The purpose of this registry was to provide insights into the characteristics, treatments and survival of patients with PAH-CHD in China.
Patients diagnosed with PAH-CHD were enrolled in this national multicenter prospective registry. Baseline and follow-up data on clinical characteristics, PAH-targeted treatments and survival were collected.
A total of 1060 PAH-CHD patients (mean age 31 years; 67.9% females) were included, with Eisenmenger syndrome (51.5%) being the most common form and atrial septal defects (37.3%) comprising the most frequent underlying defect. Approximately 33.0% of the patients were in World Health Organization functional class III-IV. The overall mean pulmonary arterial pressure and pulmonary vascular resistance were 67.1 (20.1) mmHg and 1112.4 (705.9) dyn/s/cm5, respectively. PAH-targeted therapy was utilized in 826 patients (77.9%), and 203 patients (19.1%) received combination therapy. The estimated 1-, 3-, 5-, and 10-year survival rates of the overall cohort were 96.9%, 92.9%, 87.6% and 73.0%, respectively. Patients received combination therapy had significantly better survival than those with monotherapy (P=0.016). NT-proBNP >1400 pg/mL, SvO2 ≤ 65% and Borg dyspnea index ≥ 3 and PAH-targeted therapy were independent predictors of mortality. Hemoglobin > 160g/L was a unique predictor for mortality in Eisenmenger syndrome.
Chinese PAH-CHD patients predominantly exhibit Eisenmenger syndrome and have significantly impaired exercise tolerance and right ventricular function at diagnosis, which are closely associated with long-term survival. PAH-targeted therapy including combination therapy showed a favorable effect on survival in PAH-CHD. The long-term survival of Chinese CHD-PAH patients remains to be improved.
Abbreviations:PAH (pulmonary arterial hypertension), CHD (congenital heart disease), RHC (right heart catheterization), mPAP (mean pulmonary artery pressure), PAWP (pulmonary artery wedge pressure), PVR (pulmonary vascular resistance), mRAP (mean right atrial pressure), SvO2 (mixed venous oxygen saturation), CI (cardiac index), Qp/Qs (pulmonary-to-systemic blood flow ratio), 6MWD (six-minute walking distance), WHO-FC (World Health Organization functional class), BAS (balloon atrial septostomy), NT-proBNP (N-terminal pro-B-type natriuretic peptide), ASD (atrial septal defect), VSD (ventricular septal defect), PDA (patent ductus arteriosus), PDE-5i (phosphodiesterase type-5 inhibitors), ERA (endothelin receptor antagonists)
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