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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.
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.
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