摘要目的 分析肺动脉闭锁合并室间隔缺损和大的体肺动脉侧支的患者肺循环的病理解剖特点,并探讨其临床意义.方法 回顾性分析2002年4月至2010年6月33例肺动脉闭锁合并室间隔缺损和大的体肺动脉侧支的患者,男性21例,女性12例,年龄11个月～29岁.根据1999年国际儿童心脏外科数据和命名会议的分型标准,B型22例,C型11例.29例(87.9%)存在自身肺动脉,其中发育较好的6例(18.2%),发育不良或左右肺动脉无汇合的23例(69.7%).4例(12.1%)自身肺动脉完全缺如.其中31例进行了33次手术治疗,包括主动脉-肺动脉分流手术8例,一期单源化手术2例,矫治手术23例.结果 分流手术和一期单源化手术的患者术后动脉氧饱和度83%～90%.矫治手术早期死亡1例,死亡原因为多脏器功能衰竭;低心排血量综合征4例,低氧血症(氧合指数<150 mmHg,1 mmHg=0.133 kPa)3例.16例矫治手术患者随访超过1年,右心室压41～99 mmHg;肺动脉瓣中度反流2例;射血分数>50%者14例,<50%者2例.结论 肺动脉闭锁合并室间隔缺损和大的体肺动脉侧支应根据肺血管的解剖特点采取个体化治疗的方案,存在中央肺动脉、左右肺动脉有汇合、体肺动脉侧支与肺动脉之间存在肺内交通是决定一期单源化和心内矫治手术的关键.

abstractsObjectives To analyze the anatomy features of the pulmonary circuits in the patients with pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCA), and discuss the clinical significance. Methods From April 2002 to June 2010, the anatomy features of pulmonary circuits in 33 patients with PA/VSD/MAPCA were examined and analyzed. There were 21 male and 12 female patients. The age ranged from 11 months to 29 years. The anatomic types of PA/VSD included group B for 22 cases, group C for 11 cases. Thirty-one patients of them underwent 33 operative procedures. The operations included aorta-pulmonary shunt in 8 cases, one stage unifocalization with VSD open in 2 cases, complete repair in 23 cases. Results Twenty-nine (87.9%) patients had native pulmonary arteries, 6 of them were normal size and 23 were hypoplastic size. Four patients (12. 1%) had no native pulmonary arteries. The postoperative oxygen saturation of the patients undergone shunt and one stage unifocalization was increased to 83% to 90%. There was one early death after complete repair because of multiorgan function failure. There were 4 cases of severe low cardiac output and 3 cases of respiratory function failure. Sixteen patients after complete repair were followed up more than one year. The postoperative right ventricular pressure was 41 to 99 mmHg (1 mmHg =0. 133 kPa). The ejection fraction value was more than 50% in 14 patients and less than 50% in 2 patients. Two patients had medium pulmonary insufficiency. Conclusions An individualized approach based on the anatomy of the pulmonary circuits permits achievement in the patients with PA/VSD/MAPCA. The surgical strategy for PA/VSD/MAPCA mainly depends on the anatomy features of native pulmonary arteries, confluent pulmonary arteries and MAPCA.