Right atrial myxoma in a patient with Budd-Chiari syndrome (RCD code: VI-1A.1)

Abstract

CASE REPORT Cardiac tumorsJournal of Rare Cardiovascular Diseases 2018; 3 (6): 218-220 www.jrcd.eu Case presentationA 29-year-old female was referred for cardiac magnetic resonance (CMR) for evaluation of a right atrial mass, found on a computed tomography (CT) scan and an echocardiogram. She presented with right hypochondrial pain and abdominal distension and was diagnosed with Budd-Chiari syndrome (BCS). CT scan showed evidence of thrombosis in the hepatic veins and hepatic and suprahepatic portion of the inferior vena cava (IVC). The CT scan revealed a filling defect in the right atrium and evidence of subsegmental pulmonary embolism. Echocardiogram showed a lesion in the right atrium, with thrombus or tumour being the differential diagnoses. No evidence of haematological malignancy or polycythaemia was found on routine blood tests.CMR with gadolinium enhancement was performed, which showed a large, mobile, irregular shaped, pedunculated mass in the right atrium. It measured 32 mm × 21 mm and was attached to the interatrial septum at the site of the fossa ovalis, obstructing the tricuspid valve. The mass was isointense on T1 weighted images and iso-to hyperintense on T2 weighted images. The mass showed some perfusion on first pass perfusion imaging with gadolinium injection, ruling out the possibility of thrombus. No hyperenhancement was seen in late gadolinium images (Figures 1-3).A diagnosis of right atrial myxoma was made on the basis of the above findings. Patient management and follow-upThe patient underwent surgical removal of the mass and histopathology confirmed it to be a myxoma (Figures 4-5). The patient's clinical condition improved after surgical removal of the mass. Discussion & review of literatureBCS is a rare clinical condition caused by hepatic venous outflow obstruction. The obstruction may be at any level between the small hepatic veins and the IVC junction with the right atrium. It results in centrilobular hepatic congestion with the subsequent development of fibrosis, portal hypertension, and cirrhosis.The BCS is called primary when the obstruction is due to thrombosis or congenital web and is classified as secondary when the obstruction is caused by an extrinsic compression or invasion by an abscess, cyst, or tumour [1][2][3][4]. The risk factors for thrombosis and hypercoagulable state are polycythaemia vera, myeloproliferative disorders, paroxysmal nocturnal haemoglobinuria, and condi- AbstractBudd-Chiari syndrome (BCS) is a rare clinical disorder which is caused by obstruction of the major hepatic veins or suprahepatic portion of the inferior vena cava. Either thrombosis or a mechanical venous obstruction may be present. BCS has been shown to be associated with a number of diseases including polycythaemia vera, myeloproliferative disorders, malignancy or congenital web of the gastro-intestinal tract. Right-sided cardiac tumours, although rarely reported as a cause for BCS, are generally considered as a surgically curable cause for this otherwise potentially lethal clinical condition. ...