Abstract
A child with heterotaxia, azygous continuation of an interrupted inferior vena cava, single ventricle, and pulmonary atresia underwent the Kawashima modification of the Fontan procedure, which excluded hepatic venous return from the pulmonary blood flow. After the operation, the patient had development of pulmonary arteriovenous malformations, increasing cyanosis, and ventricular dysfunction. He underwent orthotopic heart transplantation at 7 years of age with prompt resolution of the pulmonary arteriovenous malformations.
MeSH terms
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Arteriovenous Malformations / etiology*
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Arteriovenous Malformations / therapy
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Arteriovenous Shunt, Surgical / adverse effects*
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Azygos Vein / abnormalities
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Azygos Vein / surgery
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Child
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Cyanosis / etiology
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Fontan Procedure / adverse effects
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Fontan Procedure / methods
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Heart Defects, Congenital / pathology
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Heart Defects, Congenital / surgery
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Heart Transplantation*
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Hepatic Veins / surgery
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Humans
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Lung / blood supply*
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Male
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Pulmonary Atresia / pathology
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Pulmonary Atresia / surgery
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Pulmonary Circulation
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Transposition of Great Vessels / pathology
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Transposition of Great Vessels / surgery
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Vena Cava, Inferior / abnormalities
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Vena Cava, Inferior / surgery
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Ventricular Dysfunction / etiology