Congenital heart block: development of late-onset cardiomyopathy, a previously underappreciated sequela

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Implantation of Total Artificial Heart in Congenital Heart Disease
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Implantation of Total Artificial Heart in Congenital Heart Disease

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J P Moak1, K S Barron, T J Hougen

  • 1Department of Cardiology, Children's National Medical Center, Washington, DC 20010, USA. jmoak@CNMC.org

Journal of the American College of Cardiology
|January 12, 2001
PubMed

Insights

Infants with congenital heart block (CHB) can develop dilated cardiomyopathy even with early pacing. Close monitoring of ventricular function is crucial for these patients.

Area of Science:

  • Pediatric Cardiology
  • Congenital Heart Disease
  • Electrophysiology

Background:

  • Isolated congenital heart block (CHB) typically has a good prognosis after pacemaker implantation.
  • Delayed pacing or associated heart defects can lead to early mortality in CHB patients.

Purpose of the Study:

  • To identify clinical features and risk factors for late-onset dilated cardiomyopathy in infants with CHB.
  • To assess the long-term outcomes of infants diagnosed with congenital heart block.

Main Methods:

  • A multi-institutional study analyzed 16 infants with congenital CHB.
  • Clinical data, serologic findings, echocardiograms, and myocardial biopsies were reviewed.
  • Cardiac pacing was initiated early in most patients.

Main Results:

  • 10 of 16 infants had findings consistent with neonatal lupus syndrome (NLS).
  • Despite early pacing, left ventricular function significantly decreased over time in most patients.
  • 12 of 16 infants developed congestive heart failure before 24 months; 4 died, and 7 required transplantation.

Conclusions:

  • Early cardiac pacing does not prevent the development of left ventricular cardiomyopathy in some infants with CHB.
  • Continuous monitoring of ventricular function is essential for infants with congenital heart block, in addition to rate and rhythm management.
Abstract

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