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

[II-JCKAP3-4] Real world data of PH with CHD in Japan: Insights from Japanese association of congenital heart disease with pulmonary hypertension registry JACPHR

^○Shozaburo Doi¹, Tak Ishii², Keiko Uchida³, Shigetoyo Kogaki⁴, Hidekazu Ishida⁵, Shinichi Takatsuki⁶, Hiroyuki Fukushima⁷, Kei Inai⁸, Susumu Hosokawa⁹, Hiroyuki Yamagishi¹⁰ (1.Tachikawa Faculty of Nursing, Tokyo Healthcare University, Tokyo, 2.Tokyo Medical and Dental University, Tokyo, 3.Keio University, Tokyo, 4.Osaka General Medical Center, Osaka, 5.Toho University, Tokyo, 6.Osaka University, Osaka, 7.Tokyo Dental College Ichikawa General Hospital, Chiba, 8.Tokyo Women's Medical University, Tokyo, 9.Japanese Red Cross Musashino Hospital, Tokyo, 10.Tokyo Metropolitan Children's Medical Center,Tokyo)

Keywords:right heart catheterization, CHD-PH registry, children to adults

Objecive: To clarify detailed characteristics, operability and prognosis of patients of pulmonary hypertension with congenital heart disease (CHD-PH) Methods: Japanese association of congenital heart disease with pulmonary hypertension registry (JACPHR) is a multicenter, prospective observational registry study for patients diagnosed with CHD-PH from children to adults. Inclusion criteria required a mean pulmonary artery pressure (PAP) > 20 mmHg and pulmonary vascular resistance index > 3 WU･m2 by right heart catheterization. Patients with single ventricle are eligible for enrollment according to different criteria (TPG > 6 mmHg and PVRI >3 WU･m2 for single ventricle). Results: 419 patients were registered during 2.5 years. Patients were classified into seven groups according to clinical diagnosis as follows; Eisenmenger syndrome (ES), systemic-pulmonary shunts, coincidental PH, post-operative PH, left heart disease, single ventricle and segmental PH. Median mean PAP (mPAP) at enrollment excluding single ventricle patients was 27mmHg. Both mPAP and TPG were decreased at enrollment compared with on diagnosis in patients with single ventricle. Combination therapy was held in patients with higher mPAP, ES, coincidental and segmental PH. Macitentan and tadarafil were most frequently used in patients. Decrease in mPAP was not associated with or without medication, hereditary syndrome or respiratory disorders, but age and classification on diagnosis. Conclusion: We will continue prospective follow-up to elucidate the prognosis of CHD-PH patients in Japan.