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Eur J Cardiothorac Surg 1999;15:103-107
© 1999 Elsevier Science NL

Case report

Mitral valve repair for anterior leaflet papillary fibroelastoma: two case descriptions and a literature review

^a Department of Thoracic and Cardiovascular Surgery, Luigi Sacco Hospital, Via G.B. Grassi, 74, 20157 Milan, Italy
^b Division of Cardiology, Luigi Sacco Hospital, Milan, Italy
^c Pathology Service, Luigi Sacco Hospital, Milan, Italy

Received 15 July 1998; received in revised form 26 October 1998; accepted 2 November 1998.

Corresponding author. Tel.: +39-02-3579-9333; Fax: +39-02-835-7513.

	Abstract

Top Abstract Introduction Case reports Discussion Conclusion References

 
Cardiac papillary fibroelastomas are rare cardiac tumors and have been considered a `benign' incidental finding that may have significant clinical manifestations. In this paper we report two cases of mitral valve fibroelastoma: one was discovered by chance with transthoracic echocardiography in a young healthy man, the other was an intraoperative incidental finding in a middle aged man with a recent history of acute myocardial infarction. The mitral valve was repaired in both cases after excising the tumor. The patients did well and remain asymptomatic. A literature review was compiled which comprises previous case reports of 34 patients with mitral valve papillary fibroelastomas. Most were asymptomatic, but when symptoms occurred, they could be disabling, such as stroke, cardiac heart failure, myocardial infarction, and sudden death. Papillary fibroelastoma is amenable to simple surgical excision or in addition to mitral valve repair or replacement. Recurrence has not been reported.

Key Words: Papillary fibroelastoma • Mitral valve repair

	Introduction

Top Abstract Introduction Case reports Discussion Conclusion References

 
A papillary fibroelastoma is a rare tumor of the heart. This histologically benign neoplasm up to the last decade had been identified primarily during autopsy or as an incidental finding during cardiac surgery. The advent of transthoracic and transesophageal echocardiography has greatly enhanced the ability to make this diagnosis in a timely fashion, and surgical treatment has been proposed to prevent catastrophic cerebral or coronary embolization. We report on two patients with this type of tumor who underwent mitral valve repair after excising the mass. To the best of our knowledge there have been just 34 cases reports in the English literature [1] [2] [3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] [19] [20] [21] [22] [23] [24] [25] [26] [27] [28] [29] of mitral valve papillary fibroelastoma who have undergone successful resection and/or mitral valve repair or replacement.

	Case reports

Top Abstract Introduction Case reports Discussion Conclusion References

 
Case 1
A 40-year-old healthy male underwent annual clinical examination, a routine check-up for sports activity. A grade II/VI pansystolic murmur was detected at the cardiac apex. As a result of this murmur further instrumental examination by echocardiography was requested. Two-dimensional echocardiography revealed a 10-mm sessile mass involving the anterior leaflet of the mitral valve. The data were confirmed with a transesophageal exam ( Fig. 1 ). The remainder of the cardiac structures and dimensions were normal. Laboratory studies, electrocardiogram and chest roentgenogram were unremarkable. Left atriotomy through a medial sternotomy and exploration of the mitral valve on cardiopulmonary bypass confirmed a solitary verrucous tumor (10x6x6 mm) on the ventricular surface of the anterior mitral leaflet involving also some chordae tendineae ( Fig. 2 A). The tumor was excised ( Fig. 3 ) with the chordae tendineae involved exiting in a 8 mm diameter lack of cuspidal tissue and chordae along the free edge of the anterior mitral leaflet. The anterior leaflet was repaired by means of a transposition of cuspidal tissue with its chordae from the posterior mitral leaflet to the anterior leaflet ( Fig. 2B). The surgical procedure was completed by a posterior emianuloplasty with a GoreTex ring. Intraoperative transesophageal echocardiography confirmed a competent valve repair.

View larger version (84K): [in this window] [in a new window]   Fig. 1. Case 1. Preoperative transesophageal echocardiogram. Mesoesophageal view. The tumor adherence to the tip of the anterior mitral leaflet is shown.

View larger version (37K): [in this window] [in a new window]   Fig. 2. Case 1. (A) Tumor position on the mitral valve leaflet. Dotted line shows the cuspidal tissue that will be utilized for the chordal transposition. (B) Tumor excision and chordal transposition from the posterior mitral leaflet to the anterior mitral leaflet.

View larger version (194K): [in this window] [in a new window]   Fig. 3. Case I. Gross anatomical appearance of the tumor.

Case 2
A 50-year-old man was hospitalized for sudden constrictive chest pain. Electrocardiography showed evidence of an acute infero-lateral myocardial infarction confirmed by a positive enzymatic curve.

After the acute phase of the myocardial infarction had been resolved without complications an echocardiogram was performed and severe mitral valve insufficiency was found due to an anterior leaflet prolapse.

Cardiac catheterization and angiography showed mildly depressed left ventricular contractility with normal coronary arteries except for a proximal angiographic clipping of the circumflex artery in the first marginal branch.

Two months after the onset of the myocardial infarction the patient underwent surgery to repair the anterior leaflet prolapse. On cardiopulmonary bypass a coronary artery bypass graft with a saphenous vein was first performed on the marginal branch. After left atriotomy, intraoperative exploration of the mitral valve showed a solitary unexpected tumor (12x8x5 mm) on the cuspid–chordal junction of the anterior mitral leaflet, in the central position. The tumor enclosed two marginal chordae thendinae. It was removed excising a limited free edge portion together with the chordae involved. The mitral anterior leaflet was repaired by means of a cuspid–chordal transposition from the posterior mitral leaflet. The surgical procedure was completed by a quadrangular resection of the involved posterior leaflet, the anulus plication, and a posterior emianuloplasty with a GoreTex ring.

Also in this case, histopathologic examination of the tissue revealed a benign papillary fibroelastoma, so we proposed an embolic marginal branch occlusion occurred during the previous myocardial infarction. After surgery the patient had an uneventful recovery and was discharged on dicumarolic on the 8th day.

A 1-month postoperative echocardiography control showed satisfactory valve function with no residual mitral regurgitation or stenosis.

	Discussion

Top Abstract Introduction Case reports Discussion Conclusion References

 
A papillary fibroelastoma is a rare benign tumor of the heart with a tendency towards valvular involvement. Although most patients with these lesions are adults more than 50 years of age, the overall age range goes from newborn babies to 92-year-old patients [30] [31]. These tumors comprise 4.4% of all heart benign tumors detected in a retrospective study of surgical pathology [32]. No data are available concerning the incidence in asymptomatic patients.

Primary cardiac mitral valve fibroelastomas are uncommon and often asymptomatic during life [33] [34].

Until the advent of echocardiography, which has altered the natural course of these lesions by means of earlier diagnosis, these lesions were rarely diagnosed ante mortem. The majority of patients with papillary fibroelastoma are asymptomatic and treatment of cardiac fibroelastoma is controversial because minimal data are available about therapeutic efficacy. However, surface thrombus is common with these tumors; Topol et al. theorize that the tumor is a nidus for platelet and fibrin aggregation [5], posing a significant potential for embolization [1] [35] [36]. Therefore we think these tumors should be promptly managed surgically even if they are asymptomatic and there are no major controindications to operation. In fact, previous cases offer abundant proof that papillary fibroelastomas cause cerebral [1] [4] [5] [6] [7] [8] [12] [13] [19] [20] [22] [23] [24] [25] [26] [27] [28] [29] and coronary [1] [11] [12] [16] emboli. In the present review, 19 patients had symptoms of cerebral embolization, five patients sustained myocardial infarction despite a normal angiocoronarography, four patients had congestive heart failure, only two were clinically asymptomatic. There were 20 males and 16 females with no sex-related incidence [30]. The patients' mean age was 43 years (36 patients, range 3–77 years), with 24 patients (66.7%) less than 50 years old, not confirming the literature reports [30] [31], pointing out patients with such lesions as adults over 50 years of age.

When a papillary fibroelastoma occurs, simple tumor excision is the gold standard. However, valve replacement or repair may be required when the tumor has invaded the valve or when there is combined degeneration of the valve. Twenty patients underwent simple tumor excision, seven patients needed mitral valve replacement, and in nine patients mitral valve repair was performed.

In our patients, after tumor excision because of tumor location and dimension, and because of chordal involvement, valvular incompetence was present. In order to avoid mitral valve replacement we ought to perform a transposition of cuspidal tissue and chordae from the posterior leaflet to the anterior mitral leaflet completed by a posterior emiannuloplasty with a GoreTex ring. Because no cases of local recurrence have been reported, whenever repair is allowed by the anatomical condition, that is the primary surgical goal for the patient's future best quality of life.

	Conclusion

Top Abstract Introduction Case reports Discussion Conclusion References

 
We have presented two cases of primary mitral valve papillary fibroelastoma and reviewed the English literature. These are benign neoplasms with the propensity for severe symptoms if systemic embolization or valve obstruction occurs. We noticed that in the 36 cases considered two-dimensional echocardiography detected lesions in all patients but three. However, sharper definition of the tumor was possible in six patients who were also studied with transesophageal echocardiography. After diagnosis, systemic anticoagulation should accompany the treatment of the tumor. Prompt excision, preserving the original valvular tissue and function when possible, is indicated for all symptomatic patients and in those who are asymptomatic as well, considering the high risk of embolization with such tumors.

	Acknowledgments

 
We would thank Miss Laura Grignani for her assistance in preparing the illustrations.

	References

Top Abstract Introduction Case reports Discussion Conclusion References

 

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