Secondary Budd–Chiari syndrome complicating calcified right atrial thrombosis related to ventriculoatrial shunt

Abstract

A 33-year-old female patient underwent diagnostic gynecologic laparoscopy for assessment of a cystic ovarian mass and moderate amounts of ascites. Operative exploration, unexpectedly, uncovered a coarsely multinodular liver surface suspicious of cirrhosis, and surgery was terminated by resection of the left adnexa. Histopathology of the surgical specimen and ascitic fluid analysis yielded no indication of underlying malignancy, and the patient was referred for hepatology work-up. Liver function tests were unremarkable; however, ultrasound revealed an inhomogeneous, nodular liver parenchyma with portal vein dilation and reduced portal venous flow suggestive of portal hypertension. Furthermore, the hepatic veins appeared compressed and duplex sonography failed to trace an unequivocal hepatic venous Doppler flow signal. Upper gastrointestinal endoscopy identified small esophageal varices. Comprehensive serological work-up was negative with respect to viral, metabolic, hereditary, or autoimmune liver disease. Given the insufficient visualization of hepatic veins on ultrasound, the patient underwent magnetic resonance imaging (MRI) indicating inhomogeneous hepatomegaly with regenerative nodules. Furthermore, hepatic vein and, in part, inferior vena cava (IVC) obstruction by a calcified mass extending up towards the right heart was detected. To better characterize the cardiac mass, transthoracic and transesophageal echocardiogram as well as cardiac MRI were performed. Echocardiography depicted a tumor-like right atrial (RA) structure spreading alongside the tricuspid valve (Fig. 1a, Supplemental Videos 1–2). Continuous-wave Doppler across the structure and along the right ventricular inflow tract demonstrated a pressure gradient of 8 mmHg, comparable to a relevant tricuspid stenosis. Likewise, cardiac MRI and CT reinforced the suspicion of a large calcified thrombus arising from the basal RA with appositional growth into the IVC and the hepatic veins (Fig. 1b–d, Supplemental Video 3). No signs of pulmonary embolism were found. Given the clinical diagnosis of subacute Budd–Chiari syndrome (BCS), a comprehensive laboratory assessment for potential prothrombotic conditions including JAK2 V617F testing remained without pathological finding [1]. However, a thorough review of medical history revealed that the Electronic supplementary material The online version of this article (doi:10.1007/s00392-012-0510-9) contains supplementary material, which is available to authorized users.