JCK-AP session 4 (II-JCKAP4)
Fontan

[II-JCKAP4-3] Incidence and current clinical practice for all-cause morbidity in patients with Fontan circulation (Japan Fontan Registry: JFR)

^○Hideo Ohuchi¹, Kota Takei², Jun Muneuchi³, Shingo Kasahara⁴, Yuichi Ishikawa⁵, Masanori Tsukada⁶, Masaki Nii⁷, Shin Ono⁸, Hirofumi Saiki⁹ (1.Department of Pediatric Cardiology, National Cerebral and Cardiovascular Center, Osaka, 2.Pediatric Cardiology, Nagano Children's Hospital, Nagano, 3.Department of Pediatrics, Kyushu Hospital Community Healthcare Organization, Fukuoka, 4.Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences and Okayama University Hospital, Okayama, 5.Department of Pediatric Cardiology, Fukuoka Children's Hospital, Fukuoka, 6.Department of Pediatrics, Niigata University Graduate School of Medical and Dental Sciences, Niigata, 7.Department of Pediatric Cardiology, Shizuoka Children's Hospital, Shizuoka, 8.Department of Pediatric Cardiology, Kanagawa Children's Medical Center, Kanagawa, 9.Department of Pediatrics, Iwate Medical University, Iwate)

Keywords:Fontan circulation, morbidity, hospitalization

Objective: To clarify the incidence and clinical practice of unscheduled hospitalization (USH) in patients with Fontan circulation (FC). Method: We conducted a multi-center prospective study with 3-year registration of USH FC patients.Results: We followed 3225 FC patients in 20 institutions and 243 (7.5%) patients encountered USH. The most common cause was heart failure (HF, 19.3%), followed by infection (18.9%) and protein losing enteropathy (PLE, 16.5%), hemorrhage (13.2%), arrhythmia (9.5%), thromboembolism (TE, 2.5%), plastic bronchitis (1.6%), and others (17.7%). O2 inhalation was the most common non-pharmacological managements (38%), followed by surgery (12%), and catheter intervention (10%), while diuretics (iv) was the most common pharmacological managements (28%), followed by antibiotics (iv) (28%), and heparin (iv) (11%). Overall median USH-stay (days) was 9 and the longest stay was due to PLE (20), followed by TE (17) and HF (13). After discharge, 76 (31%) patients re-hospitalized with the highest cause of PLE (34%), followed by HF (16%) and hemorrhage and infection (13% for each). Leukopenia and hypoproteinemia at discharge were independently associated with a high risk of re-USH (p<0.05). Conclusions: We clarified current practices in FC patients in Japan. Further analyses of each USH are necessary for establishing management strategy.