Abernethy syndrome is a rare congenital portosystemic vascular anomaly in which portal venous blood partially or completely bypasses the liver. Porto-pulmonary hypertension is one of its most severe complications and may determine long-term prognosis. We report a patient with Abernethy syndrome characterized by complete agenesis of the intrahepatic and common portal vein and a large splenorenal portosystemic shunt, complicated by severe porto-pulmonary hypertension. Given the absence of intrahepatic portal venous branches, shunt closure was not feasible; and liver transplantation is not possible as long as severe pulmonary hypertension persists. Serial multimodality imaging demonstrated progressive giant dilatation of the main pulmonary artery, reaching 7 cm in diameter, associated with chronic in situ thrombosis. Despite advancedpulmonary vascular disease, right ventricular systolic function and functional pulmonary capacity remained preserved under targeted pulmonary hypertension therapy, delaying the need for lung and subsequent liver transplantation. This case underscores the importance of individualized, multidisciplinary management in high-risk patients with Abernethy syndrome, an underrecognized condition that may be associated with severe complications.
Ferdinande et al. (Wed,) studied this question.
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