Abstract

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. 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. 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). 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.

Highlights

  • The natural history of hypoplastic left heart syndrome (HLHS), which is generally fatal within the first month of life [1], was changed by the ground-breaking work of Norwood et al, who published the first successful cases in a series of children surgically treated between 1979 and 1981 [2,3]

  • The immediate survival rate of the 78 patients with HSLS submitted to Norwood surgery was 74.35%, with 67.57% in the modified BlalockTaussig (mBT) Group and 80.49% in the right ventricle-to-pulmonary artery (RV-pulmonary artery (PA)) Tube Group (p=0.21)

  • cardiopulmonary bypass (CPB) duration was less in the mBT Group, as anastomosis of the PTFE tube in the subclavian artery was performed during the reheating of the patient

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Summary

Introduction

The natural history of hypoplastic left heart syndrome (HLHS), which is generally fatal within the first month of life [1], was changed by the ground-breaking work of Norwood et al, who published the first successful cases in a series of children surgically treated between 1979 and 1981 [2,3] This procedure consists of connecting the pulmonary artery trunk to the previously extended aortic arch, thereby forming a new aorta. In 2003, Sano et al [9] reported good results with a modification of the Norwood technique using a PTFE graft anastomosed between the right ventricle (RV) and the pulmonary artery (PA), allowing blood flow to the pulmonary artery only during ventricular systole. This facilitates treatment of the patient in the postoperative period, because it avoids a drop in the coronary flow to the lung during diastole caused by the “steal phenomenon” [9,10,11]

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