Characteristics and outcome of congenital left ventricular aneurysm and diverticulum: Analysis of 809 cases published since 1816.

Background: Congenital left ventricular aneurysm (LVA) or diverticulum (LVD) is rare cardiac anomalies. We aimed to analyse the clinical characteristics and outcome in all ever published patients.

Methods: MEDLINE, Web of science, Google and EMBASE, and reference lists of relevant articles were searched for publications reporting on LVA or LVD patients.

Results: We identified 809 patients published since 1816 [354 (49.1%) LVA, 453 (50.6%) LVD, 2 (0.3%) both]. Mean age at diagnosis was 34.1±27 (LVA) and 29.7±27.6years (LVD; p=0.05). 48.9% were male. LVA was larger (38.7±22.5mm versus 31.4±21.2mm; p=0.002) and frequently found in submitral location (33% versus 4.9%; p<0.001), LVD was frequently located at the LV-apex (61.2% versus 28.7%; p<0.001). LVD was often associated with cardiac (34.2% versus 11%; p<0.001) or extracardiac anomalies (32.7% versus 3%; p<0.001). LVA patients presented more frequently with ventricular tachycardia/fibrillation (18.1% versus 13.1%; p=0.01), the incidences of rupture (4% versus 4.5%; p=0.9), syncope (8.3% versus 5.1%; p=0.1), and embolic events (4.9% versus 3.6%; p=0.4) at presentation were not different between LVA and LVD. Mean follow-up was 56.3±43months. Cardiac death occurred more frequently in the LVA group (11.5% versus 5.0%; p=0.05) at a median age of 0.8 [LVA] and 2.5 [LVD] years. The leading cause of cardiac death was congestive heart failure in the LVA-group (50.0% versus 0.0%; p=0.01), and rupture in the LVD-group (75.0% versus 27.3%; p=0.04).

Conclusions: LVA and LVD are distinct congenital anomalies with different clinical and morphological characteristics. The prognosis of LVA is significantly worse during long-term follow-up.