Pulmonary arterial hypertension after congenital heart defect correction: a call for timely diagnosis and careful risk stratification to improve outcomes
Li, Qiangqiang He, Yuan Constantine, Andrew Dimopoulos, Konstantinos Zhang, Chen Wang, Qiang Gu, Hong
Li, Qiangqiang
He, Yuan
Constantine, Andrew
Dimopoulos, Konstantinos
Zhang, Chen
Wang, Qiang
Gu, Hong
Affiliation
Beijing Anzhen Hospital; Capital Medical University; University Hospitals Birmingham NHS Foundation Trust; University of Birmingham; Royal Brompton & Harefield NHS Foundation Trust; Imperial College London
Other Contributors
Publication date
2025-12-18
Collections
Research Projects
Organizational Units
Journal Issue
Abstract
Aims: Patients with pulmonary arterial hypertension (PAH) after congenital heart disease (CHD) correction (PAH-CHDcor) are becoming the most prevalent and rapidly expanding group within PAH associated with CHD (PAH-CHD), yet data on its presentation, long-term outcomes and prognostic variables are lacking. We report on a large paediatric and adult population with PAH-CHDcor, focusing on clinical presentation and long-term survival.
Methods and results: We studied 127 PAH-CHDcor patients (mean age 21.5 ± 10.5 years; 74.8% female) diagnosed via cardiac catheterization from 2006 to 2022. The majority had post-tricuspid shunts (73.2%), with combined pre- and post-tricuspid (11.8%) and complex shunts (6.3%) less frequent. Pulmonary vascular resistance (PVR) at diagnosis averaged 13.2 ± 8.9 WU. Diagnosis occurred late (>5 years post-repair) in 43.3% of patients. Median follow-up was 4.0 (IQR 2.0-6.4) years. Kaplan-Meier estimates for survival at 3 and 5 years were 93.3% and 89.6%, respectively. Higher baseline PVR predicted mortality (HR 1.10, 95% CI 1.03-1.16, P = 0.003) and was the strongest multivariable predictor of a composite endpoint (death, heart failure hospitalization, or parenteral prostacyclin initiation; HR 1.11, 95% CI 1.05-1.18, P < 0.001). An exploratory application of a paediatric prognostic score (GOSH) showed excellent discriminative power for mortality (AUC 0.867) and the composite endpoint (AUC 0.856) at 5 years in this independent cohort.
Conclusion: Mortality and morbidity are considerable in patients with PAH-CHDcor despite modern management. Regular, careful screening of all patients with repaired CHD is essential to ensure early diagnosis and risk stratification, with proactive evidence-based treatment to improve outcomes in this expanding population.
Citation
Li Q, He Y, Constantine A, Dimopoulos K, Zhang C, Wang Q, Gu H. Pulmonary arterial hypertension after congenital heart defect correction: a call for timely diagnosis and careful risk stratification to improve outcomes. Eur Heart J Open. 2025 Dec 18;6(1):oeaf172. doi: 10.1093/ehjopen/oeaf172.
Type
Article