Key result
A young man with isolated noncompaction of the right ventricle presented with progressive right heart failure and atrial fibrillation, confirmed by echocardiography and cardiac MRI.
Case Report (n=1)
This case highlights a rare presentation of isolated noncompaction of the right ventricle, manifesting with right heart failure and atrial fibrillation, and diagnosed via multimodality imaging.
Raises suspicion for isolated RV noncompaction in unexplained right heart failure with AF; leaves open prevalence and management needs.
Isolated noncompaction of ventricular myocardium (INVM) is a genetic cardiomyopathy due to abnormal arrest in endomyocardial embryogenesis between fetal 5th and 8th week. Noncompaction of right ventricle alone is rare. Here we present one such case where a young man presented with progressive right heart failure and atrial fibrillation. Subsequent evaluation by echo and cardiac magnetic resonance imaging confirmed our diagnosis. The cardinal manifestations of INVM are heart failure, arrhythmia, and embolic events and our case presented with former two manifestations. Echocardiographic criteria for diagnosing INVM are discussed.
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Ranganathan et al. (2012) conducted a case report in Isolated noncompaction of right ventricle (n=1). Isolated noncompaction of right ventricle was evaluated. A young man with isolated noncompaction of the right ventricle presented with progressive right heart failure and atrial fibrillation, confirmed by echocardiography and cardiac MRI.
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