Ann Thorac Surg 2007;84:1396-1397
© 2007 The Society of Thoracic Surgeons
Case Reports
Left Atrial Papillary Fibroelastoma: A Rare Cause of Multiple Cerebral Emboli
Siamak Mohammadi, MD,
Andre Martineau, MD,
Pierre Voisine, MD,
François Dagenais, MD*
Department of Cardiac Surgery, Laval Hospital, Québec City, Québec, Canada
Accepted for publication May 18, 2007.
* Address correspondence to Dr Dagenais, Department of Cardiac Surgery, Laval Hospital, 2725 Chemin Sainte-Foy, Québec, G1V 4G5, Canada (Email: francois.dagenais{at}chg.ulaval.ca).
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Abstract
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Papillary fibroelastomas have been increasingly described since the widespread use of cardiac echocardiography. Most often diagnosed incidentally, papillary fibroelastomas may embolize, mainly to the cerebral circulation. The aortic valve is predominantly affected. The left ventricle is the most frequent nonvalvular location. We present a case of a 59-year-old man who had recurrent episodes of cerebral ischemic attacks and required coronary artery bypass surgery. The overall neurologic investigation was normal, including a transthoracic echocardiography. A 5 x 5 mm pedunculated, mobile mass was described on the intraoperative transesophageal echocardiography. The excision was performed through the left atrial appendage during the coronary artery bypass surgery. The microscopic examination demonstrated a papillary fibroelastoma. The unusual localization and management of papillary fibroelastomas are discussed.
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Introduction
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Papillary fibroelastomas (PFs) are a rare form of benign cardiac tumor. The majority of PFs are asymptomatic and arise from the valvular endocardium. Left-sided lesions may embolize resulting in a cerebrovascular accident. We describe a case of a fortuitous discovery of a left atrial PF during coronary artery bypass surgery in a patient with previous multiple ischemic cerebral events.
A 59-year-old man was referred for coronary artery bypass grafting owing to a three-vessel coronary artery disease with unstable angina. During the preceding 4 years, he experienced three episodes of transient ischemic attacks and one cerebrovascular accident with a residual partial left hemiparesthesia. The medical history was also positive for high blood pressure and a 25 pack-year smoking history. The patient had no prior history of arrhythmia or other neurologic symptoms. Other than the partial left hemiparesthesia, physical examination was normal. Investigations in search of a potential cause of cerebrovascular accident included a Doppler of the carotid arteries, a transthoracic echocardiography, and a thrombophilia screening, all of which were within normal limits. A head computed tomographic scan showed sequelae of his previous ischemic cerebrovascular accident. The coronary angiogram demonstrated significant left main and right coronary artery stenoses. Intraoperative transesophageal echocardiography showed the absence of a patent foramen ovale on the saline bubble test, but revealed a 5 x 5 mm pediculated, mobile and spherical mass with stippled edges attached to the left atrium at the base of the appendage (Fig 1). The ascending aorta showed a grade I atherosclerosis.
Under cardiopulmonary bypass and cardioplegic arrest, the left atrium appendage was amputated. A 5-mm yellow-white, soft, thinly fronded tumor with a short stalk was found attached to the ridge between the left superior pulmonary vein and the base of appendage. Complete excision of the tumor (Fig 2) was performed. The patient also underwent a triple coronary artery bypass procedure to the left anterior descending artery, first obtuse marginal artery, and the right coronary arteries using bilateral internal mammary arteries and one radial artery. The patient had an uncomplicated perioperative course and was discharged from the hospital on postoperative day 7. At his 4-month follow-up, the patient was well without experiencing a recurrent neurologic event.
Histopathologic examination showed a lesion consisting of a central connective tissue core lined by a single layer of endothelial cells compatible with the diagnosis of a papillary fibroelastoma.
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Comment
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The advent of echocardiography has increased the number of reported cases of PF. Papillary fibroelastoema is a rare benign intracardiac tumor that typically affects the heart valves. The aortic valve is predominantly affected. The left ventricle is the most frequent nonvalvular tumor site. The left atrium is rarely involved. In a large, comprehensive analysis of 725 cases of PF, only 10 cases have been reported to originate from the left atrium [1]. The vicinity of the left pulmonary vein and the left atrial appendage seem to be the most frequent location of PF within the left atrium, as three cases have been described on the ridge between the left atrial appendage and the left upper pulmonary vein, two cases in the left atrial appendage, and one case in the left inferior pulmonary vein [2–7]. The differential diagnosis of an unknown mass in this region should include a PF.
Most PFs are found incidentally at the time of cardiac disease investigations or autopsy. Although symptoms related to the PF are rare, thromboembolic events are the most frequent clinical manifestation [1]. Embolisms to the coronary, cerebral, and systemic circulations have been reported in cases of left heart PFs [8]. Embolization may occur from particles of the friable tumor or thrombus formed on the tumor. Tumor fragments have been isolated from embolic material [9]. It has been suggested that tumor location may influence the patient’s clinical presentation. Interestingly, all 6 patients with PF located in the vicinity of the left atrial appendage and the left pulmonary vein presented with recurrent cerebrovascular thromboembolic events. The low flow and stasis in the area of the left atrial appendage may promote thrombus deposition on the tumor with subsequent embolization.
The diagnosis is usually made by echocardiography. Although transthoracic echocardiography is highly precise for the diagnosis of most cardiac tumors, small tumors such as the PF may be missed, as evidenced by the present case. Transesophageal echocardiography has been shown to be superior for the diagnosis of PF [10]. Surgical excision is the recommended therapy for left atrial PF, regardless of the size and symptoms, given the risk of systemic embolism.
In conclusion, left atrial PF is a histologically benign tumor but a potential source of cerebrovascular emboli due to its strategic position. Transesophageal echocardiography is diagnostic and should be considered in a patient with an unexplained neurologic ischemic event. Treatment in symptomatic patients mandates surgical excision.
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References
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Abstract
Introduction
