ATS
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Abstract Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Author home page(s):
Friedrich Stefan Eckstein
Hans-Joachim Schäfers
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Eckstein, F. S.
Right arrow Articles by Borst, H.-G.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Eckstein, F. S.
Right arrow Articles by Borst, H.-G.

Ann Thorac Surg 1995;60:206-208
© 1995 The Society of Thoracic Surgeons

Case Reports

Papillary Fibroelastoma of the Aortic Valve Presenting With Myocardial Infarction

Friedrich Stefan Eckstein, MD, Hans-Joachim Schäfers, MD, Jochen Grote, MD, Andreas Mügge, MD, Hans-Georg Borst, MD

Divisions of Thoracic and Cardiovascular Surgery and Cardiology, Hannover Medical School, Hannover, Germany


   Abstract
Top
 Abstract
Introduction
 Comment
 References
 
We describe the case of a 56-year-old woman who presented with myocardial infarction. Noninvasive and invasive investigations revealed coronary embolism from a papillary tumor attached to the right coronary cusp of the aortic valve as the underlying process. The tumor, which histologically proved to be a papillary fibroelastoma, was excised surgically and the resulting defect in the aortic valve leaflet was closed with a patch of autologous pericardium, effectively reconstructing the aortic valve. The current literature on intracardiac papillary fibroelastoma as a source of embolism and its surgical treatment is summarized.


   Introduction
Top
 Abstract
 Introduction
Comment
 References
 
Intracardiac papillary fibroelastomas of the heart valves are extremely rare, benign, and often asymptomatic tumors usually found at autopsy. When the tumor becomes symptomatic during life, loss of valve function, peripheral embolism, stroke, myocardial infarction, or sudden death can occur. The only logical therapy at present is surgical excision with replacement or reconstruction of the native valve. We describe a case of a patient with a papillary fibroelastoma of the aortic valve that was treated successfully by surgical excision.

A 56-year-old woman was admitted to a peripheral hospital with severe chest pain and enzymatic as well as electrocardiographic signs of an inferolateral myocardial infarction. Systemic lysis therapy with streptokinase was started, the peak creatine kinase level being 1744 U/L (creatine kinase-MB level, 100 U/L). The patient became asymptomatic and the postinfarction period was without complications. Two weeks later a transthoracic two-dimensional echocardiogram was performed revealing an inferolateral hypokinesia, as well as an 18 x 11 mm mass attached to the right coronary cusp of the aortic valve. There was no stenosis and only mild insufficiency of the aortic valve. Because there were no clinical signs of endocarditis, a tumor of unknown origin was presumed. The patient was treated with warfarin and was transferred to a cardiac referral center. Cardiac catheterization and angiography showed no evidence of coronary artery disease. Aortogram revealed a filling defect on the right coronary cusp of the aortic valve. Echocardiography was repeated, and the mass attached to the aortic valve was classified as a mural thrombus most probably caused by subclinical endocarditis. Conservative therapy with warfarin was continued and the patient discharged after rehabilitation.

Four months later, repeat aortogram demonstrated an unchanged mass attached to the aortic valve. It was thought that the mass most likely represented a tumor arising from the aortic valve. Because embolization into the right coronary artery already had occurred, this was considered a clear indication for surgical intervention.

The patient was operated on by means of extracorporeal circulation in moderate hypothermia (30°C). The aorta was cross-clamped and opened with a longitudinal incision, and cardioplegia was given into the coronary ostia. Inspection of the aortic root revealed a tumor measuring 18 x 11 mm arising from the free wall of the right coronary cusp of the aortic valve, its appearance resembling a sea anemone (Fig 1Go). The tumor was excised with the adjacent part of the valve leaflet (Fig 2Go). This resulted in a defect in the cusp of approximately 10 x 5 mm. The defect was reconstructed by implantation of an autologous pericardial patch measuring 10 x 5 mm using a continuous suture. The aortotomy was closed and the patient was weaned from extracorporeal circulation in normal sinus rhythm. Intraoperative transoesophageal echocardiography revealed no aortic insufficiency.



View larger version (2K):
[in this window]
[in a new window]
  		Fig 1. . Excised tumor and part of the cusp. The tumor is submerged in water to show its similarity to a sea anemone.

 


View larger version (121K):
[in this window]
[in a new window]
  		Fig 2. . Field of operation after aortotomy. The tumor is attached to the right coronary cusp, and the excision line is marked by the dotted line. (Painting by K. H. Richardt, Hannover Medical School.)

 
The postoperative course was unremarkable. Follow-up echocardiography performed 6 months postoperatively revealed normal function with minimal central reflux of the aortic valve without a sign of tumor recurrence. Histologic examination of the resected mass revealed a primary cardiac tumor classified as a benign papillary fibroelastoma.


   Comment
Top
 Abstract
 Introduction
 Comment
References
 
Primary tumors of the heart and pericardium are extremely rare, with an incidence between 0.0017% and 0.28% in reported or collected autopsy series [1]. More than 70% of these tumors are benign, and only 8% are papillary fibroelastomas, mostly located on heart valves [1]. The most frequent location is, as in the presented case, the valvular endocardium of the aortic valve [1, 2]. The macroscopic appearance of the papillary fibroelastoma reminds many authors of a sea anemone. Histologically the tumor consists of the normal components of the endocardium, fibrous tissue, elastic fibers, and smooth muscle cells set in a mucopolysaccharide matrix and covered by hyperplastic endocardial cells [1]. Although there is a characteristic morphology of the described tumor, other names such as giant Lambl's excrescence [3, 4], papillary tumor [5, 6], fibroma [7–11], and papillary fibroma [12–14] have been used in the past.

Most fibroelastomas are incidental findings on surgically excised heart valves or are found at postmortem examination [6, 15–18]. Although they are classified as benign tumors they can result in serious complications. Striking clinical aspects are embolization or angina pectoris [11, 18–20], myocardial infarction [12, 21, 22], and stroke or neurologic deficit [8, 12–14, 21, 23–29]. Although the first successful removal of a cardiac tumor was in 1952 [20], reliable preoperative diagnosis of cardiac tumors only became easily available with the aid of transthoracic and especially transesophageal echocardiography [3, 7, 13, 14, 26, 27, 29–33].

Surgical resection is considered the treatment of choice for cardiac neoplasms even without clinical signs [7, 11, 12, 34], because the potential complications may be debilitating or lethal. The operative risk is low, and the short-term and long-term results are excellent [7, 9–12, 21, 22, 30, 31, 35]. This likewise applies to tumors affecting cardiac valves. Tumors must be excised and reconstruction of the valve should be performed if resection involves a minor part of a valve leaflet. Valve replacement is indicated if a significant part of the valve is destructed by the tumor or restoration is not possible [17]. Reconstruction of the mitral valve appears easily feasible and has been reported previously [26, 29]. Reconstruction of the aortic valve is considered more difficult; consequently valve replacement after resection of the tumor has been preferred [3, 22]. Valve reconstruction, however, avoids prosthesis-related complications, such as heart block, paravascular leak, or the need for anticoagulation. Although the follow-up is still limited, disadvantages of valve replacement, ie, anticoagulation or degeneration of biologic valves, possibly can be avoided. Long-term follow-up of our patient will be necessary to determine the functional longevity of the reconstructed aortic valve.


   References
Top
 Abstract
 Introduction
 Comment
 References
 

  1. Kroll W, Nellessen U, Hofig M, Luttges J, Sievers H, Simon R. Young patient with left brain infarct and transient right sided hemiparesis in cardiac papillary fibroelastoma. Z Kardiol 1991;80:234–6.[Medline]
  2. Marvasti MA, Obeid AI, Cohen PS, Giambartolomei A, Parker FB. Successful removal of papillary endocardial fibroma. Thorac Cardiovasc Surg 1983;31:254–5.[Medline]
  3. Lichtenstein HL, Lee JCK, Stewart S. Papillary tumor of the heart: incidental finding at surgery. Hum Pathol 1979;4:473–5.
  4. Seib HJ, Wildenauer M, Luther M, Pitzl H, Henselmann L. [Papillary fibroelastoma of the aortic valve. Sudden death due to a rare tumor of the heart]. Z Kardiol 1984;73:409–13.[Medline]
  5. Edwards FH, Hale D, Cohen A, Thompson L, Pezzella AT, Virmani R. Primary cardiac valve tumors. Ann Thorac Surg 1991;52:1127–31.[Abstract]
  6. Almagro UA, Perry LS, Choi H, Pintar K. Papillary fibroelastoma of the heart. Report of six cases. Arch Pathol Lab Med 1982;106:318–21.[Medline]
  7. Pomerance A. Papillary ``tumours'' of the heart valves. J Pathol Bacteriol 1961;81:135–40.
  8. Reece IJ, Cooley DA, Frazier OH, Hallman GL, Powers PL, Montero CG. Cardiac tumors. J Thorac Cardiovasc Surg 1984;88:439–46.[Abstract]
  9. Kasarskis EJ, O'Connor W, Earle G. Embolic stroke from cardiac papillary fibroelastomas. Stroke 1988;19:1171–3.[Abstract/Free Full Text]
  10. Ong LS, Nanda NC, Barold SS. Two-dimensional echocardiographic detection and diagnostic features of left ventricular papillary fibroelastoma. Am Heart J 1982;103:917–8.[Medline]
  11. Larrieu AJ, Jamieson WRE, Tyers GFO, et al. Primary cardiac tumors. J Thorac Cardiovasc Surg 1982;83:339–48.[Abstract]
  12. Dapper F, Görlach G, Hoffmann C, Fitz H, Marck P, Scheld HH. Primary cardiac tumors—clinical experiences and late results in 48 patients. Thorac Cardiovasc Surg 1988;36:80–5.[Medline]
  13. Miller JI, Mankin HT, Broadbent JC, Giuliani ER, Danielson GK. Primary cardiac tumors. Circulation 1972;(Suppl 1):134–8.
  14. Levinsky L, Srinivasan V, Gingell RL, et al. Papillary fibroelastoma of aortic and mitral valves following myectomy for idiopathic hypertrophic subaortic stenosis. Thorac Cardiovasc Surg 1981;29:187–91.[Medline]
  15. McAllister HA Jr, Fenoglio JJ Jr. Tumors of the cardiovascular system. In: Atlas of Tumor pathology, second series, fascicle 15. Washington DC: Armed Forces Institute of Pathology, 1978:20–5.
  16. Cha SD, Incarrito J, Fernandez J. Giant Lambl's excrescence of papillary muscle and aortic valve. Echocardiographic, angiographic, and pathologic findings. Clin Cardiol 1981;4:51–4.[Medline]
  17. Fowles RE, Miller DC, Egbert BM, Fitzgerald JW, Popp RL. Systemic embolization from a mitral valve papillary endocardial fibroma detected by two-dimensional echocardiography. Am Heart J 1981;102:128–30.[Medline]
  18. Shub C, Tajik AJ, Seward JB, et al. Cardiac papillary fibroelastomas. Mayo Clin Proc 1981;56:629–33.[Medline]
  19. Abu Nassar SG, Parker JC. Incidental papillary endocardial tumor. Arch Pathol 1971;92:370–6.[Medline]
  20. Gorton ME, Soltanzadeh H. Mitral valve fibroelastoma. Ann Thorac Surg 1989;47:605–7.[Abstract]
  21. Gerbode F, Kerth WJ, Hill JD. Surgical management of tumors of the heart. Surgery 1967;61:94–101.[Medline]
  22. Maurer ER. Successful removal of tumor of the heart. J Thorac Surg 1952;23:479–85.
  23. Dein JR, Frist WH, Stinson EB, et al. Primary cardiac neoplasms. J Thorac Cardiovasc Surg 1987;93:502–11.[Abstract]
  24. Butterworth JS, Poindexter CA. Papilloma of cusp of the aortic valve. Circulation 1973;48:213–5.[Abstract/Free Full Text]
  25. Heath D, Best PV, Davis BT. Papilliferous tumours of the heart valves. Br Heart J 1961;23:20–4.
  26. McFadden PM, Lacy JR. Intracardiac papillary fibroelastoma: an occult cause of embolic neurologic deficit. Ann Thorac Surg 1987;43:667–9.[Abstract]
  27. Ortiz de Murua JA, Hernando E, Arenas C, Duarte J, Fernandez-Calella D. [Repeated cerebral embolism in a patient with papillary fibroelastoma of the mitral valve detected by two-dimensional echocardiography]. Rev Esp Cardiol 1990;43:587–9.[Medline]
  28. Topol EJ, Biern RO, Reitz BA. Cardiac papillary fibroelastoma and stroke. Echocardiographic diagnosis and guide to excision. Am J Med 1986;80:129–32.[Medline]
  29. Castaneda AR, Varco RL. Tumors of the heart: surgical considerations. Am J Cardiol 1968;21:357–62.[Medline]
  30. Heath D. Pathology of cardiac tumors. Am J Cardiol 1968;21:315–27.[Medline]
  31. Israel DH, Sherman W, Ambrose JA, Sharma S, Harpaz N, Robbins M. Dynamic coronary ostial obstruction due to papillary fibroelastoma leading to myocardial ischemia and infarction. Am J Cardiol 1991;67:104–5.[Medline]
  32. Rieckmann C, Liebau G, Hellberg K, Seeliger H. [Papillary fibroelastoma of aortic valve: primary echocardiographic diagnosis in an asymptomatic case]. Z Kardiol 1990;79: 208–10.[Medline]
  33. Flotte T, Pinar H, Feiner H. Papillary fibroelastoma of the left ventricular septum. Am J Surg Pathol 1980;4:585–8.[Medline]
  34. Melo J, Ahmad A, Chapman R, Wood J, Starr A. Primary tumors of the heart: a rewarding challenge. Am Surg 1979;45:681–3.[Medline]
  35. Lambl VA. Papilläre Exkreszenzen an der Semilunar-Klappe der Aorta. Wien Med Wochenschr 1856;6:244–7.



This article has been cited by other articles:


Home page
 Eur J EchocardiogrHome page
G. Truscelli, C. Torromeo, F. Miraldi, C. Vittori, P. F. Silenzi, A. Caso, P. Gallo, C. Gaudio, and L. Tritapepe
The role of intraoperative transoesophageal echocardiography in the diagnosis and management of a rare multiple fibroelastoma of aortic valve: a case report and review of literature
Eur J Echocardiogr, October 1, 2009; 10(7): 884 - 886.
[Abstract] [Full Text] [PDF]

Home page
 ANGIOLOGYHome page
D. Kumbala, T. Sharp, and M. Kamalesh
"Perilous Pearl"--Papillary Fibroelastoma of Aortic Valve: A Case Report and Literature Review
Angiology, October 1, 2008; 59(5): 625 - 628.
[Abstract] [PDF]

Home page
 J. Thorac. Cardiovasc. Surg.Home page
J.-M. Grinda, J. P. Couetil, S. Chauvaud, N. D'Attellis, A. Berrebi, J.-N. Fabiani, A. Deloche, and A. Carpentier
CARDIAC VALVE PAPILLARY FIBROELASTOMA: SURGICAL EXCISION FOR REVEALED OR POTENTIAL EMBOLIZATION
J. Thorac. Cardiovasc. Surg., January 1, 1999; 117(1): 106 - 110.
[Abstract] [Full Text] [PDF]

Home page
 Eur. J. Cardiothorac. Surg.Home page
D. Pacini, P. A. Farneti, O. Leone, and R. Galli
Cardiac papillary fibroelastoma of the mitral valve chordae
Eur. J. Cardiothorac. Surg., March 1, 1998; 13(3): 322 - 324.
[Abstract] [Full Text] [PDF]

Home page
 Ann. Thorac. Surg.Home page
K. Minatoya, H. Okabayashi, T. Yokota, and E. L. Hoover
Cardiac Papillary Fibroelastomas: Rationale for Excision
Ann. Thorac. Surg., November 1, 1996; 62(5): 1519 - 1521.
[Abstract] [Full Text]

Home page
 Ann. Thorac. Surg.Home page
A. T. Pezzella
Primary Cardiac Valve Tumors
Ann. Thorac. Surg., March 1, 1996; 61(3): 1041 - 1042.
[Full Text]

This Article
Right arrow Abstract Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to Personal Folders
Right arrow Download to citation manager
Right arrow Author home page(s):
Friedrich Stefan Eckstein
Hans-Joachim Schäfers
Right arrow Permission Requests
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Eckstein, F. S.
Right arrow Articles by Borst, H.-G.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Eckstein, F. S.
Right arrow Articles by Borst, H.-G.

HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
ANN THORAC SURG ASIAN CARDIOVASC THORAC ANN EUR J CARDIOTHORAC SURG
J THORAC CARDIOVASC SURG ICVTS ALL CTSNet JOURNALS