Ventricular and Pulmonary Vascular Reserve After the Fontan Operation

Study Overview
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Brief Summary
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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
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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.

Official Title
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Ventricular and Pulmonary Vascular Reserve After the Fontan Operation

Conditions
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Heart Failure, Congenital Heart Disease

Intervention / Treatment
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* Diagnostic Test: rest-exercise echocardiography


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Fontan Palliation Age ≥18 years Exclusion Criteria: -No clinical indication for cardiac catheterization

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 99 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Prospective Fontan patients<br>Fontan patients to be enrolled and complete study tests/procedures | Diagnostic Test: rest-exercise echocardiography<br>* an echocardiogram performed during rest and exercise<br>|
| Retrospective Fontan patients<br>Fontan patients to be retrospectively reviewed and used as control subjects | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Fontan Associated Liver Disease | 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; | 5 year | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Mortality | Mortality | 5 years |