Single-ventricle, Stenosis
Conditions
Keywords
Stent, fontan, Single-ventricle
Brief summary
The goal of this observational study is to assess the clinical impact following Fontan stent placement for extracardiac Fontan conduit (EFC) stenosis in patients with single-ventricle Fontan-palliated patients ages 12-21 years old. The main question it aims to: -Assess changes in exercise capacity by cardiopulmonary exercise testing and liver stiffness assessed by shear wave elastography before and after EFC stenting. Participants will undergo two cardiopulmonary exercise tests and three shear wave elastography scans.
Interventions
DIAGNOSTIC_TESTCardiopulmonary Exercise Testing (CPET)
CPET will be performed on a cycle ergometer using a ramp protocol designed to achieve an exercise time of 8-12 min. The entire procedure duration is approximately 60 min. CPET will be completed 6 months to 1 day before the cardiac catheterization and will be repeated 3-6 months following cardiac catheterization.
DIAGNOSTIC_TESTShear Wave Elastography Scan
The study lasts approximately 20 minutes. Shear Wave Elastography will be completed 6 months to 1 day before the cardiac catheterization. The Scan will be repeated 2-4 weeks following cardiac catheterization, and 3-6 months following cardiac catheterization.
Sponsors
Children's Hospital Los Angeles
Study design
Observational model
CASE_ONLY
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
12 Years to 21 Years
Inclusion criteria
* Functionally single-ventricle Fontan-palliated patients undergoing clinically indicated cardiac catheterization * Anticipated Fontan stent placement for extracardiac Fontan conduit stenosis at the discretion of the primary operator * Weight ≥ 50 kg * 12-21 years of age
Exclusion criteria
* Interrupted IVC * Greater than mild atrioventricular valve regurgitation on most recent echocardiogram or MRI (RF \> 20%). * Greater than mild systemic ventricular dysfunction on most recent echo or MRI (EF \< 40%). * Non-sinus rhythm * Pacemaker * Inability to perform exercise test due to developmental or physical disability
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Determine change from baseline in exercise capacity after Fontan stenting. | 1 year post enrollment | Peak oxygen consumption (VO2) as measured by Vyntus CPX metabolic cart. |
| Determine changes from baseline in liver stiffness as measured by shear wave elastography after Fontan stenting. | 1 year post enrollment | FibroScan will be used to assess liver stiffness before catheterization, at 2-4 weeks following stent placement, and 3-6 months following stent placement. |
Countries
United States
Contacts
Primary ContactMolly Moxness
Outcome results
None listed