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Rate-dependent left bundle-branch block in a child with propionic aciduria

Kipp B Ardoin1, Douglas S Moodie, Christopher S Snyder

  • 1Section of Pediatric Cardiology, Department of Pediatrics, Ochsner Clinic Foundation, New Orleans, LA.

Ochsner Journal
|May 24, 2011
PubMed

Insights

A rare case of rate-dependent left bundle-branch block was observed in a pediatric patient with propionic acidemia and dilated cardiomyopathy. This electrocardiogram finding is typically postoperative but can occur in specific cardiac conditions.

Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Genetics
  • Metabolic Disorders

Background:

  • Left bundle-branch block (LBBB) on electrocardiogram is often a postoperative finding.
  • Rarely, LBBB can be associated with myocardial infarction, hypertrophic cardiomyopathy, or exercise.
  • Propionic acidemia is a rare metabolic disorder that can affect the heart.

Purpose of the Study:

  • To describe a unique case of rate-dependent LBBB in a pediatric patient with propionic acidemia and dilated cardiomyopathy.
  • To highlight the association between metabolic disorders and cardiac conduction abnormalities.

Main Methods:

  • Case report of a pediatric patient.
  • Electrocardiogram (ECG) analysis.
  • Review of relevant medical literature.

Main Results:

  • The patient presented with dilated cardiomyopathy and rate-dependent LBBB.
  • The LBBB pattern was linked to the underlying metabolic disorder and cardiac condition.

Conclusions:

  • Rate-dependent LBBB can occur in pediatric patients with propionic acidemia and dilated cardiomyopathy.
  • This case expands the known associations of LBBB beyond typical etiologies.

Keywords:
Bundle-branch blockcardiac manifestations in propionic aciduriaexercise-induced (rate dependent) bundle-branch blockheart blocknormal heart conductionpropionic aciduria

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