Lymphatic Morphology of Fontan Patients

Completed

• Conditions: Lymphatic Abnormality; Protein-Losing Enteropathies; Plastic Bronchitis; Congenital Heart Disease; Univentricular Heart
• Interventions: Diagnostic Test: Non-contrast MRI; Diagnostic Test: Cardiopulmonary exercise test

• Registration Number: NCT04163653
• Lead Sponsor: University of Aarhus

Brief Summary

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.

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.

Basic Information
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Recruitment & Eligibility
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Study & Design
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Trial Locations

Study Timeline

• Study Start: 2018-01-01
• Study First Submitted: 2019-11-11
• Study First Submitted QC: 2019-11-13
• Study First Posted: 2019-11-15
• Primary Completion: 2020-01-01
• Status Verified: 2021-04
• Study Completion: 2022-01-01
• Last Update Submitted: 2023-06-15
• Last Update Posted: 2023-06-18

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 41

Inclusion Criteria

• Fontan Circulation

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Exclusion Criteria

• Age <18 years
• Mental illness
• Genetic syndromes

Read More

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Fontan Group	Cardiopulmonary exercise test	Fontan patients operated at the two centres between 1991 and 2014.
Healthy Control Group	Non-contrast MRI	Age, gender and weight matched healthy controls.
Fontan Group	Non-contrast MRI	Fontan patients operated at the two centres between 1991 and 2014.
Healthy Control Group	Cardiopulmonary exercise test	Age, gender and weight matched healthy controls.

Primary Outcome Measures

Name	Time	Method
Lymphatic classification	1 hour	Classified by third party, according to following: (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6542623/)
Relative measure of tortuosity	1 hour	Straight length/full length (mm/mm)
CPX Vo2	1 hour	VO2 max
Measurement of thoracic duct diameter	1 hour	Diameter at inlet (mm)
CPX heart rate	1 hour	Maximum heart rate (bpm)
Measurement of thoracic duct volume	1 hour	Volume (mm\^2)
The International Physical Activity Questionnaire (IPAQ-SF)	1 hour	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.

Trial Locations

Locations (1)

Department of Cardiothoracic and Vascular Surgery; 🇩🇰 Aarhus N, Denmark