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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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Cohort | Single ventricle fontan. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Cardiopulmonary Exercise Testing (CPET) | Diagnostic Test | 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. |
| Measure | Description | Time Frame |
|---|---|---|
| Determine change from baseline in exercise capacity after Fontan stenting. | Peak oxygen consumption (VO2) as measured by Vyntus CPX metabolic cart. | 1 year post enrollment |
| Determine changes from baseline in liver stiffness as measured by shear wave elastography after Fontan stenting. | FibroScan will be used to assess liver stiffness before catheterization, at 2-4 weeks following stent placement, and 3-6 months following stent placement. | 1 year post enrollment |
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Inclusion Criteria:
Exclusion Criteria:
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Patients 12-21 years old with functioning single-ventricle Fontan-palliated who are scheduled to undergo clinically indicated fontan conduit stenting.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Molly Moxness | Contact | 3233619823 | mmoxness@chla.usc.edu |
| Name | Affiliation | Role |
|---|---|---|
| Neil Patel, MD | Children's Hospital Los Angeles | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Children's Hospital Los Angeles | Recruiting | Los Angeles | California | 90027 | United States |
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| ID | Term |
|---|---|
| D000080039 | Univentricular Heart |
| D003251 | Constriction, Pathologic |
| ID | Term |
|---|---|
| D006330 | Heart Defects, Congenital |
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D006331 | Heart Diseases |
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| ID | Term |
|---|---|
| D005080 | Exercise Test |
| ID | Term |
|---|---|
| D006334 | Heart Function Tests |
| D003935 | Diagnostic Techniques, Cardiovascular |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
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|
| Shear Wave Elastography Scan | Diagnostic Test | 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. |
|
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D020763 | Pathological Conditions, Anatomical |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D012129 | Respiratory Function Tests |
| D003948 | Diagnostic Techniques, Respiratory System |
| D016552 | Ergometry |
| D008919 | Investigative Techniques |