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The lymphatics regulate the interstitial fluid by removing excessive fluid. It represents an extremely important step in the prevention of edema. The Fontan-Kreutzer procedure has revolutionized the treatment of univentricular hearts. However, it is associated with severe complications such as protein-losing enteropathy (PLE) and peripheral edema that may involve the lymphatic circulation.
Our hypothesis is that patients with a univentricular circulation have a reduced functionality of the lymphatic vasculature, which predisposes them to developing complications such as edema and PLE.
The functional state of lymphatics is investigated using near infrared fluorescence imaging, NIRF. The anatomy is described using non-contrast MRI and the capillary filtration rate is measured using plethysmography.
Background:
The lymphatics regulate the interstitial fluid by removing excessive fluid. It represents an extremely important step in the prevention of edema. The Fontan-Kreutzer procedure has revolutionized the treatment of univentricular hearts. However, it is associated with severe complications such as protein-losing enteropathy (PLE) and peripheral edema that may involve the lymphatic circulation.
Hypothesis:
Patients with a univentricular circulation have a reduced functionality of the lymphatic vasculature, which predisposes them to developing complications such as edema and PLE.
Material and Methods:
The functional state of lymphatics is investigated using near infrared fluorescence imaging, NIRF. The anatomy is described using non-contrast MRI and the capillary filtration rate is measured using plethysmography. The study population is patients with Fontan-Kreutzer circulation operated at Aarhus University hospital. Exclusion criteria is BMI>30 and age (years) < 18. The Fontan-Kreutzer group will be compared with an age, gender and weight matched control group of healthy volunteers.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Fontan-Kreutzer operated patients | Patients with a Fontan-Kreutzer circulation. No interventions are done. (The intervention is the operation done 15-20 years ago) |
| |
| Healthy Control subjects | Age, gender and weight matched control subjects |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Near Infrared Fluorescence Imaging | Diagnostic Test | The functional state of lymphatics is investigated using near infrared fluorescence imaging, NIRF. |
|
| Measure | Description | Time Frame |
|---|---|---|
| Pumping pressure | Occlusion of lymphatic flow by inflating a cuff (Hokanson E20 Rapid cuff inflator, Hokanson AG101 air source) to 70mmHg and then reducing the pressure with 5mmHg each 5th minute. The highest pressure under which the fluorescent dye is able to cross the inflatable cuff is labelled Ppump. | 1 hour |
| Contraction Frequency | Contraction frequency in vessels in the main drainage pathway of the legs (ventromedial bundle) as well as vessels draining the area behind the medial malleolus. | 6 min |
| Refill time | The time it takes for a 10 cm long vessel to refill after emptying the vessel with massage | 20 min |
| Velocity | The velocity measured in cm/s for a packet of lymph moving from one region of interest (ROI) to another through atlas a 5 cm straight vessel | 6 min |
| Capillary Filtration Rate | A 5-step 20-min venous congestion protocol will be used to measure capillary filtration. The capillary filtration rate (µl·100 ml-1 ·min-1 ) is measured as the slope of the time-volume change (%) curve at steady state at the end of each pressure phase. | 25 min |
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Inclusion Criteria:
Exclusion Criteria:
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Patients born with an univentricular heart, who surgically is corrected with a Fontan-Kreutzer Circulation
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| Name | Affiliation | Role |
|---|---|---|
| Vibeke E Hjortdal, MD, PhD, Dr.Med | Aarhus University Hospital, Department of Cardiothoracic and Vascular Surgery | Principal Investigator |
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| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 30943769 | Derived | Mohanakumar S, Telinius N, Kelly B, Lauridsen H, Boedtkjer D, Pedersen M, de Leval M, Hjortdal V. Morphology and Function of the Lymphatic Vasculature in Patients With a Fontan Circulation. Circ Cardiovasc Imaging. 2019 Apr;12(4):e008074. doi: 10.1161/CIRCIMAGING.118.008074. |
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| ID | Term |
|---|---|
| D044148 | Lymphatic Abnormalities |
| D008209 | Lymphedema |
| D000080039 | Univentricular Heart |
| 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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|
| Non-contrast MRI | Diagnostic Test | The anatomy is described using non-contrast MRI. |
|
| Strain Gauge Plethysmography | Diagnostic Test | The capillary filtration rate is measured using plethysmography |
|
| D006330 | Heart Defects, Congenital |
| D018376 | Cardiovascular Abnormalities |
| D002318 | Cardiovascular Diseases |
| D006331 | Heart Diseases |