【摘要】 目的采用超声心动图对先天性矫正型大动脉转位(ccTGA)的解剖特征进行分析。方法超声心动图检查,且诊断为ccTGA的患儿45例,采用Van Praagh顺序节段分析法进行诊断。结果 (1)心房-心室连接不一致、心室-大动脉连接不一致:{S,L,L}40例(包括左位心30例,孤立性右位心10例);{I,D,D}4例(包括左位心2例,镜像右位心2例);{S,L,D}1例,为左位心。(2)合并房室瓣异常:房室瓣结构畸形7例。(3)其他合并畸形40例(88.9%),其中,最常见的是肺动脉瓣狭窄或肺动脉瓣下狭窄(29例,64.4%)和室间隔缺损(25例,55.6%)。结论 {S,L,L}是ccTGA最常见的病理解剖形式,此外也可以表现为{I,D,D}和{S,L,D};在ccTGA患者中,解剖三尖瓣功能性反流较常见且较严重;肺动脉瓣狭窄或肺动脉瓣下狭窄和室间隔缺损是其最常见的合并畸形。更多还原

【Abstract】 Objective To elucidate the pathological anatomy of congenitally corrected transposition of the great arteries(ccTGA)by echocardiography.Methods Forty-five consecutive patients,diagnosed as ccTGA by echocardiography were included.According to Van Praagh sequential segmental analysis,transthoracic echocardiography was performed.Results(1)Combination of atrioventricular discordance and ventriculoarterial discordance was diagnosed in all of the 45 cases.The position relationship{S,L,L}was detected in 40 cases,including 30 cases with levocardia and 10 cases with isolated dextrocardia,{I,D,D}in 4 cases,including 2 cases with levocardia and 2 cases with mirror-image dextrocardia,and{S,L,D}in 1case with levocardia.(2)Coexistence of atrioventricular valvular abnormalities was seen in 7 cases.(3)Other coexisted abnormalities were seen in 40 cases(88.9%),among which,pulmonary valve stenosis or subvalvular pulmonary stenosis(29 cases,64.4%)and ventricular septal defect(25 cases,55.6%)were the most common structural lesions.Conclusions Based on the findings of echocardiography,{S,L,L}was the most frequent pathological anatomy of ccTGA,and pulmonary valve stenosis or subvalvular pulmonary stenosis and ventricular septal defect were the most common coexisted abnormalities.Additionally,attention should be paid to the functional tricuspid regurgitation in patients with ccTGA.更多还原