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

[II-JCKAP3-2] Pharmacological treatment for pediatric patients with congenital heart disease with pulmonary hypertension: Insights from nationwide registry in Japan

^○Taku Ishii¹, Susumu Hosokawa², Naofumi Sumitomo³, Hidekazu Ishida⁴, Shinichi Takatsuki⁵, Keiko Uchida⁶, Hiroyuki Fukushima⁷, Shigetoyo Kogaki⁸, Hiroyuki Yamagishi⁹, Shozaburo Doi¹⁰ (1.Department of Pediatrics, Tokyo Medical and Dental University, Tokyo, 2.Department of Pediatricsese Red Cross Musashino Hospital, Tokyo, 3.Department of Pediatrics, Keio University, Tokyo, 4.Department of Pediatrics, Osaka University, Osaka, 5.Department of Pediatrics, Toho University, Tokyo, 6.Department of Physiology, Tokyo Medical University, Tokyo, 7.Department of Pediatrics, Tokyo Dental Colledge Ichikawa General Hospital, Chiba, 8.Department of Pediatrics, Osaka Medical Center, Osaka, 9.Department of Pediatrics, Tokyo Metropolitan Children's Medical Center, Tokyo, 10.Department of Nursing Discipline, Tokyo Health Care University, Tokyo)

キーワード：CHD-PAH, pharmacological treatment, efficacy

Background: The efficacy of pharmacological treatment for pediatric patients with congenital heart disease with pulmonary hypertension (CHD-PH) remains unclear. Methods: The multi-center registry study on CHD-PH in Japan began in August 2021. From this registry, patients aged 15 years old or younger at the time of diagnosis were selected, while those with a single ventricle were excluded. Results: 215 patients were included in this study. The median age of patients at enrollment was 8.5 years old. Approximately 60% of the enrolled patients had some form of comorbidity, including trisomy 21. Pharmacological treatments received by the enrolled patients were as follows: no medication in 74 cases (34.4%), monotherapy in 61 cases (28.4%), and combination therapy in 80 cases (37.2%). The medication used predominantly were endothelin receptor blocker and PDE-5 inhibitors, each being administered to approximately half of the patients. In the 85 cases where past and registration catheter results were comparable, 19 patients (22.4%) showed an increase in mPAP of over 20%. In logistic regression analysis, even after adjusting for age at diagnosis, mPAP at diagnosis, and clinical classification, the odds ratio for worsening mPAP remained high in the group not receiving medication.Conclusion: In pediatric patients with CHD-PH, pharmacological treatment for pulmonary hypertension is essential.