American heart journal plus : cardiology research and practice · 2026
Mid-term prognosis and risk stratification in patients with post-operative pulmonary hypertension: Insights from the Japanese Association of Congenital Heart Disease Registry (JACPHR).
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Sumitomo Naofumi F, Ishii Taku, Uchida Keiko, Hosokawa Susumu, Ishizaki Reina, Furutani Yoshiyuki, Ishida Hidekazu, Takatsuki Shinichi, Inai Kei, Kogaki Shigetoyo, Fukushima Hiroyuki, Tamura Yuichi, Yamagishi Hiroyuki, Doi Shozaburo, Japanese Association of Congenital Heart Disease Registry (JACPHR) members
Аннотация
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.
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.