Hypoplastic left heart syndrome (HLHS) is a severe anomaly. In France prenatal diagnostic is systematically associated with proposal for pregnancy termination. However still many newborns are referred for surgical management. The aim of the present study was to evaluate our recent experience with Norwood type univentricular palliation with particular emphasis to the surgical technique used.

The records of 44 consecutive patients who underwent procedure by Marie-Lannelongue and Jacques-Cartier Hospitals’ team between 2010 and 2018 were reviewed. The study group included 28 (64%) HLHS, 6 (14%) hypolplastic left heart complex, 2 (4.5%) unbalanced AV canal.

Norwood-Sano procedure was performed in 29 (66%), conventional Norwood in 7 (16%). A modification of Norwood- procedure using a intravascular Aorta-pulmonaru artery separation patch with hole was performed in 6 and the remaining 2 underwent hybrid-2 stage management. Three patients who underwent conventional Norwood procedure had been managed for biventricular pathway initially however converted in single ventricle pathway. The mortality occurred in 8 (18%, 70% CL 13-25%) patients including all death until stage 2, superior cavopulmonary shunt procedure. The outcome after Norwood-Sano/conventional Norwood procedures were superior to the modified technique (P=0.19). From 36 survivors, 16 had their 2nd stage and 6 their 3rd stage with total cavo-pulmonary shunt procedure. Two patients were converted to biventricular repair. One patient died after 2nd stage.

Despite the lack of conviction for referral, the management of HLHS and similar conditions were managed with favourable outcome. Technical modification that excludes conventional techniques, associated with poorer outcomes, must be avoided.