Eur J Cardiothorac Surg 2001;20:1249-1251
© 2001 Elsevier Science NL
Papillary fibroelastoma of a mitral valve chorda
Jean-Marc Marnettea,d,
Hassan Hassana,
Joêlle Sarotb,
Karolien Jacobsc
a Department of Cardiac Surgery, Namur General Hospital (Centre hospitalier régional de Namur), 185 avenue Albert 1°, 5000 Namur, Belgium
b Department of Cardiology, Namur General Hospital (Centre hospitalier régional de Namur), 185 avenue Albert 1°, 5000 Namur, Belgium
c Department of Neurology, Namur General Hospital (Centre hospitalier régional de Namur), 185 avenue Albert 1°, 5000 Namur, Belgium
d C.N.A.C. (Centre Namurois de Cardiologie et de Chirurgie Cardiaque), Namur, Belgium
Received 15 May 2001;
received in revised form 22 August 2001;
accepted 3 September 2001.
Corresponding author. Tel.: +32-8172-6943; fax: +32-8172-6913
e-mail: chicarnam{at}skynet.be
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Abstract
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A 43-year-old woman presented with an ischemic stroke in the right middle cerebral artery territory. Cardiac echography disclosed a tumor of a primary chordae of the anterior leaflet of the mitral valve. After neurologic recovery, the patient was referred to surgery for excision of the tumor and plastic reconstruction using a chordal transfer technique. Histological examination of the tumor showed a typical papillary fibroelastoma. Papillary fibroelastoma is the third most frequent cardiac benign tumor. The high embolic potential of this tumor is in favor of an aggressive surgical attitude.
Key Words: Ischemic stroke • Mitral valve chordae • Fibroelastoma • Mitral valve reconstructive surgery
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1. Introduction
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Cardiac papillary fibroelastoma (CPFE) represent less than 16% of all primary cardiac tumors, but is the third most common primary cardiac neoplasm after myxoma and lipoma in necropsy series [1] and the most common valvular tumor [2]. In 1996, Hicks et al. [3] reviewed four cases of isolated chordal CPFE. We report here a new case in this location.
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2. Case report
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A 43-year-old woman presented at our hospital with a sudden onset of right upper-limb weakness and aphasia on January 6, 2000. Her past medical history was unremarkable except for an episode of aphasia subsiding in a few minutes in August 1999. On physical examination, a left facial palsy was associated with a motor deficit of the left upper limb. No other anomalies were present and routine investigations (electrocardiography (EKG), chest X-ray and usual laboratory work) were negative.
Cerebral computerized tomography (CT) scan and magnetic resonance imaging put in evidence an ischemic lesion with mass effect in the territory of the right middle cerebral artery, suggesting an embolic process.
Carotid duplex ultrasound exploration was normal. Transthoracic echocardiography (TTE) disclosed a mobile mass on the mitral valve apparatus. Transesophageal echocardiography (TEE) showed the mass to originate from a mitral chordae attached to the free edge of the central portion of the anterior leaflet, and close to this attachment (Fig. 1
). The mass was estimated to be 5 mm wide. A small patent foramen ovale was also present.
Doppler ultrasonography examination of the lower limb veins, 24 h EKG and blood pressure recordings, coagulation screening in search of hypercoagubility disorders, right heart catheterization and coronary arteriography were performed and showed no abnormalities.
Diagnostic conclusion was embolic cerebral stroke related to a tumor of the chordal mitral valve apparatus, probably a papillary fibroelastoma.
Surgical excision of the tumor was programed, but belated until full complete neurologic recovery. On March 3, 2001, the mitral valve was approached through a biatrial transeptal incision. A 6 mm diameter polylobular translucent mass was attached to a main paramedial chordae of the anterior leaflet, 2 mm far from the edge of this leaflet (Fig. 2
). This mass and the adjacent chordae were excised. A secondary chordae was mobilized and reimplanted on the free edge of the leaflet. The minimal atrial septal defect was closed by suturing the biatrial incision. Immediate and post-operative TEE showed a normally functioning mitral valve. The post-operative course was uneventful. Histopathologic examination of the mass confirmed the diagnosis of papillary fibroelastoma.
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Fig. 2. Operative view of the mitral valve – tumor is visible as a polylobular mass attached to a main medial chorda, 2 mm far from the edge of the anterior leaflet.
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One year later, the patient remains asymptomatic without any medication.
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3. Discussion
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CPFE derive from normal components of the endocardium and resemble a sea anemone with multiple papillary fronds. Histologically they present a central core of dense connective tissue. Surrounding this central core is a layer of hyperplastic endocardial cells, loose connective tissue and a mesh of elastic fibers. The tumor rarely exceeds 1 cm in diameter [2].
Grinda et al. [4] in a review of 198 cases of CPFE found the location of CPFE to be aortic in 29%, mitral in 25%, tricuspid in 17%, pulmonic in 13% and non-valvular in 16%. In the same year, Di Mattia et al. [5] collected only 34 observations of surgical resection of a mitral papillary fibroelastoma. Chordal CPFE as an isolated location appears more rarely in the literature [6–8].
The real incidence of CPFE remains controversial as evolution can be asymptomatic for years [8]. However, all patients are at risk of embolic neurological strokes [9]. Embolization may occur from the fragile papillary fronds [3] or from a platelet and fibrin aggregation forming on the tumor [10]. Chordal CPFE as other mitral valve tumors are potentially life-threatening tumors, likely to produce serious neurological symptoms or sudden death [2].
Transthoracic and specially transesophageal echography is the most sensitive and reliable investigation to establish the diagnosis, exclude multiple locations and plan the optimal surgical approach [4]. In the reported case, echography disclosed another potential cause of emboli, a patent foramen ovale; so the embolic responsibility of CPFE could not be fully established.
Even small CPFEs (3 mm) can be responsible for strokes [4]. The effectiveness of preventive systemic anticoagulation remains controversial [4,5,11]. Even if the patients are asymptomatic and if there are no major contraindications to operation, they should be surgically managed. In case of post-embolic brain damage, a delay of 4–6 weeks is generally recommended between the embolic episode and intracardiac surgery [12].
Conservative management of mitral lesions is generally possible [4,5]. Chordal lesions can be repaired by plication, chordal transfer or use of artificial chordae. Recurrence has not been reported as long as the tumor has been completely resected [6].
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Acknowledgments
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We are grateful to Dr Y. Couvreur for the histopathologic examination and to Dr Yvan Pamjanov (Indianapolis medical centre) for reviewing it, and to Dr Alain Brunet (CHU St Pierre-Bruxelles) for reviewing the manuscript.
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References
|
|---|
-
McAllister H.A., Jr, Fengolio J.J., Jr Tumors of the cardiovascular system. Atlas of Tumor Pathology. Washington, DC: Armed Forces Institute of Pathology, 1978 p. 20–5.
-
Edwards F.H., Hale D., Cohen A., Thompson L., Pezzela A.T., Virmani R. Primary cardiac valve tumors. Ann Thorac Surg 1991;52(5):1127-1131.[Abstract]
-
Hicks K.A., Kovach J.A., Frishberg D.P., Wiley T.M., Gurczak P.B., Vernalis M.N. Echocardiographic evaluation of papillary fibroelastoma: a case report and review of the literature. J Am Soc Echocardiogr 1996;9(3):353-360.[Medline]
-
Grinda J.M., Couetil J.P., Chauvaud S., D'Attelis N., Berrebi A., Fabiani J.N., Deloche A., Carpentier A. Cardiac valve papillary fibroelastoma: surgical excision for revealed or potential embolization. J Thorac Cardiovasc Surg 1999;117(1):106-110.[Abstract/Free Full Text]
-
Di Mattia D.G., Assaghi A., Mangini A., Ravagnan S., Bonetto S., Fundaro P. Mitral valve repair for anterior leaflet papillary fibroelastoma: two cases descriptions and a literature review. Eur J Cardiothorac Surg 1999;15:103-107.[Abstract/Free Full Text]
-
Gallo R., Kumar N., Prabhakar G., Awada A., Maalouf Y., Duran C.M.G. Papillary fibroelastoma of mitral valve chorda. Ann Thorac Surg 1993;55:1576-1577.[Medline]
-
Pacini D., Farneti Pa., Leone O., Galli R. Cardiac papillary fibroelastoma of the mitral valve chordae. Eur J Cardiothorac Surg 1998;13:322-324.[Abstract/Free Full Text]
-
Kücükoglu S., Arat A., Mutlu H., Okcün B., Bakay C., Oz B., Uner S. A cardiac papillary fibroelastoma with chordal location. J Am Soc Echocardiogr 1999;92:1023-1028.
-
Kasarkis E.J., O'Connor W., Earle G. Embolic stroke from cardiac papillary fibroelastoma. Stroke 1988;19:1171-1173.[Abstract/Free Full Text]
-
Fowles R.E., Miller D.C., Egbert B.M., Fitzgerald J.W., Popp R.L. Systemic embolization from a mitral valve papillary endocardial fibroma detected by two-dimensional echocardiography. Am Heart J 1981;102:128-130.[Medline]
-
Loire R., Pinède L., Donsbeck A.V., Nighoghossian N., Perinetti N. Fibroélastomes papillaires cardiaques (excroissances de Lambl géantes) Etude anatomo-clinique de 10 cas opérés. Presse Med 1998;27(16):753-757.
-
Eishi K., Kawazoe K., Kuriyama Y., Kitoh Y., Kawashima Y., Omae T., for the 174 centers of the Japanese Association of Thoracic Surgery. Surgical management of infective endocarditis associated with cerebral complications. Multi-center retrospective study in Japan. J Thorac Cardiovasc Surg 1995;110(6):1745-1755.[Abstract/Free Full Text]
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Abstract
1. Introduction
