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Case Report
Recurrent Myocardial Infarction Due to Obstruction of the Right Coronary Artery Ostium by an Aortic Papillary Fibroelastoma
November 2002
Papillary fibroelastomas (PFEs) are rare benign cardiac tumors which usually develop on valvular or other endocardial surfaces.1 They may be clinically silent and fortuitously discovered on echocardiogram,2 or may be the source of various, sometimes grave pathologic manifestations, including sudden death, myocardial infarction or systemic embolization, notably to the brain, when located in the left heart or aorta.1–5 This report describes recurrent myocardial inferior infarction caused by a PFE penetrating the right coronary artery (RCA) ostium, successfully treated with surgery.
Case Report. A 69-year-old previously healthy woman without coronary risk factors other than age and menopause managed with hormone replacement was originally admitted to a community hospital for treatment of evolving inferior myocardial infarction complicated by transient, complete atrioventricular block and paroxysmal atrial fibrillation. Following apparently successful thrombolysis with rtPA, the patient was transferred to our institution, where, on day 4, coronary angiography revealed the presence of a normal left coronary arterial system and, at first injection, a patent, dominant RCA system free of atheromatous disease. However, at the next injection, sudden subocclusion of the RCA was noted, consistent with catheter-induced spasm, soon associated with a thrombus-like appearance. After multiple unsuccessful intracoronary injections of molsidomine, isosorbide dinitrate and verapamil, angioplasty of the RCA ostium, insertion of a 16 mm stent without balloon predilation, and administration of abciximab were carried out successfully and without complication. Follow-up echocardiographic findings were consistent with a small infarction. Treatment with verapamil, aspirin and clopidogrel was initiated for the presumed diagnosis of coronary spasm. After 4 uneventful weeks, the patient was urgently readmitted to the community hospital for management of recurrent inferior myocardial infarction, for which she received a second course of rtPA, followed by seemingly effective reperfusion and uncomplicated recovery. Follow-up angiography revealed a near occlusion at the mid-proximal RCA, and retrograde perfusion of the distal vessel from the left coronary artery system, while a left anterior oblique contrast left ventriculography showed a mobile, spherical mass at the RCA ostium (Figure 1). Transesophageal echocardiography confirmed the presence of a 2-cm diameter spherical, mobile, heterogeneous mass, partially adherent to the RCA ostium (Figure 2). Furthermore, computed tomography showed the intraaortic lesion apparently attached to the previously implanted RCA ostial stent, without intraparietal or extraaortic extension (Figure 3). After confirmation of a normal brain scan, a surgical exploration showed the presence of a gelatinous, translucent, berry-like tumor penetrating the RCA ostium and stent. The latter was excised, along with the tumor in its entirety, and saphenous vein bypass grafting of the RCA was performed, followed by an uncomplicated post-operative recovery. Histologic examination of the tumor was consistent with a benign PFE. After a follow-up of 17 months, the patient has remained asymptomatic. On transthoracic echocardiography, aortic valve function was preserved, no intraaortic regrowth of the tumor was visible, and overall and segmental left ventricular systolic function was intact.
Discussion. PFEs are rare tumors representing 16% of all primary cardiac tumors, ranking third among benign cardiac neoplasms, behind myxomas and lipomas.6 In 90% of instances, the tumor is attached to the valvular endocardium.6–8 In an autopsy series, PFEs have been described in the right as well as left heart, though left-sided lesions contribute to the majority of symptoms. Over three quarters of PFEs are attached to the aortic or mitral valves, usually on their aortic and atrial surfaces, respectively. These typically solitary tumors measure between 0.2 and 5.0 cm, though most are
1. Abu Nassar SG, Parker J Jr. Incidental papillary endocardial tumor: Its potential significance. Arch Pathol 1971;92:370–376.
2. Yee HC, Nwosu JE, Lii AD, et al. Echocardiographic features of papillary fibroelastoma and their consequences and management. Am J Cardiol 1997;80:811–814.
3. Grote J, Mugge A, Schfers HJ, et al. Multiplane transoesophageal echocardiography detection of a papillary fibroelastoma of the aortic valve causing myocardial infarction. Eur Heart J 1995;16:426–429.
4. Israel DH, Sherman W, Ambrose JA, et al. Dynamic coronary ostial obstruction due to papillary fibroelastoma leading to myocardial ischemia and infarction. Am J Cardiol 1991;67:104–105.
5. Mayer K, Niederhaeuser U, Jenni R. Cardiac papillary fibroelastoma with cerebral and coronary embolic events. Heart 1998;79:307.
6. Edwards FH, Hale D, Cohen A, et al. Primary cardiac valve tumors. Ann Thorac Surg 1991;52:1127–1130.
7. Grinda JM, Latremouille CH, Berrebi A, et al. Cardiac fibroelastoma. Six operated cases and review of the literature. Arch Mal Coeur 2000;93:727–732.
8. Sun JP, Asher CR, Yang X, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: A retrospective and prospective study in 162 patients. Circulation 2001;103:2687–2693.
9. Grinda JM, Couetil JP, Chauvaud S, et al. Cardiac valve papillary fibroelastoma: Surgical excision for revealed or potential embolization. J Thorac Cardiovasc Surg 1999;117:106–110.
10. Etienne Y, Jobic Y, Houel JF, et al. Papillary fibroelastoma of the aortic valve with myocardial infarction: Echocardiographic diagnosis and surgical excision. Am Heart J 1994;127:443–445.
11. Bussani R, Silvestri F. Images in cardiovascular medicine. Sudden death in a woman with fibroelastoma of the aortic valve chronically occluding the right coronary ostium. Circulation 1999;100:2204.
12. Bossert T, Diegeler A, Spyrantis N, Mohr FW. Papillary fibroelastoma of the aortic valve with temporary occlusion of the left coronary ostium. J Heart Valve Dis 2000;9:842–843.
