[II-JCKAP3-1] Predicting Outcome of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease using the Index of Pulmonary Vascular Disease
Keywords:congenital heart disease, index of pulmonary vascular disease, pulmonary arterial hypertension
Objective: Limited data exist on long-term outcome risk factors for pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD-PAH). We focused on the index of pulmonary vascular disease (IPVD), an assessment system classifying pulmonary vascular lesions. This study aimed to investigate the relationship between IPVD and CHD-PAH long-term outcomes.Methods: A retrospective study examined lung pathology images of 764 CHD-PAH patients (>20 years) submitted to the Japanese Research Institute of Pulmonary Vasculature from 2001 to 2020. Clinical information was collected retrospectively. The primary endpoint was cardiovascular death.Results: The 5-, 10-, 15-, and 20-year cardiovascular death-free survival rates for all patients were 92.0%, 90.4%, 87.3%, and 86.1%, respectively. The group with IPVD 2.0 or more had significantly poorer survival than IPVD >2.0 (P = .037). Adjusted Cox proportional hazards model confirmed similar results (HR 4.46; 95% CI: 1.45-13.73; P = .009).Conclusions: The IPVD scoring system predicts CHD-PAH long-term outcomes. For IPVD 2.0 or more, careful consideration of treatment strategies, including palliative procedures and postponement of intracardiac repair, is recommended.