Advances in lymphatic imaging and interventions in patients with congenital heart disease

The lymphatic system was discovered in the early days of medicine, however, the ability to image and treat lymphatic abnormalities has only developed over the last 10 to 15 years. In this time, the presence of lymphatic disorders with congenital heart disease has been described and better understood. Advances in imaging modalities have led to novel MRI techniques including dynamic contrast enhanced magnetic resonance lymphangiograms (DCMRL) as well as conventional lymphangiograms to better delineate lymphatic pathology. Using these modalities, it is now possible to diagnose and treat a wide array of lymphatic abnormalities including chylous effusions, chylous ascites, plastic bronchitis, and protein losing enteropathy. Therapies have advanced to involve percutaneous, transabdominal thoracic duct access with treatment of lymphatic problems using liquid embolic material, covered stents, and coils. In cases of thoracic duct occlusion, re-establishing flow is the first line of treatment which can be performed using a novel microsurgical lympho-venous anastomosis technique. In patients with single ventricle physiology, it is essential to image the lymphatics using intra-nodal, intra-hepatic, and intra-mesenteric DCMRL prior to intervening. In these patients, care must be taken to prevent complete occlusion of the thoracic duct to avoid creating another source of lymphatic problems. Current and future lymphatic management involves a combination of catheter-based and surgical interventions as well as the use of novel medical therapies, such as MEK inhibitors, to remodel abnormal lymphatic architecture. Synopsis: This article strives to review the current status of the field of lymphatic imaging and interventions in congenital heart diseases and briefly outlines future directions as the field evolves.

Automated summary

The lymphatic system was discovered early in medicine, but imaging and treatment of lymphatic abnormalities have only advanced in the past 10 to 15 years. Improved imaging techniques have enabled better understanding and diagnosis of lymphatic disorders in patients with congenital heart disease. Current and future management strategies involve a combination of catheter-based and surgical interventions as well as novel medical therapies to remodel abnormal lymphatic architecture.