Hypoplastic left heart syndrome: the report of a surgical strategy and comparative results of Norwood x Norwood-Sano approach.

Objectives: To report a surgical strategy for the Norwood procedure in the hypoplastic left heart syndrome (HLHS) that enables short hypothermic circulatory arrest time and aortic arch reconstruction with autologous pericardium patch. To compare the results of the modified Blalock-Taussig (mBT) shunt and the right ventricle-to-pulmonary artery (RV-PA) conduit procedures as the source of pulmonary blood flow.

Method: Retrospective study of 78 newborns consecutively operated between March, 1999 and June 2006. One technique for reconstruction of the neoaorta and two different techniques for reestablishment of the pulmonary blood flow: the mBT shunt in the first 37 newborns and RV-PA conduit in the last 41. Cannulation of the ductus arteriosus for systemic perfusion was the main part of the surgical strategy to reduce the hypothermic circulatory arrest time.

Results: In-hospital survival for the entire cohort was 74,35%, or 67.57% for the mBT shunt and 80,49% for RV-PA conduit groups (p=0,21). Hypothermic circulatory arrest times were 45.79+/-1.99 min and 36,8+/-1,52 min (p=0,0012), respectively. Mortality rates between first and second stages were 40% for the mBT shunt and 6,9% for RV-PA conduit groups (p=0,007). Late coarctation of the aorta occurred in five patients Actuarial survival curves(Kaplan-Meier) comparison showed better results with VD-AP conduit (p=0,003).

Conclusions: This surgical strategy resulted in short circulatory arrest time, low mortality and low incidence of aortic coarctation. Although the higher rate of survival to first palliation stage with the RV-PA conduit was not significant, the lower interstage mortality and superior medium-term survival in RV-AP group were statistically advantageous.