Double-chambered left ventricle (DCLV) is an extremely rare congenital heart disease in which a muscular band or fibromuscular structure divides the left ventricle into two compartments. This condition is less frequent than double-chambered right ventricle and is usually not associated with other congenital heart defects. The aim of this study is to review reported cases in the literature and clarify the diagnosis, clinical features, and management strategies for this anomaly.

We performed a literature review of previously reported cases, focusing on anatomical features, clinical presentation, diagnostic techniques, and therapeutic approaches for DCLV.

DCLV may present in asymptomatic patients or manifest with clinical symptoms such as dyspnea, chest pain, or arrhythmias. Diagnosis relies primarily on imaging, with cardiac magnetic resonance (CMR) being the gold standard due to its ability to assess anatomical features, ventricular function, and myocardial viability. Management is typically conservative, although medical therapy may be required for symptomatic patients. Surgical intervention is indicated in cases of significant obstruction.

Despite its rarity, DCLV may have significant clinical implications, including arrhythmogenic risk, making timely diagnosis and appropriate follow-up essential for patient management.