A 35-yr-old man presented to his primary care physician with a 7 mo history of palpitations and substernal chest pressure. To evaluate his new symptoms, the patient’s physician ordered an electrocardiogram, a transthoracic echocardiogram (TTE), and a chest roentgenogram. The chest roentgenogram and electrocardiogram findings were normal. TTE demonstrated normal left and right ventricular size and function, with structurally normal valves. A linear echodensity was noted in the aortic arch, which showed an absence of blood flow when interrogated with color flow Doppler.
The patient subsequently underwent cardiac computed tomography, magnetic resonance imaging (MRI), and a transesophageal echocardiography (TEE), all of which displayed a 2.5 cm × 2.75 cm homogenous mass adjacent to the roof the left atrium, between the aortic root and the pulmonary arteries. Figure 1 illustrates the transverse sinus located posterior to the ascending aorta and main pulmonary trunk where the hemangioma was located. The transverse sinus is formed from lines of deflection where the visceral and parietal pericardium come together in utero.1 Figure 2 displays a TEE image and measurements of the hemangioma located in the transverse pericardial sinus.
TEE additionally revealed that the mass was spongiform in appearance, and contained multiple vascular channels with low velocity blood flow, consistent with venous lakes. There was no apparent filling of the mass with left ventricular blood after IV perflutren microspheres (Optison®) administration, which was administered to provide left ventricular contrast during the echocardiogram. Additionally, there was no obvious mass effect on any adjacent mediastinal structures. Subsequent cardiac catheterization revealed a highly vascular mass supplied by a branch artery arising from the proximal left main coronary artery.
The patient was subsequently taken to the operating room, a median sternotomy was performed and the mass was excised under cardiopulmonary bypass. Clips 1 and 2 (please see clips available at www.anesthesia-analgesia.org) are intraoperative TEE clips, both from the midesophageal long axis and inflow–outflow views, which prominently display the location of the hemangioma. Final pathology revealed that the mass was a mixed cavernous and capillary hemangioma located in the transverse sinus.
Primary tumors of the pericardium and heart are uncommon (0.001 and 0.28% incidence).2 Among benign cardiac and pericardial tumors in adult patients, only 2.8% are hemangiomas. Cardiac hemangiomas have been found to arise from all aspects of cardiac structures: the pericardium, endocardium, myocardium, and epicardium.3,4 Histologically, they have been classified as cavernous, capillary, or arteriovenous hemangiomas and it is not uncommon for hemangiomas to have features of more than one classification,5 as in to our patient. The differential diagnosis included hemangioma, myxoma, angiosarcoma, or any one of many tumors that may arise from the heart or pericardium.2 Certainly, knowledge of patient age and associated morbidities help refine the preoperative differential diagnosis.
Echocardiography and cardiac MRI are often used to evaluate tumor location, size, and tissue characteristics. Preoperative TTE noted a linear echocardiographic density on examination, however it was unable to clearly characterize the tumor. TEE provided information on size, location and functional significance. Gulati et al.6 suggested MRI may offer benefits over TEE and TTE for assessment of cardiac masses in terms of better evaluating the extent and determining etiology. Others have supported the use of MRI for defining the nature of cardiac tumors.7 Clinical follow-up for patients with these lesions is unpredictable; however, if the lesion is resectable, the clinical course is reported to be favorable. Unresectable lesions have been documented to produce ventricular tachyarrhythmias, along with other associated symptoms of cardiac compression.5
Echocardiography and newer imaging modalities are critical for diagnostic purposes and for delineating the extent and functional significance of tumors. The additional role of intraoperative TEE inpatient management is certainly dependent on tumor location and planned surgical strategy. TEE was used to delineate any change in tumor size or characteristics and to access the functional impact in real-time. In addition, should coronary anatomy have been disrupted after tumor excision (vascular supply from the left main coronary artery), regional wall motion abnormalities would have alerted the care team to pursue additional interventions, such as bypass grafting or repair of the damaged vessel.
1. Sadler TW. Langman’s medical embryology, 9th ed. Philadelphia: Lippincott Williams and Wilkins, 2004:255
2. McAllister HA Jr. Primary tumors and cysts of the heart and pericardium. In: Harvey WP, ed. Current problems in cardiology. Chicago: Year Book Medical Publishers, 1979:8–38
3. Luna A, Ribes R, Caro P, Vida J, Erasmus J. Evaluation of cardiac tumors with magnetic resonance imaging. Eur Radiol 2005;15:1446–55
4. Vander Salm T. Unusual primary tumors of the heart. Semin Thorac Cardiovasc Surg 2000;12:89–100
5. Burke A, Virmani R. Tumors of the heart and great vessels. In: Atlas of tumor pathology, Series 3, Fascicle 16. Washington, D.C.: Armed Forces Institute of Pathology, 1993;78–86
6. Gulati G, Sharma S, Kothari S, Juneja R, Saxena A, Talwar K. Comparison of echo and MRI in the imaging evaluation of intracardiac masses. Cardiovasc Intervent Radiol 2004;27:459–69
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7. Alsaileek A, Tepe S, Alveraz L, Miller D, Tajik J, Breen J. Diagnostic features of cardiac hemangioma on cardiovascular magnetic resonance, a case report. Int J Cardiovasc Imaging 2006;22:699–702