201. A new variant of Abernethy malformation treated by transhepatic interventional closure: a case report.
- Author
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Sieverding, Ludger, Hofbeck, Michael, Michel, Jörg, Hornung, Andreas, Scheckenbach, Christian, Grözinger, Gerd, Sturm, Ekkehard, Warmann, Steven W., and Hanser, Anja
- Subjects
ATRIAL septal defects ,HUMAN abnormalities ,VENOUS pressure ,PORTAL vein ,PULMONARY veins ,PULMONARY hypertension ,PORTAL vein surgery ,HEPATIC encephalopathy ,BLOOD-vessel abnormalities ,SURGICAL arteriovenous shunts ,DISEASE complications - Abstract
Background: Congenital portosystemic shunts (CPSS) are rare vascular malformations and can be classified into extrahepatic and intrahepatic shunts. Extrahepatic CPSS, also termed Abernethy malformations are associated with severe long-term complications including portopulmonary hypertension, liver atrophy, hyperammoniemia and hepatic encephalopathy. We report a hitherto undescribed variant of Abernethy malformation requiring an innovative approach for interventional treatment.Case Presentation: We describe a 31-year-old patient following surgical repair of atrioventricular septal defect at the age of 6 years. In the long-term follow-up he showed persistent pulmonary hypertension which deteriorated despite dual pulmonary vasodilative treatment. When he developed arterial desaturation and symptomatic hyperammoniemia detailed reassessment revealed as underlying cause a hitherto undescribed variant of Abernethy malformation connecting the portal vein with the right lower pulmonary vein. Following interdisciplinary discussions we opted for an interventional approach. Since the malformation was un-accessible to interventional closure via antegrade venous or retrograde arterial access, a transhepatic percutaneous puncture of the portal vein was performed. Temporary balloon occlusion of the malformation revealed only a slight increase in portal venous pressure. Interventional occlusion of the large vascular connection was achieved via this transhepatic approach by successive implantation of two large vascular occluding devices. The postinterventional course was unremarkable and both ammonia levels and arterial saturation normalized at follow-up of 12 months.Conclusions: Portal vein anomalies should be included in the differential diagnoses of pulmonary hypertension or pulmonary arterio-venous malformations. Based on careful assessment of the anatomy and testing of portal vein hemodynamics interventional therapy of complex Abernethy malformations can be performed successfully in specialized centers. [ABSTRACT FROM AUTHOR]- Published
- 2022
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