Double-outlet left ventricle: single-center experience and literature review

Double-outlet left ventricle (DOLV) is an abnormal ventriculo-arterial connection characterized by origin of both great arteries, or more than 50% of each arterial root, from the morphological left ventricle. The aim of our paper is to describe the anatomic, echocardiographic, and multi-modality imaging characteristics of DOLV and associated malformations, and to assess its surgical outcomes. Methods: From 2011 to 2022, we retrospectively reviewed case records, intra-operatory reports and follow-up data of patients diagnosed with DOLV at Bambino Gesu Children’s Hospital. A systematic search was developed in MEDLINE, EMBASE and Web of Science databases, to identify original reports between January 1, 1975 and May 30, 2022, assessing the morphology and surgical outcomes of DOLV. Retrospective cohort studies, cross-sectional and case series were included in the analysis. Single case reports and reviews were excluded. Results: At our center, four cases of DOLV were identified. Patient 1 was diagnosed with (S,D,D) DOLV and hypoplastic right ventricle. The aorta overrode a large, doubly-committed VSD with absence of infundibular septum. A tenuous mitro-aortic discontinuity and a well-developed subpulmonary conus were present. Associated abnormalities included crossed pulmonary arteries and two adjacent, side-by-side coronary ostia, located in the anterior facing sinus, which gave origin to the left anterior descending (LAD) and the right coronary artery (RCA). Left circumflex artery (LCx) had a retro-aortic course and originated from the RCA. After pulmonary artery banding, Damus-Kaye-Stansel and Glenn intervention were proposed as first-stage of univentricular palliation. Patient 2 and 3 were diagnosed with (S,D,D) DOLV, subaortic VSD and pulmonary stenosis. Patient 2 underwent Rastelli operation and no anatomic detail were available. Patient 3 showed absence of the infundibular septum and mitro-pulmonary continuity, whereas subaortic conus was well developed. Anomalous origin of the LCx, originating from the posterior facing sinus with retro-aortic course was present. Rastelli procedure was performed to reconstruct right ventricular outflow tract. LCA and RCA were respectively caudal to subvalvular and supravalvular segments of the RV-to-PA conduit. After a 6-years follow-up, severe stenosis of the RV-to-PA conduit was present, nevertheless percutaneous conduit dilatation was contraindicated, due to coronary abnormality, and an aortic homograft was implanted Patient 4 was diagnosed with (S,D,L) DOLV with subaortic VSD and mitro-pulmonary fibrous continuity. A large subaortic conus was present. Reparation à l’etage ventriculaire was performed to reconstruct RVOT. Follow-up MRI at 8 years showed severe pulmonary artery regurgitation with mild RV dilatation (indexed volume 99mL/m2) and normal RV ejection fraction (54 %) Systematic review:Through our systematic research strategy we scrutinized 96 records for inclusion criteria (Figure 4). After systematic evaluation, a total of 9 reports fulfilled eligibility criteria and were included in our study. Morphological findings and surgical outcomes are summarized in Table 1. Among 191 cases of DOLV included, the most common subtypes of VSD were subaortic (128/191), subpulmonary (23/191) or doubly committed (14/191) (Figure 5). d-transposition of the aorta was present in 117/191 (61%) cases, whereas l-transposition was reported in 63/191 (32%) (Figure 6)