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Review
. 2023 Sep 27;3(1):101174.
doi: 10.1016/j.jscai.2023.101174. eCollection 2024 Jan.

Lymphatic Imaging and Intervention in Congenital Heart Disease

Christoph Bauer  1   2 , Mario Scala  2   3 , Jonathan J Rome  4 , Gerald Tulzer  1   2 , Yoav Dori  4
Affiliations
Review

Lymphatic Imaging and Intervention in Congenital Heart Disease

Christoph Bauer et al. J Soc Cardiovasc Angiogr Interv. .

Abstract

The lymphatic system plays a central role in some of the most devastating complications associated with congenital heart defects. Diseases like protein-losing enteropathy, plastic bronchitis, postoperative chylothorax, and chylous ascites are now proven to be lymphatic in origin. Novel imaging modalities, most notably, noncontrast magnetic resonance lymphangiography and dynamic contrast-enhanced magnetic resonance lymphangiography, can now depict lymphatic anatomy and function in all major lymphatic compartments and are essential for modern therapy planning. Based on the new pathophysiologic understanding of lymphatic flow disorders, innovative minimally invasive procedures have been invented during the last few years with promising results. Abnormal lymphatic flow can now be redirected with catheter-based interventions like thoracic duct embolization, selective lymphatic duct embolization, and liver lymphatic embolization. Lymphatic drainage can be improved through surgical or interventional techniques such as thoracic duct decompression or lympho-venous anastomosis.

Keywords: congenital heart disease; dynamic contrast magnetic resonance lymphangiography; lymphatic insufficiency; lymphatic interventional techniques; plastic bronchitis; protein-losing enteropathy.

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Figures

None
Graphical abstract
Figure 1
Figure 1
(A) Maximal intensity projection coronal projection of intranodal dynamic contrast magnetic resonance lymphangiography in a patient with plastic bronchitis showing bilateral pulmonary lymphatic perfusion syndrome (arrow). (B) Maximal intensity projection coronal projection of IH-DCMRL in a patient with protein-losing enteropathy demonstrating leak into the duodenum (arrow). (C) IM-DCMRL in a patient with retrograde mesenteric flow (arrow).
Figure 2
Figure 2
Fluoroscopy images in theanterior-posteriorprojection of IH lymphangiography. (A) Liver lymphatic channels (arrow), (B) biliary tree (arrow), (C) portal veins (arrow), and hepatic veins (arrowhead).
Central Illustration
Central Illustration
Novel lymphatic imaging techniques (ie, dynamic contrast magnetic resonance lymphangiography) and minimally invasive procedures (thoracic duct embolization) used for successful treatment of lymphatic complications (plastic bronchitis and chylothorax) in a patient with a congenital heart defect. (A) Maximal intensity projection coronal projection of intranodal-DCMRL showing bilateral pulmonary lymphatic perfusion syndrome (arrow). (B) Fluoroscopic lymphangiography in the anterior-posterior projection shows a thoracic duct (arrow) and bilateral pulmonary lymphatic perfusion syndrome. (C) Coil (arrow) and glue (arrowhead) seen in the thoracic duct after thoracic duct embolization.
Figure 3
Figure 3
(A) Fluoroscopic lymphangiography in the anterior-posterior projection shows the dilated thoracic duct (arrow) and mediastinal as well as bilateral pulmonary lymphatic perfusion syndrome (arrowhead). (B) The thoracic duct (arrow) is seen after selective lymphatic duct embolization of the abnormal channels (hashed arrow) showing no residual perfusion of the mediastinum or lungs with the duct draining into the vein (arrowhead).
Figure 4
Figure 4
(A) Blue dye injected into the liver lymphatic channels is seen via endoscopy leaking into the duodenal lumen (arrow). (B) Fluoroscopy in the anterior-posterior projection after glue embolization of periduodenal lymphatic networks. (C) Glue is seen inside the duodenal lumen after embolization (arrow).
Figure 5
Figure 5
(A) Contrast injection in the left internal jugular vein in the anterior-posterior projection after surgical thoracic duct decompression showing a patent connection of the innominate vein to the atrium (arrow), (B) contrast injection in the left internal jugular vein after percutaneous TD decompression showing a patent connection of the stent to the atrium (arrow).
See this image and copyright information in PMC

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