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Asian Cardiovasc Thorac Ann 2001;9:71-72
© 2001 Asia Publishing EXchange Pte Ltd


CASE STUDY

Transaortic Excision of Left Ventricular Papillary Fibroelastoma

Suhas C Bendre, MD, Alec Baker, PA-C, Todd M Grehl, MD

Division of Cardiovascular Surgery Good Samaritan Hospital Corvallis, Oregon, USA
For reprint information contact: Todd M Grehl, MD Tel: 1 541 766 3662 Fax: 1 541 757 5014 email: tmg{at}goodsam.com Division of Cardiovascular Surgery, Good Samaritan Hospital, 3600 N.W. Samaritan Drive, Suite 3116, Corvallis, OR 97330, USA.

    Abstract
 TOP
 Abstract
 Introduction
 Case Report
 Discussion
 References
 
Transthoracic echocardiography in a 75-year-old woman with fatigue, dizziness, and left-sided weakness, demonstrated a 3.5 x 3-cm mass in the left ventricle, near the apex. The mass was excised via the aorta. Histology indicated a papillary fibroelastoma. Such tumors rarely arise from the ventricular endocardium.


    Introduction
 TOP
 Abstract
 Introduction
 Case Report
 Discussion
 References
 
Papillary fibroelastoma is a rare benign neoplasm with a frequency of 0.002% to 0.33%, comprising 7.9% of benign primary cardiac tumors.1,2 It is the third most frequent benign tumor of the heart, after myxoma and lipoma. It typically affects the cardiac valves, mainly the aortic and mitral valves, and very rarely the endocardium. It may present with serious complications such as a cerebro-vascular event, myocardial ischemia or infarction, or pulmonary embolism.


    Case Report
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 Abstract
 Introduction
 Case Report
 Discussion
 References
 
A 75-year-old woman was admitted with increasing fatigue, dizziness, and left-sided weakness associated with difficulty in walking. She had no history of neurologic events or cardiac disease. Neurologic evaluation by carotid Doppler ultrasound revealed minimal bilateral intimal thickening of the common carotid arteries. A computed tomography scan of the head showed no evidence of infarction or a space-occupying lesion. Transthoracic echocardiography demonstrated a 3.5 x 3-cm mobile but broad-based mass firmly attached to the anterior apical segment and septal portion of the left ventricle near the apex (Figure 1Go). The mass was not associated with the papillary muscles or chordae of the mitral valve. The mitral valve appeared anatomically and functionally normal. There was no other cardiac valvular or cavitary pathology on echocardiography. Ventricular function was normal. Although the neurologic symptoms cleared quickly, they were assumed to be due to embolic material emanating from the left ventricular mass, thus the patient was referred for surgical intervention. She underwent coronary angiography before surgery, which revealed normal coronary arteries. Excision of the mass was carried out under cardiopulmonary bypass using standard techniques. The ascending aorta was opened in an oblique fashion with the incision carried down into the non-coronary sinus. The aortic valve leaflets were retracted carefully and the mass was excised by sharp dissection. Further inspection of the left ventricular chamber after resection of the mass revealed the papillary muscles and chordae to be intact. The patient was weaned from bypass without difficulty. Postoperative recovery was uneventful. Histology confirmed the mass was a papillary fibroelastoma.



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Figure 1. Two-dimensional echocardiogram showing the left ventricular tumor.

 

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 Abstract
 Introduction
 Case Report
 Discussion
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These tumors are never present at birth.3 The etiology remains elusive but their incidence increases with age. The youngest patient reported was a 3.5-year-old boy who presented with a stroke.4 Most of these tumors remain silent and are detected incidentally at surgery or autopsy. At least 10 cases of left ventricular fibroelastoma treated by surgical excision have been reported in the literature.5 Cardiac papillary fibroelastoma can present as solitary or multiple intracavitary tumors.6,7 Although benign, it may cause devastating complications such as coronary or cerebral emboli. Embolization may occur from the fragile papillary fronds of the tumor itself or from a thrombus forming on the tumor. The exact cause of the neurologic symptoms in our patient was difficult to ascertain. Histological examination of the excised tumor showed elastic fibers, fibrous tissue, and collagen fibers with an outer layer consisting of regular endothelial cells.

Various methods have been proposed for complete excision of a left ventricular tumor. It is most important to avoid a left ventriculotomy with its subsequent complications such as bleeding and myocardial fibrosis. This leaves only 2 modes of access to a left ventricular tumor: transaortic and trans-left atrial. Papillary fibroelastoma commonly arises from the valvular endocardium. When associated with the mitral valve, it is best approached via a left atriotomy. When associated with the aortic valve and left ventricular outflow tract, it is best approached via the transaortic route. When the tumor is close to the apex deep within the left ventricular cavity, different techniques have been described, particularly the use of a videoscope passing through the aortic or mitral valve to avoid damage to the valvular apparatus and to achieve complete surgical excision. Along with this technique, transthoracic and intraoperative transesophageal echocardiography can help in making surgical decisions. In our patient, there was an isolated tumor of reasonable size, which we were able to visualize completely and thus could excise through the aorta.

Even though it is histologically benign and clinically asymptomatic, cardiac papillary fibroelastoma should be excised because of the potential embolic complications. The diagnosis of this tumor should be considered in patients with unexplained cardiovascular or cerebro-vascular ischemia.


    Acknowledgments
 
We are grateful to Cindy Fessler for her help in the preparation of the manuscript.


    References
 TOP
 Abstract
 Introduction
 Case Report
 Discussion
 References
 

  1. Bailbe M, Coisne D, Babin P, Corbi P, Menu P, Rosier MP, et al. Papillary fibroelastoma. A rare etiology of strokes in young patients [French]. Rev Med Interne 1998;19: 119–22.[Medline]

  2. Hicks KA, Kovachi JA, Frishberg DP, Wiley TM, Gurczak PB, Vernalis MN. Echocardiographic evaluation of papillary fibroelastoma: a case report and review of literature. J Am Soc Echocardiogr 1996;9:353–60.[Medline]

  3. Roberts WC. Papillary fibroelastoma of the heart. Am J Cardiol 1997;80:973–5.[Medline]

  4. Demenezes IC, Fragota J, Martins FM. Papillary fibroelastoma of the mitral valve in a 3-year-old child: a case report. Pediatr Cardiol 1996;17:194–5.[Medline]

  5. Howard RA, Aldea GS, Shapira OM, Kasznica JM, Davidoff R. Papillary fibroelastoma: increasing recognition of a surgical disease. Ann Thorac Surg 1999;68:1881–5.[Abstract/Free Full Text]

  6. Kalman JM, Lubicz S, Brennan JB, Vernon Roberts SE, Calafiore P. Multiple papillary fibroelastomas. Aus NZ J Med 1991;21:744–6.[Medline]

  7. de Virgilio C, Dubrow TJ, Robertson JM, Siegel S, Ginzton L, Nussmeier M, et al. Detection of multiple cardiac papillary fibroelastomas using transesophageal echocardiography. Ann Thorac Surg 1989;48:119–21.[Abstract]





This Article
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