Hideo Ohuchi1, Kei Inai2, Makoto Nakamura3, In-Sam Park4, Mamie Watanabe5, Ono Hiroshi6, Ki-Sung Kim7, Hisanori Sakazaki8, Kenji Waki9, Hiroyuki Yamagishi10, Kenichiro Yamamura11, Kenji Kuraishi12, Masaru Miura13, Michikazu Nakai14, Kunihiro Nishimura14, Koichiro Niwa15. 1. Department of Pediatric Cardiology and Adult Congenital Heart Disease, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan. Electronic address: hohuchi@ncvc.go.jp. 2. Pediatric Cardiology, Tokyo Women's Medical University, Shinjuku, Tokyo, Japan. 3. Cardiology, Fukuoka Children's Hospital, Fukuoka, Fukuoka, Japan. 4. Pediatric Cardiology, Sakakibara Heart Institution, Fuchu, Tokyo, Japan. 5. Pediatric Cardiology, Kyushu Hospital (JCHO), Kitakyushu, Fukuoka, Japan. 6. Cardiology, National Center for Child Health and Development, Setagaya-ku, Tokyo, Japan. 7. Cardiology, Kanagawa Children's Medical Center, Yokohama, Kanagawa, Japan. 8. Pediatric Cardiology, Hyogo Prefectural Amagasaki General Medical Center, Amagasaki, Hyogo, Japan. 9. Pediatric Cardiology, Kurashiki Central Hospital, Kurashiki, Okayama, Japan. 10. Pediatrics, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan. 11. Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Fukuoka, Japan. 12. Pediatric Cardiology and Neonatology, Ogaki Municipal Hospital, Ogaki, Gifu, Japan. 13. Cardiology, Tokyo Metropolitan Children's Medical Center, Fuchu, Tokyo, Japan. 14. Preventive Medicine and Epidemiologic Informatics, Center for Cerebral and Cardiovascular Center, Japan. 15. Cardiology, St Luke's International Hospital, Chuo-ku, Tokyo, Japan.
Abstract
BACKGROUND: Mortality rates may be high in adult Fontan patients; however, the clinical determinants remain unclear. PURPOSE: We conducted a prospective multicenter study of adult Fontan survivors to determine the 5-year mortality rate and clarify the determinants. METHOD AND RESULTS: We followed 600 adult Fontan survivors from 40 Japanese institutions (307 men, 28 ± 7 years old, follow-up: 18 ± 6 years). The New York Heart Association (NYHA) functional class I and II was 51% and 42%, respectively. During the follow-up period of 4.1 ± 1.6 years, 33 patients died, and the 5-year survival rate was 93.5%. The mode of death was heart failure in 11 patients (34%), arrhythmia or sudden death in 8 (24%), cancer in 5 (15%), perioperative problems and hemostatic problems in 4 each (12% for each), and infection in 1 (3%). Left isomerism, prior hospitalization, protein losing enteropathy (PLE), pulmonary arteriovenous fistulae, NYHA functional class, impaired hemodynamics, hyponatremia, hepatorenal dysfunction, and use of diuretics were associated with a high mortality rate (p < 0.05-0.0001). Further, PLE (hazard ratio [HR]: 14.4), left isomerism (HR: 3.5), and NYHA (HR: 2.4) independently predicted a high 5-year high mortality (p < 0.05 for all). The incidence of cancer-related mortality increased markedly with age >40 years. CONCLUSIONS: Majority of the Japanese adult Fontan survivors had good functional status, with an acceptable 5-year survival rate. However, the significant prevalence of non-cardiac mortality highlights Fontan pathophysiology as a multi-organ disease that requires a multidisciplinary management strategy to improve the long-term outcome.
BACKGROUND: Mortality rates may be high in adult Fontan patients; however, the clinical determinants remain unclear. PURPOSE: We conducted a prospective multicenter study of adult Fontan survivors to determine the 5-year mortality rate and clarify the determinants. METHOD AND RESULTS: We followed 600 adult Fontan survivors from 40 Japanese institutions (307 men, 28 ± 7 years old, follow-up: 18 ± 6 years). The New York Heart Association (NYHA) functional class I and II was 51% and 42%, respectively. During the follow-up period of 4.1 ± 1.6 years, 33 patients died, and the 5-year survival rate was 93.5%. The mode of death was heart failure in 11 patients (34%), arrhythmia or sudden death in 8 (24%), cancer in 5 (15%), perioperative problems and hemostatic problems in 4 each (12% for each), and infection in 1 (3%). Left isomerism, prior hospitalization, protein losing enteropathy (PLE), pulmonary arteriovenous fistulae, NYHA functional class, impaired hemodynamics, hyponatremia, hepatorenal dysfunction, and use of diuretics were associated with a high mortality rate (p < 0.05-0.0001). Further, PLE (hazard ratio [HR]: 14.4), left isomerism (HR: 3.5), and NYHA (HR: 2.4) independently predicted a high 5-year high mortality (p < 0.05 for all). The incidence of cancer-related mortality increased markedly with age >40 years. CONCLUSIONS: Majority of the Japanese adult Fontan survivors had good functional status, with an acceptable 5-year survival rate. However, the significant prevalence of non-cardiac mortality highlights Fontan pathophysiology as a multi-organ disease that requires a multidisciplinary management strategy to improve the long-term outcome.
Authors: Ja-Kyoung Yoon; Gi Beom Kim; Mi Kyoung Song; Sang Yun Lee; Seong Ho Kim; So Ick Jang; Woong Han Kim; Chang-Ha Lee; Kyung Jin Ahn; Eun Jung Bae Journal: Korean Circ J Date: 2022-03-16 Impact factor: 3.101