Am Heart J Plus. 2026 Apr 15;65:100784. doi: 10.1016/j.ahjo.2026.100784. eCollection 2026 May.
ABSTRACT
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: Among 84 patients (median age 11.6 years; 55% men), eight cardiovascular events occurred during a mean follow-up of 18.2 months: three all-cause deaths, three hospitalizations for heart failure, and two cases of worsening functional class. Mean pulmonary artery pressure ≥30 mmHg, pulmonary vascular resistance index ≥4.0 WU·m2, B-type natriuretic peptide ≥40 pg/mL or NT-proBNP ≥300 pg/mL, and WHO functional class ≥III were significant prognostic factors. An equal-weighted score (0-4 points) stratified patients into low- (0-1), moderate- (2-3), and high-risk (4) groups with distinct survival (hazard ratio, 3.61; p <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.
PMID:42028443 | PMC:PMC13101661 | DOI:10.1016/j.ahjo.2026.100784