Biventricular Noncompaction Cardiomyopathy: Rare Case with MRI Diagnosis Insights

Noncompaction cardiomyopathy (NCC) is a rare congenital cardiomyopathy caused by the arrest of endomyocardial morphogenesis, leading to prominent trabeculations and deep intertrabecular recesses. Emerging evidence suggests that noncompaction may also occur secondary to other myocardial pathologies or have a genetic basis. A 59-year-old male with no coronary risk factors presented with 1-year history of chest pain, palpitations, orthopnea, and fatigue. Clinical examination revealed atrial fibrillation, normal pulmonary findings, and no peripheral edema. Echocardiography showed inferior wall hypokinesia, grade I diastolic dysfunction, with ejection fraction of 48%. Cardiac magnetic resonance imaging (MRI) confirmed excessive myocardial trabeculations with reduced biventricular function. Petersen, Stacey, and Jacquier criteria established the diagnosis of biventricular noncompaction (BVNC), a rare form of NCC. The patient was managed with standard heart failure therapy. BVNC, while rare, poses diagnostic challenges and carries significant morbidity due to heart failure and arrhythmias. This case describes the importance of MRI for accurate diagnosis and tailored management in BVNC.