Lymphatic Abnormality, Plastic Bronchitis, Protein-Losing Enteropathies, Univentricular Heart, Congenital Heart Disease
Conditions
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.
Interventions
DIAGNOSTIC_TESTNon-contrast MRI
Classification and characterization of the lymphatic vasculature using non-contrast MRI.
DIAGNOSTIC_TESTCardiopulmonary exercise test
Cardiopulmonary exercise test, measurement of VO2 and heart rate.
Sponsors
InCor Heart Institute
Hospital das Clínicas de Ribeirão Preto
University of Aarhus
Study design
Observational model
CASE_CONTROL
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
18 Years to 100 Years
Healthy volunteers
Yes
Inclusion criteria
\- Fontan Circulation
Exclusion criteria
* Age \<18 years * Mental illness * Genetic syndromes
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| 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) |
| Measurement of thoracic duct volume | 1 hour | Volume (mm\^2) |
| Measurement of thoracic duct diameter | 1 hour | Diameter at inlet (mm) |
| 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. |
| CPX heart rate | 1 hour | Maximum heart rate (bpm) |
| CPX Vo2 | 1 hour | VO2 max |
Countries
Denmark
Outcome results
None listed