Key result
Higher pulmonary arterial compliance predicts ~33% lower all-cause mortality in PAH-CHD.
Why the study?
Pulmonary arterial compliance predicts outcome in pulmonary arterial hypertension, but its prognostic role in PAH associated with congenital heart disease is poorly defined.
Does pulmonary arterial compliance predict all-cause mortality in adult patients with PAH-CHD?
Cohort (n=434)
Yes
Does pulmonary arterial compliance predict all-cause mortality in adult patients with PAH-CHD?
Effect estimate: HR 0.665 (95% CI 0.503-0.878)
p-value: p=0.004
Pulmonary arterial compliance is an independent predictor of all-cause mortality in adult patients with PAH-CHD, providing incremental value for risk stratification.
BACKGROUND: Pulmonary arterial compliance (PAC) reflects the pulsatile load and predicts outcome in pulmonary arterial hypertension (PAH). The prognostic role of PAC in the heterogeneous patient population of PAH associated with congenital heart disease (PAH-CHD) is poorly defined. This study aimed to explore the prognostic value of PAC in patients with PAH-CHD. METHODS: Adult patients diagnosed with PAH-CHD were collected from a PAH multicenter prospective registry between August 2009 and December 2019. The primary endpoint was all-cause mortality. Multivariable Cox regression and restricted cubic spline (RCS) analysis were used to evaluate the association between PAC and the primary endpoint. Subgroup and interaction analysis between PAC and shunts or defect characteristics were explored. Incremental predictive performance was evaluated by calculating the C-index, continuous net reclassification improvement, and integrated discrimination improvement. RESULTS: A total of 434 adult PAH-CHD patients were enrolled. The median follow-up time was 52.2 months. The survival rate of patients in the lower PAC group was significantly worse than those in the higher PAC group (Log-rank P < 0.001). Multivariable Cox regression analysis showed that PAC independently predicted all-cause mortality after adjustment for other prognostic factors, whether as a continuous variable (HR = 0.665, 95%CI 0.503-0.878, P = 0.004) or a dichotomous variable (HR = 0.251, 95%CI 0.124-0.507, P < 0.001). A linear relationship between PAC and all-cause mortality was identified by RCS analysis. Subgroup analysis revealed that the impact of PAC might be affected by the presence of post-tricuspid shunt. Incorporating PAC into the validated risk models significantly improved the reclassification and discrimination ability for all-cause mortality. CONCLUSION: PAC was significantly associated with all-cause mortality in patients with PAH-CHD and provided additional value on risk assessment. The role of PAC may vary across different clinical subgroups. TRIAL REGISTRATION: ClinicalTrials.gov (NCT01417338), registered 16th August 2011.
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Li et al. (2025) conducted a cohort in Pulmonary arterial hypertension associated with adult congenital heart disease (PAH-CHD) (n=434). Pulmonary arterial compliance (PAC) was evaluated on All-cause mortality (HR 0.665, 95% CI 0.503-0.878, p=0.004). Pulmonary arterial compliance independently predicted all-cause mortality in patients with PAH-CHD when evaluated as a continuous variable (HR 0.665; 95% CI 0.503-0.878; P=0.004).
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