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Conduction abnormalities in pediatric patients with restrictive cardiomyopathy.

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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  • 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