The Role of the Pulmonary Vasculature in the Fontan Circulation
1 other identifier
observational
20
1 country
1
Brief Summary
This study aims to explore the structural and functional characteristics of the pulmonary vasculature in adult Fontan patients. Objectives:
- Determine the effect of pulmonary vasodilatation on indexed cardiac output during simulated exercise.
- Characterization of structural properties of small pulmonary arteries.
Trial Health
Trial Health Score
Automated assessment based on enrollment pace, timeline, and geographic reach
participants targeted
Target at below P25 for all trials
Started Jun 2015
Longer than P75 for all trials
1 active site
Health score is calculated from publicly available data and should be used for screening purposes only.
Trial Relationships
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Study Timeline
Key milestones and dates
First Submitted
Initial submission to the registry
March 30, 2015
CompletedFirst Posted
Study publicly available on registry
April 10, 2015
CompletedStudy Start
First participant enrolled
June 1, 2015
CompletedPrimary Completion
Last participant's last visit for primary outcome
June 30, 2024
CompletedStudy Completion
Last participant's last visit for all outcomes
October 30, 2024
CompletedNovember 28, 2023
November 1, 2023
9.1 years
March 30, 2015
November 27, 2023
Conditions
Outcome Measures
Primary Outcomes (2)
vasodilatation
change in indexed cardiac output between baseline and after pulmonary vasodilatation
change in indexed cardiac output between baseline and after 10 minutes of pulmonary vasodilatation at heart catheterization
number of intimal lesions in small pulmonary arteries
number of intimal lesions in small pulmonary arteries
baseline at heart catheterization
Study Arms (3)
Fontan group
Right heart catheterization: * pulmonary artery OCT analysis * dobutamine stress test * pulmonary vascular response test (nitric oxide) * trans-thoracic echocardiography
Control group
Right heart catheterization: \- pulmonary artery OCT analysis
PAH group
Right heart catheterization: \- pulmonary artery OCT analysis
Eligibility Criteria
All adult patients (≥18 years), who attend the University Medical Center Groningen for follow-up after a Fontan completion. Additionally 5 adult patients (≥18 years) without a Fontan circulation and without a univentricular heart-like diagnosis but with a clinical indication for right heart catheterization and normal values at right heart catheterization measurements will be included to serve as controls for the pulmonary artery OCT analysis.
You may qualify if:
- Fontan group
- written informed consent
- Clinical indication for cardiac catheterization
- Control group
- written informed consent
- Clinical indication for right heart catheterization
- Absence of pulmonary vascular disease
- Normal pulmonary vascular hemodynamic profile
You may not qualify if:
- Fontan group
- Obstruction in Fontan conduit
- Inability to measure a reliable cardiac index and PVR (rhythm instability, hemodynamic or anatomic reasons)
- Control Group
Contact the study team to confirm eligibility.
Sponsors & Collaborators
Study Sites (1)
University Medical Center Groningen
Groningen, 9713 GZ, Netherlands
Related Publications (7)
Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971 May;26(3):240-8. doi: 10.1136/thx.26.3.240.
PMID: 5089489BACKGROUNDWolff D, van Melle JP, Ebels T, Hillege H, van Slooten YJ, Berger RM. Trends in mortality (1975-2011) after one- and two-stage Fontan surgery, including bidirectional Glenn through Fontan completion. Eur J Cardiothorac Surg. 2014 Apr;45(4):602-9. doi: 10.1093/ejcts/ezt461. Epub 2013 Sep 25.
PMID: 24067749BACKGROUNDde Leval MR, Deanfield JE. Four decades of Fontan palliation. Nat Rev Cardiol. 2010 Sep;7(9):520-7. doi: 10.1038/nrcardio.2010.99. Epub 2010 Jun 29.
PMID: 20585329BACKGROUNDGewillig M, Brown SC, Eyskens B, Heying R, Ganame J, Budts W, La Gerche A, Gorenflo M. The Fontan circulation: who controls cardiac output? Interact Cardiovasc Thorac Surg. 2010 Mar;10(3):428-33. doi: 10.1510/icvts.2009.218594. Epub 2009 Dec 7.
PMID: 19995891BACKGROUNDGoldberg DJ, Avitabile CM, McBride MG, Paridon SM. Exercise capacity in the Fontan circulation. Cardiol Young. 2013 Dec;23(6):824-30. doi: 10.1017/S1047951113001649.
PMID: 24401254BACKGROUNDKhambadkone S, Li J, de Leval MR, Cullen S, Deanfield JE, Redington AN. Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation. Circulation. 2003 Jul 1;107(25):3204-8. doi: 10.1161/01.CIR.0000074210.49434.40. Epub 2003 Jun 23.
PMID: 12821557BACKGROUNDRidderbos FJ, Wolff D, Timmer A, van Melle JP, Ebels T, Dickinson MG, Timens W, Berger RM. Adverse pulmonary vascular remodeling in the Fontan circulation. J Heart Lung Transplant. 2015 Mar;34(3):404-13. doi: 10.1016/j.healun.2015.01.005. Epub 2015 Jan 16.
PMID: 25813767BACKGROUND
MeSH Terms
Conditions
Condition Hierarchy (Ancestors)
Study Officials
- PRINCIPAL INVESTIGATOR
Rolf M Berger, MD PhD
University Medical Center Groningen
Central Study Contacts
Study Design
- Study Type
- observational
- Observational Model
- COHORT
- Time Perspective
- CROSS SECTIONAL
- Sponsor Type
- OTHER
- Responsible Party
- PRINCIPAL INVESTIGATOR
- PI Title
- Professor and Head of Department of Pediatric Cardiology
Study Record Dates
First Submitted
March 30, 2015
First Posted
April 10, 2015
Study Start
June 1, 2015
Primary Completion
June 30, 2024
Study Completion
October 30, 2024
Last Updated
November 28, 2023
Record last verified: 2023-11