The Role of the Pulmonary Vasculature in the Fontan Circulation

November 27, 2023 updated by: Prof. dr. R.M.F. Berger, University Medical Center Groningen

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.

Study Overview

Status

Recruiting

Conditions

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 Type

Observational

Enrollment (Estimated)

20

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Contact Backup

Study Locations

  • Netherlands
      • Groningen, Netherlands, 9713 GZ
        • Recruiting
        • University Medical Center Groningen
        • Principal Investigator:
          • Rolf MF Berger, MD PhD
        • Sub-Investigator:
          • Elke S Hoendermis, MD PhD
        • Sub-Investigator:
          • Floris-Jan S Ridderbos, MD

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

18 years and older (Adult, Older Adult)

Accepts Healthy Volunteers

No

Sampling Method

Non-Probability Sample

Study Population

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.

Description

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.

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

Cohorts and Interventions

Group / Cohort
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

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
vasodilatation
Time Frame: 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
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
Time Frame: baseline at heart catheterization
number of intimal lesions in small pulmonary arteries
baseline at heart catheterization

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

University Medical Center Groningen

Investigators

  • Principal Investigator: Rolf M Berger, MD PhD, University Medical Center Groningen

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start

June 1, 2015

Primary Completion (Estimated)

June 30, 2024

Study Completion (Estimated)

October 30, 2024

Study Registration Dates

First Submitted

March 30, 2015

First Submitted That Met QC Criteria

April 7, 2015

First Posted (Estimated)

April 10, 2015

Study Record Updates

Last Update Posted (Actual)

November 28, 2023

Last Update Submitted That Met QC Criteria

November 27, 2023

Last Verified

November 1, 2023

More Information

Terms related to this study

Additional Relevant MeSH Terms

Other Study ID Numbers

  • NL51128.042.15

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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