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.

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Publisher Full Text

Publisher Full Text

Authors

Walsh MA, Grenier MA, Jefferies JL, Towbin JA, Lorts A, Czosek RJ

Institution

Heart Institute, Division of Pediatric Cardiology, Cincinnati Children's Hospital; Medical Center, Cincinnati, OH 45229, USA.

Source

Circulation. Heart failure 5:2 2012 Mar 1 pg 267-73

MeSH

Adolescent
Arrhythmias, Cardiac
Cardiomyopathy, Restrictive
Cause of Death
Child
Child, Preschool
Death, Sudden, Cardiac
Echocardiography
Electrocardiography
Female
Follow-Up Studies
Heart Catheterization
Heart Conduction System
Humans
Incidence
Infant
Male
Ohio
Prognosis
Retrospective Studies
Risk Factors
Survival Rate

Pub Type(s)

Comparative Study
Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

22260945