Mode of death and predictors of mortality in adult Fontan survivors: A Japanese multicenter observational study

• Published in: International journal of cardiology 2019-02, Vol.276, p.74-80
• Main Authors: Ohuchi, Hideo, Inai, Kei, Nakamura, Makoto, Park, In-Sam, Watanabe, Mamie, Hiroshi, Ono, Kim, Ki-Sung, Sakazaki, Hisanori, Waki, Kenji, Yamagishi, Hiroyuki, Yamamura, Kenichiro, Kuraishi, Kenji, Miura, Masaru, Nakai, Michikazu, Nishimura, Kunihiro, Niwa, Koichiro
• Format: Article
• Language: English
• Subjects: Adult Fontan; Arrhythmia; Cancer; Heart failure; Mortality
• ISSN: 0167-5273; 1874-1754
• DOI: 10.1016/j.ijcard.2018.09.002

Mortality rates may be high in adult Fontan patients; however, the clinical determinants remain unclear. We conducted a prospective multicenter study of adult Fontan survivors to determine the 5-year mortality rate and clarify the determinants. 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 40 years.