An observational study in Lymphatic Abnormality, Plastic Bronchitis and Protein-Losing Enteropathies, sponsored by University of Aarhus. Completed at 1 site in Denmark. Open to participants aged 18 Years to 100 Years, including healthy volunteers. Per ClinicalTrials.gov, last updated 2023-06-18.
Sponsored by University of Aarhus · Observational
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.
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.
42 studies on the registry are indexed under Lymphatic Abnormalities; 18 are open to participants now.
This study's enrollment of 41 is below the median of 56 across 13 observational studies indexed under Lymphatic Abnormalities.
Browse Lymphatic Abnormalities studies →University of Aarhus is the lead sponsor of 1,299 studies on the registry; 189 are open to participants now.
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Patients with a Fontan circulation.
Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.
- Fontan Circulation
Exclusion Criteria:
Fontan patients operated at the two centres between 1991 and 2014.
Diagnostic Test: Non-contrast MRI · Diagnostic Test: Cardiopulmonary exercise test
Age, gender and weight matched healthy controls.
Diagnostic Test: Non-contrast MRI · Diagnostic Test: Cardiopulmonary exercise test
Classification and characterization of the lymphatic vasculature using non-contrast MRI.
Cardiopulmonary exercise test, measurement of VO2 and heart rate.
Lymphatic classification
Classified by third party, according to following: (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6542623/)
Time frame: 1 hour
Relative measure of tortuosity
Straight length/full length (mm/mm)
Time frame: 1 hour
Measurement of thoracic duct volume
Volume (mm\^2)
Time frame: 1 hour
Measurement of thoracic duct diameter
Diameter at inlet (mm)
Time frame: 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.
Time frame: 1 hour
CPX heart rate
Maximum heart rate (bpm)
Time frame: 1 hour
CPX Vo2
VO2 max
Time frame: 1 hour
Ran from Jan 2018 to primary completion in Jan 2020, 24 months.
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This study is completed, as verified in Apr 2021. You cannot join it, but the record below documents what was studied.
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