主动脉弓缩窄的产前超声诊断

Prenatal diagnosis of fetal aortic coarctation by ultrasonography

目的探讨胎儿主动脉弓缩窄(CoA)的产前超声诊断线索、诊断方法与技巧及畸形特征。方法所有胎儿均常规获得四腔心切面、左右心室流出道切面、三血管气管(3VT)切面(即主动脉弓横切面)的灰阶和彩色多普勒血流显像。当四腔心切面发现左心系统偏小、3VT切面显示主动脉弓和动脉导管内径比例失调疑CoA时,进一步获得主动脉弓纵切面和冠状切面(Y平面)并测量峡部内径。结果产前超声共诊断56例CoA患儿,28例有解剖或新生儿超声心动图和手术结果,产前超声误诊和漏诊4例。其中8例合并单心室、心内膜垫缺损、右心室双出口、大动脉转位、永存动脉干等其他严重复杂心脏畸形,7例合并心脏外严重畸形。22例合并室间隔缺损(VSD)和永存左上腔静脉(LSVC)等非复杂心脏畸形。产前超声诊断的28例CoA患儿均首先于3VT切面发现两大动脉内径比例失调,主动脉弓异常小,四腔心切面显示左、右心室比例不对称,左心明显小于右心。此28例CoA患儿中有23例(82.1%)同时获得了3VT切面、主动脉弓纵切面及Y平面,14例于主动脉弓纵切面显示有来自降主动脉的反流。而误诊和漏诊的4例CoA患儿均未能获得满意的主动脉弓纵切面和Y平面。结论 3VT切面显示主动脉弓和动脉导管内径比例失调和四腔心切面不对称、左心偏小是CoA的诊断线索,进一步获取主动脉弓的特殊切面以获得至少2个平面的印证是提高CoA产前诊断率的主要技巧。CoA可以单独发生,也可合并其他心内外畸形。

Objective To study prenatal diagnosis of fetal aortic coarctation （CoA） and the pattern of CoA by ultrasonography. Methods The routine fetal echocardiography included four-chamber view and outflow tracts and/or the great arteries view and three-vessel trachea （3VT） view（transverse view of the aortic arch） in grey scale and color flow Doppler image. The longitude view and coronal view （Y-plane） of the aortic arch were obtained and the isthmus inner diameter was measured when CoA was suspected in disproportion presence of the great arteries, or the ventricles, or both, with smaller left heart structures in the four-chamber view or in the 3VT view. Results Fifty-six cases of CoA were suspected by prenatal ultrasoungraphy. Twenty-eight of them were performed by postmortem or postnatal echocardiography and surgery. Four cases with CoA were misdiagnosis and misseddiagnosis. Eight cases were associated with intracardiac malformations including single ventricle, endocardial cushion defect, double right out tract, transposition of the great artery, and persistent truncus arteriosus. Seven cases had additional extracardiac malformations. Twenty-two cases had ventricular septal defect and persistent left superior vena cava non-complex intracardiac defects. All 28 cases prenatal diagnosed with CoA were presented with disproportion of the great arteries by prenatal sonograms, or the ventricles, or both, with smaller left ventricle and aorta diameter in the four-chamber view or in 3VT view. The 3VT view, longitudinal view and Y-plane of the aorta arch were all obtained in 23 of 28 （82.1%） cases with CoA. Fourteen of them had aortic arch regurgitation from the descend aorta. Only the 3VT view was obtained in misdiagnosis and misseddiagnosis of four cases with CoA. No clear longitude view and Y-plane of the aortic arch were obtained.Conclusion The ventricular and/or great atrial disproportion detected with smaller left ventricle and aorta diameter via fetal routine echocardiography are clues for diagnosis of CoA. Two orthogonal special views of the aortic arch is the skill for diagnosis of fetal CoA. The CoA may present to be isolated or associated with intracardiac and extracardiac malformations.