Patients with hyplastic left heart syndrome:

5 patients aged between 3 months and 15 years with hypoplastic left heart syndrome and chylothorax, in 3 patients also with protein-losing enteropathy. DCMRL demonstrated patency of the thoracic duct in all patients. In 3 patients, DCMRL showed that the contrasted lymph from the lower half of the body flows retrogradely into the thickened duodenal wall and from there passes into the intestinal lumen (protein-losing enteropathy). Since patency of the thoracic duct could be demonstrated, the cause of the chylothoraces and protein-losing enteropathy must be assumed to be the increased central venous pressure in Failing Fontan, and not an anomaly or postoperative occlusion of the lymphatics. Heart transplantation was therefore performed in 3 of the patients.This is planned for 2 of the patients who are currently undergoing conservative therapy.

5-year-old patient with Pulmonary Lymphatic Perfusion Syndrome in Failing Fontan:

DCMRL showed a discontinuity of the thoracic duct. There was a clear retrograde flow of lymph from the mediastinum into the lungs (Pulmonary Lymphatic Perfusion Syndrome PLPS) and spillover of the contrasted lymph into the pleural cavity. The patient died before planned surgical treatment could be performed.

Patient with Uhl's disease and chylothorax:

2-month-old female patient with postoperative development of a chylothorax. In the DCMRL evidence of varicose dilated mediastinal lymphatic vessels. The drainage into the thoracic duct is preserved. Decision on drug therapy for cardiac insufficiency was made, under which a regression of the chylothoraces could be achieved.

Patient with chyluria: 

An 18-year-old patient presented after large operation in the retroperitoneal space and subsequent development of high-grade chyluria. It was demonstrated that there was a subtotal occlusion of the retroperitoneal lymphatics at the site of the previous surgery. The thoracic duct was accessed via several fine collaterals, and the thoracic duct was freely patent. The subtotal occlusion of the lymphatic vessels proximal to the thoracic duct resulted in a inversion of lymph flow, causing retrograde flow into the left kidney and subsequent excretion of lymph via urine.  Selective lipiodol injection was chosen as the treatment, successfully stopping the retrograde lymph flow into the kidney and facilitating the formation of new collaterals draining into the thoracic duct. The chyluria ceased.

Patient with transcutaneous chyle leakage:

12-year-old female patient with transcutaneous chyle leakage. Intra-abdominal numerous dilated lymphatic vessels, thoracic duct freely patency. Focal sclerotherapy of the dilated lymphatic vessels was successfully performed.

Patient with pericardial effusion:

16-year-old female patient with a large chylous pericardial effusion. The DCMRL revealed multiple dilated lymphatic vessels, mainly located mediastinally and pericardially, which led to an overflow of the chyle into the pericardium. The cause was a generalized lymphatic anomaly (GLA). As a result, therapy with the mTOR inhibitor Sirolimus was started, under which the pericardial effusion regressed.