Ventricular and Pulmonary Vascular Reserve After the Fontan Operation

Completed

• Conditions: Heart Failure; Congenital Heart Disease
• Interventions: Diagnostic Test: rest-exercise echocardiography

• Registration Number: NCT03853837
• Lead Sponsor: Mayo Clinic

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 Timeline

• Study First Submitted: 2018-08-09
• Study Start: 2018-08-30
• Study First Submitted QC: 2019-02-22
• Study First Posted: 2019-02-26
• Primary Completion: 2022-01-04
• Study Completion: 2022-07-21
• Status Verified: 2022-10
• Last Update Submitted: 2022-10-04
• Last Update Posted: 2022-10-06

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 32

Inclusion Criteria

• Fontan Palliation
• Age ≥18 years

Exclusion Criteria

-No clinical indication for cardiac catheterization

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Prospective Fontan patients	rest-exercise echocardiography	Fontan patients to be enrolled and complete study tests/procedures

Primary Outcome Measures

Name	Time	Method
Fontan Associated Liver Disease	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 Outcome Measures

Name	Time	Method
Mortality	5 years	Mortality

Trial Locations

Locations (1)

Mayo Clinic; 🇺🇸 Rochester, Minnesota, United States