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- Title:
Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry.
- Document type:
- Article; Journal Article
- Author(s):
- 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; Clift, Paul
- 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.
- Journal title abbreviation:
- Cardiol Young
- Year:
- 2022
- Journal volume:
- 32
- Journal issue:
- 4
- Pages contribution:
- 597-605
- Fulltext / DOI:
- doi:10.1017/S1047951121002791
- Pubmed ID:
- http://view.ncbi.nlm.nih.gov/pubmed/34294186
- Print-ISSN:
- 1047-9511
- TUM Institution:
- Klinik für Kinderkardiologie und angeborene Herzfehler (Prof. Hess)
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