Congenital Heart Disease, Fontan Operation, Pulmonary Vascular Remodeling, Pulmonary Vascular Resistance
Conditions
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.
Interventions
None listed
Sponsors
University Medical Center Groningen
Study design
Observational model
COHORT
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| 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 |
| number of intimal lesions in small pulmonary arteries | baseline at heart catheterization | number of intimal lesions in small pulmonary arteries |
Countries
Netherlands
Contacts
Primary ContactFloris-Jan S Ridderbos, MD
Backup ContactElke S Hoendermis, MD PhD
Outcome results
None listed