CASE REPORT

Dynamic Coronary Ostial Obstruction Papillary Fibroelaktoma Leading to Myocardial lschemia and Infarction

Due to

Douglas H. Israel, MD, Warren Sherman, MD, John A. Ambrose, MD, Samin Sharma, MD, Noam Harpaz, MD, and Michael Robbins, MD apillary fibroelastoma is an uncommon cardiac tumor rarely P diag, losedduring life. Although mosi

cal chest discomfort, with inferior ST-segment elevation. Left ventriculography revealedposterobasal hyare incidental findings at autopsy, a pokinesia, without opacification of small number of caseshave beenas- the right coronary artery. The right sociatedwith cardiac symptomsthat coronary artery was recannulated include angina, embolism and sud- and selective injection filled the arden death. We describea caseof pap tery normally, but with markedly illary fibroelastoma detected angio- delayed distal runoff. Ostial obgraphically and shown to causecor- struction was suspected, and an aoronary obstruction and myocardial togram, performed to better visualize the ostium, revealed no filling ischemia. (Figure I). Several moments later, A 50-year-old woman without coronary risk factors developed ex- the patient’s chest pain resolved and ertional chest pain I month before the electrocardiogram reverted to admission. Electrocardiogram was baseline. Recannulation of the right normal. On stress testing, she devel- coronary artery at this time showed oped chest pain with ST-segment el- normal opacification and runoff. evation in the inferior leads. Q-wave However, chest pain recurred spontaneously minutes later and repeat myocardial infarction subsequently evolved, with a peak creatine kinase injection again revealed absent distal runoff. On review, the tine of 1,188 (14.5% MB). After further episodes of chest pain at rest, the showed a filling defect that moved with the aortic valve and prolapsed patient was referred for cardiac catheterization. toward the right coronary ostium. Thus, with ongoing evidence of myoCoronary arteries were angiocardial ischemia, and a suspected graphically normal. After the pigtail catheter was passed into the left ven- aortic valve mass, the patient underwent aortic root exploration. At surtricle, the patient developed her typigery, a pedunculated mass, 1.5 cm in From the Department of Cardiology, Mount Sinai Medical Center, New York, New York diameter, was found attached to the 10029.Manuscript receivedJune 7, 1990;re- upper margin of the right coronary vised manuscript received and accepted July cusp of the aortic valve, overlying 25, 1990. and obstructing the right coronary Address for reprints: John A. Ambrose, MD, Mount Sinai Hospital, Annenberg 8, Box ostium. Microscopic analysis revealed a 1030,One GustaveL. Levy Place,New York, hyalinized, multilobulated pedicle New York 10029.

FtGURE 1. Aartogram showing a lucencyinthertghtaorttcsiWs.Thereis nvttuingoftherightcoronaryartery andOllCilWGfitlllhlW3UyCanbeSeen pdapsinginandoutofrightcoroMly ostbn.Whentnjecthwasmade,patienthadchestpainwithidemic changeson ekcbcadiogram.

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with papillary fronds radiating haphazardly from its surface (Figure 2). The pedicle consisted of fused lobules of fibroelastic tissue composed of condensed, granular elastic tissue cores surrounded by lamellar whorls of elasticfibers and acellular, homogeneouscollagen. The papillae were similarly organized, the coarser ones consisting of lobules of elastic tissue and collagen, and the more delicate fends of collagen only. Each frond was investedby a single layer offlat endothelial-like cells, beneath which was a distinct light-blue myxoid layer that stained with Alcian blue.

Papillary fibroelastoma constitutes lessthan 1% of all primary intracardiac tumors.’ Most often found attached to heart valves, such tumors are usually lOO cases have been published in the world,L-3 but only a fraction have beenassociated with symptoms.Three casesof papillary fibroelastomahavebeenreported at autopsyin patients who had had a history of stroke,3e5although direct causeand effect relations were not proved. More convincing evidenceof embolismwaspresentedin a report of suddendeath in a man with an aortic valve tumor and a left anterior descendingartery emboluscomposedof histologically identical tissue.6 Other cases of sudden death possibly due to coronary obstruction have beenreported.7,8A small number of other case reports implicate papillary fibroelastoma as the cause of angina pectorisgor episodiccyanosis due to right ventricular outflow tract obstruction.‘0 To date, there have been 6 previously reported casesof papillary fibroelastomadetectedduring life,2JIl4 usually discovered incidentally during echocardiographic investigation of other underlying cardiac disorders.‘2-‘4 In one of the reported cases,14cardiac catheterization revealedan aortic root lucency in a 4 lyear-old man with episodesof nonexertional chest pain and angiographitally normal coronary arteries. Although transient coronary ob-

aortic root angiography may be helpful in establishing the diagnosis.

PtGURE 2. Papillary fibroelastoma consisting of thick pedick and papillary fronds. Pe&te consists of mdtipte fused lddes ot hyatinixed connective tissue. Entire mass layer (hematoxylin-eodn stain, X40). is invested by ttat edotheM-tike

1. McAllister HA, Fenoglio JJ Jr. Tumors of the cardiovascular system. Atlas of Tumor Pathology. Armed Forces Institute of Pathology, 1978;20-25. 2. Almagro UA, Perry LS, Choi H, Pintar K. Papillary fibroelastoma of the heart. Arch Pathol Lab Med 1982;106:318-321. 3. Abu Nassar SC, Parker JC Jr. Incidental papillary endocardial tumor: its potential significance. Arch Pathol Lab Med I97 I ;92:370-376. 4. Yater WM. Tumors of the heart and pericardium. Pathology, symptomatology and report of nine cases. Arch Intern Med 1931;48:627-666. 5. J&ski TC. Myxoma of the heart valves: report of a case. Am J Pathol 1934;10:399-405. 6. Harris LS, Adelson L. Fatal coronary embolism from a myxomatous polyp of the aortic valve. An unusual cause of sudden death. Am J C/in Pathol 1964;43:61-64. 7. Butterworth JS, Poindexter CA. Papilioma of cusp of the aortic valve. Report of a patient with sudden death. Circulation 1973;48:213-215. 8. Rona G, Feeney N, Kahn DS. Fibroelastic hamartoma of the aortic valve producing ischemic heart disease. Associated pulmonary glomus bodies. Am J

Cardiol 1963;12:869-874.

struction was an obvious etiology for the patient’s symptoms, at surgery one could not show mobility of the massinto either coronary ostium, in contrast to our case. In the patient reported here, an aortic valve papillary fibroelastoma was discovered at angiography as a filling defect in the right aortic sinus. To our knowledge, this is the only case where there is definite symptomatic, electrocardiographic, enzymatic and gross intraoperative evidence of papillary fibroelastoma resulting in myocardial infarction. The actual mechanismof infarction is not entirely clear. Embolization of tumor fragmentsor thrombotic materi-

al cannot be excluded,but it is possible that the tumor prolapsedinto the right coronary ostium, as it did during the catheterization, perhaps guided in part by the increasedforce and velocity of ejection of blood during the patient’s exercise test. We therefore have provided for the first time definite arteriographic evidence of dynamic coronary obstruction by tumor as the causeof chestpain with electrocardiographic evidence of myocardial ischemia. As excision of suchtumors is now feasible,papillary fibroelastoma should be considered as a rare but curable causeof myocardial ischemia.When indicated, 2dimensional echocardiography and

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9. Heath D, Best PV. Davis BT. Papilliferous tumows of the heart valves. Er Heart J I96 I ;23:20-24. 10. Anderson KR, Fiddler GI, Lie JT. Congenital papillary tumor of the tricuspid valve: an unusual cause of right ventricular outflow tract obstruction in a neonate with trisomy E. Mayo Clin Proc 1977;52:

665-669. 11. Lichtenstein HL, Lee JCK, tumor of the heart: incidental

Stewart finding

S. Papillary at surgery.

Hum Pathol 1979;10:473-475. 12. Cha SD, Incarrito J, Fernandez J. Giant Lambl’s excrescence of papillary muscle and aortic valve. Echocardiographic, angiographic, and pathologic C/in Cardiol I98 I :4:5 l-54. findings. 13. Flotte T, Pinar H, Feiner H. Papillary elastofibroma of the left ventricular septum. Am J Swg Pathol 1979;10:473-475. 14. Shub C, Tajik AJ, Seward JB, Edwards WD, Pruitt RD, Orszulak TA, Pluth JR. Cardiac papillary fibroelastomas. Two-dimensional echocardioMayo Clin Proc 198 1;56:629graphic recognition.

633. 15. Campbell H, Carting WR. Sudden death due to a fibrinous polyp of the aortic valve. Guy’s Hasp Rep 1934;84:41-42.

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