Mid-term prognosis and risk stratification in patients with post-operative pulmonary hypertension: Insights from the Japanese Association of Congenital Heart Disease Registry (JACPHR)

Background: Post-operative pulmonary hypertension (PH) following biventricular repair of congenital heart disease (CHD) remains a high-risk condition, but its prognosis and risk stratification are poorly defined. This study aimed to identify prognostic factors and develop a simple clinical risk score. Methods: Patients with post-operative PH enrolled in the Japan CHD-Pulmonary Hypertension Registry (2021-2024) were analyzed. Continuous and categorical variables were assessed by log-rank testing, and a composite risk score was developed to predict cardiovascular events. Prognostic performance was evaluated by Kaplan-Meier and Cox regression analyses. Results: <0.001). Conclusions: This nationwide, registry-based study provides new insights into prognostic determinants and risk stratification in patients with post-operative PH. This study identified practical prognostic factors and proposed a simple, exploratory equal-weighted risk score. Prognostic significance was evident even at thresholds below conventional criteria, highlighting the vulnerability of this population and supporting the score's potential clinical utility pending external validation.