Ann Thorac Surg 2004;78:1472-1475
© 2004 The Society of Thoracic Surgeons
Case report
Recurrent Pericardial Tamponade from Atrial Hemangioma
Naoyuki Sata, MDa,*,
Yukinori Moriyama, MDb,
Naokazu Hamada, MDa,
Takashi Horinouchi, MDa,
Kenkichi Miyahara, MDa
a Divisions of Cardiology, Shinkyo Hospital, Kagoshima, Japan
b Division of Cardiovascular Surgery, Shinkyo Hospital, Kagoshima, Japan
Accepted for publication July 10, 2003.
* Address reprint requests to Dr Sata, Division of Cardiology, Shinkyo Hospital, 3-41-1 Usuki, Kagoshima 890-0073, Japan
satasata{at}m.kufm.kagoshima-u.ac.jp
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Abstract
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We encountered a 72-year-old woman with a left atrial hemangioma arising in the appendage and growing like an extracardiac mass. Life-threatening cardiac tamponade, recurrent over a 5-year clinical course, was the only sign of this rare tumor. The extraatrial growth pattern of the tumor made it difficult to distinguish the cardiac origin from a paracardiac mass. With the aid of cardiopulmonary bypass, the tumor was removed from the left atrium at the base of the appendage. Pathologic diagnosis was a combination of cavernous- and venous-type hemangioma. The postoperative course was uneventful, and the patient was doing well with no pericardial effusion at the 10-month follow-up.
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Introduction
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Primary cardiac tumors are uncommon, with an incidence of 0.001% to 0.03% at autopsy. Of these, hemangiomas are extremely rare, accounting for less than 3.5% of all cardiac tumors [1, 2]. Recent advances in various imaging modalities have facilitated the diagnosis and surgical treatment of such lesions. In general, patients with intracardiac masses may present with exertional dyspnea, thromboembolic episodes, pericarditis, arrhythmia, or congestive heart failure, depending on the size and location of the mass. However, tumors with extracardiac extension may be rarely symptomatic and are occasionally found incidentally. We report herein a case of left atrial hemangioma arising in the appendage and growing like an extracardiac mass, with recurrent cardiac tamponade as the only sign.
A 72-year-old woman was brought to our hospital by ambulance with a sudden onset of exertional dyspnea and chest pain. Blood pressure was 70 mm Hg on palpation, and the pulse rate was 130 beats per minute. Physical examination revealed distant cardiac sounds with no murmur. Chest roentgenography revealed mild cardiomegaly and signs of pulmonary congestion. Electrocardiography identified a normal sinus rhythm with low voltage and nonspecific ST-T wave changes. Transthoracic echocardiography revealed massive pericardial effusion, which was treated by pericardiocentesis. Approximately 300 mL of serosanguinous fluid was removed. Cytologic examination of the fluid showed no tumor cells. Results of laboratory tests were all within normal limits. The patient's history was remarkable for cardiac tamponade. Five years earlier, she had been admitted to our hospital with severe dyspnea caused by massive pericardial effusion. At that time, pericardiocentesis was also performed, and a large amount of serosanguineous fluid was aspirated. After making a rapid recovery, the patient was transferred to another institution for further examination. Despite repeated echocardiography and computed tomography (CT), no definite diagnosis was made (Fig 1a). Since then, the patient had been asymptomatic and had no medical consultations until recurrent pericardial effusion was found on the current admission. For a workup of the effusion, transesophageal echocardiography was performed, and a large extraatrial mass was identified that exerted no external compression on any cardiac structure (Fig 2). Subsequent magnetic resonance imaging (MRI) revealed reaccumulating pericardial fluid and a heterogeneous mass 4.5 cm in diameter located between the left atrium and left pulmonary artery (Fig 3). However, chest CT did not yield sharp images of the mass indicated by MRI (Fig 1b). Angiocardiography including the coronary system was not performed. Given the clinical course and the results of imaging studies, a mediastinal tumor invading the pericardial cavity was suspected. At operation, the patient underwent a left anterolateral thoracotomy by way of the fourth intercostal space. Contrary to preoperative expectations, however, the tumor was found to be growing within the left atrial appendage and extending into the pericardial sac. The pericardium adjacent to the tumor had become thick with inflammatory changes. In view of the necessity for wide resection of this tumor with the full thickness of the atrial wall, femorofemoral cardiopulmonary bypass with pulmonary artery venting under mild hypothermia was instituted. The tumor was removed from the left atrium at the base of the appendage under beating heart. Special care was taken not to injure the left coronary artery. The excised specimen comprised an encapsulated soft, spongy mass measuring 4.5 x 4.5 cm and weighing 15 g (Fig 4). The epicardial surface of the tumor was thickened by inflammatory changes. The pathologic diagnosis was a combination of cavernous- and venous-type hemangioma (Fig 5). The postoperative course was uneventful, and the patient was doing well, with no pericardial effusion, as of 10 months after operation. Periodic follow-up with echocardiography and MRI surveillance is planned for the future.
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Fig 1. (a) Chest computed tomography (CT) after the first pericardiocentesis 5 years earlier, showing no definite mass lesion at the left atrial level. (b) CT at the current admission revealing an ill-defined mass lesion with a stenotic lumen of the appendage and massive pericardial effusion at the same slice level.
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Fig 2. Transesophageal echocardiography demonstrating a large mass lesion adjoining the left atrium, with no external compression. (LA = left atrium; LV = left ventricle.)
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Fig 3. Sagittal T2-weighted magnetic resonance image showing a high–signal-intensity mass located between the left pulmonary artery and left atrium.
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Fig 4. An encapsulated soft, spongy mass measuring 4.5 cm in diameter and weighing 15 g was removed from the base of the left atrial appendage. The tumor arose within the wall of the appendage.
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Fig 5. Histologic examination of the tumor showing an area of dilated vascular space with a cavernous hemangioma. (Hematoxylin & eosin; x400 before 65% reduction.)
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Comment
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Hemangioma of the heart is a rare tumor that affects all ages and has the potential to occur anywhere in the heart. These vascular tumors are composed of capillaries or cavernous vascular channels and often display combined features [3]. In general, differential diagnoses of mass lesions in the heart include thrombi, myxoma, lipoma, fibroma, cyst, and other malignant tumors such as angiosarcoma [1]. In contrast to myxoma, hemangioma rarely involves left atrial tissue in adults, and little information about these entities is available. Four adult cases of left atrial hemangioma, including our patient, have been reported in the English-language literature [4–6]. Among these, this case was the only one in which the tumor arose from the appendage and grew into the pericardial cavity with resultant life-threatening cardiac tamponade.
Clinical symptoms of cardiac hemangioma include chest pain, dyspnea, embolic episodes, arrhythmia, and right heart failure [1]. In this case, the fact that isolated pericardial effusion, recurrent over a 5-year clinical course, was the only sign of the hemangioma is particularly remarkable. The mechanisms of intermittent pericardial effusion are unclear, although pericardial irritation caused by mechanical friction with the tumor or spontaneous rupture of epicardial tumor vessels seems the most likely. Diagnosis is usually made by noninvasive imaging modalities such as echocardiography, CT, and MRI [1, 2]. Echocardiography usually provides the information needed for surgical treatment, such as tumor size, location, and mobility. However, as shown in our patient, extraatrial growth of the tumor makes a cardiac tumor difficult to distinguish from a paracardiac mass. Even with high-resolution imaging techniques, limitations remain in our ability to delineate the extent and location of masses and determine tissue characteristics. In fact, careful review of the chest CT of our patient taken after the first pericardiocentesis could not clearly discriminate the tumor from surrounding cardiac structures, although tumor size may have been small.
The natural history of cardiac hemangiomas is unpredictable. The tumors may remain dormant and clinically silent but rarely resolve spontaneously [7]. It is notable that patients with extracardiac tumor extension may be rarely symptomatic compared with those with intracardiac masses. However, according to previous reports in addition to our experience, even asymptomatic epicardial tumors may cause life-threatening cardiac tamponade, irrespective of tumor size [8]. Radical resection should therefore be performed for well-circumscribed hemangiomas whenever possible. Incomplete resection of huge, extensive hemangiomas have a potential risk of tumor recurrence, with resultant poor prognosis. Taking all these points into account, all patients with hemangioma must be placed under follow-up with imaging examinations after operation to detect new lesions or recurrences of this kind of rare tumor.
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References
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- Perchinsky MJ, Lichtenstein SV, Tyers GF. Primary cardiac tumors: forty years' experience with 71 patients. Cancer. 1997;79:1809–1815[Medline]
- Thomas J. Unusual primary tumors of the heart. Semin Thorac Cardiovasc Surg. 2000;12:89–100[Medline]
- Tabry IF, Nassar VH, Rizk G, Touma A, Dagher IK. Cavernous hemangioma of the heart. Case report and review of the literature. J Thorac Cardiovasc Surg. 1975;69:415–420[Abstract]
- Miralles A, Bracamonte L, Soncul H, et al. Cardiac tumors: clinical experience and surgical results in 74 patients. Ann Thorac Surg. 1991;52:886–895[Abstract]
- Abad C, Campo E, Estruch R, et al. Cardiac hemangioma with papillary endothelial hyperplasia: report of a resected case and review of the literature. Ann Thorac Surg. 1990;49:305–308[Abstract]
- Lo LJ, Nucho RC, Allen JW, Rohde RL, Lau F. Left atrial cardiac hemangioma associated with shortness of breath and palpitation. Ann Thorac Surg. 2002;73:979–981[Abstract/Free Full Text]
- Palmer TE. Spontaneous resolution of a large cavernous hemangioma of the heart. Am J Cardiol. 1986;58:184–185[Medline]
- Chrissos DN, Agelopoulos NG, Garyfallos DJ, Stergiopoulou PD. Follow-up of an unresectable hemangioma in the heart. Echocardiography. 1998;15:239–241[Medline]
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Abstract
Introduction
