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Background Plastic bronchitis (PB) and protein-losing enteropathy (PLE) are devastating complications after Fontan palliation that lead to uncontrolled loss of protein-rich lymphatic fluid into extra lymphatic compartments. Decompression of the thoracic duct is a new treatment option that effectively restores lymphatic system integrity by redirecting lymphatic flow into the low-pressure levels of the common atrium. Case summary We report a patient with severe failing Fontan circulation where surgical thoracic duct decompression leads to resolution of PLE and PB symptoms but worsening hypoxaemia that could be managed with banding of internal jugular vein. Discussion Thoracic duct decompression in patients with failing Fontan circulation can be a simple and effective treatment for PLE and PB. Hypoxaemia may occur but can be managed with banding of internal jugular vein.

european heart journal. case reports

Thoracic duct decompression and jugular vein banding—an effective treatment option for protein-losing enteropathy and plastic bronchitis in severe failing Fontan circulation: a case report