Ann Thorac Surg 2005;79:1765-1767
© 2005 The Society of Thoracic Surgeons
Case report
Surgical Treatment of a Huge Cavernous Hemangioma Surrounding the Right Coronary Artery
Riza Turkoz, MD*,a,
Oner Gulcan, MDa,
Levent Oguzkurt, MDb,
Hakan Atalay, MDa,
Bulent Bolat, MDa,
Alpay Sezgin, MDc
a Department of Cardiovascular Surgery, Baskent University, Adana, Turkey
b Department of Radiology, Baskent University, Adana, Turkey
c Department of Cardiology, Baskent University, Adana, Turkey
Accepted for publication October 28, 2003.
* Address reprint requests to Dr Turkoz, Baskent Universitesi Tip Fakültesi, Adana Uygulama ve Arastirma Hastanesi, Dadaloglu mah 39 sok No. 6, Adana 01250, Turkey
drgulcan{at}yahoo.com
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Abstract
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Hemangioma of the heart is an extremely rare benign cardiac tumor. A 61-year-old woman presented with a huge hemangioma on the right ventricle. The tumor was completely surrounding the right coronary artery. Under cardiopulmonary bypass, the right coronary artery was transected from the aortic sinus and the supplying arteries of the tumor were divided. The tumor was successfully resected, and the right coronary artery was reanastomosed to the aortic sinus.
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Introduction
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Three-quarters of primary cardiac tumors are benign, most of them are myxomas. Nonmyxomatous benign cardiac tumors are rare and most commonly consist of lipoma, papillary fibroelastoma, and rhabdomyoma. Hemangiomas of the heart are extremely rare with an incidence of 1% to 2% of benign cardiac tumors. Cardiac hemangioma may occur anywhere in the heart, but it is usually found on the lateral wall of the left ventricle and on the anterior wall of the right ventricle. Diagnosis of a cardiac hemangioma is always difficult. The characteristic sign on coronary angiography is a tumor blush and the maps of blood supply to the tumor. A review of the cases reported in the literature reveals that preoperative diagnosis of cardiac hemangiomas has been achieved in only 34% of these patients [1]. We report a case of huge hemangioma located on the right ventricle surrounding the right coronary artery (RCA), which was completely removed with the aid of cardiopulmonary bypass.
A 61-year-old woman was admitted to our hospital with palpitation and tightness of the chest. No abnormalities were detected on physical examination. Her heart was in sinus rhythm (rate, 84 beats/min), and her blood pressure was 110/70 mm Hg. An electrocardiogram showed a normal sinus rhythm and nonspecific T-wave inversion in leads V1 through V6. The cardiac silhouette and pulmonary vasculature were normal on the chest roentgenogram. The erythrocyte sedimentation rate was 21 mm/h.
Transthoracic and transesophageal echocardiograms showed a huge mass localized on the anterior wall of the right ventricle and atrium. Computed tomography confirmed a mass compressing the right cardiac chambers. The left ventricular ejection fraction was 62%. Images in cardiac catheterization showed that the right atrium was considerably compressed by the tumor. Coronary angiography revealed 30% stenosis of the middle left anterior descending artery and a contrast blush arising from the RCA (Fig 1).
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Fig 1. Tumoral feeders originating from proximal RCA (arrows). There are feeding arteries, early venous filling, and some tumoral blush. Stretching of the RCA (by the tumor) early venous fillings and tumoral blush are also demonstrated.
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Surgery was planned to resect the tumor. The patient underwent a median sternotomy. Parietal pericardium was normal. There was a huge spongy lobulated mass (12 x 9 cm) extending from the right ventricle around the acute margin of the heart to the ventricular groove extending up to the aortic root and down to the right atrial appendix (Fig 2). The mass looked like a dilated right atrial appendix and was cystic on palpation. After aortic and bi-caval cannulation, the patient was placed on cardiopulmonary bypass. The aorta was cross clamped and the myocardium was arrested by antegrade blood cardioplegia. Exploration revealed that the tumor was encasing the RCA 1 cm distal to its origin. The RCA was transected from the aortic sinus and the supplying arteries of the tumor originating from the RCA were divided. The tumor was carefully separated from the heart and complete removal was achieved (Fig 3). The RCA was reanastomosed to the aortic sinus (Fig 4). The resected tumor (dimensions, 12 x 9 x 6 cm) appeared purplish, elastic, soft, and multilobular. Pathologic examination diagnosed the tumor as a cavernous hemangioma.
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Fig 4. Reanastomosis of the right coronary artery to its origin at the aorta after total excision of the tumor.
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The patient had an uneventful recovery. She remains well without medication 14 months postoperatively, and the echocardiogram showed no recurrence of the tumor.
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Comment
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Hemangioma is a benign proliferative lesion characterized by increased endothelial cell turnover. It can be found anywhere in the body, but most frequently it is localized to the cervicofacial region [2]. It can be superficial, deep, or visceral in location. Visceral hemangiomas are frequently located in deeper organs such as the liver, colon, or brain. Hemangiomas of the heart are extremely rare tumors with an incidence of 0.0017% at autopsy [3]. The epicardium is the most common location for cardiac hemangiomas, but they may also be found in the myocardium and endocardium. Histologic appearance may be either a capillary or cavernous type. The most frequent clinical symptoms depend on the location, size, and mobility of the tumor. Patients with hemangiomas may present with dyspnea on exertion, arrhythmias, angina, sign of right heart failure, pericarditis or pericardial effusion, and thromboembolic events, whereas a small percent are asymptomatic. Diagnosis of the cardiac tumors is usually made by echocardiography, computed tomography, or magnetic resonance imaging, but preoperative pathologic diagnosis is not always possible. The characteristic sign of a cardiac hemangioma on a coronary angiography is a patch of neovascularity termed a tumor blush. In cardiac hemangiomas, complete resection is the choice of treatment. The prognosis for hemangiomas is generally favorable with a low recurrence rate. Moreover, the prognosis of the patients has been quite good even if they have undergone incomplete resection. Spontaneous resolution of a large, cavernous hemangioma of the heart at a similar location and size has been reported in a case after a follow-up of 2 years [4]. For this particular case, the tumor was deemed unresectable and a specific therapy has not been prescribed. Regression has also been reported in extensive unresectable cardiac hemangioma after steroid therapy [5]. Incomplete resection may result in tumor growth and compression of the coronary vessels, clinically simulating myocardial infarction, arrhythmia, hemopericardium, and even lethal tamponade. Orthotopic heart transplantation was described for complete cure of inoperable tumors. Cardiomyoplasty has also been recommended as an alternative to heart transplantation for therapy of large ventricular tumors [6]. For our case, the difficulties encountered were: (1) encasement of the RCA by the tumor and (2) presence of multiple feeding arteries of the tumor that originated from the right coronary artery. To overcome these difficulties we proceeded as follows: After separating the RCA from its aortic origin, we divided the supplying arteries. The coronary artery is mobilized completely from the tumor. After resection of the tumor, elongation of the RCA because of stretching the tumor enabled us to implant it directly into its origin at the RCA. The surgical technique we have used overcame the difficulty of the large size and intratumoral course of the RCA so that a complete resection was possible.
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References
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- Brizard C, Latremouille C, Jebara VA, et al. Cardiac hemangiomas. Ann Thorac Surg. 1993;56:390–394[Abstract]
- Gampper vascular anomalies. Hemangiomas. Plast Reconstr Surg 2002;110:572–85
- Strauss R, Merlis R. Primary tumors of the heart. Arch Pathol. 1945;39:74
- Palmer TE, Tresch DD, Bonchek LI. Spontaneous resolution of a large, cavernous hemangioma of the heart. Am J Cardiol. 1986;58:184–185[Medline]
- Chang JS, Young ML, Lue HC. Infantile cardiac hemangioendothelioma. Pediatr Cardiol. 1992;13:52–55[Medline]
- Chachques JC, Argyriadis PG, Latremouille C, et al. Cardiomyoplasty. Ventricular reconstruction after tumor resection. J Thorac Cardiovasc Surg. 2002;123:889–894[Abstract/Free Full Text]
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Abstract
Introduction
