关键词: 室间隔完整型肺动脉闭锁, Fontan手术, Glenn手术

Abstract:

Objective To investigate a more effective surgical approach to treat pulmonary atresia with intact ventricular septum (PA-IVS). Methods From January 2001 to June 2004, 11 patients with PA-IVSwere enrolled in a retrospective study, aged from 34 d to 32 month old, averaged (8.00±9.04) months, with body weight from 4.2 kg to 10 kg, (6.67±1.86) kg. All of 11 patients complicated with PDA and ASD/PFO, 10 patients with RVOTO, 2 patients with right ventricle or tricaspid valve hypoplasia. One patient received pulmonary valvotomy without CPB, and eight patients had transventricular pulmonary valvotomy with patch repaired, one patient received operation for Glenn shunt, and the other one had Fontan procedure. Results The early post-operation mortality was 18.1% (2 cases), and the 2 cases were complicated with severe right ventricular, one case had hypoplasia pulmonary valvotomy and enlarged right ventricle with Gore-tex patch, the another one had Fontan procedure. Eight patients were followed-up for 1 month to 3 years; the blood O₂ saturation of eight patients was over 0.9. Two cases remained in mild stenosis of the pulmonary valve or in the right ventricular outflow tract. Conclusion PA-IVS patients should be surgically repaired as early as possible and the surgical results depends on the growth of right ventricle and tricuspid valve.

Key words: pulmonary atresia intact ventricular septum, surgical procedures, Fontan procedures, Glenn shunt