Lymphatic Morphology of Fontan Patients

Congenital Heart Disease Clinical Trial

Official title:

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

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT04163653
• Other study ID #: BRALYMF
• Status: Completed
• Start date: January 1, 2018
• Est. completion date: January 1, 2022

Study information

• Verified date: April 2021
• Source: University of Aarhus
• Contact: n/a
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 41
• Est. completion date: January 1, 2022
• Est. primary completion date: January 1, 2020
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: All
• Age group: 18 Years to 100 Years

Eligibility

Inclusion Criteria:

• Fontan Circulation

Exclusion Criteria:

• Age <18 years

• Mental illness

• Genetic syndromes

Related Conditions & MeSH terms

• Bronchitis
• Congenital Heart Disease
• Heart Defects, Congenital
• Heart Diseases
• Lymphatic Abnormalities
• Lymphatic Abnormality
• Plastic Bronchitis
• Protein-Losing Enteropathies
• Univentricular Heart

Intervention

Diagnostic Test:
• Non-contrast MRI
Classification and characterization of the lymphatic vasculature using non-contrast MRI.
• Cardiopulmonary exercise test
Cardiopulmonary exercise test, measurement of VO2 and heart rate.

Locations

Country	Name	City	State
Denmark	Department of Cardiothoracic and Vascular Surgery	Aarhus N

Sponsors (3)

Lead Sponsor	Collaborator
University of Aarhus	Hospital das Clínicas de Ribeirão Preto, InCor Heart Institute

Country where clinical trial is conducted

Denmark, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Lymphatic classification	Classified by third party, according to following: (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6542623/)	1 hour
Primary	Relative measure of tortuosity	Straight length/full length (mm/mm)	1 hour
Primary	Measurement of thoracic duct volume	Volume (mm^2)	1 hour
Primary	Measurement of thoracic duct diameter	Diameter at inlet (mm)	1 hour
Primary	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
Primary	CPX heart rate	Maximum heart rate (bpm)	1 hour
Primary	CPX Vo2	VO2 max	1 hour