Ventricular and Pulmonary Vascular Reserve After the Fontan Operation
Trial Snapshot
- Phase
- Not Applicable
- Status
- Completed
- Sponsor
- Mayo Clinic
- Enrollment
- 32
- Locations
- 1
- Primary Endpoint
- Fontan Associated Liver Disease
Study Overview
Brief Summary
Aim #1: Define and determine the prevalence of pulmonary vascular disease and diastolic dysfunction as assessed by the gold standard of invasive hemodynamic cardiopulmonary exercise testing.
Aim #2: Determine the role of rest-exercise echocardiography for the assessment hemodynamics in Fontan physiology.
Aim #3: Evaluate the clinical impact of pulmonary vascular disease and ventricular diastolic dysfunction.
Detailed Description
Define and determine the prevalence of pulmonary vascular disease and diastolic dysfunction as assessed by the gold standard of invasive hemodynamic cardiopulmonary exercise testing. Subjects with Fontan palliation will undergo cardiac catheterization during exercise. The investigators hypothesize that invasive hemodynamic assessment during supine exercise will enable identification of early stage pulmonary vascular disease and diastolic dysfunction that is not apparent from invasive studies performed at rest.
Determine the role of rest-exercise echocardiography for the assessment hemodynamics in Fontan physiology. Transthoracic echocardiography will be performed at rest and during exercise simultaneously with catheterization in the subjects participating in Aim #1 to determine correlations between invasive and noninvasive hemodynamics and identify noninvasive alternatives to assess functional reserve.
Evaluate the clinical impact of pulmonary vascular disease and ventricular diastolic dysfunction. Multi-organ function and patient reported outcomes will be evaluated at baseline, 12 months (optional), and 24 months (optional) in subjects participating in Aims #1 and #2. The investigators hypothesize that subjects with poorer ventricular-vascular reserve will display more clinical and end-organ deterioration in longitudinal follow up.
Study Design
- Study Type
- Observational
- Observational Model
- Cohort
- Time Perspective
- Prospective
Eligibility Criteria
- Ages
- 18 Years to 99 Years (Adult, Older Adult)
- Sex
- All
- Accepts Healthy Volunteers
- No
Inclusion Criteria
- •Fontan Palliation
- •Age ≥18 years
Exclusion Criteria
- •No clinical indication for cardiac catheterization
Outcomes
Primary Outcomes
Fontan Associated Liver Disease
Time Frame: 5 year
Fontan associated liver disease measured via magnetic resonance elastography to find liver stiffness measured in kiloPascals: \<2.5kPa = Normal; 2.5 to 3.0 kPa = Normal or inflammation; 3.0 to 3.5 kPa = Stage 1-2 fibrosis; 3.5 to 4 kPa = Stage 2-3 fibrosis; 4 to 5 kPa = Stage 3-4 fibrosis; \>5 kPa = Stage 4 fibrosis;
Secondary Outcomes
- Mortality(5 years)
Investigators
Alexander C. Egbe
Principal Investigator
Mayo Clinic