Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry
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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.
| Originalsprog | Engelsk |
|---|---|
| Tidsskrift | Cardiology in the Young |
| Vol/bind | 32 |
| Udgave nummer | 4 |
| Sider (fra-til) | 597-605 |
| Antal sider | 9 |
| ISSN | 1047-9511 |
| DOI | |
| Status | Udgivet - 2022 |
Bibliografisk note
Funding Information:
Conflicts of interest. LS has nothing to disclose. JA has received institutional research funding from Janssen Pharmaceutical Companies of Johnson & Johnson. Yd’U has received consultant fees from Janssen Pharmaceutical Companies of Johnson & Johnson and MSD, and travel fees from Berlin Heart. Yd’U is a Clinician Practitioner Fellow of the NHMRC (1082186). Murdoch Children’s Research Institute is supported by the Victorian Government’s Operational Infrastructure Support Program. CF is an employee of Mapi (an ICON Plc company). Actelion (a Janssen Pharmaceutical Company of Johnson & Johnson) contracted Mapi to conduct this study. WJF is a consultant for Actelion US, a Janssen Pharmaceutical Company of Johnson & Johnson. AH is a consultant for Bayer. In the last 5 years, he has received speaker honoraria from Janssen Pharmaceutical Companies of Johnson & Johnson, OMT, AOP Orphan, Pfizer, GlaxoSmithKline, Medtronic, and Schiller. He is shareholder (<25,000 EUR) of Johnson & Johnson, Gilead, and Merck & Co. Inc. (MSD), as well as of funds investing in healthcare companies. His institution has participated in studies for Janssen Pharmaceutical Companies of Johnson & Johnson, Lilly, Bayer, Bristol Myers Squibb, Medtronic, Novartis, Pfizer, Edwards, and Occlutec. His institution has received unrestricted grants for investigator-initiated trials from Janssen Pharmaceutical Companies of Johnson & Johnson and Medtronic. YK has received institutional research funding from Janssen Pharmaceutical Companies of Johnson & Johnson. EM was an employee of Actelion Pharmaceuticals Ltd, a Janssen Pharmaceutical Company of Johnson & Johnson, at the time of the submitted work and is now an employee of F. Hoffmann-La Roche Ltd. DR is an employee of Actelion Pharmaceuticals Ltd, a Janssen Pharmaceutical Company of Johnson & Johnson. MS has received institutional research funding from Janssen Pharmaceutical Companies of Johnson & Johnson. PC is a paid consultant for Actelion Pharmaceuticals Ltd, a Janssen Pharmaceutical Company of Johnson & Johnson.
Funding Information:
Financial support. The PREpArE-Fontan registry was sponsored by Actelion Pharmaceuticals Ltd, a Janssen Pharmaceutical Company of Johnson & Johnson.
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