Value of echocardiography in the integrated management of fetuses with critical pulmonary stenosis or pulmonary atresia with intact ventricular septum

OBJECTIVE: The aim of this study was to analyze the echocardiographic characteristics, intrauterine progression, postnatal outcomes, and follow-up results of fetuses diagnosed with critical pulmonary stenosis with an intact ventricular septum (CPS/IVS) and pulmonary atresia with an intact ventricular septum (PA/IVS) using echocardiography. METHODS: A retrospective cohort study was conducted on fetuses with isolated PA/IVS and CPS/IVS, collecting both morphological and hemodynamic echocardiographic data and following them postoperatively. RESULTS: A total of 48 cases were included in the study. On the initial ultrasound, eight cases were diagnosed with PA/IVS and 40 cases with CPS/IVS. The right ventricle/left ventricle long axis (RV/LV) ratio, right ventricle long axis (RV-L) z-score, and pulmonary artery/aorta (PA/AO) ratio for PA/IVS were significantly lower than those for CPS/IVS (RV/LV P < 0.001; RV-L P = 0.002; PA/AO P = 0.003). Severe tricuspid regurgitation (TR) was predominant in both PA/IVS and CPS/IVS, with six and 28 cases, respectively. A significant increase in RV-L, tricuspid valve (TV) annulus, and pulmonary valve (PV) was observed with gestational age and fetal growth. In an exploratory analysis, earlier gestational age at first ultrasound was associated with neonatal intervention (hypothesis-generating due to small sample size). Post-surgical follow-up showed improvements in RV/LV ratio, tricuspid valve/mitral valve (TV/MV) ratio, and PV z-score (P < 0.05), suggesting favorable short-term echocardiographic postoperative outcomes. CONCLUSION: In this cohort in which all infants achieved biventricular circulation, PA/IVS fetuses showed more compromised RV and PV annular development than CPS/IVS. Serial echocardiography demonstrated measurable growth of RV/TV/PV with gestation, while postnatal and post-intervention follow-up showed improved RV/LV ratio and PV z-scores. These findings support the descriptive value of echocardiography for longitudinal assessment and perioperative follow-up within an integrated management pathway.