Reflection of the perioperative managements of Norwood stage I procedure for hypoplastic left heart syndrome

Abstract

Objective Norwood Stage Ⅰ is the standard procedure to cope with hypoplastic left heart syndrome(HLHS), which continues to be the most challenging congenital heart disease. The aim of this study is to retrospectively compare the classical perioperative management of Norwood StageⅠwith the modified strategy. Methods Between June 2010 and November 2017, totally 10 patients with HLHS underwent the standard Norwood Stage Ⅰ procedure. They are stratified to two tiers: Group A, from June 2010 to August 2014, there were 5 boys. Age at surgeries ranged from 29 to 75 days, and weight 2.57-3.50 kg with median of 3.13 kg. Group B, from August 2014 to November 2017, there were 4 boys and 1 girl. Age at surgeries ranged from 6 to 22 days, and weight from 2.0-3.1 kg. In Group A, all 5 cases underwent the standard Norwood Stage Ⅰ procedure under deep hypothermic circulatory arrest, including 4 cases of modified Blalock-Taussig shunt(MBTS) and 1 case of RV-PA shunt. In Group B, all 5 cases adopted side graft technique and RV-PA shunt, aortic arch and ascending aorta were reconstructed with treated bovine pericardial patch. Group B used incubators to adjust systemic vascular resistance instead of vasodilators. Results Group A’s early mortality is 40%; Group B’s early mortality is 20%, 1 case died of tamponade. Conclusion The standard Norwood Stage Ⅰ procedure is a complex procedure, which demands multidisplinary cooperation, to palliatively correct HLHS. To adjust and find a suitable perioperative managements can improve the results. Sharing experiences on perioperative managements of Norwood Stage Ⅰ between heart centers in China will be helpful to decrease the mortality and morbidity in relatively short period. Key words: Heart defects/congenital; Hypoplastic left heart syndrome; Norwood procedures; Side graft