Naofumi F. Sumitomo, Taku Ishii, Keiko Uchida, Susumu Hosokawa, Reina Ishizaki, Yoshiyuki Furutani, Hidekazu Ishida, Shinichi Takatsuki, Kei Inai, Shigetoyo Kogaki, Hiroyuki Fukushima, Yuichi Tamura, Hiroyuki Yamagishi, Shozaburo Doi, on behalf of The Japanese Association of Congenital Heart Disease Registry (JACPHR) members
Keio University School of Medicine. Institute of Science Tokyo. Tokyo Medical University. Japanese Red Cross Mushashino Hospital. Tokyo Women’s Medical University. University of Osaka, Toho University Omori Medical Center. Osaka General Medical Center. Tokyo Dental College Ichikawa General Hospital. International University of Health and Welfare Mita Hospital. Tokyo Metropolitan Children’s Medical Center. Tokyo Health Care University.
Japan
American Heart Journal Plus
Am Heart J Plus 2026; 65:
DOI: 10.1016/j.ahjo.2026.100784
Abstract
Background: Post-operative pulmonary hypertension (PH) following biventricular repair of congenital heart disease (CHD) remains a high-risk condition, but its prognosis and risk stratification are poorly defined. This study aimed to identify prognostic factors and develop a simple clinical risk score.
Methods: Patients with post-operative PH enrolled in the Japan CHD-Pulmonary Hypertension Registry (2021-2024) were analyzed. Continuous and categorical variables were assessed by log-rank testing, and a composite risk score was developed to predict cardiovascular events. Prognostic performance was evaluated by Kaplan-Meier and Cox regression analyses.
Results: Among 84 patients (median age 11.6 years; 55% men), eight cardiovascular events occurred during a mean follow-up of 18.2 months: three all-cause deaths, three hospitalizations for heart failure, and two cases of worsening functional class. Mean pulmonary artery pressure ≥30 mmHg, pulmonary vascular resistance index ≥4.0 WU·m2, B-type natriuretic peptide ≥40 pg/mL or NT-proBNP ≥300 pg/mL, and WHO functional class ≥III were significant prognostic factors. An equal-weighted score (0-4 points) stratified patients into low- (0-1), moderate- (2-3), and high-risk (4) groups with distinct survival (hazard ratio, 3.61; p <0.001).
Conclusions: This nationwide, registry-based study provides new insights into prognostic determinants and risk stratification in patients with post-operative PH. This study identified practical prognostic factors and proposed a simple, exploratory equal-weighted risk score. Prognostic significance was evident even at thresholds below conventional criteria, highlighting the vulnerability of this population and supporting the score’s potential clinical utility pending external validation.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing
Diagnostic Testing for Pulmonary Vascular Disease. Risk Stratification
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
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Free PDF File or Full Text Article Available Through PubMed or DOI: Yes