Abstract
An eight-year-old girl was evaluated for unexplained cyanosis. A contrast echo was suggestive of pulmonary arteriovenous fistula. Further evaluation revealed Abernethy malformation (type 2) leading to hepatopulmonary syndrome. Percutaneous device closure of Abernethy malformation was done after anatomical and physiological evaluation of the portal circulation. Prior to closure, it is important to ensure adequate portal radicals into the liver and normal portal pressure after test balloon occlusion. Subcostal echocardiography can diagnose and guide device closure of Abernethy malformation, a treatable cause of hepatopulmonary syndrome.
Keywords:
Pediatric interventions; fistulas/shunts; pulmonary angiography.
Copyright © 2013 Wiley Periodicals, Inc.
Publication types
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Case Reports
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Video-Audio Media
MeSH terms
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Arteriovenous Fistula / diagnosis
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Arteriovenous Fistula / etiology
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Arteriovenous Fistula / therapy
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Child
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Diagnosis, Differential
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Echocardiography, Doppler, Color
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Endovascular Procedures / instrumentation*
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Female
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Hepatorenal Syndrome / diagnosis
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Hepatorenal Syndrome / etiology*
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Hepatorenal Syndrome / physiopathology
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Humans
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Magnetic Resonance Angiography
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Portal Vein / abnormalities*
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Portal Vein / physiopathology
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Prosthesis Design
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Pulmonary Artery / abnormalities
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Pulmonary Veins / abnormalities
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Regional Blood Flow
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Septal Occluder Device*
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Splanchnic Circulation
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Treatment Outcome
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Vascular Malformations / complications
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Vascular Malformations / diagnosis
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Vascular Malformations / physiopathology
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Vascular Malformations / therapy*
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Vena Cava, Inferior / abnormalities*
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Vena Cava, Inferior / physiopathology
Supplementary concepts
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Patent Ductus Venosus
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Pulmonary Arteriovenous Fistulas