title: Thoracic Duct Decompression for Protein-Losing Enteropathy in Failing Fontan Circulation.
abstract: 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.
authors.affiliation: Department of Pediatric Cardiology, Hospital de Santa Marta, Centro Hospitalar Lisboa Central, Nova Medical School, Lisbon, Portugal. Electronic address: marta.antonio@gmail.com.
authors.forename: Marta
authors.initials: M
authors.lastname: António
authors.affiliation: Department of Cardiothoracic Surgery, Hospital de Santa Marta, Centro Hospitalar Lisboa Central, Nova Medical School, Lisbon, Portugal.
authors.forename: Andreia
authors.initials: A
authors.lastname: Gordo
authors.affiliation: Department of Pediatric Cardiology, Hospital de Santa Marta, Centro Hospitalar Lisboa Central, Nova Medical School, Lisbon, Portugal.
authors.forename: Conceição
authors.initials: C
authors.lastname: Pereira
authors.affiliation: Department of Pediatric Cardiology, Hospital de Santa Marta, Centro Hospitalar Lisboa Central, Nova Medical School, Lisbon, Portugal.
authors.forename: Fátima
authors.initials: F
authors.lastname: Pinto
authors.affiliation: Department of Anesthesia, Hospital de Santa Marta, Centro Hospitalar Lisboa Central, Nova Medical School, Lisbon, Portugal.
authors.forename: Isabel
authors.initials: I
authors.lastname: Fragata
authors.affiliation: Department of Cardiothoracic Surgery, Hospital de Santa Marta, Centro Hospitalar Lisboa Central, Nova Medical School, Lisbon, Portugal.
authors.forename: José
authors.initials: J
authors.lastname: Fragata
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D054537:Atrioventricular Block / Q000209:etiology / Q000628:therapy; D016121:Brachiocephalic Veins / Q000000981:diagnostic imaging / Q000601:surgery; D002304:Cardiac Pacing, Artificial; D003131:Combined Modality Therapy; D019299:Decompression, Surgical / Q000379:methods*; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects*; D006325:Heart Atria / Q000000981:diagnostic imaging / Q000601:surgery; D006330:Heart Defects, Congenital / Q000601:surgery; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000601:surgery; D008208:Lymphatic System / Q000503:physiopathology; D008297:Male; D058327:Norwood Procedures; D011504:Protein-Losing Enteropathies / Q000209:etiology / Q000601:surgery*; D011667:Pulmonary Veins / Q000601:surgery; D013897:Thoracic Duct / Q000601:surgery*; D014057:Tomography, X-Ray Computed; D014665:Vasodilator Agents / Q000627:therapeutic use; D014683:Vena Cava, Superior / Q000601:surgery
chemical_list: D000305:Adrenal Cortex Hormones; D014665:Vasodilator Agents