Abstract: Background: Pulmonary atresia with intact ventricular septum (PA/IVS) and critical pulmonary stenosis (CPS) are rare congenital heart diseases. It is vital for cardiologists and pregnant women to perform detailed echocardiography evaluation in order to predict postnatal cardiac conditions. Objective: This study is to classify and evaluate the fetal, neonatal and postoperative cases with PA/IVS or CPS. Design: Case series report. Methods: Medical records of the fetal, neonatal and postoperative cases diagnosed with PA/IVS or CPS by echocardiograghy were retrospectively analyzed, and the echocardiographic characteristics of fetal, neonatal cases and follow-up after treatment were compared. Main outcome measures: The progress and correlation of the growth of right ventricle and pulmonary valve opening during fetal, neonatal stages and follow-up after treatment. Results: Of the 100 fetuses with PA/IVS or CPS, the number of typeⅠ, type Ⅱ and type Ⅲ was 69, 19, and 12 respectively. Type Ⅰ and type Ⅱ had moderate to severe tricuspid regurgitation (TR), while type Ⅲ had no TR. Type Ⅲ cases were all PA/IVS, in which seven fetuses had ventriculocoronary connection. Eleven fetuses with PA/IVS showed obvious pulmonary aterary dysplasia. Of the 23 fetuses treated after birth, there were 15 for PA/IVS, 8 for CPS, 21 for typeⅠand 2 for type Ⅱ. Two patients developed from moderate and severe PS in the fetus to PA/IVS at birth, respectively. The ratio of right to left ventricular length (RV/LV) was lower in neonatal period than that in fetal period. Moderate-severe TR were 22 in both fetal and neonatal stages. After hybrid therapy, one patient with PA/IVS died 7 days after operation, and 22 patients were followed up in the out-patient setting. TV Z-score, TV/MV and RV/LV increased significantly compared with that of pre-operation, and moderate to severe TR decreased from 22 cases to 1 case after operation and follow-up. Conclusion: Fetuses with PA/IVS and CPS can be classified and evaluated by echocardiography. The degree of TR is mild, while right ventricular dysplasia is more serious. During the prenatal period, part of moderate to severe PS can progress to PA/IVS, and the degree of right ventricular dysplasia is aggravated.

Key words: Pulmonary atresia with intact ventricular septum, Critical pulmonary stenosis, Dysplasia of right ventricule, Tricuspid regurgitation, Echocardiography