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

Christoph Bauer; Roland Mair; Rudolf Mair; Gerald Tulzer

doi:10.1093/ehjcr/ytaa170

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

Eur Heart J Case Rep. 2020 Aug 23;4(5):1-4. doi: 10.1093/ehjcr/ytaa170. eCollection 2020 Oct.

Authors

Christoph Bauer¹, Roland Mair², Rudolf Mair², Gerald Tulzer¹

Affiliations

¹ Department of Pediatric Cardiology, Johannes Kepler University Linz, Krankenhausstrasse 26-30, 4020 Linz, Austria.
² Department of Pediatric Cardiac Surgery, Johannes Kepler University Linz, Krankenhausstrasse 26-30, 4020 Linz, Austria.

Abstract

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.

Keywords: Case report; Fontan-operation; Hypoxaemia; Internal jugular vein banding; Plastic bronchitis; Protein-losing enteropathy; Thoracic duct decompression.

Publication types

Case Reports