Thoracic Duct Decompression for Protein-Losing Enteropathy in Failing Fontan Circulation

António, M; Gordo, A; Pereira, C; Pinto, MF; Fragata, I; Fragata, J

http://hdl.handle.net/10400.17/2507

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Abstract(s)

An infrequent but devastating late complication of Fontan circulation is protein-losing enteropathy (PLE), which results from unbalanced lymphatic homeostasis. Surgical decompression of the thoracic duct by redirecting its drainage to the pulmonary venous atrium has been introduced recently as a possible treatment. This report describes a single-institution experience with this innovative procedure in 2 patients with failing Fontan circulation with PLE refractory to optimized medical therapy.

Keywords

HSM CAR PED HSM ANS HSM CCT Adrenal Cortex Hormones/therapeutic use Atrioventricular Block/etiology Atrioventricular Block/therapy Adolescent Brachiocephalic Veins/diagnostic imaging Brachiocephalic Veins/surgery Cardiac Pacing, Artificial Combined Modality Therapy Decompression, Surgical/methods Fontan Procedure/adverse effects Heart Atria/diagnostic imaging Heart Atria/surgery Heart Defects, Congenital/surgery Hypoplastic Left Heart Syndrome/surgery Lymphatic System/physiopathology Norwood Procedures Protein-Losing Enteropathies/etiology Protein-Losing Enteropathies/surgery Pulmonary Veins/surgery Thoracic Duct/surgery Tomography, X-Ray Computed Vasodilator Agents/therapeutic use Vena Cava, Superior/surgery