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.

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