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. 2019 Apr;12(4):e008074.
doi: 10.1161/CIRCIMAGING.118.008074.

Morphology and Function of the Lymphatic Vasculature in Patients With a Fontan Circulation

Sheyanth Mohanakumar  1   2 , Niklas Telinius  1   2 , Benjamin Kelly  1   2 , Henrik Lauridsen  3 , Donna Boedtkjer  2   4 , Michael Pedersen  2   3 , Marc de Leval  5 , Vibeke Hjortdal  1   2
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Morphology and Function of the Lymphatic Vasculature in Patients With a Fontan Circulation

Sheyanth Mohanakumar et al. Circ Cardiovasc Imaging. 2019 Apr.

Abstract

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.

Keywords: Fontan procedure; edema; humans; lymphatic abnormalities; lymphography; magnetic resonance imaging; thoracic duct.

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Comment in

  • Imaging the Lymphatic System in Fontan Patients.
    Udink Ten Cate FEA, Tjwa ETTL. Udink Ten Cate FEA, et al. Circ Cardiovasc Imaging. 2019 Apr;12(4):e008972. doi: 10.1161/CIRCIMAGING.119.008972. Circ Cardiovasc Imaging. 2019. PMID: 30943768 No abstract available.

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