Asian Cardiovasc Thorac Ann 2006;14:e58-e59
© 2006 Asia Publishing EXchange Ltd
Mitral Valve Papillary Fibroelastoma: Surgical Considerations
Gian M Novaro, MD,
Rafael E Cabrales, MD2,
W Douglas Boyd, MD1
Department of Cardiology
1 Department of Cardiothoracic Surgery
2 Department of Anesthesia, Cleveland Clinic Florida, Weston, USA
For reprint information contact: Gian M Novaro, MD Tel: 1 954 659 5313 Fax: 1 954 659 5292 Email: novarog{at}ccf.org, Department of Cardiology, Desk A23, Cleveland Clinic Florida, 2950 Cleveland Clinic Boulevard, Weston, FL33331, USA
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ABSTRACT
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Cardiac papillary fibroelastomas are uncommon primary cardiac tumors. Due to their infrequent occurrence, an appropriate management strategy has been difficult to define. We report a 51-year-old female with a papillary fibroelastoma of the mitral valve diagnosed during a source of embolism evaluation, where the association between symptoms and the tumor were equivocal. The mitral valve papillary fibroelastoma was electively surgically excised, based on the proposed guidelines that allow for identification of patients at greatest risk of embolic events. We review the evaluation and selection of those patients who might benefit from surgical therapy even in the absence of symptoms.
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INTRODUCTION
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Cardiac papillary fibroelastomas (CPF) are rare primary benign cardiac tumors.1–5 They may be incidentally noted or discovered during an evaluation of source of embolism.2 Due to their infrequent occurrence, an appropriate management strategy has been difficult to define. From the available literature, mainly consisting of case series, a reasonable approach to their management has been proposed to help select those who might benefit from surgical therapy.1,5
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CASE REPORT
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A 51-year-old female with hypercholesterolemia and tobacco use presented with new-onset transient symptoms of ataxia and blurred vision. Physical examination revealed an afebrile, non-toxic appearing female with a normal cardiovascular and neurological exam. Head and neck magnetic resonance angiography revealed an isolated severe stenosis of the mid-brachiocephalic artery. No additional abnormalities were noted. Transesophageal echocardiography was performed to exclude source of embolism and intracardiac shunt, and showed a small, round, homogeneous echodensity on the anterior mitral leaflet (Figure 1
). The echodensity measured 0.8 x 1.0 cm and appeared mobile with a probable stalk. Left ventricular function was normal, and there was no significant mitral regurgitation, shunts, or co-existing valvular or intracardiac lesions. Blood cultures, sedimentation rate, and testing for thrombophilia disorders, antiphospholipid antibodies and lupus erythematosus were negative. Based on its appearance, the working diagnosis of a CPF was made. With her presenting symptomatology, the decision was made to address her peripheral arterial disease.
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Figure 1. Transesophageal echocardiogram in a long-axis view, showing a round homogeneous echodensity on the mid portion of the anterior mitral leaflet (arrow), most consistent with a papillary fibroelastoma
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The patient underwent successful stent angioplasty of the brachiocephalic obstructive lesion without complications. During her follow-up, attention was then directed to the appropriate management of the CPF, including whether or not her symptoms might be attributable to a cardioembolic event. Given the patient’s age, low-risk surgical profile, and size and mobility of the CPF, it was recommended she undergo elective surgical resection. Via a mini-median sternotomy, she underwent successful excision of the mitral leaflet mass. Intraoperatively the anterior mitral leaflet mass was identified (Figure 2); no additional masses or valvular abnormalities were noted. Grossly the mass was round with a small stalk, and described as having a characteristic frond-like appearance. Pathologic examination confirmed the diagnosis of a CPF. During follow-up, now 2 years after surgery, the patient has had no further symptoms or evidence of tumor recurrence.
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Figure 2. Intraoperative gross appearance of the mitral valve papillary fibroelastoma. The tumor is attached to the leaflet by a small stalk (not seen). The characteristic sea anemone appearance can be appreciated.
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DISCUSSION
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Cardiac papillary fibroelastomas are rare, but are one of the more common benign cardiac tumors. The largest published series reports on 162 pathologically confirmed CPFs.1 As in our patient, they most commonly are located on valvular endocardium, favoring the aortic followed by the mitral valve. When CPFs are on the mitral valve, they are most often on the atrial side at the leaflet’s midportion, and not near the free edge.3 The tumors are small, ranging in size from 2 to 28 mm (median 8 mm), with 99% being < 20 mm.1 Nearly half of all CPFs are mobile with a stalk, and in about 9% of cases can be multiple.1
Histologically, they are composed of an inner connective tissue matrix enveloped in acid mucopolysaccharide and an outer endothelium. Echocardiography is the most common imaging modality used in the diagnosis of CPF. Although the typical characteristics have been described, a definitive echocardiographic diagnosis may be challenging with a differential diagnosis that includes vegetations, thrombi, degenerative valve lesions, and other tumors. When possible, transesophageal echocardiography should be used in patients with a suspected CPF to accurately define its size, attachments, number, and presence of a stalk.1
Surgical considerations specific to CPF include defining the risk of embolization in order to consider elective resection, and the surgical approach.1,2,4,5 Significant valve dysfunction is rare but the major associated risk with CPF is cardioembolic events.1,2 Features favoring a higher embolic risk include large tumor size (
1 cm) and mobility, particularly in the presence of a stalk.1 Therefore, elective surgical resection should be considered in the younger patient with left-sided lesions who are low-risk surgical candidates, and who present with CPFs with high-risk features: even if the patient is asymptomatic. The younger the patient, the greater the cumulative lifetime risk of embolization. Right-sided CPFs should be resected only if large and obstructive, or hemodynamically significant. Regarding surgical excision of CPFs, a minimally invasive approach can often be used, typically with no significant valvular damage. In the largest series of CPFs, there were no tumor recurrences after surgical resection at a mean follow-up of 630 days.1
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CONCLUSION
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This case demonstrates a potential clinical scenario encountered in the evaluation and diagnosis of a presumed CPF. The association of CPF with symptoms, and the presence of high-risk tumor features form the basis for surgical considerations in a newly diagnosed CPF. Although CPFs are a surgically treatable cause of cardioembolism2 and urgent resection after diagnosis has been proposed,5 implementation of the proposed guidelines1 may help determine the low- versus high-risk CPF asymptomatic patient.
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REFERENCES
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- Sun JP, Asher CR, Yang XS, Cheng GG, Scalia GM, Massed AG, et al. Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients. Circulation 2001;103:2687–93.[Abstract/Free Full Text]
- 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]
- Edwards FH, Hale D, Cohen A, Thompson L, Pezzella AT, Virmani R. Primary cardiac valve tumors. Ann Thorac Surg 1991;52:1127–31.[Abstract]
- Koolbergen DR, Voigt P, Kolowca M, Smit VT, Somer ST, Dion RA. Elective surgery for fibroelastoma of the aortic valve. Ann Thorac Surg 2004;77:725.[Free Full Text]
- Bossert T, Gummert JF, Mohr F. Papillary fibroelastomas and other cardiac tumors should be resected on an urgent basis. Ann Thorac Surg 2005;79:756.[Free Full Text]
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ABSTRACT
INTRODUCTION
