الملخص
To explore the prenatal diagnosis classification and prognostic evaluation of fetal pulmonary atresia with ventricular septal defect ( PA/VSD ) . Methods T hirty‐one fetal pulmonary atresia with ventricular septal defect were classified Ⅰ － Ⅳ type by Boston classification ,and the McGoon indexes were calculated ,w hether associated with malformation and chromosomal abnormalities ,and follow‐up . Results T hirteen fetuses were diagnosed type Ⅰ PA/VSD , 6 fetuses were associated with malformation ,2 fetuses were chromosomal abnormalities , 7 fetuses′ McGoon index ＞ 1 .20 ,6 fetuses′McGoon index＜1 .20 ,8 cases had operation ( 6 cases had radical operation and had a good follow up ,2 cases had palliative operation and were waiting for radical operation) , 5 cases received termination of pregnancy . Six fetuses were diagnosed as type Ⅱ PA/VSD ,5 fetuses were associated with malformation ,1 fetus was chromosomal abnormalities ,1 fetus′s McGoon index＞ 1 .20 ,5 fetuses′ McGoon index＜ 1 .20 ,2 cases had operation ( 1 case had radical operation and had a good follow up ,1 case had palliative operation and was waiting for radical operation) ,4 fetuses received termination of pregnancy . Four fetuses were diagnosed as type Ⅲ PA/VSD ,3 fetuses were associated with malformation ,no fetus was chromosomal abnormalities ,4 fetuses′McGoon index＜1 .20 ,1 case had palliative operation and was waiting for radical operation , 3 cases received termination of pregnancy . Eight fetuses were diagnosed as type Ⅳ PA/VSD ,3 fetuses were associated with malformation , 3 fetuses were chromosomal abnormalities , 1 case had unifocalization operation ,but died after operation in one day ,7 cases received termination of pregnancy . T he area under the ROC curve of McGoon index in hinting PA/VSD postnatal radical operation was 1 .000 ( P ＝ 0 .002 ,95%CI :1 .0000 － 1 .000 ) , the border value was 1 .255 , the sensitivity and specificity were 100% ,85 .7% , respectively . Conclusions Echocardiography can diagnose the classification of fetal PA/VSD . The radical operation for cases of McGoon index ＞1 .255 is feasible ,the cases of type Ⅳ PA/VSD and PA/VSD with associated malformation and chromosomal abnormalities have a poor follow up .