Lymphatic Morphology of Fontan Patients

Lymphatic Morphology and Classification in Patients With a Fontan Circulation - A Multicenter Study

The Fontan procedure has revolutionized the treatment of patients born with a congenital univentricular heart defect. However, over time, it is associated with severe lymphatic complications such as plastic bronchitis, protein-losing enteropathy (PLE) and peripheral edema.

The hypothesis is that patients with a univentricular circulation have a changed morphology which may be associated with both the degree of lymphatic complications and their physical capacity.

The morphology will be described using T2-weighted non-contrast MRI.

Study Overview

• Status: Completed
• Conditions: Congenital Heart Disease; Univentricular Heart; Protein-Losing Enteropathies; Plastic Bronchitis; Lymphatic Abnormality
• Intervention / Treatment: Diagnostic test: Non-contrast MRI; Diagnostic test: Cardiopulmonary exercise test

Detailed Description

The Fontan procedure has revolutionized the treatment of patients born with a congenital univentricular heart defect. However, over time, it is associated with severe lymphatic complications such as plastic bronchitis, protein-losing enteropathy (PLE) and peripheral edema. It has been proposed that these complications arise due to a elevated central venous pressure of the changed circulation.

The hypothesis of the study is that patients with a univentricular circulation have a changed morphology which may be associated with both the degree of lymphatic complications and their physical capacity.

The morphology will be described using T2-weighted non-contrast MRI. The main lymphatic vessel, the thoracic duct, will be rendered and measured using 3D software, and the lymphatic changes characterized by Dr. Yoav Dori (CHOP, Pennsylvania, USA), using a newly published grading system. The above will be correlated with the amount of complications experienced by the patients, and their physical capacity measured by both IPAQ questionaire and CPX test.

The study population consists of patients with a Fontan circulation operated at InCor Heart Institute, São Paulo, Brasil and Hospital das Clínicas, Ribeirão Preto, Brasil. Exclusion criteria were mental illness, genetic syndromes and age <18 years. The patient group will be compared with age, gender and weight matched healthy controls.

• Study Type: Observational
• Enrollment (Actual): 41

Contacts and Locations

Study Locations

• Denmark
  • Aarhus N, Denmark, 8200
    • Department of Cardiothoracic and Vascular Surgery

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: Yes

• Sampling Method: Probability Sample

Study Population

Patients with a Fontan circulation.

Description

Inclusion Criteria:

- Fontan Circulation

Exclusion Criteria:

• Age <18 years
• Mental illness
• Genetic syndromes

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Fontan Group; Fontan patients operated at the two centres between 1991 and 2014.	Diagnostic test: Non-contrast MRI; Classification and characterization of the lymphatic vasculature using non-contrast MRI.; Diagnostic test: Cardiopulmonary exercise test; Cardiopulmonary exercise test, measurement of VO2 and heart rate.
Healthy Control Group; Age, gender and weight matched healthy controls.	Diagnostic test: Non-contrast MRI; Classification and characterization of the lymphatic vasculature using non-contrast MRI.; Diagnostic test: Cardiopulmonary exercise test; Cardiopulmonary exercise test, measurement of VO2 and heart rate.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Lymphatic classification	Classified by third party, according to following: (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6542623/)	1 hour
Relative measure of tortuosity	Straight length/full length (mm/mm)	1 hour
Measurement of thoracic duct volume	Volume (mm^2)	1 hour
Measurement of thoracic duct diameter	Diameter at inlet (mm)	1 hour
The International Physical Activity Questionnaire (IPAQ-SF)	IPAQ-SF estimation of weekly activity. (Total Physical Activity min/wk: 2 × time spent on vigorous + moderate + walking) Higher value equals higher level of activity.	1 hour
CPX heart rate	Maximum heart rate (bpm)	1 hour
CPX Vo2	VO2 max	1 hour

Collaborators and Investigators

• Sponsor: University of Aarhus
• Collaborators: InCor Heart Institute; Hospital das Clínicas de Ribeirão Preto

Study record dates

Study Major Dates

• Study Start (Actual): January 1, 2018
• Primary Completion (Actual): January 1, 2020
• Study Completion (Actual): January 1, 2022

Study Registration Dates

• First Submitted: November 11, 2019
• First Submitted That Met QC Criteria: November 13, 2019
• First Posted (Actual): November 15, 2019

Study Record Updates

• Last Update Posted (Estimated): June 19, 2023
• Last Update Submitted That Met QC Criteria: June 15, 2023
• Last Verified: April 1, 2021

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Digestive System Diseases; Cardiovascular Diseases; Infections; Respiratory Tract Infections; Respiratory Tract Diseases; Lymphatic Diseases; Lung Diseases; Congenital Abnormalities; Gastrointestinal Diseases; Bronchial Diseases; Intestinal Diseases; Lung Diseases, Obstructive; Cardiovascular Abnormalities; Heart Diseases; Heart Defects, Congenital; Univentricular Heart; Bronchitis; Protein-Losing Enteropathies; Lymphatic Abnormalities
• Other Study ID Numbers: BRALYMF

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No