P=0.01) were significantly longer in those who had an acute cardiac event. Older age at presentation was associated with sudden cardiac events (P<0.01). No other functional or echocardiographic variables were associated with a sudden cardiac event. Conclusions-Pediatric patients with restrictive cardiomyopathy are at risk for acute high-grade heart block, and, in this cohort, bradycardic events represented a significant portion of all arrhythmic events. Aggressive ECG monitoring strategies looking for conduction system disease should be ongoing in all patients with restrictive cardiomyopathy. Implantation of a defibrillator/pacemaker should be considered as prophylactic management.

Original languageEnglish (US)
Pages (from-to)267-273
Number of pages7
JournalCirculation: Heart Failure
Volume5
Issue number2
DOIs
StatePublished - Mar 1 2012

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Restrictive Cardiomyopathy
Heart Block
Pediatrics
Defibrillators
Electrocardiography
Syncope
Sudden Death
Tachycardia
Cardiac Arrhythmias
Retrospective Studies
Hemodynamics
Demography
Population

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cite this

Conduction abnormalities in pediatric patients with restrictive cardiomyopathy. / Walsh, Mark A.; Grenier, Michelle A.; Jefferies, John; Towbin, Jeffrey; Lorts, Angela; Czosek, Richard J.

In: Circulation: Heart Failure, Vol. 5, No. 2, 01.03.2012, p. 267-273.

Research output: Contribution to journalArticle

Walsh, Mark A. ; Grenier, Michelle A. ; Jefferies, John ; Towbin, Jeffrey ; Lorts, Angela ; Czosek, Richard J. / Conduction abnormalities in pediatric patients with restrictive cardiomyopathy. In: Circulation: Heart Failure. 2012 ; Vol. 5, No. 2. pp. 267-273.
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