Diagnosis and Management of Lymphatic Disorders in Congenital Heart Disease
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CONGENITAL HEART DISEASE (RA KRASUSKI AND G FLEMING, SECTION EDITORS)
Diagnosis and Management of Lymphatic Disorders in Congenital Heart Disease Benjamin Kelly 1,2
&
Sheyanth Mohanakumar 2,3 & Vibeke Elisabeth Hjortdal 2,3
Accepted: 1 September 2020 # The Author(s) 2020
Abstract Purpose of Review Lymphatic disorders have received an increasing amount of attention over the last decade. Sparked primarily by improved imaging modalities and the dawn of lymphatic interventions, understanding, diagnostics, and treatment of lymphatic complications have undergone considerable improvements. Thus, the current review aims to summarize understanding, diagnostics, and treatment of lymphatic complications in individuals with congenital heart disease. Recent Findings The altered hemodynamics of individuals with congenital heart disease has been found to profoundly affect morphology and function of the lymphatic system, rendering this population especially prone to the development of lymphatic complications such as chylous and serous effusions, protein-losing enteropathy and plastic bronchitis. Summary Although improved, a full understanding of the pathophysiology and targeted treatment for lymphatic complications is still wanting. Future research into pharmacological improvement of lymphatic function and continued implementation of lymphatic imaging and interventions may improve knowledge, treatment options, and outcome for affected individuals. Keywords Lymphatic complications . Congenital heart disease . Chylothorax . Prolonged effusion . Protein-losing enteropathy . Plastic bronchitis
Introduction The lymphatic vasculature is widely distributed throughout the entire body. In health, it functions as a unidirectional drainage and transport system originating in the interstitial space and terminating with the return of the lymphatic fluid back This article is part of the Topical Collection on Congenital Heart Disease * Benjamin Kelly [email protected] Sheyanth Mohanakumar [email protected] Vibeke Elisabeth Hjortdal [email protected] 1
Department of Cardiothoracic and Vascular Surgery, Aarhus University Hospital, Aarhus, Denmark
2
Department of Clinical Medicine, Aarhus University, Aarhus, Denmark
3
Department of Cardiothoracic Surgery, Rigshospitalet, Copenhagen, Denmark
into the great veins of the neck or thorax. The initial lymphatic capillaries are composed of a single layer of lymphatic endothelial cells with interstitially anchored filaments preventing collapse. For initial uptake and transport, the fluid is dependent on favorable pressure gradients to reach the precollecting and collecting lymphatic vessels. From here, an increasing concentration of smooth muscle cells intertwiningly weaved around the lymphatic vessel contract in order to propel the fluid forward [1–3]. The initiation of contractions and maintenance of an adequate frequency is complexly regulated. Similar to the heart and intestines, pacemaker cells have been proposed to secure contractions and continuous movement, with ion channe
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