Qiangqiang Li, Yuan He, Andrew Constantine, Konstantinos Dimopoulos, Chen Zhang, Qiang Wang, Hong Gu
Beijing Anzhen Hospital and Capital Medical University. Queen Elizabeth Hospital Birmingham and University Hospitals Birmingham NHS Foundation Trust. Royal Brompton Hospital and Royal Brompton and Harefield NHS Foundation Trust. Imperial College London.
China and United Kingdom
European Heart Journal Open
Eur Heart J Open 2025; 6:
DOI: 10.1093/ehjopen/oeaf172
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.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes