Research
Print page Print page
Switch language
The Capital Region of Denmark - a part of Copenhagen University Hospital
Published

Adolescents and adults with Fontan circulation: insights from the PREpArE-Fontan registry

Research output: Contribution to journalJournal articleResearchpeer-review

  1. Chronic foetal hypoxaemia does not cause elevation of serum markers of brain injury

    Research output: Contribution to journalJournal articleResearchpeer-review

  2. Left ventricular thrombus in multisystem inflammatory syndrome in children associated with COVID-19

    Research output: Contribution to journalJournal articleResearchpeer-review

  3. Liver fibrosis in patients with tetralogy of Fallot, an unrecognised complication?

    Research output: Contribution to journalJournal articleResearchpeer-review

  1. Does Subclinical Leaflet Thrombosis Impact the Durability of Bioprosthetic Aortic Valves?

    Research output: Contribution to journalEditorialResearchpeer-review

  2. Percutaneous left atrial appendage closure in a surgically ligated left atrial appendage

    Research output: Contribution to journalJournal articleResearchpeer-review

  3. Mitral Valve Infective Endocarditis after Trans-Catheter Aortic Valve Implantation

    Research output: Contribution to journalJournal articleResearchpeer-review

  4. Microcirculatory Function in Nonhypertrophic and Hypertrophic Myocardium in Patients With Aortic Valve Stenosis

    Research output: Contribution to journalJournal articleResearchpeer-review

  • Lars Søndergaard
  • Jamil Aboulhosn
  • Yves d'Udekem
  • Céline Faure
  • Wayne J Franklin
  • Alfred Hager
  • Yuli Y Kim
  • Erwan Muros-Le Rouzic
  • Daniel Rosenberg
  • Markus Schwerzmann
  • Paul Clift
View graph of relations

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.

Original languageEnglish
JournalCardiology in the Young
Volume32
Issue number4
Pages (from-to)597-605
Number of pages9
ISSN1047-9511
DOIs
Publication statusPublished - Apr 2022

    Research areas

  • Congenital heart disease surgery, Fontan physiology, univentricular heart

ID: 72633629