PMID- 30201381
OWN - NLM
STAT- MEDLINE
DCOM- 20190827
LR  - 20190827
IS  - 1874-1754 (Electronic)
IS  - 0167-5273 (Linking)
VI  - 276
DP  - 2019 Feb 1
TI  - Mode of death and predictors of mortality in adult Fontan survivors: A Japanese 
      multicenter observational study.
PG  - 74-80
LID - S0167-5273(18)34483-8 [pii]
LID - 10.1016/j.ijcard.2018.09.002 [doi]
AB  - 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.
CI  - Copyright (c) 2018. Published by Elsevier B.V.
FAU - Ohuchi, Hideo
AU  - Ohuchi H
AD  - Department of Pediatric Cardiology and Adult Congenital Heart Disease, National 
      Cerebral and Cardiovascular Center, Suita, Osaka, Japan. Electronic address: 
      hohuchi@ncvc.go.jp.
FAU - Inai, Kei
AU  - Inai K
AD  - Pediatric Cardiology, Tokyo Women's Medical University, Shinjuku, Tokyo, Japan.
FAU - Nakamura, Makoto
AU  - Nakamura M
AD  - Cardiology, Fukuoka Children's Hospital, Fukuoka, Fukuoka, Japan.
FAU - Park, In-Sam
AU  - Park IS
AD  - Pediatric Cardiology, Sakakibara Heart Institution, Fuchu, Tokyo, Japan.
FAU - Watanabe, Mamie
AU  - Watanabe M
AD  - Pediatric Cardiology, Kyushu Hospital (JCHO), Kitakyushu, Fukuoka, Japan.
FAU - Hiroshi, Ono
AU  - Hiroshi O
AD  - Cardiology, National Center for Child Health and Development, Setagaya-ku, Tokyo, 
      Japan.
FAU - Kim, Ki-Sung
AU  - Kim KS
AD  - Cardiology, Kanagawa Children's Medical Center, Yokohama, Kanagawa, Japan.
FAU - Sakazaki, Hisanori
AU  - Sakazaki H
AD  - Pediatric Cardiology, Hyogo Prefectural Amagasaki General Medical Center, 
      Amagasaki, Hyogo, Japan.
FAU - Waki, Kenji
AU  - Waki K
AD  - Pediatric Cardiology, Kurashiki Central Hospital, Kurashiki, Okayama, Japan.
FAU - Yamagishi, Hiroyuki
AU  - Yamagishi H
AD  - Pediatrics, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan.
FAU - Yamamura, Kenichiro
AU  - Yamamura K
AD  - Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, 
      Fukuoka, Japan.
FAU - Kuraishi, Kenji
AU  - Kuraishi K
AD  - Pediatric Cardiology and Neonatology, Ogaki Municipal Hospital, Ogaki, Gifu, 
      Japan.
FAU - Miura, Masaru
AU  - Miura M
AD  - Cardiology, Tokyo Metropolitan Children's Medical Center, Fuchu, Tokyo, Japan.
FAU - Nakai, Michikazu
AU  - Nakai M
AD  - Preventive Medicine and Epidemiologic Informatics, Center for Cerebral and 
      Cardiovascular Center, Japan.
FAU - Nishimura, Kunihiro
AU  - Nishimura K
AD  - Preventive Medicine and Epidemiologic Informatics, Center for Cerebral and 
      Cardiovascular Center, Japan.
FAU - Niwa, Koichiro
AU  - Niwa K
AD  - Cardiology, St Luke's International Hospital, Chuo-ku, Tokyo, Japan.
CN  - JSACHD Fontan Investigators
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
PT  - Observational Study
DEP - 20180905
PL  - Netherlands
TA  - Int J Cardiol
JT  - International journal of cardiology
JID - 8200291
SB  - IM
CIN - Int J Cardiol. 2019 Feb 1;276:110-111. PMID: 30340849
MH  - Adolescent
MH  - Adult
MH  - Cause of Death/*trends
MH  - Female
MH  - Follow-Up Studies
MH  - Fontan Procedure/*mortality/*trends
MH  - Heart Diseases/diagnosis/*mortality
MH  - Humans
MH  - Japan/epidemiology
MH  - Male
MH  - Middle Aged
MH  - Neoplasms/diagnosis/*mortality
MH  - Predictive Value of Tests
MH  - Prospective Studies
MH  - Survival Rate/trends
MH  - Young Adult
OTO - NOTNLM
OT  - Adult Fontan
OT  - Arrhythmia
OT  - Cancer
OT  - Heart failure
OT  - Mortality
EDAT- 2018/09/12 06:00
MHDA- 2019/08/28 06:00
CRDT- 2018/09/12 06:00
PHST- 2018/07/16 00:00 [received]
PHST- 2018/08/28 00:00 [revised]
PHST- 2018/09/03 00:00 [accepted]
PHST- 2018/09/12 06:00 [pubmed]
PHST- 2019/08/28 06:00 [medline]
PHST- 2018/09/12 06:00 [entrez]
AID - S0167-5273(18)34483-8 [pii]
AID - 10.1016/j.ijcard.2018.09.002 [doi]
PST - ppublish
SO  - Int J Cardiol. 2019 Feb 1;276:74-80. doi: 10.1016/j.ijcard.2018.09.002. Epub 2018 
      Sep 5.