Mode of death and predictors of mortality in adult Fontan survivors

A Japanese multicenter observational study

On behalf of the JSACHD Fontan Investigators

Research output: Contribution to journalArticle

4 Citations (Scopus)

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.

Original languageEnglish
JournalInternational Journal of Cardiology
DOIs
Publication statusAccepted/In press - 2018 Jan 1

Fingerprint

Multicenter Studies
Observational Studies
Survivors
Mortality
Survival Rate
Protein-Losing Enteropathies
Isomerism
Hyponatremia
Hemostatics
Sudden Death
Diuretics
Cardiac Arrhythmias
Hospitalization
Heart Failure
Hemodynamics
Prospective Studies
Infection
Neoplasms

Keywords

  • Adult Fontan
  • Arrhythmia
  • Cancer
  • Heart failure
  • Mortality

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Mode of death and predictors of mortality in adult Fontan survivors : A Japanese multicenter observational study. / On behalf of the JSACHD Fontan Investigators.

In: International Journal of Cardiology, 01.01.2018.

Research output: Contribution to journalArticle

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title = "Mode of death and predictors of mortality in adult Fontan survivors: A Japanese multicenter observational study",
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.",
keywords = "Adult Fontan, Arrhythmia, Cancer, Heart failure, Mortality",
author = "{On behalf of the JSACHD Fontan Investigators} and Hideo Ohuchi and Kei Inai and Makoto Nakamura and Park, {In Sam} and Mamie Watanabe and Ono Hiroshi and Kim, {Ki Sung} and Hisanori Sakazaki and Kenji Waki and Hiroyuki Yamagishi and Kenichiro Yamamura and Kenji Kuraishi and Masaru Miura and Michikazu Nakai and Kunihiro Nishimura and Koichiro Niwa",
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T1 - Mode of death and predictors of mortality in adult Fontan survivors

T2 - A Japanese multicenter observational study

AU - On behalf of the JSACHD Fontan Investigators

AU - Ohuchi, Hideo

AU - Inai, Kei

AU - Nakamura, Makoto

AU - Park, In Sam

AU - Watanabe, Mamie

AU - Hiroshi, Ono

AU - Kim, Ki Sung

AU - Sakazaki, Hisanori

AU - Waki, Kenji

AU - Yamagishi, Hiroyuki

AU - Yamamura, Kenichiro

AU - Kuraishi, Kenji

AU - Miura, Masaru

AU - Nakai, Michikazu

AU - Nishimura, Kunihiro

AU - Niwa, Koichiro

PY - 2018/1/1

Y1 - 2018/1/1

N2 - 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.

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.

KW - Adult Fontan

KW - Arrhythmia

KW - Cancer

KW - Heart failure

KW - Mortality

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