Ann Thorac Surg 2009;87:1953-1954. doi:10.1016/j.athoracsur.2008.11.039
© 2009 The Society of Thoracic Surgeons
Case Reports
Papillary Fibroelastoma of the Aortic Wall With Partial Occlusion of the Right Coronary Ostium
Can Yerebakan, MD*,
Andreas Liebold, MD, PhD,
Gustav Steinhoff, MD, PhD,
Christian Alfons Skrabal, MD, PhD
Department of Cardiac Surgery, Medical Faculty, University of Rostock, Rostock, Germany
Accepted for publication November 13, 2008.
* Address correspondence to Dr Yerebakan, Department of Cardiac Surgery, Medical Faculty, University of Rostock, Schillingallee 35, Rostock, 18057, Germany (Email: can.yerebakan{at}med.uni-rostock.de).
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Abstract
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Papillary fibroelastomas are the most common cardiac valve tumors, although they represent less than 10% of all cardiac tumors. These benign tumors are increasingly incidentally discovered as the result of the widespread use of echocardiography. After a definitive diagnosis has been made, surgical resection is strongly advocated due to the risk of cardioembolic complications. We present a very rare finding of an aortic wall papillary fibroelastoma with a resultant partial occlusion of the right coronary ostium.
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Introduction
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Cardiac papillary fibroelastomas are rare, benign, and often asymptomatic tumors that are usually found at autopsy. They mostly originate from valvular endocardium and are often located in the left heart. In symptomatic cases, valvular dysfunction, embolic stroke, peripheral embolism, or sudden cardiac death can occur. We present a patient with a symptomatic papillary fibroelastoma of the aortic wall with resultant angina pectoris and stroke. The tumor was surgically removed.
A 74-year-old woman with a recent medical history of recurrent dizziness and embolic stroke in the brainstem was referred to our clinic after echocardiographic examination revealed a 1.0- x 1.5-cm tumorous formation in the ascending aorta in close to the right coronary aortic valve leaflet (Fig 1). Further questioning revealed that the patient had experienced chest pain at the time of the stroke, in addition to an episode of nausea and vertigo accompanied by transient visual disturbances.
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Fig 1. Transesophageal echocardiographic image shows the papillary fibroelastoma (PF) adherent to the aortic wall close to the right coronary ostium. There is no connection to the aortic valve cusps. (AV = aortic valve; RCA = right coronary artery.)
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Thrombus formation was suspected due to the signs of subacute ischemic stroke as found in magnetic resonance imaging. Therefore, the referring clinic had already initiated thrombolytic therapy with alteplase. Although the treatment was well tolerated by the patient, the size of the tumor remained unchanged in the echocardiographic follow-up examination. No clinical or laboratory signs of an inflammatory process or endocarditis developed.
Surgical intervention was indicated due to the risk of possible further embolization. After median sternotomy, partial cardiopulmonary bypass was established through cannulation of the aorta and inferior vena cava through the right atrial appendage in the usual fashion. After aortic cross-clamping and transverse aortotomy, an approximately 1.5- x 1.0-cm glossy, transparent, elastic tumor with whitish filaments was found (Fig 2A). The tumor arose partially from the aortic wall immediately next to the right coronary ostium, which itself was partially obstructed by an extension of the mass. The aortic valve itself was not affected. The entire tumor was removed, including the very superficial endothelial layer of the aortic wall (Fig 2B). Total cardiopulmonary bypass time was 39 minutes, and the patient was weaned in a stable hemodynamic state.
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Fig 2. (A) Intraoperative view after aortic valve exposure shows papillary fibroelastoma (PF) with partial occlusion of the right coronary ostium. (B) Papillary fibroelastoma after excision.
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The histopathologic examination of the tumor revealed a papillary fibroelastoma without thrombotic or inflammatory components. The patient had an uneventful recovery on the cardiac surgical ward and was discharged on postoperative day 10.
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Comment
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Papillary fibroelastomas are the most common cardiac valve tumors. They are thought to represent less than 10% of all cardiac tumors [1], but their true incidence is unknown. These tumors are the third most frequent benign primitive cardiac neoplasms after myxomas and fibromas [2]. The tumor appears as a solitary mass in 90% of patients, and in 95% it is located within the left heart. The tumor usually reaches a maximum size of 15 mm, but larger tumors have also been found [3, 4]. With the exception of only few congenital cases that have been reported, there seems to be neither a specific genetic causality nor sex predominance. The etiology of these tumors is still unclear. Grandmougin and colleagues [5] have advocated a possible virus-induced mechanism as a result of findings of cytomegalovirus in the tumor mass.
The diagnosis is usually made incidentally in asymptomatic patients who undergo echocardiography for some other reason. However, embolic stroke attacks, valvular dysfunction, arrhythmias, angina pectoris, or even myocardial infarction due to coronary occlusion are possible complications that may lead to a diagnostic workup that reveals a papillary fibroelastoma as the underlying pathology [6–8]. These tumors are located on semilunar and atrioventricular valves with an almost equal frequency. Papillary fibroelastomas attached to the aortic valve have rarely been reported to cause obstruction of coronary artery ostia. This patient had the very rare presentation of a papillary fibroelastoma originating from the aortic wall and partially obstructing the right coronary ostium.
Tumor embolization seems to have been the cause of the ischemic stroke. The patient's concomitant complaint of chest pain may also be attributed to the partial flow disturbance through the right coronary ostium. The exclusion of endocarditis coupled with the lack of size reduction during thrombolytic treatment in addition to the typical echocardiographic appearance led to the suspicion of a tumorous process.
Because of the high embolic risk of the mass, the decision for surgical removal was made in accordance with the general recommendations in the literature. Various surgical strategies have been discussed, all of which include the successful removal of valvular or intraventricularly located papillary fibroelastomas. In addition to the standard institution of cardiopulmonary bypass with full median sternotomy and open resection, minimally invasive techniques with limited sternotomy or thoracoscopic approaches have also been successful [9, 10].
In summary, cardiac papillary fibroelastomas may be incidental findings in asymptomatic patients or they may present with serious complications such as embolism, coronary obstruction, or valvular dysfunction. In any case, they need to be removed surgically owing to possible or existing life-threatening complications.
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References
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- McAllister HA, Fenoglio JJ. Tumors of the cardiovascular systemIn: Hartmann WH, Cowan WR, editors. Atlas of tumor pathology. 2nd series. Washington, DC: Armed Forces Institute of Pathology; 1978. pp. 20-25Fascicle 15.
- Shahian D. Papillary fibroelastomas Semin Thorac Cardiovasc Surg 2000;12:101-110.[Medline]
- Sun JP, Asher CR, Yang XS, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients Circulation 2001;103:2687-2693.[Abstract/Free Full Text]
- Crestanello JA, Orszulak TA. Giant papillary fibroelastoma of the right atrium: an unusual presentation Ann Thorac Surg 2002;74:1252-1254.[Abstract/Free Full Text]
- Grandmougin D, Fayad G, Moukassa D, et al. Cardiac valve papillary fibroelastomas: clinical, histological and immunohistochemical studies and a physiopathogenic hypothesis J Heart Valve Dis 2000;9:832-841.[Medline]
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- Pasteuning WH, Zijnen P, van der Aa MA, Peters JH, van Geldorp TR. Papillary fibroelastoma of the aortic valve in a patient with an acute myocardial infarction J Am Soc Echocardiogr 1996;9:897-900.[Medline]
- Butterworth JS, Poindexter CA. Papilloma of cusp of the aortic valve. Report of a patient with sudden death. Circulation 1973;48:213-215.[Abstract/Free Full Text]
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- Hsu VM, Atluri P, Keane MG, Woo YJ. Minimally invasive aortic valve papillary fibroelastoma resection Interact Cardiovasc Thorac Surg 2006;5:779-781.[Abstract/Free Full Text]
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C. Yerebakan, G. Steinhoff, and C. A. Skrabal
Papillary fibroelastoma of the left heart with multiple origins?
Interact CardioVasc Thorac Surg,
November 1, 2010;
11(5):
677 - 678.
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Abstract
Introduction
