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J Thorac Cardiovasc Surg 2006;132:690-691
© 2006 The American Association for Thoracic Surgery
Brief Communication |
a Department of Cardiac Surgery, "Maggiore della Carità" Hospital, Novara, Italy
c Department of Cardiology, "Maggiore della Carità" Hospital, Novara, Italy
b Department of Medical Sciences, Unit of Pathology, University School of Medicine, Novara, Italy
Received for publication April 15, 2006; accepted for publication May 3, 2006. * Address for reprints: Mario Vivirito, MD, U.O. Cardiochirurgia, Azienda Ospedaliera "Maggiore della Carità," Corso Mazzini 18, 28100 Novara, Italy (Email: mvivirito{at}libero.it).
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Cardiac hemangiomas, although histologically identical1
to those localized elsewhere in the body, are rare. In a classic autoptical review by McAllister and colleagues,2 they represent only 2.8% of primary tumors and cysts of the heart and pericardium. Probably because cardiac valves are essentially avascular structures, valvular hemangiomas are even rarer. To the best of our knowledge, up to March 2006, only 7 valvular hemangiomas have been reported in the English literature, all arising from tricuspid or mitral valves. The case we report is the first one located in the aortic valve.
Clinical Summary
An 81-year-old man was referred from an outside hospital to our department with a history of recent onset of thoracic pain, effort dyspnea, and moderate fever. A computed tomographic scan showed no signs of aortic dissection. A transesophageal echocardiogram showed a moderate aortic insufficiency, with a little mass attached to the noncoronary cusp (Figure 1). Coronary angiography demonstrated only a negligible lesion located in the distal part of the posterior descending artery and failed to show any tumor blush. A pericardiocentesis was performed, draining 800 mL of hematic fluid.
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The histopathology and the immunohistochemical staining demonstrated a capillary hemangioma of the noncoronary cusp of the aortic valve. We waited until the results of the cultural examinations were reported to be negative, and then the patient was discharged on the 13th postoperative day.
At 6 months' follow-up, the patient is doing well, and a transthoracic echocardiography shows a well-functioning prosthesis without any sign of recurrence of the tumor.
Discussion
From a clinical point of view, only a small minority of patients (8%) with cardiac hemangiomas are asymptomatic, whereas the vast majority are symptomatic, with symptoms usually depending on the location and extension of the tumor. The more frequent symptoms are as follows: dyspnea on exertion (43%), arrhythmias (17%), pseudoangina (13%), signs of heart failure (13%), pericarditis or pericardial effusion (8%), and failure to thrive (8%).3
About diagnosis, echocardiography obviously plays a primary role, and coronary angiography, when showing a feeding artery and the characteristic tumor blush, might be diagnostic. Recently, contrast-enhanced computed tomography and magnetic resonance imaging have been proposed for a more precise evaluation of the extension of the tumor.4
Even though technology now provides all these diagnostic means, because of the various settings of clinical and anatomic presentation, a correct preoperative diagnosis is not always made. In the review by Brizard and coworkers3 of 23 patients, only 34.8% had a preoperative diagnosis of cardiac hemangiomas, 21.7% underwent operations with a suspicion of cardiac tumor, and in the remaining 43.5% the diagnosis was "other." When the diagnosis is made or suspected, surgical treatment is indicated to make diagnosis certain, to relieve symptoms, to avoid the risk of sudden death, and to avoid the risk of malignant transformation.5
Because signs, symptoms, and instrumental findings did not allow a univocal interpretation, our patient was essentially operated on for the suspicion of a potentially embolizing infective endocarditis. Because of the rarity of cardiac hemangiomas and the exceptionality of valvular ones, we did not even suspect the presence of such a tumor.
In recent years, an increasing number of cardiac and also valvular hemangiomas has been reported, raising the doubt that in the past this kind of pathology could have been underestimated. Even in those recent articles, the right diagnosis is mostly still made postoperatively.
In conclusion, we believe that making a correct preoperative diagnosis of cardiac and especially valvular hemangiomas, because clinical settings and instrumental findings are often atypical, is first of all essential in the suspicion of this pathology because probably this is not so exceptional.
References
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