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Abernethy malformation: Our experience from a tertiary cardiac care center and review of literature


1 Department of Pediatric Cardiology and Congenital Heart Disease, Fortis Escorts Heart Institute, New Delhi, India
2 Department of Radiodiagnosis, Fortis Escorts Heart Institute, New Delhi, India

Correspondence Address:
Dr. Sushil Azad
Department of Pediatric Cardiology and Congenital Heart Disease, Fortis Escorts Heart Institute, Okhla, New Delhi - 110 025
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_185_18

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Year : 2019  |  Volume : 12  |  Issue : 3  |  Page : 240-247

 

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Abernethy malformation, also called as congenital extrahepatic portosystemic venous shunt, is a rare anomaly involving the portal venous system. Although rare, it is increasingly being reported and is important to diagnose given the adverse clinical consequences in untreated patients. It has myriad of clinical presentations, from being completely asymptomatic to causing hepatic carcinoma, hepatic encephalopathy, severe pulmonary hypertension, and diffuse pulmonary arteriovenous malformation. We describe our experience with five cases in a tertiary pediatric cardiac care center with Abernethy malformation, with review of literature and also discuss possible therapeutic implications.






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1 Department of Pediatric Cardiology and Congenital Heart Disease, Fortis Escorts Heart Institute, New Delhi, India
2 Department of Radiodiagnosis, Fortis Escorts Heart Institute, New Delhi, India

Correspondence Address:
Dr. Sushil Azad
Department of Pediatric Cardiology and Congenital Heart Disease, Fortis Escorts Heart Institute, Okhla, New Delhi - 110 025
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_185_18

Rights and Permissions

Abernethy malformation, also called as congenital extrahepatic portosystemic venous shunt, is a rare anomaly involving the portal venous system. Although rare, it is increasingly being reported and is important to diagnose given the adverse clinical consequences in untreated patients. It has myriad of clinical presentations, from being completely asymptomatic to causing hepatic carcinoma, hepatic encephalopathy, severe pulmonary hypertension, and diffuse pulmonary arteriovenous malformation. We describe our experience with five cases in a tertiary pediatric cardiac care center with Abernethy malformation, with review of literature and also discuss possible therapeutic implications.






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