The prenatal diagnosis, classification and outcome of anomalous origin of pulmonary artery from ascending aorta

Abstract

Objective To investigate the clinical value of fetal echocardiography in prenatal diagnosis, classification and outcome of abnormal origin of pulmonary artery from ascending aorta (AOPA). Methods From January 2014 to June 2018, 18 cases of AOPA diagnosed by echocardiography in 43 966 fetuses from Shaanxi Fetal Congenital Heart Disease Diagnostic Center were retrospectively analyzed. The echocardiographic features, pathological and anatomical classification, genetic characteristics and outcome of postnatal echocardiography were summarized. Results Abnormal origin of pulmonary artery branches could be demonstrated by color Doppler imaging system in 18 cases. Ten cases (55.6%) of right pulmonary artery abnormalities originated from ascending aorta (AORPA), in which 6 cases (60%) were distal pulmonary artery abnormalities, 4 cases (40%) were proximal pulmonary artery abnormalities. Eight cases (44.4%) of left pulmonary artery abnormalities originated from ascending aorta (AOLPA), including 7 cases (87.5%) of distal pulmonary artery abnormalities and 1 case (12.5%) of proximal pulmonary artery abnormalities. Twelve AOPAs were associated with other intracardiac malformations with 6 right ventricular double outlet (DORV) accompanying with pulmonary stenosis, 3 tetralogy of fallots(TOF), 2 atrioventricular septal defects(AVSD), 1 single ventricle with single atrium, and 1 Berry syndrome, and no casese were associated with extracardiac malformations.Amniocentesis karyotype analysis and gene chip detection in 5 cases showed normal results. Four of 18 cases were born, in which 3 cases died and 1 case was progressively suffered with right pulmonary artery atresia, 10 cases were inducted of labor (4 autopsy) and following-up was lost in 4 cases. Conclusions Incidence of AOLPA and distal type in fetal AOPA is higher than that in child and adult from references, DORV is abnormal type mostly associated with intracardiac malformation. AOPA has no obvious genetic result for less cases. The main risk after AOPA birth is pulmonary infection. Echocardiography is of great value for AOPA in prenatal diagnosis, outcome observation and surgical planning after birth. Key words: Ultrasonography, prenatal; Fetus; Abnormal origin of pulmonary artery from ascending aorta