Ventricular and Pulmonary Vascular Reserve After the Fontan Operation

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.

Study Overview

• Status: Completed
• Conditions: Heart Failure; Congenital Heart Disease
• Intervention / Treatment: Diagnostic test: rest-exercise echocardiography

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 Type: Observational
• Enrollment (Actual): 32

Contacts and Locations

Study Locations

• United States
  • Minnesota
    • Rochester, Minnesota, United States, 55905
      • Mayo Clinic

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 99 years (ADULT, OLDER_ADULT)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Fontan palliation

Description

Inclusion Criteria:

• Fontan Palliation
• Age ≥18 years

Exclusion Criteria:

-No clinical indication for cardiac catheterization

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Prospective Fontan patients; Fontan patients to be enrolled and complete study tests/procedures	Diagnostic test: rest-exercise echocardiography; an echocardiogram performed during rest and exercise
Retrospective Fontan patients; Fontan patients to be retrospectively reviewed and used as control subjects

What is the study measuring?

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

Collaborators and Investigators

• Sponsor: Mayo Clinic
• Collaborators: National Heart, Lung, and Blood Institute (NHLBI)
• Investigators: Principal Investigator: Alexander Egbe, MD, Mayo Clinic

Publications and helpful links

Helpful Links

• Mayo Clinic Clinical Trials

Study record dates

Study Major Dates

• Study Start (ACTUAL): August 30, 2018
• Primary Completion (ACTUAL): January 4, 2022
• Study Completion (ACTUAL): July 21, 2022

Study Registration Dates

• First Submitted: August 9, 2018
• First Submitted That Met QC Criteria: February 22, 2019
• First Posted (ACTUAL): February 26, 2019

Study Record Updates

• Last Update Posted (ACTUAL): October 6, 2022
• Last Update Submitted That Met QC Criteria: October 4, 2022
• Last Verified: October 1, 2022

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Cardiovascular Diseases; Congenital Abnormalities; Cardiovascular Abnormalities; Heart Diseases; Heart Defects, Congenital
• Other Study ID Numbers: 17-010090; 1K23HL141448-01 (NIH)

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No