Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry.
Søndergaard, Lars Aboulhosn, Jamil d'Udekem, Yves Faure, Céline Franklin, Wayne J Hager, Alfred Kim, Yuli Y Muros-Le Rouzic, Erwan Rosenberg, Daniel Schwerzmann, Markus
Søndergaard, Lars
Aboulhosn, Jamil
d'Udekem, Yves
Faure, Céline
Franklin, Wayne J
Hager, Alfred
Kim, Yuli Y
Muros-Le Rouzic, Erwan
Rosenberg, Daniel
Schwerzmann, Markus
Abstract
The Patient Registry for Adolescents and Adults with Stable Fontan Circulation aims to describe a contemporary cohort of Fontan patients who could be eligible for a clinical trial investigating macitentan, an endothelin receptor antagonist. This international, non-interventional, multicentre, cross-sectional, observational registry enrolled patients with "stable" Fontan circulation ≥10 years following extra-cardiac conduit or lateral tunnel procedure. Main exclusion criteria were NYHA functional class IV, reoperation of Fontan circulation, or signs of disease worsening. Patient characteristics at enrolment are described; available data were collected during a single registration visit. Of the 266 screened patients, 254 were included in this analysis. At enrolment, median (interquartile range) age was 24 (20;30) years, 37%/63% of patients were from the USA/Europe, 54% were male, 54%/47% had undergone extra-cardiac conduit/lateral tunnel procedures, and 95% were in NYHA functional class I or II. History of arrhythmia was more common in older patients and patients with lateral tunnel; overall prevalence was 19%. Most laboratory values were within the normal range but mean creatinine clearance was abnormally low (87.7 ml/min). Angiotensin-converting enzyme inhibitors were used by 48% of patients and their use was associated with creatinine clearance <90 ml/min (p = 0.007), as was Fontan completion at an older age (p = 0.007). 53.4% of patients had clinical characteristics that could potentially meet an endothelin receptor antagonist trial's eligibility criteria. The PREpArE-Fontan registry describes a cohort of patients who could potentially participate in an endothelin receptor antagonist trial and identified early subtle signs of Fontan failure, even in "stable" patients.
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Date
2021-07-23
Type
Article
Subject
Paediatrics, Public health. Health statistics. Occupational health. Health education
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Citation
Søndergaard L, Aboulhosn J, d'Udekem Y, Faure C, Franklin WJ, Hager A, Kim YY, Muros-Le Rouzic E, Rosenberg D, Schwerzmann M, Clift P. Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry. Cardiol Young. 2022 Apr;32(4):597-605. doi: 10.1017/S1047951121002791. Epub 2021 Jul 23
Journal / Source Title
Cardiology in the Young
DOI
10.1017/S1047951121002791
PMID
34294186
Publisher
Cambridge University Press
Publisher’s URL
https://www.cambridge.org/core/journals/cardiology-in-the-young