JCK-AP session 3 (II-JCKAP3)
Pulmonary Circulation

[II-JCKAP3-1] Predicting Outcome of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease using the Index of Pulmonary Vascular Disease

^○Ayako Chiba-Nagai^1,2,3, Naoki Masaki², Kay Maeda², Konosuke Sasaki², Hiroki Sato⁴, Atsushi Manabe¹, Yoshikatsu Saiki¹ (1.Department of Pediatrics, Hokkaido University, Hokkaido, 2.Division of Cardiovascular Surgery, Tohoku University Graduate School of Medicine, Miyagi, 3.Department of Pediatric Cardiology and Adult Congenital Cardiology, Tokyo Women's Medical University, Tokyo, 4.Department of Cardiology and Clinical Examination, Oita University, Oita)

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.