The Role of the Pulmonary Vasculature in the Fontan Circulation

Congenital Heart Disease Clinical Trial

Official title:

The Role of the Pulmonary Vasculature in the Fontan Circulation

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT02414321
• Other study ID #: NL51128.042.15
• Status: Recruiting
• Start date: June 2015
• Est. completion date: October 30, 2024

Study information

• Verified date: November 2023
• Source: University Medical Center Groningen
• Contact: Floris-Jan S Ridderbos, MD
• Phone: +31503611506
• Email: f.j.s.ridderbos[@]umcg.nl
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

Description:

Rationale:

The Fontan circulation is a palliation for patients with a functionally univentricular heart. The Fontan circulation is characterized by impaired exercise capacity and gradual attrition over time. To date treatment options are extremely limited. It has been proposed that pulmonary vascular resistance (PVR) is the controlling and limiting factor of cardiac output in the Fontan circulation. Remodeling of the pulmonary vasculature and increasing PVR are possible key factors in the long term failure of the Fontan circulation. Optical Coherence Tomography (OCT) is an intravascular imaging modality used for structural characterization of blood vessels. Nitric oxide is a pulmonary vasodilator used in pulmonary vascular response tests, aimed at lowering PVR which may improve cardiac output in the Fontan circulation. These two diagnostic procedures are tools to study the structural and functional characteristics of the pulmonary vasculature in the Fontan circulation. The results of this study could identify the pulmonary circulation as a future treatment target in Fontan patients and may provide clues for new therapeutic treatment strategies to improve the long term outcome of these patients.

Study procedure:

The study protocol will be performed during a clinically indicated cardiac catheterization. In the context of this study additional measurements will be performed which will include a pulmonary vascular response test, trans-thoracic echocardiography and pulmonary artery OCT measurements.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 20
• Est. completion date: October 30, 2024
• Est. primary completion date: June 30, 2024
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

Inclusion criteria:

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

Exclusion criteria:

Fontan group

• Standard exclusion criteria for cardiac catheterization

• Obstruction in Fontan conduit

• Inability to measure a reliable cardiac index and PVR (rhythm instability, hemodynamic or anatomic reasons)

Control Group

• No specific exclusion criteria apart from the standard exclusion criteria for right heart catheterization.

Related Conditions & MeSH terms

• Congenital Heart Disease
• Fontan Operation
• Heart Defects, Congenital
• Heart Diseases
• Pulmonary Vascular Remodeling
• Pulmonary Vascular Resistance
• Vascular Remodeling

Locations

Country	Name	City	State
Netherlands	University Medical Center Groningen	Groningen

Sponsors (1)

Lead Sponsor	Collaborator
University Medical Center Groningen

Country where clinical trial is conducted

Netherlands, 

References & Publications (7)

de 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. — View Citation

Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971 May;26(3):240-8. doi: 10.1136/thx.26.3.240. — View Citation

Gewillig 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. — View Citation

Goldberg 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. — View Citation

Khambadkone 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. — View Citation

Ridderbos 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. — View Citation

Wolff 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. — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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
Primary	number of intimal lesions in small pulmonary arteries	number of intimal lesions in small pulmonary arteries	baseline at heart catheterization