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Biventricular Noncompaction Cardiomyopathy in a Patient Presenting With a New Cerebrovascular Event

Himani Madnawat1, Issam Atallah1, Ali Ahmad1

  • 1Divisions of Cardiology, Department of Internal Medicine, Saint Louis University School of Medicine, St. Louis, Missouri.

The American Journal of Cardiology
|January 9, 2023
PubMed

Insights

Noncompaction (NC) cardiomyopathy (NCCM) is a rare heart muscle disease from failed myocardial compaction. It increasingly affects adults with heart failure, stroke, and arrhythmias.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • Noncompaction (NC) cardiomyopathy (NCCM) is a rare, genetically heterogeneous cardiomyopathy (CM).
  • It results from the incomplete compaction of the embryonic myocardial wall, leading to prominent trabeculations and deep intertrabecular recesses.

Observation:

  • NCCM primarily affects the left ventricle's apical segment but can present with basal, biventricular, or right ventricular predominance.
  • While predominantly diagnosed in pediatric patients, there is growing recognition in adult populations.

Findings:

  • Adult NCCM cases are increasingly identified in patients presenting with heart failure, stroke, and arrhythmias.
  • The genetic heterogeneity of NCCM contributes to its varied clinical presentations and age of diagnosis.

Implications:

  • Early recognition of NCCM in adults is crucial for timely management.
  • Treatment strategies include managing heart failure symptoms, anticoagulation for stroke prevention, and considering implantable cardiac defibrillators for arrhythmia management.

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