A Single-Center Clinical Analysis of 121 Pediatric Cases with Pulmonary Atresia and Ventricular Septal Defect Undergoing the Pulmonary Artery Flow Study

Abstract

Background

This study analyzed the utility of the pulmonary artery flow study (PAFS) in prognostic assessment for children with pulmonary atresia and ventricular septal defect (PA/VSD).

Methods

We reviewed 121 patients with type B or C pulmonary atresia and ventricular septal defect (PA/VSD) who underwent the PAFS between August 2016 and August 2024. Demographic, operative, and follow-up data were collected. Statistical analyses included Spearman's correlation, ROC curves, and Kaplan-Meier survival analysis.

Results

The cohort included 60 males and 61 females, with 97 type B and 24 type C cases. Mean age was 44 months and median weight was 12.8 kg. Mean pulmonary artery pressure (mPAP) strongly correlated with postoperative right ventricular systolic pressure/left ventricular systolic pressure (RVSP/LVSP) (P = 0.000). An mPAP cutoff of 24.5 mmHg predicted VSD closure with 79.2% sensitivity and 91.2% specificity. Mean pulmonary artery pressure and RVSP/LVSP were associated with prolonged ventilation and critical care unit stay. During follow-up, 15/121 children (12.4%) died, with significantly poorer survival in those with mPAP > 25 mmHg (P = 0.009).

Conclusions

The pulmonary artery flow study is a valuable predictor of successful VSD closure and postoperative outcomes. An mPAP threshold of 25 mmHg may help identify high-risk patients requiring more intensive management.