The case report of Abernethy malformation type Ib in an adult patient with pulmonary arterial hypertension

Abstract

This clinical case illustrates the diagnosis of a rare congenital pathology – an adult patient with pulmonary arterial hypertension and Abernethy malformation type Ib.Clinical observation: Patient S., 45 years old, with pulmonary arterial hypertension, was admitted with complaints of shortness of breath during intense physical exertion. Based on the anamnesis morbi, the diagnosis of idiopathic pulmonary arterial hypertension has been verified since 2017. Results of comprehensive examination: functional status (six-minute walk test), NTpro-BNP level, chest x-ray, echocardiography, right heart catheterization were assessed during the hospitalization. According to these there were signs of the high pulmonary arterial hypertension with systolic pressure in the lung arteries 68-70 mm Hg, extension of the trunk, right and left branches of the pulmonary artery. In order to exclude objective evidence of cardiac dysfunction, portal hypertension, an abdominal ultrasound was performed. We didn’t obtaine the information about an increase in central venous pressure, the presence of signs of portal hypertension, such as ascites, increased pressure in the portal system. However, echo signs of congenital pathology were determined - Abernethy malformation type Ib: porto-caval shunt between the superior mesenteric vein and the inferior vena cava. According to multispiral computed tomography of abdominal organs with contrast: the superior mesenteric vein and splenic vein join to form a short extra-hepatic portal vein which drains into a systemic vein (inferior vena cava). Taking into account the results of risk stratification, the patient underwent an escalation of pathogenetic therapy for the treatment of pulmonary arterial hypertension, and dynamic monitoring was recommended.Conclusion: The presented clinical case is unique in terms of diagnosing a rare congenital pathology of the hepatic blood flow – Abernethy malformation type Ib in an adult patient with pulmonary arterial hypertension.