|Number 15-13: Budd-Chiari: An Unexpected Imaging Journey That Ends With Congenital CMR.|
Number 15-13: Budd-Chiari: an unexpected imaging journey that ends with congenital CMR.
Case from: Daniel Devos1, Peter Smeets1, Alex Dik1, Xavier Verhelst2, Daniel De Wolf3, Hans Van Vlierberghe2
Institutes: 1Department of Radiology, 2Department of Gastroenterology, 3Department of Paediatric Cardiology, Ghent University Hospital, Ghent, Belgium.
Clinical history: A 46 year old woman was referred to our hospital for investigation of vaginal blood loss. Relevant medical history was Behçet’s syndrome, chronic venous insufficiency and deep venous thrombosis in lower extremities, pulmonary embolism, and recent symptomatic iron-deficiency anaemia needing blood transfusion. The patient was not using any drugs, alcohol or medication.
To further investigate liver vein pressure, a venous catheterisation was performed. Approaches through the left and right femoral vein (Figure 2) and right jugular vein were unsuccessful for contrast filling of the inferior caval vein. It was cautiously decided not to venture into an invisible IVC. Collateral veins were shown. Liver vein pressure could not be measured.
An MR study of the liver showed a Budd-Chiari liver with patent but morphologically abnormal veins (Figure 3).
A multiphase contrast enhanced CT study was performed to investigate IVC and liver veins (Figure 4). A homogenous density was found in the IVC of normal size, but passage of contrast appeared delayed. A sharp delineation between IVC blood and intra-atrial blood was present in all phases. Suspicion was raised of an inflow obstruction to the right atrium, and CMR was performed.
4a. Arterial phase (25s) 4b. Portovenous phase (50s)
4c. Venous phase (70s) 4d. Equilibrium phase (2min)
CMR Findings: A membranous obstruction was found to obstruct inflow of blood from IVC towards right atrium. Cine imaging showed a turbulent jet indicating a small aperture in the membrane, as well as very slow retrograde flow in the IVC, explaining the unsuccessful invasive cavography earlier (Movie 1). This aberrant flow was confirmed by phase contrast images (not shown) and dynamic MR angiography (Figure 5).
Movie 1. Long axis cine of the right heart, showing (the right ventricle, right atrium and) both superior and inferior caval venous inflow into the right atrium. The membrane covering the inferior caval venous entry is clearly depicted, with a turbulent jet from blood flow through a small orifice pointing upwards. In the IVC there is slow retrograde flow. There is no evidence for mass or thrombus.
This obstruction was relieved by balloon plasty. Gynecologic surgery was safely performed within two weeks, with normal liver function.
The Eustachian valve is a structure at the junction of IVC and right atrium that during fetal life directs blood toward the foramen ovale. Normally only a rim at the anterolateral aspect of caval vein entry into the atrium remains. MR images of a membrane virtually spanning the complete entrance of the right atrium from the IVC have not yet been published in an adult patient.
It is remarkable that this patient has suffered from pulmonary embolism; with the flow reversal in the IVC, thrombus from deep venous thrombosis cannot have reached the heart. Presumably, the patient’s Behçet’s disease combined with slow flow in the IVC or in collateral veins caused the emboli. The patient’s chronic venous deficiency in the lower extremities will have been largely due to this flow obstruction.
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