Abstract
Congenital portosystemic shunts are rare congenital vascular malformations characterized by a partial or complete portal blood diversion into the systemic circulation. Congenital extrahepatic portosystemic shunts, known as Abernethy malformations, pose a diagnostic challenge due to their low incidence and clinical presentations.
 A 15-year-old male with a history of chronic epigastric pain and nausea, high arterial blood pressure, recurrent nose bleeds, chest pain, dizziness, dyspnea, low exercise tolerance, hematochezia, and itching was diagnosed with Abernethy malformation type Ib. Imaging studies revealed a dilated portal vein conduit flowing into the inferior vena cava, bypassing the porta hepatis. Multiple liver nodules, heart chamber dilatation, myocardial hypertrophy, and pulmonary hypertension were also discovered. Following multidisciplinary panel meetings, liver transplantation was advised due to the severity of the patients symptoms and shunt anatomy.
 Furthermore, diagnostic algorithms and other treatment options are discussed.
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