Right Atrial Myxoma Presenting as Budd-Chiari Syndrome

Čujec, Bibiana; Ulmer, Brian; McKaigney, John; Bharadwaj, B

doi:10.1016/s0003-4975(10)62158-3

Cited by 21 publications

(16 citation statements)

References 7 publications

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“…The risk factors for thrombosis and hypercoagulable state are polycythaemia vera, myeloproliferative disorders, paroxysmal nocturnal haemoglobinuria, and condi-tions such as protein C and protein S deficiency or factor V Leiden mutations. Cardiac tumours are rarely associated with the development of BCS [2,3].…”

Section: Discussion and Review Of Literaturementioning

confidence: 99%

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

Sultan

Hussain

Rasheed

2018

Journal of Rare Cardiovascular Diseases

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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. ...

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Section: Discussion and Review Of Literaturementioning

confidence: 99%

“…They are usually located in the left atrium and only 20% of cardiac myxomas originate from the right atrium [9]. There are a few cases of BCS reported in the literature which are associated with right atrial myxoma [2,3].…”

Section: Discussion and Review Of Literaturementioning

confidence: 99%

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

Sultan

Hussain

Rasheed

2018

Journal of Rare Cardiovascular Diseases

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“…PR segment prolongation has been associated with RA myxomas; atrial arrhythmias, especially atrial fibrillation and flutter, and sino-atrial node dysfunction have also been described (6,7). While myxomas usually originate at the fossa ovalis or the base of the intraatrial septum, rare reports of myxoma stalks arising from the junction of the right atrial wall and anteriosuperior aspect of the inferior vena cava (8), the eustachian valve (9), and the suprahepatic inferior vena cava (10) have been described. To date, there is no description in the literature of a myxoma arising from the lateral SVC-RA junction.…”

Section: Discussionmentioning

confidence: 99%

Right atrial myxoma associated with vascular malformation and atypical chest pain

Fox

Bonavita

Sethi

1992

Cathet. Cardiovasc. Diagn.

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“…A myxoma arising from the Eustachian valve, although rare, has been described (3). A solitary cyst attached to the Eustachian valve has also been reported (4).…”

Section: Introductionmentioning

confidence: 99%

Intracardiac Eustachian Valve Cyst in an Adult Detected with Other Cardiac Anomalies: Usefulness of Multidetector CT in Diagnosis

et al. 2012

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We present an unusual case of an intracardiac Eustachian valve cyst observed concurrently with atresia of the coronary sinus ostium, a persistent left superior vena cava (LSVC) and a bicuspid aortic valve. There have been several echocardiographic reports of Eustachian valve cysts; however, there is no report of multidetector computed tomography (MDCT) findings related to a Eustachian valve cyst. Recently, we observed a Eustachian valve cyst diagnosed on MDCT showing a hypodense cyst at the characteristic location of the Eustachian valve (the junction of the right atrium and inferior vena cava). MDCT also demonstrated additional cardiovascular anomalies including atresia of the coronary sinus ostium and a persistent LSVC and bicuspid aortic valve.

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Right Atrial Myxoma Presenting as Budd-Chiari Syndrome

Cited by 21 publications

References 7 publications

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

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

Right atrial myxoma associated with vascular malformation and atypical chest pain

Intracardiac Eustachian Valve Cyst in an Adult Detected with Other Cardiac Anomalies: Usefulness of Multidetector CT in Diagnosis

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