Major Pulmonary Arterial Hypertension Secondary to Congenital Portosystemic Intra Hepatic Shunt: A Case Report

Abstract

Congenital portosystemic shunt, also known as Abernethy malformation, is a rare vascular malformation in which the portal blood drains towards the systemic circulation eluding the liver. These shunts arise through disturbances that intervene in the embryonic development of the splanchnic circulation, or when elements that are specific to the fetal circulation (especially those regarding the ductus venous) persist beyond the intrauterine life. We present the case of an 23 men years old, followed for ventricular septal defect (VSD) and pulmonary hypertension (PHA), who was addressed to our department to realize a Computed tomography pulmonary angiography (CTPA) for suspicion an acute pulmonary embolism (APE) due to a high d-dimer level. This examination does not show direct or indirect signs of pulmonary embolism but on the other hand objective a major pulmonary arterial hypertension associated with cardiomegaly secondary to a portosystemic intrahepatic shunt. An abdominal CT scan was requested in order to detail this vascular malformation and to objectify the presence of the portal trunk by the confluence of the spleno-mesaraic trunk and the inferior mesenteric vein with the demonstration of the communication of the right portal branch with inferior vena cava. Angio-CT should be the performed whenever a vascular malformation is suspected in order to establish a correct diagnosis, because portosystemic shunts carry a high risk of severe complications.