A Right Atrial Hemangioma Mimicking Thrombus In A Patient With Atrial Arrhythmias


Cardiac hemangiomas are rare tumors, accounting for only 2.8% of all benign primary cardiac tumors and occur at any age. Clinical presentations vary depending on the tumor location (myocardial, endocardial or pericardial). In many cases, this may be an incidental finding. We report the case of a patient with paroxysmal atrial fibrillation who had a right atrial hemangioma detected with transesophageal echocardiography prior to having percutaneous pulmonary vein isolation performed


A 50-year-old male with history of paroxysmal atrial fibrillation for one year presented for evaluation for pulmonary vein isolation. He had a history of palpitations, but denied syncope, chest pain, or dyspnea. Physical examination was unremarkable except for an irregular heart rate (approximately 90 beats per minute). Computed Tomographic Angiography (CTA) of the pulmonary veins and left atrium was normal. However, a large mass was noted in the right atrium. A presumptive diagnosis of tumor or a large thrombus was made. Transesophageal Echocardiography (TEE) revealed normal cardiac chamber sizes and normal left and right ventricular systolic function. A mobile mass measuring approximately 2.5 x 3.5 cm was identified (Fig. 1). The mass was cystic with septations and was attached to the interatrial septum near the inferior vena cava. Cardiac angiography revealed normal coronaries. The patient underwent open heart surgery to resect the mass and had intra-operative pulmonary vein isolation performed. The mass on gross examination was pedunculated and polypoid (Fig. 2). On sectioning, the cut surface was hemorrhagic, friable and partially cystic. This led to the preliminary diagnosis of an atrial thrombus. Multiple microscopic sections reveal a fibrotic hemorrhagic stroma with dilated cavernous vessels and focal small capillary channels (Fig. 3). The mass was covered with endocardium. The final diagnosis was an intracavitary cardiac hemangioma (cavernous and capillary type) of the right atrium. The patient did well and was discharged home in sinus rhythm.

Fig. (1).

(TEE) Cystic and pedunculated mobile mass with septations measuring approximately 2.5 x 3.5 cm in the right atrium.

Fig. (2).

Pedunculated and polypoid gross specimen.

Fig. (3).

Microsopic section of the specimen depicting focal small capillary channels (A) and dilated cavernous vessels (B).


Primary cardiac tumors are far less common than metastatic tumors, with an incidence reported to be approximately 0.02% in a large autopsy series [1]. Most cardiac tumors are benign; however, when found in the right side of the heart, they are more likely to be malignant (75%) than benign (25%) [2]. The most common form of benign cardiac tumor is the myxoma, accounting for 50% of neoplasms, while other forms including rhabdomyomas, hemangiomas,

lipoma, and fibromas account for the rest. Hemangiomas are very rare, accounting for 2.8% of all benign primary cardiac tumors and usually occur in the left side of the heart making this an unusual case and interesting cause of atrial arrhythmia. They occur in the right side of the heart only in 1 out of 5000 cases. These vascular tumors are made up of a proliferation of endothelial cells, and histologically are similar to hemangiomas in other parts of the body [3]. They are classified histologically into 3 types:

Cavernous: Composed of multiple dilated thin walled vessels.

Capillary: Composed of small capillary like vessels.

Arterio-venous: Composed of dysplastic malformed arteries and veins.

The more commonly encountered forms are the cavernous and capillary types; however, the tumor may have features of all three types [4,5]. The tumor may be located in the myocardium, endocardium or the epicardium. The clinical presentation varies depending on the location and includes arrhythmias, symptoms of heart failure, right ventricular outflow obstruction and cardiac tamponade [5,6,7]. In the absence of right atrial wall invasion, pericardial effusion or tumor originating from the inferior vena cava, a right atrial tumor can be presumed to be benign [8]. However, since statistically, the majority of right sided tumors are malignant, a biopsy and tissue histology are required for a definitive diagnosis. Establishing a diagnosis of cardiac hemangioma can be difficult intraoperatively and indeed initial gross examination of this specimen led to a diagnosis of organizing thrombus given the patient’s history of atrial arrhythmias.

While echocardiography has been used to establish a diagnosis of cardiac hemangioma, there are no standardized criteria [6]. While the cardiac hemangioma is commonly described as a large multiloculated mass with multiple trabeculae and numerous echo free spaces [9], other descriptions can be found in the literature [7]. The most helpful echocardiographic features noted in our case were the recognition of the cystic and septated nature of the mass. Although it was not used in our case, further characterization of the tumor can be made using contrast enhanced echocardiography which can demonstrate the vascularity of the tumors and differentiate it from other relatively avascular structures such as myxomas, lipomas and fibromas [10, 11]. Thus in the assessment of a cardiac mass, use of a combination of echocardiographic features may be helpful in characterizing the nature of the mass. The TEE appearance and location of this mass was inconsistent with the initial pathologic diagnosis of atrial thrombus and led to further staining and the correct characterization of the operative specimen.


Reyen K. Frequency of primary tumors of the heart Am J Cardiol 1996; 77: 107A.
Gopal AS, Stathopoulos JA, Arora N, et al. Differential diagnosis of intracavitary tumors obstructing the right ventricular outflow tract J Am Soc Echocardiogr 2001; 14: 937-40.
McAllister HA, Fenoglio JJ Jr. Tumors of the cardiovascular system. In: Atlas of tumor pathology, 2nd series, Fascicle 15 In: In: Atlas of tumor pathlogy. Washington DC: Armed forces Institute of Pathology 1978; pp. 46-52.
Burke A, Virmani R. Tumors of the heart and great vessels In: In: Atlas of tumor pathlogy,3rd series, Fascicle 16. Washington DC: Armed forces Institute of Pathology 1996; pp. 78-86.
Sobrinho AF, Ferreira JA, Borem MP, et al. Capillary type cardiac hemangioma in the left atrium Braz J Cardiovasc Surg 2005; 20(4): 1-6.
Landolphi DR, Belkin RN, Hjemdahl-Monsen CE, et al. Cardiac cavernous hemangioma mimicking pericardial cyst: atypical echocardiographic appearance of a rare cardiac tumor J Am Soc Echocardiogr 1997; 10: 579-81.
Soberman MS, Plauth WH, Winn KJ, et al. Hemangioma of the right ventricle causing outflow tract obstruction J Thorac Cardiovasc Surg 96: 307-9.
Lynch M, Clements SD, Shanewise JS, et al. Right-sided cardiac tumors detected by transesophageal echocardiography and its usefulness in differentiating the benign from the malignant ones Am J Cardiol 1977; 79: 781-84.
Engberding R, Daniel WG, Erbel R. Diagnoses of heart tumors by transesophageal echocardiography; a multicenter study in 154 patients European Cooperative Study Group Eur Heart J 1993; 14: 1223-28.
Kirkpatrick JN, Wong T, Bednarz JE, et al. Differential diagnoses of cardiac masses using contrast enhanced echocardiographic perfusion imaging J Am Coll Cardiol 2004; 43: 1412-9.
Lepper W, Shivalkar B, Rinkevich D, et al. Assessment of the vascularity of a left ventricular mass using myocardial contrast echocardiography J Am Soc Echocardgr 2002; 15: 1419-22.