Atypical left bundle branch block in dilative "burned-out" phase of hypertrophic cardiomyopathy

Juan Benezet-Mazuecos1, Borja Ibanez, Jeronimo Farre

  • 1Servicio de Cardiología, Fundación Jiménez Díaz, Universidad Autónoma de Madrid, Madrid, Spain. jbenezet@yahoo.es

Insights

This case study highlights a rare instance of hypertrophic cardiomyopathy (HC) progressing to an end-stage, or "burned-out," phase. Unusual electrocardiogram (ECG) findings may signal this advanced stage of heart failure.

Area of Science:

  • Cardiology
  • Internal Medicine
  • Pathophysiology

Background:

  • Hypertrophic cardiomyopathy (HC) is a genetic heart condition.
  • Left ventricular remodeling is a key feature in HC pathophysiology.
  • Some HC patients paradoxically develop left ventricular wall thinning and systolic dysfunction.

Observation:

  • A 70-year-old male presented with congestive heart failure.
  • The patient had a 22-year history of hypertrophic cardiomyopathy.
  • Electrocardiogram (ECG) revealed a unique left bundle branch block (LBBB).
  • 2D-echocardiogram showed a dilated left ventricle with a 25% ejection fraction.

Findings:

  • The patient's presentation represents the rare "end-stage" or "burned-out" phase of HC.
  • This phase is characterized by systolic dysfunction and heart failure, even without coronary artery disease.
  • The specific LBBB pattern observed is a unique manifestation of this advanced HC stage.

Implications:

  • ECG changes, particularly rare LBBB patterns, may serve as early indicators of the "burned-out" phase in HC.
  • Understanding these ECG alterations can aid in early detection and management of advanced hypertrophic cardiomyopathy.
  • Further research into the mechanisms of left ventricular wall thinning in HC is warranted.

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