Abstract
Background
The purpose of this registry was to provide insights into the characteristics, treatments
and survival of patients with PAH-CHD in China.
Methods
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.
Results
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.
Conclusions
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.
Keywords
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)To read this article in full you will need to make a payment
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