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Morphology and Function of the Lymphatic Vasculature in Patients With a Fontan Circulation
Sheyanth Mohanakumar1,2, Niklas Telinius1,2, Benjamin Kelly1,2
1Department of Cardiothoracic and Vascular Surgery, Aarhus University Hospital, Denmark (S.M., N.T., B.K., V.H.).
Insights
Patients with Fontan circulation have impaired lymphatic pumping and altered thoracic ducts, suggesting lymphatic system challenges contributing to complications. This study assessed lymphatic function in these patients.
Area of Science:
- Cardiovascular Research
- Lymphatic Physiology
- Pediatric Cardiology
Background:
- The Fontan procedure is crucial for treating univentricular hearts but can lead to complications like protein-losing enteropathy and edema.
- These complications may involve the lymphatic circulation, highlighting a need to understand lymphatic function in Fontan patients.
Purpose of the Study:
- To assess lymphatic function and morphology in patients with Fontan circulation.
- To investigate potential links between lymphatic system abnormalities and Fontan-associated complications.
Main Methods:
- Near-infrared fluorescence imaging was used to evaluate lymphatic vessel function in the lower extremities of 10 Fontan patients and 10 healthy controls.
- Lymphatic morphology was assessed using T2-weighted MRI, and microvascular permeability was measured via strain gauge plethysmography.
Main Results:
- Fontan patients exhibited 17% lower lymphatic pumping pressure and 62% higher contraction frequency compared to controls.
- The thoracic duct was found to be elongated and abnormally coursed in Fontan patients.
- No significant differences in lymph movement velocity, refill time, or microvascular permeability were observed between groups.
Conclusions:
- Patients with Fontan circulation demonstrate impaired lymphatic pumping capacity and morphological changes in the thoracic duct.
- These findings suggest a compromised lymphatic vasculature in Fontan circulation, potentially contributing to the pathogenesis of associated complications.
Background:
The Fontan procedure has revolutionized the treatment of univentricular hearts. However, it is associated with severe complications such as protein-losing enteropathy, plastic bronchitis, and peripheral edema that may involve the lymphatic circulation. We aimed to assess lymphatic function and morphology in patients with a univentricular circulation.
Methods:
The functional state of lymphatic vessels in the lower extremities of patients with a Fontan circulation (n=10) was investigated using the novel technique near-infrared fluorescence imaging and compared with an age-, sex-, and weight-matched control group of healthy volunteers (n=10). The lymphatic morphology was described using T2-weighted magnetic resonance imaging, and microvascular permeability was estimated by strain gauge plethysmography.
Results:
The Fontan patients had 17% lower lymphatic pumping pressure (50±3.1 mm Hg) compared with controls (60±2.8 mm Hg; P=0.0341) and a 62% higher contraction frequency (0.8±0.1 min-1) compared with the healthy controls (0.5±0.1 min-1; P=0.0432). Velocity by which the lymph is moved and refill time after manual emptying of the lymphatic vessels showed no differences between the 2 groups. The thoracic duct was elongated 10% ( P=0.0409) and with an abnormal course in the Fontan patients compared with normal. No difference in microvascular permeability was found between the 2 groups.
Conclusions:
Patients with a Fontan circulation have an impaired lymphatic pumping capacity and morphologically changed thoracic duct. Our results indicate a challenged lymphatic vasculature in the Fontan circulation and may play a role in the pathogenesis of the complications that are seen in Fontan patients.
Clinical Trial Registration:
URL: https://www.clinicaltrials.gov . Unique identifier: NCT03379805.
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