Background: Stage I palliation of hypoplastic left heart syndrome (HLHS) and its variants is usually performed by a Norwood operation. The management of pulmonary blood flow during this procedure remains controversial. The RV‐to-PA conduit (RVPAC) has been proposed as the better alternative compared to a systemic-to-pulmonary shunt (SPS). Methods: A retrospective single center chart review of consecutive patients who underwent a Norwood I procedure between 01/1997 and 09/2006 was performed. All patients were operated in deep hypothermia, with or without circulatory arrest, using different shunt modifications according to surgeon's preference. Patients were divided into two groups depending on surgical management for pulmonary blood flow (modified BT shunt [BT] and non-valved RVPAC [Sano]). Results: Fifty-four patients were included in the study (BT: 31 patients vs. Sano: 23 patients). Diastolic blood pressure during the first 24 hours postoperatively was significantly lower in the BT group (BT: 38.6 ± 6.9 mmHg vs. Sano: 42.4 ± 7.2 mmHg; p < 0.01) with a trend towards a higher systolic blood pressure (BT: 74.1 ± 13.5 mmHg vs. Sano: 69.8 ± 12.1 mmHg; p = 0.08). Mean circulatory arrest time in the BT group was significantly longer compared to the Sano patients (BT: 41 ± 21 min vs. Sano: 25 ± 23 min; p < 0.01). The mean hospital stay was 18.5 days for BT patients and 20 days for Sano patients (p = 0.45). Early mortality for the total cohort was 14.8 % (n = 8) (BT 19.4 % [n = 6] vs. Sano 8.7 % [n = 2]; p = 0.12). There was no significant difference in inter-stage mortality between the two groups (BT: 18.2 % vs. Sano: 21.1 %; p = 0.47). Conclusion: The results for both established surgical methods (BT and Sano) for the palliation of HLHS and its variants have improved over time and are reaching acceptable early mortality rates. There was a trend towards a favorable early outcome for Sano patients, which did not reach statistical significance in this study due to the low patient numbers.

Aortic atresia (AA) with interrupted aortic arch (IAA) is an exceptional condition. In absence of associated lesion, the flow to the brain and the retrograde flow to the coronary arteries can only come from vessels on the descending aorta. This flow needs to be large enough to supply the brain and the myocardium. The only IAA type compatible with survival is Type C where the flow from descending aorta could be insured by the left carotid and the left vertebral artery, branch of the left subclavian artery (SCA). Only one such a case was described in the literature. All the other surviving cases had an associated lesion including: a double aortic arch, an AP window, an aberrant right SCA or a bilateral PDA. We report a case of AA with IAA type B associated with a double aortic arch that underwent a successful bi-ventricular one-stage neonatal Norwood-Rastelli repair and is doing well after 18 months.

This retrospective multicentre study aims to present late surgical outcomes of Double Outlet Right Ventricle (DORV) coming from emerging countries. Mécénat Chirurgie Cardiaque brings to France for surgery selected children with simple and complex congenital diseases, including DORV. The patients are operated in nine hospitals, specialized in paediatric cardiac surgery. heart Data are collected from the MCC comprehensive database, with a strict post-operative follow-up. Patients included only biventricular repair of DORV with 2 viable ventricles. According to the classification of Eleventh Revision of the International Classification of Diseases (ICD11), DORV was defined as a congenital cardiovascular malformation in which both great arteries arise entirely or predominantly from the morphologically right ventricle. From Jan. 1996 to Jan. 2022, 81 consecutive DORV biventricular repair (BVR) were performed. There were 6 subtypes of DORV divided in two groups:—DORV-committed VSD: DORV-VSD (n:25), DORV-Fallot (n:34), DORV-TGA (n:5);—and DORV-ncVSD: DORV-nc-VSD-no PS (n: 7), DORV-nc-VSD-PS (n: 5), and DORV-AVSD-PS (n: 5). Four Fontan patients were excluded. Three patients were lost to follow-up, (3.4%). The overall peri-operative mortality was 7.4% ± 2.6%, 6/81, [95% CI: 2.8% - 15.4%] ranging from 0% in DORV-AVSD-PS to 14% for DORV-ncVSD-no PS. The overall 10Y survival was 86%. The early mortality of DORV-non committed VSD at 5.9% ± 2.4%, (1/17) was similar to DORV-committed VSD at 7.8% ± 2.7%, (5/64), p: 0.79 There was a trend for optimal outcome for arterial switch operation and DORV-AVSD-PS repair. VSD enlargement was significantly more frequent in DORV-non committed VSD, 42% (5/12), p: 0.001. There were low numbers in complex groups. The number of Fontan is noticeably low. The fundamental anomaly and surgical challenge in DORV is the aorta standing entirely on the right ventricle. Overall survival at 10Y was 86%. This study shows a trend towards satisfactory early and late outcomes in biventricular repair of simple DORV with committed VSD, compared to complex DORV with non-committed VSD.