182 Twenty years of hypoplastic left heart syndrome in Croatia

Hypoplastic left heart syndrome (HLHS) is rare congenital heart defect in which the left side of the heart is severely underdeveloped. It has been a lethal congenital heart anomaly until the last four decades until three palliative surgeries were established (operation by Norwood, Glenn and Fontan). The aim of this study was to evaluate the outcomes of treating patients with HLHS. The main methods were statistical, and the clinical characteristics were retrospectively reviewed. We included 132 patients in 20-year period who have been treated at University Hospital Centre Zagreb, operated in our and in foreign centers. We followed them before, in the meantime and after final operation. Of all patients, 69 survived and 63 died. The highest mortality was in period between operation by Norwood and Glenn in early infancy and accounts for almost 48% all lethal outcomes. The most common anatomic variant is the mitral atresia and aortic atresia (MA-AA) subtype and the rarest is mitral stenosis and aortic atresia (MS-AA) subtype. Apart of a three – staged operation procedures, 53 patients required one or more interventions involving implantation of the stent into the pulmonary branches, isthmus the aorta, the Sano or mBT junction, and the dilatation of the same, then coiling of the arteriovenous malformations, electrostimulator implantation and the closure of fenestra. The most common interventions are stent implantation into the pulmonary branches and dilatation of the aortic recoarctation and stenosis of the pulmonary branch stent. In twelve patients, fenestra was closed with an Amplatzer occluder. The mean follow-up age operation by Fontan (TCPC) is 7.64 years (1.1 – 16.5 years). Two patients were transferred to the GUCH population. This retrospective study included 132 patients with hypoplastic left heart syndrome in twenty-year period who have been treated at University Hospital Centre Zagreb. The overall survival of all patients is 52.2%. The highest mortality was in period between operation by Norwood and Glenn in early infancy. The most common interventions are stent implantation into the pulmonary branches and dilatation of recoarctation of the aorta. Mean follow-up age operation by Fontan (TCPC) is 7.64 years.