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Abernathy syndrome: A rare cause of pulmonary arterial hypertension.

Onkar B Auti, Murugan K, Anil Sapare, Karthik GA, Vimal Raj

Background: Abernathy syndrome, also known as Congenital Extra-hepatic Portosystemic Shunt (CEPS) is an entity where the portal venous system drains directly into the systemic vein, bypassing the liver completely or partially. Patients with this entity usually present with liver parenchymal abnormalities such as nodular liver lesions, hepatoblastoma or hepatocellular carcinoma. This may also cause liver dysfunction, hepatopulmonary syndrome, hepatic encephalopathy or hyperammonemia. Initial presentation with pulmonary arterial hypertension (PAH) related symptoms is relatively rare in Abernathy syndrome.

Case history: Here we present 3 cases whose initial presentation were with PAH and did not have any associated cardiac abnormality on echocardiography. CT pulmonary angiography covering the upper abdomen was done in all cases and found to have Abernathy syndrome. We described features of Abernathy syndrome on CT angiography.

Conclusion: Abernathy syndrome is a rare but treatable cause of PAH and should be considered in all patients being investigated for PAH. Computed Tomography protocols may require to be altered to ensure the correct diagnosis.