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| Name | Class |
|---|---|
| InCor Heart Institute | OTHER |
| Hospital das ClÃnicas de Ribeirão Preto | OTHER |
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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 ClÃnicas, 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.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Fontan Group | Fontan patients operated at the two centres between 1991 and 2014. |
| |
| Healthy Control Group | Age, gender and weight matched healthy controls. |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Non-contrast MRI | Diagnostic Test | Classification and characterization of the lymphatic vasculature using non-contrast MRI. |
|
| 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 |
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Inclusion Criteria:
- Fontan Circulation
Exclusion Criteria:
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Patients with a Fontan circulation.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Department of Cardiothoracic and Vascular Surgery | Aarhus N | 8200 | Denmark |
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| ID | Term |
|---|---|
| D044148 | Lymphatic Abnormalities |
| D011504 | Protein-Losing Enteropathies |
| D000080039 | Univentricular Heart |
| D006330 | Heart Defects, Congenital |
| ID | Term |
|---|---|
| D008206 | Lymphatic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
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| ID | Term |
|---|---|
| D005080 | Exercise Test |
| ID | Term |
|---|---|
| D006334 | Heart Function Tests |
| D003935 | Diagnostic Techniques, Cardiovascular |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
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| Cardiopulmonary exercise test | Diagnostic Test | Cardiopulmonary exercise test, measurement of VO2 and heart rate. |
|
| D007410 | Intestinal Diseases |
| D005767 | Gastrointestinal Diseases |
| D004066 | Digestive System Diseases |
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D006331 | Heart Diseases |
| D012129 | Respiratory Function Tests |
| D003948 | Diagnostic Techniques, Respiratory System |
| D016552 | Ergometry |
| D008919 | Investigative Techniques |