Congenital left ventricular diverticulum revisited: a systematic review of clinical phenotypes, imaging features, and therapeutic strategies

Congenital left ventricular diverticulum (LVCD) is a rare cardiac malformation characterized by a contractile outpouching that includes all three layers of the ventricular wall. Although most frequently diagnosed in infancy, LVCD may also be identified in adults, either incidentally or in association with embolic events, arrhythmias, or heart failure. This systematic review synthesizes current clinical evidence on LVCD, encompassing demographic patterns, imaging characteristics, associated malformations, treatment strategies, and outcomes. All published case reports and case series describing LVCD were systematically reviewed in accordance with PRISMA guidelines. The literature search included PubMed/MEDLINE, Embase, and Scopus. Extracted data included demographics, clinical presentation, imaging findings, associated anomalies, interventions, and follow-up. In total, 157 patients were identified. LVCD was diagnosed across a wide age range, from fetuses and newborns to adults up to 80 years (mean 33 years). At diagnosis, 29% of patients were asymptomatic, while chest pain (21%), pulsating epigastric mass (12%), and arrhythmias (10%) were the most common symptomatic presentations. Echocardiography was the primary diagnostic modality (73%), with cardiac computed tomography and/or cardiac magnetic resonance performed in 52% of cases. Initial management was conservative in 44.6% (70/157) and surgical in 43.3% (68/157), with one case each of percutaneous closure and pregnancy termination. The outcome was reported just in 140/157. Overall survival was 95% (133/140), with 5% (7/140) deaths during a mean follow-up of 18 months. Reported complications included stroke, ventricular arrhythmias, systemic embolism, and, rarely, rupture. LVCD is a clinically heterogeneous condition with variable presentation and prognosis. Although many cases are asymptomatic, the potential for serious complications highlights the importance of early detection and individualized management. This review provides the largest synthesis of LVCD to date and proposes a pragmatic framework for diagnostic evaluation and management, emphasizing the need for greater awareness and standardized approaches to this rare disorder.