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Noncompaction cardiomyopathy: a substrate for a thromboembolic event

Marcelo Dantas Tavares de Melo1, José Arimateia Batista de Araújo Filho, Jose Rodrigues Parga Filho

  • 1Heart Institute (InCor) do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. marcelo_dtm@yahoo.com.br.

BMC Cardiovascular Disorders
|January 26, 2015
PubMed

Insights

Noncompaction cardiomyopathy (NCC) is a rare heart condition. Anticoagulation is recommended for patients with ventricular dysfunction, but its safety and benefit in NCC require further clinical trials.

Area of Science:

  • Cardiology
  • Genetics
  • Internal Medicine

Background:

  • Noncompaction cardiomyopathy (NCC) is a rare genetic heart muscle disease.
  • Characterized by a thin compacted epicardial layer and an extensive noncompacted endocardial layer.
  • Clinical issues include ventricular arrhythmia, heart failure, and systemic thromboembolism.

Observation:

  • A 43-year-old male with a history of pulmonary thromboembolism presented with progressive dyspnea.
  • Cardiovascular magnetic resonance imaging revealed severe biventricular trabeculation.
  • Findings included a low ejection fraction (15%), high noncompacted/compacted myocardial thickness ratio (3.2), and biventricular apical thrombus.

Findings:

  • The case highlights the complex management of noncompaction cardiomyopathy.
  • Severe biventricular dysfunction and thrombus formation were observed.
  • The patient's presentation underscores the risk of thromboembolic events in NCC.

Implications:

  • The optimal anticoagulation strategy for NCC patients remains under discussion.
  • Current recommendations include anticoagulation for ventricular systolic dysfunction, embolism history, thrombus, and atrial fibrillation.
  • Clinical trials are needed to establish the safety and efficacy of oral anticoagulation in NCC.
Abstract

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