The Role of the Pulmonary Vasculature in the Fontan Circulation

Recruiting

• Conditions: Fontan Operation; Pulmonary Vascular Remodeling; Congenital Heart Disease; Pulmonary Vascular Resistance

• Registration Number: NCT02414321
• Lead Sponsor: University Medical Center Groningen

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.

Detailed 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.

Study Timeline

• Study First Submitted: 2015-03-30
• Study First Submitted QC: 2015-04-07
• Study First Posted: 2015-04-10
• Study Start: 2015-06
• Status Verified: 2023-11
• Last Update Submitted: 2023-11-27
• Last Update Posted: 2023-11-28
• Primary Completion: 2024-06-30
• Study Completion: 2024-10-30

Recruitment & Eligibility

• Status: RECRUITING
• Sex: All
• Target Recruitment: 20

Inclusion Criteria

Not provided

Exclusion Criteria

Not provided

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
number of intimal lesions in small pulmonary arteries	baseline at heart catheterization	number of intimal lesions in small pulmonary arteries
vasodilatation	change in indexed cardiac output between baseline and after 10 minutes of pulmonary vasodilatation at heart catheterization	change in indexed cardiac output between baseline and after pulmonary vasodilatation

Trial Locations

Locations (1)

University Medical Center Groningen; 🇳🇱 Groningen, Netherlands