Hypoplastic left heart syndrome: the influence of surgical strategy on outcomes

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, and to compare the results of the modified Blalock-Taussig (mBT) shunt with the right ventricle-to-pulmonary artery (RV-PA) conduit procedures as the source of pulmonary blood flow.

Retrospective study of 71 newborns with HLHS consecutively operated between March, 1999 and February, 2006. One technique for reconstruction of the neoaorta and two different techniques for reestablishment of the pulmonary blood flow were used: the mBT shunt in the first 37 newborns and RV-PA conduit in the last 34. Cannulation of the ductus arteriosus for arterial perfusion was the main part of the surgical strategy to reduce the hypothermic circulatory arrest time.

In-hospital survival for the entire cohort was 74.64%, or 67.57% and 82.35% for the mBT shunt and RV-PA conduit groups, respectively (p=0.1808). Mortality rates between the first and second palliation stages were 40% and 4.4% for the mBT shunt and RV-PA conduit groups, respectively (p=0.0054). Hypothermic circulatory arrest times were 45.79+/-1.99 min and 36.62+/-1.62 min (p=0.0012), respectively. Late coarctation of the aorta occurred in five patients (7.2%).

This surgical strategy resulted in short circulatory arrest time, low mortality and favorable morphology of the neoaorta, with low incidence of late coarctation of the aorta. The higher rate of survival to first palliation stage with the RV-PA conduit was not significant, but interstage mortality was statistically lower when compared with the modified Blalock-Taussig shunt procedure.