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J Thorac Cardiovasc Surg 2006;132:691-692
© 2006 The American Association for Thoracic Surgery

Brief Communication

Asymptomatic hemangioma of the interatrial septum

^a Department of Pathology, Abderrahmen Mami Hospital, Ariana, Tunisia
^b Department of Cardiothoracic Surgery, Abderrahmen Mami Hospital, Ariana, Tunisia
^c Department of Cardiology, Siliana Hospital, Siliana, Tunisia

Received for publication April 28, 2006; accepted for publication May 9, 2006.

^_* Address for reprints: Aïda Ayadi-Kaddour, MD, Department of Pathology, Abderrahmen Mami Hospital, 2080 Ariana, Tunisia (Email: kaddour1fr{at}yahoo.fr).

A cardiac hemangioma is a rare form of primary cardiac tumor, occurring with an incidence of 1% to 2% of all detected benign heart neoplasms.¹ To date, only 45 cases involving surgical treatment have been discussed in the literature.¹

We present a patient with hemangioma of the interatrial septum that was found incidentally and successfully resected.

Clinical Summary

A 74-year-old man was referred to our institution with a cardiac tumor detected incidentally by means of thoracic echocardiography during a medical checkup. The patient was asymptomatic. Physical examination revealed an arrhythmia. Electrocardiography showed an irregular rhythm caused by atrial fibrillation and left ventricular hypertrophy. Transesophageal echocardiography revealed a 24-cm² echogenic mass arising from the interatrial septum (Figure 1). Computed tomography confirmed a huge oval tumor (6.1 x 6 cm) with hypointense signal intensity; inhomogeneous enhancement after intravenous contrast administration is shown in Figure 1. The patient was taken to the operating room for resection of the cardiac mass during cardiopulmonary bypass. The defect of the interatrial septum was repaired with a patch of a synthetic tissue. The patient's recovery was simple. The macroscopic examination showed a mass measuring 6 x 4 x 3 cm, with a well-demarcated border and spongy quality (Figure 2 ). Microscopic examination revealed large, endothelial-lined, blood-containing spaces with thick fibroblastic walls (Figure 2). Areas of capillary-type vessels were present. The channels were separated by sparse connective tissue. Immunohistochemical staining with the endothelial marker CD34 confirmed the diffuse presence of an internal layer of thin endothelial cells. Thus the diagnosis of mixed hemangioma was made. At 2 years' follow-up, the patient is doing well.

View larger version (92K): [in this window] [in a new window]   Figure 1. Computed tomographic scan showing a septal tumor.

View larger version (121K): [in this window] [in a new window]   Figure 2. The excised gross tumor. Inset, Histologic section showing irregular, relatively large, endothelial-lined vascular structures.

Primary neoplasm of the heart is rare and often diagnosed postmortem because of the lack of specific clinical symptoms and signs.² Among benign cardiac tumors, cavernous hemangioma is an extremely rare tumor. These tumors can present at all ages, with a mean presenting age of 43 years and a slight male predominance.³ Subendocardial hemangiomas have been described in all the cardiac chambers, but most occur on the right side of the heart and the left atrium.³ Clinical manifestations include dysrhythmia, pericardial effusion, heart failure, outflow tract obstruction, and coronary insufficiency.¹ A right atrial location on the rim of the foramen ovale or interatrial septum is typically silent.³ Less invasive diagnostic techniques, such as transthoracic echocardiography, allow for the diagnosis to be made during life with definitive surgical treatment.⁴ Our patient presented with atrial fibrillation, and transthoracic echocardiography showed a tumor arising from the atrial septum mimicking atrial myxoma. Preoperative diagnosis of cardiac hemangiomas is always difficult and could be established in only 34% of a previously reported series.⁴ With the availability of new noninvasive imaging techniques, diagnosis of these lesions might now be more frequent. Enhanced-contrast computed tomography or magnetic resonance imaging shows the vascular nature of the tumor and helps to evaluate the extent of extracardiac invasion and myocardial involvement.⁵ Coronary angiography often helps to establish the diagnosis by showing the characteristic tumor blush. Cardiac hemangiomas are classified as cavernous, capillary, mixed, or arteriovenous.⁵ Cardiac hemangioma can be successfully excised, and surgical resection is the treatment of choice for symptomatic lesions or when the diagnosis is in question.² The long-term outcome of patients with surgically treated symptomatic lesions is excellent. Spontaneous regression of a cardiac hemangioma has been reported, and therefore surgical intervention might not always be necessary, particularly for an extensive but asymptomatic hemangioma that would require complex and potentially hazardous excision.^3,4

References

1. Ugras S, Bayram I. Cavernous haemangioma of the mitral valve in a child. report of a case and review of the literature. Pathology. 2005;37:396-398.[Medline]
   
2. Kipher B, Englberger L, Stauffer E, Carrel T. Rare presentation of cardiac hemangioma. Ann Thorac Surg. 2000;70:977-979.[Abstract/Free Full Text]
   
3. Thomas JE, Eror AT, Kenney M, Caravalho J. Asymptomatic right atrial cavernous hemangioma. a case report and review of the literature. Cardiovasc Pathol. 2004;13:341-344.[Medline]
   
4. Lapenne E, De Bonis M, Torracca L, La Canna G, Dell' Antonio G, Alffieri O. Cavernous hemangioma of the tricuspid valve. minimally invasive surgical resection. Ann Thorac Surg. 2003;76:2097-2099.[Abstract/Free Full Text]
   
5. Burke A, Virmani R. Tumors of the heart and great vessels. minimally invasive surgical resection. In: Rosai J, editor. Atlas of tumor pathology, third series, fascicle 16. Washington (DC): Armed Forces Institute of Pathology; 1996. pp. 79-90.
   

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This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Ayadi-Kaddour, A. Articles by El Mezni, F. Search for Related Content PubMed PubMed Citation Articles by Ayadi-Kaddour, A. Articles by El Mezni, F. Related Collections Cardiac - other