Decreased pulmonary artery compliance (<1.04 mL/mmHg) was associated with worse prognosis and was an independent predictor of all-cause mortality in patients with APAH-CHD (HR 1.359; P<0.001).
Cohort (n=175)
Does decreased pulmonary artery compliance predict all-cause mortality in patients with pulmonary arterial hypertension associated with adult congenital heart disease?
Pulmonary artery compliance is inversely correlated with pulmonary vascular resistance and serves as a strong independent predictor of mortality in patients with PAH associated with adult congenital heart disease.
Effect estimate: HR 1.359
p-value: p=< 0.001
In congenital heart disease (CHD), the presence of pulmonary arterial hypertension (PAH) is associated with a poor prognosis. In this study, we aim to investigate the role of pulmonary artery compliance (Cp) in predicting the mortality of PAH associated with adult congenital heart disease (APAH-CHD). One-hundred and seventy-five patients of APAH-CHD who underwent a comprehensive clinical evaluation were included in this study. All patients were followed up in a 6-month interval and the primary end point was all cause of death. The duration of mean follow-up was 67±26 months, of which there were twenty-three death. Cp had an inverse correlation with pulmonary artery resistance (PVR), regardless of the clinical phenotype (Eisenmenger syndrome, PAH with small defect and PAH after defect correction). Patients in the lowest Cp group (Cp ≤ 0.84 mL/mmHg) had advanced WHO function class, worse exercise tolerance, liver function, and status of oxygen saturation. In univariate cox regression analysis, Cp (HR = 1.359, P < 0.001), PVR (HR = 0.972, P = 0.001), pulmonary blood flow (HR = 1.092, P = 0.001), heart rate (HR = 1.038, P = 0.028) and 6-minute walking distance (HR = 1.003, P = 0.037) were predictors of survival. After adjustment by bivariate analysis, Cp was the independent predictor. Kaplan-Meier survival curves showed that patients with Cp < 1.04 mL/mmHg had worse prognosis. In conclusion, Cp possibly reflects disease severity and decreased Cp was associated with poor prognosis in patients with APAH-CHD.
Cheng et al. (Sun,) conducted a cohort in Pulmonary arterial hypertension associated with adult congenital heart disease (APAH-CHD) (n=175). Pulmonary artery compliance (Cp) was evaluated on All cause of death (HR 1.359, p=< 0.001). Decreased pulmonary artery compliance (<1.04 mL/mmHg) was associated with worse prognosis and was an independent predictor of all-cause mortality in patients with APAH-CHD (HR 1.359; P<0.001).