Lymphatic Function in Patients With a Fontan Circulation From Brazil

Study Overview
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Brief Summary
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It is suggested that the elevated central venous pressure in patients with a Fontan circulation can provoke manifestation of lymphatic dysfunction, met as plastic bronchitis, peripheral oedema and most feared protein-losing enteropathy (PLE). An explorative study from our department at Aarhus University Hospital revealed that the function of the lymphatic vasculature in 10 young Fontan patients with no complications was abnormal compared to healthy controls. However, to further describe and confirm these findings we had to investigate the lymphatic circulation in a larger, older and more complicated group of Fontan patients. The hypothesis is that, patients with a univentricular circulation have a reduced functionality of the lymphatic vasculature and which predisposes them to developing complications such as edema and PLE.

Detailed Description
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The study population is patients with a Fontan circulation followed at InCor Heart Institute in Sao Paolo and Ribeirão Preto, Brazil. These were matched with and group of healthy controls. The functional state of superficial lymphatics in the lower leg will be investigated, using near infrared fluorescence (NIRF) imaging. The capillary filtration of fluid and leakiness of the capillaries will be measured using strain gauge plethysmography and the peripheral blood flow will be measured using venous occlusion plethysmography. Furthermore, plasma concentration of noradrenaline will be estimated.

Official Title
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Lymphatic Function and Interstitial Fluid Filtration in Patients With a Fontan Circulation From Brazil

Conditions
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Univentricular Heart, Lymphatic Abnormalities, Lymphatic Edema

Intervention / Treatment
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* Diagnostic Test: Near-Infrared Fluorescence imaging
* Diagnostic Test: Strain Gauge Plethysmography


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Fontan circulation Age above 16. Exclusion Criteria: Mental illness Syndromes.

Ages Eligible for Study
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Minimum Age: 16 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Fontan Patients<br>Fontan patients operated at the two centres between 1991 and 2014. | Diagnostic Test: Near-Infrared Fluorescence imaging<br>* .Near-infrared imaging was used to evaluate the function of the superficial lymphatic vessels in the right lower leg and foot as previously described.doi: 10.1161/CIRCIMAGING.118.008074.<br>Diagnostic Test: Strain Gauge Plethysmography<br>* Strain Gauge Plethysmography to asses capillary filtration rate og the lower limb<br>|
| Control Group<br>Age, gender and weight matched healthy controls. | Diagnostic Test: Near-Infrared Fluorescence imaging<br>* .Near-infrared imaging was used to evaluate the function of the superficial lymphatic vessels in the right lower leg and foot as previously described.doi: 10.1161/CIRCIMAGING.118.008074.<br>Diagnostic Test: Strain Gauge Plethysmography<br>* Strain Gauge Plethysmography to asses capillary filtration rate og the lower limb<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Lymphatic function assessed by Near-Infrared fluorescence Imaging 1 | Contraction frequency (contraction/minut) | 1 hour | 
| Lymphatic function assessed by Near-Infrared fluorescence Imaging 2 | Pumping Pressure (mmHg) | 1 hour | 
| Lymphatic function assessed by Near-Infrared fluorescence Imaging 3 | Packet Velocity | 1 hour | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Capillary Filtration Rate measured by Strain Gauge Plethysmography | Capillary Filtration Rate | 1 hour | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Lymphatic dysfunction, Near-Infrared Fluorescence Imaging, Fontan Circulation, Plethysmography