Characterizing the Effect of Dopamine on Markers of Lymph Re-circulation in Fontan-associated Protein-losing Enteropathy

NCT03322345 | Enrolling By Invitation

• 1 other identifier: LYMPH-DOPA-PLE
• Study Type: observational
• Target: 24
• Locations: 1 country
• Sites: 1

Brief Summary

Patients that have undergone a Fontan procedure (surgical correction for single ventricle congenital heart disease) may develop a complication known as protein-losing enteropathy (PLE). Some studies suggest PLE is primarily caused by impaired lymph flow. Use of continuous dopamine infusion can improve PLE. Evidence suggests the effect of dopamine may be through its effect on lymphatic function. This observational study looks at markers of lymph flow and PLE symptoms after treatment using dopamine and other standard therapies during disease exacerbations.

Conditions

Dopamine; Protein-Losing Enteropathies; Lymphatic System; Lymph; Catecholamine; Single-ventricle; Congenital Heart Disease

Keywords

lymph flow; Fontan

Outcome Measures

Primary Outcomes (3)

• Change in CD4 counts

  The change in the number of circulating CD4 lymphocytes will be tracked at multiple time points from baseline to 6 months after start of additional treatment (dopamine and control)

  Baseline to 6 months

• Change in fatty acid profile

  The change in the relative ratios of free fatty acids absorption into the the plasma will be tracked at multiple time points from baseline to 6 months after start of additional treatment (dopamine and control)

  Baseline to 6 months

• Change in vitamin D3

  The change in the levels of vitamin D3 absorbed and circulating in the plasma will be tracked at multiple time points from baseline to 6 months after start of additional treatment (dopamine and control)

  Baseline to 6 months

Secondary Outcomes (4)

• Change in albumin infusion requirements

  Baseline to 6 months

• Change in IVIG infusion requirements

  Baseline to 6 months

• Change in PLE symptoms

  baseline to 6 months

• Change in quality of life

  baseline to 6 months

Study Arms (2)

Dopamine Added

Patients with protein losing enteropathy (PLE) that have an exacerbation such that their treating physicians decide to add continuous dopamine infusion to their current therapies.

No dopamine added (control)

Patients with protein losing enteropathy (PLE) that have an exacerbation such that their treating physicians decide to add new therapies to their current therapies but that do not require the addition of continuous dopamine infusion.

Eligibility Criteria

• Age: 3 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

The study population will be prospective cohort of patients who have undergone Fontan surgery and are currenty followed within the University of Michigan Congenital Heart Center. The target populations are patients with protein losing enteropathy (PLE) that are having increased disease symptoms requiring additional treatment. Those that have PLE refractory to other treatments and require the addition of continuous dopamine infusion to their treatment regimen will be recruited to the dopamine group. Those that have PLE worsening that require the addition of other treatments except the initiation of continuous dopamine infusion will be recruited to the control group.

You may qualify if:

• Males and females with Fontan physiology of any age
• Must have protein losing enteropathy with current worsening who require additional therapies
• Participant consent or parental/guardian consent and participant assent

You may not qualify if:

• Patients with inflammatory bowel disease (i.e Crohn's, ulcerative colitis)
• Patients with systemic autoimmune disease (i.e. Systemic Lupus Erythematous)
• Patients with primary immunodeficiency syndromes
• Patients with nephrotic syndrome
• Patients with anemia
• Patients less than 31 pounds

Sponsors & Collaborators

• University of Michigan: lead

Study Sites (1)

University of Michigan Health System

Ann Arbor, Michigan, 48109, United States

Location

MeSH Terms

Conditions

Protein-Losing Enteropathies; Univentricular Heart; Heart Defects, Congenital

Condition Hierarchy (Ancestors)

Intestinal Diseases; Gastrointestinal Diseases; Digestive System Diseases; Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities

Study Officials

• Kurt Schumacher, MD, MS

  University of Michigan Division of Pediatric Cardiology

  PRINCIPAL INVESTIGATOR

• Joshua Meisner, MD, PhD

  University of Michigan Division of Pediatric Cardiology

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Pediatric Cardiology Fellow

Study Record Dates

• First Submitted: October 23, 2017
• First Posted: October 26, 2017
• Study Start: April 9, 2019
• Primary Completion (Estimated): April 1, 2027
• Study Completion (Estimated): April 1, 2027
• Last Updated: March 13, 2025

Record last verified: 2025-03

Locations

US (1)