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