CASE REPORT article
Front. Med.
Sec. Hepatobiliary Diseases
Volume 12 - 2025 | doi: 10.3389/fmed.2025.1604853
Pulmonary Hypertension Secondary to Abernethy Malformation with Left Inferior Vena Cava: A Case Report and Literature Review
Provisionally accepted- Department of Radiology, Qilu Hospital of Shandong University, Jinan, China
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Background: Abernethy malformation is a rare condition in which the portomesenteric blood drains into systemic circulation, bypassing the liver. With advancements in imaging techniques and increased awareness of this malformation, there has been a growing number of reported cases in recent years. Case presentation: We present a case report and literature review in an effort to further the understanding of Abernethy malformation.We report a 21-year-old male presenting with pulmonary hypertension (PH) and right heart enlargement for 7 days. Portal CT angiography (CTA) revealed a vessel communication between the portal vein (PV) and the IVC, located on the left side of the abdominal aorta below the renal vein, and multiple nodular liver lesions (NLL). Finally, Abernethy malformation type II was diagnosed, which is extremely rare due to the absence of polysplenia while co-existing with anomalies of inferior vena cava (AIVC). He was discharged with stable vital signs on symptomatic therapy. Conclusions: Abernethy malformation presents with a range of clinical manifestations.It should be considered in patients with unexplained PH.
Keywords: Abernethy malformation, Congenital extrahepatic portosystemic shunt, pulmonary hypertension, Anomalies of inferior vena cava, Nodular liver lesions
Received: 02 Apr 2025; Accepted: 06 Aug 2025.
Copyright: © 2025 Zhang, Peng, Ren, Li, Zhao, Qi and Zhang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Yang Zhang, Department of Radiology, Qilu Hospital of Shandong University, Jinan, China
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