Clinical results of combined palliative procedures for cyanotic congenital heart defects with intractable hypoplasia of pulmonary arteries  

作　　者：FAN Xiang-ming ZHU Yao-bin SU Jun-wu ZHANG Jing LI Zhi-qiang XU Yao-qiang LI Xiao-feng LIU Ying-long 

机构地区：[1]Department of Pediatric Heart Center, Department of Children Heart Center, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China

出　　处：《Chinese Medical Journal》2013年第9期1678-1682,共5页中华医学杂志（英文版）

基　　金：This study was supported by grants from Beijing Natural Science Foundation （No.7112046）, Beijing Municipal Education Committee （No. PXM2011 014226 07 000060）, Beijing Municipal Science and Technology Committee （No. Z101107050210020）, and Beijing Municipal Science and Technology Committee （No. Z11110006150000）.

摘　　要：Background Congenital heart defects with intractable hypoplasia of the pulmonary arteries without intercourse or with intercourse stenosis is unsuitable for surgical correction or regular palliative procedures. We reported our experience with combined palliative procedures for congenital heart defects with intractable hypoplasia pulmonary arteries. Methods From 2001 to 2012, a total of 41 patients with cyanotic congenital heart defects and intractable hypoplasia of the pulmonary arteries underwent surgical procedures. From among them, 31 patients had pulmonary atresia with ventricular septal defect （VSD） and the other 10 cases had complicated congenital heart defects with pulmonary stenosis. Different kinds of palliative procedures were performed according to the morphology of the right and left pulmonary arteries in every patient. If the pulmonary artery was well developed, a Glenn procedure was performed. A modified Blalock-Taussig shunt or modified Waterston shunt was performed if pulmonary arteries were hypoplastic. If the pulmonary arteries were severely hypoplastic, a Melbourne shunt was performed. Systemic pulmonary artery shunts were performed bilaterally in 25 cases. A systemic-pulmonary shunt was performed on one side and a Glenn procedure was performed contralaterally in 16 cases. Major aortopulmonary collateral arteries were unifocalized in six cases, ligated in two cases and interventionally embolized in two cases. There was one early death because of cardiac arrest and the hospital mortality was 2.4%. Results Five patients suffered from postoperative low cardiac output syndrome, three had perfusion of the lungs, and two pulmonary infections. Systemic pulmonary shunts were repeated after the original operation in three cases due to the occlusion of conduits. The mean follow-up time was 25 months. The pre- and the post-operation left pulmonary indices were （8.13±3.68） vs. （14.9±6.21） mm2/m2. The pre- and post-operation right pulmonary indices were （12.7±8.13） vs. （17.7±7.78） mmBackground Congenital heart defects with intractable hypoplasia of the pulmonary arteries without intercourse or with intercourse stenosis is unsuitable for surgical correction or regular palliative procedures. We reported our experience with combined palliative procedures for congenital heart defects with intractable hypoplasia pulmonary arteries. Methods From 2001 to 2012, a total of 41 patients with cyanotic congenital heart defects and intractable hypoplasia of the pulmonary arteries underwent surgical procedures. From among them, 31 patients had pulmonary atresia with ventricular septal defect （VSD） and the other 10 cases had complicated congenital heart defects with pulmonary stenosis. Different kinds of palliative procedures were performed according to the morphology of the right and left pulmonary arteries in every patient. If the pulmonary artery was well developed, a Glenn procedure was performed. A modified Blalock-Taussig shunt or modified Waterston shunt was performed if pulmonary arteries were hypoplastic. If the pulmonary arteries were severely hypoplastic, a Melbourne shunt was performed. Systemic pulmonary artery shunts were performed bilaterally in 25 cases. A systemic-pulmonary shunt was performed on one side and a Glenn procedure was performed contralaterally in 16 cases. Major aortopulmonary collateral arteries were unifocalized in six cases, ligated in two cases and interventionally embolized in two cases. There was one early death because of cardiac arrest and the hospital mortality was 2.4%. Results Five patients suffered from postoperative low cardiac output syndrome, three had perfusion of the lungs, and two pulmonary infections. Systemic pulmonary shunts were repeated after the original operation in three cases due to the occlusion of conduits. The mean follow-up time was 25 months. The pre- and the post-operation left pulmonary indices were （8.13±3.68） vs. （14.9±6.21） mm2/m2. The pre- and post-operation right pulmonary indices were （12.7±8.13） vs. （17.7±7.78） mm

关 键 词：congenital heart disease hypoplasia of pulmonary artery PALLIATION 

分 类 号：S829.1[农业科学—畜牧学] S858.31[农业科学—畜牧兽医]