Taku Ishii, Tatsuhiko Anzai, Keiko Uchida, Susumu Hosokawa, Naofumi F. Sumitomo, Hidekazu Ishida, Keiichi Hirono, Jun Muneuchi, Ayako Chida-Nagai, Ryo Inuzuka, Hirofumi Sawada, Sayo Suzuki, Jun Maeda, Hisaaki Aoki, Lisheng Lin, Takashi Murakami, Yusuke Nakano, Tatsuya Onishi, Takuya Wakamiya, Kei Inai, Shinichi Takatsuki, Atsushi Yao, Shigetoyo Kogaki, Hiroyuki Fukushima, Yuichi Tamura, Kunihiko Takahashi, Hiroyuki Yamagishi, Shozaburo Doi
Institute of Science Tokyo. Tokyo Medical University. Japanese Red Cross Musashino Hospital. Keio University. University of Osaka Graduate School of Medicine. Toyama University Hospital. Japan Community Health Care Organization Kyushu Hospital. Hokkaido University. University of Tokyo. Mie University. Fukuoka Children’s Hospital. Tokyo Metropolitan Children’s Medical Center. Osaka Women’s and Children’s Hospital. Ibaraki Children’s Hospital. University of Tsukuba. Yokohama City University. National Hospital Organization Shikoku Medical Center for Children and Adults. Kanagawa Children’s Medical Center. Tokyo Women’s Medical University. Toho University Omori Medical Center. Osaka General Medical Center. Tokyo Dental College Ichikawa General Hospital. International University of Health and Welfare.
Japan
International Journal of Cardiology Congenital Heart Disease
Int J Cardiol Congenit Heart Dis 2026; 25
DOI: 10.1016/j.ijcchd.2026.100679
Abstract
Background: Pulmonary hypertension (PH) is a major determinant of outcomes in congenital heart disease (CHD), yet tools for individualized prognostic estimation are limited. This study aimed to develop an exploratory, clinically oriented prediction model for estimating cardiac event-free survival in patients with CHD-PH.
Methods: Data from the nationwide Japanese Association of CHD-PH Registry were analyzed in a retrospective cohort derived from a prospectively maintained registry. Cardiac event-free survival was evaluated using Cox proportional hazards models incorporating prespecified, routinely available clinical and hemodynamic variables. Cardiac events were defined as death, transplantation, clinical worsening requiring treatment escalation, or PH-related hospitalization.
Results: A total of 224 patients were included, and 23 experienced 29 cardiac events during a median follow-up of 1.5 years. These included 7 cardiovascular deaths, 1 lung transplantation, and 1 atrial septostomy, 13 cases of clinical worsening. The cardiac event-free survival rates were 94.0% and 86.2% at 1 and 2 years, respectively. In multivariable analysis, trisomy 21 (hazard ratio [HR] 3.63), elevated pulmonary vascular resistance index (PVRI; HR 13.2), and elevated central venous pressure (CVP; HR 3.27) were associated with worse outcomes. These associations were consistent in sensitivity and subgroup analyses. Model-based estimates demonstrated risk gradients across clinically relevant profiles and were incorporated into a prototype prediction tool.
Conclusions: An exploratory pragmatic prediction model for cardiac event-free survival in CHD-PH was developed using nationwide registry data. These findings support the feasibility of a registry-based prognostic framework; however, further validation is required before clinical application.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Genetic Factors Associated with Pulmonary Vascular Disease
Acquired Patient Factors Associated with Pulmonary Vascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes