CAJ | 학술논문

目的探讨肺动脉闭锁合并室间隔缺损患儿的肺循环血供和相应的治疗对策.方法 2012年4月至2013年10月在北京安贞医院接受手术治疗的48例肺动脉闭锁合并室间隔缺损患儿,患儿术前均接受多排螺旋CT检查及经胸超声心动图检查.根据多排螺旋CT检查结果确定侧支血管起源及走形路径,计算McGoon比值及Nakata指数.结果 48例患儿中,肺循环血供主要来自于动脉导管16例(33.3％),粗大的体肺侧支动脉14例(29.1％),动脉导管与体肺侧支共存18例(37.5％);McGoon比值为(1.2±0.4),Nakata指数为(130±88) mm2/m2.术式包括侧支汇聚术2例,体肺分流术29例,双向格林术13例,体肺分流加腔肺分流术4例.术后早期死亡2例.结论肺动脉闭锁合并室间隔缺损患儿的肺动脉血供及解剖形态复杂多样化,多排螺旋CT结果结合经胸超声心动图结果综合评价肺血管情况及心内畸形可制定相应的治疗方案.
목적 탐토폐동맥폐쇄합병실간격결손환인적폐순배혈공화상응적치료대책.방법 2012년4월지2013년10월재북경안정의원접수수술치료적48례폐동맥폐쇄합병실간격결손환인,환인술전균접수다배라선CT검사급경흉초성심동도검사.근거다배라선CT검사결과학정측지혈관기원급주형로경,계산McGoon비치급Nakata지수.결과 48례환인중,폐순배혈공주요래자우동맥도관16례(33.3％),조대적체폐측지동맥14례(29.1％),동맥도관여체폐측지공존18례(37.5％);McGoon비치위(1.2±0.4),Nakata지수위(130±88) mm2/m2.술식포괄측지회취술2례,체폐분류술29례,쌍향격림술13례,체폐분류가강폐분류술4례.술후조기사망2례.결론 폐동맥폐쇄합병실간격결손환인적폐동맥혈공급해부형태복잡다양화,다배라선CT결과결합경흉초성심동도결과종합평개폐혈관정황급심내기형가제정상응적치료방안.
Objective To investigate the classifications of pulmonary arterial supply in pulmonary atresia with ventricular septal defect and treatment strategies.Methods From April 2012 to October 2013,a total of 48 patients with pulmonary atresia with ventricular septal defect were enrolled.All the patients were scanned with multidetector-row computed tomography(MDCT) and transthoracic echocardiography(TTE).All patients were evaluated in terms of the origins and the traces of major systemic-pulmonary collateral arteries (MAPCAs) as well as the McGoon ratio and Nakata Index.Results In the 48 cases,the pulmonary arterial supply was from the arterial duct(PDA) in 16 cases(33.3％),MAPCAs in 14 cases (29.1％),PDA coexist with MAPCAs in 18 cases (37.5％) ; the McGoon ratio was (1.21 ± 0.41) and the Nakata index was (130 ± 88) mm2/m2 ; During the perioperative stage,only two patients were dead.Procedures included unifocalization in 2 cases,Modified Blalock-Taussing shunt in 29 cases and bidirectional cavopulmonary anastomosis in 13 cases.Modified Blalock-Taussing shunt with bidirectional cavopulmonary anastomosis occurred in 4 cases.Conclusions Variations are found in the site of pulmonary atresia and the morphology of the pulmonary arterial supply in congenital anomaly of pulmonary atresia with ventricular septal defect.Generally,combined with MDCT and TTE,surgeons can obtain full-scale information of the specific cardiovascular abnormality and make surgical decisions to manage pulmonary atresia with ventricular septal defect.