关键词: 肺动脉闭锁, 先天性, 移植, 同种

Abstract: Objective　To observe the outcomes of the treatment of pulmomary atresia with ventricle septum defect utilizing the cryopreserved valved homograft conduits in pulmonary atresia patients. Methods Cryopreserved valved homograft conduits were implanted from October 2005 to May 2014 in 17 patients with diagnosis of pulmonary atresia with ventricle septum defect (male/female 10/7) in the age range 1year–16 years, median 6.4 years. The weight of patients was 8kg to 50kg, median 21.6kg. The hemoglobin was 127～218 g/L, median 175.3 g/L.Oxygen saturation of extremity was 0.60～0.90,median 0.78. 7 had B-T shunt for palliation and 3 had unifocalization operation. The time from primary operation was 7 months to 3.5 years, median 2.1 years. All of patiens had cyanosis. Cryopreserved pulmonary homograft conduits were implanted in 11 patients, and aortic homograft conduits were implanted in 3 patients. Average conduit size was 22.1mm. Routine electrocardiogram, transthoracic echocardiography and chest radiography was performed to assess the degree of pulmonary insufficiency and stenosis at 3 months, and then every 6-12 months postoperatively. Results　 Early death occurred in 1case. The patient is a 1-year girl, 8kg. Her diagnosis is pulmonary atresia with ventricle septum defect, patent ductus ateriosus, patent foramen ovale. Left ventricle is dysplasia and there is narrow at the beginning of left pulmonary atery. She died of low cardiac output syndrome 24 hours postoperatively. one patient had to be implanted a permanent pacemaker because of complete atrioventricular heart block 30 days after operation. Sixteen cases discharged. The mean follow-up period was 4.5years (range, 6 months to 8.5 years). During the follow- up, echocardiography showed moderate pulmonary valvular regurgitation in 1 patient and moderate tricuspid valvular regurgitation in 2patients. Fifteen patients did not show obvious pulmonary insufficiency. Gradient pressure of pulmonary vavle was 64mmHg and 58 mmHg at the 5th and 7th year after operation respectively in two patients. No late death occurred. No cryopreserved valved homograft conduit needs to replacement in 16 patients Conclusion　 Cryopreserved valved homograft conduit may provide a reliable option for reconstruction of right ventricle outflow tract in patients with pulmonary atresia. It shows excellent early results in preventing pulmonary insufficiency.

Key words: 　pulmonary atresia, congenital, transplantation, homograft