J Thorac Cardiovasc Surg 2006;132:1472-1473
© 2006 The American Association for Thoracic Surgery
An unusual case of papillary fibroelastoma "invading" the mitral valve
Georges Fayad, MDa,*,
Thomas Modine, MDa,
Thierry Le Tourneau, MD, PhDb,
Richard Azzaoui, MDa,
Christophe Decoene, MDa,
Marie Christine Copin, MD, PhDc,
Christophe Bauters, MD, PhDa,
Henri Warembourg, MDa
a Service de Chirurgie Cardiovasculaire, Hôpital Cardiologique, CHRU de Lille, France
b Service de Cardiologie C, Hôpital Cardiologique, CHRU de Lille, France
c Service d’anatomopathologie, CHRU de Lille, France.
Received for publication May 3, 2006; accepted for publication June 20, 2006.
* Address for reprints: Georges Fayad, MD, Hôpital Cardiologique, CHRU, Boulevard du Pr, J. Leclercq, 59037 Lille Cedex, France. (Email: g-fayad{at}chru-lille.fr).
We report
the case of a 28-year-old patient who was admitted to an emergency department for left-sided motor deficit. The medical history showed multiple transient ischemic attacks over the past 2 years. Preoperative cerebral magnetic resonance imaging showed right sylvian infarct. Transthoracic echocardiography revealed a round, highly mobile, pedunculated 6- by 5-mm mass attached to the anterior mitral leaflet near the posterior commissure. Transesophageal echocardiogram showed similar images (Figures 1 and 2,
arrows). This mass did not alter the mitral valve function. The ejection fraction was normal and no associated valve disease was found.
View larger version (124K):
[in this window]
[in a new window]
|
Figure 1. The broken line on the anterior leaflet marks the border between clear (normal) and rough (infiltrated) zones. The mitral valve showed infiltration of the anterior leaflet by multiple PFEs mainly on the free edge (discontinued arrows). A1, A2, and A3, Anterior mitral valve segments.
|
|
View larger version (120K):
[in this window]
[in a new window]
|
Figure 2. The biggest part of the mass (thick arrow) looked like an "active volcano," and the small lesions had a "lava flow" shape (thin arrows and between brackets). A1, A2, and A3, Anterior mitral valve segments.
|
|
Clinical Summary
The patient was referred for cardiac surgery to achieve ablation of this clinically symptomatic mass. He was operated on successfully with moderate hypothermic cardiopulmonary bypass (33°C). We used a left atriotomy access to the mitral valve. Tumor was ablated and the mitral valve preserved. The gross appearance of the analyzed tumors showed a papillary fibroelastoma (PFE) that had a characteristic frondlike appearance. The postoperative period was uneventful and the patient was discharged at the 11th day with platelet antiaggregant therapy and regular echocardiographic follow-up. Histopathologic examination diagnosed PFE. Indeed, the histopathologic description of all the samples confirmed the diagnosis of PFE by showing the presence of specific fronds connected to a common pedicle. These latter structures were found to contain three dissociated levels: (1) a superficial endothelial layer surrounding the tumor, (2) an intermediate edematous and myxomatous layer, and (3) a central core with a concentric avascular fibrosis and mesenchymal cells.
Discussion
Multiple PFEs are extremely uncommon.1,2 Indeed, they are usually single, small, and may be pedunculated. In our case, the mitral valve had an infiltrated anterior leaflet mainly on the free edge (Figure 1, discontinued arrow). The invasive characteristic of this unusual observation may be explained by the anatomic characteristic of the anterior leaflet. Indeed, it is divided into two zones, a rough zone and a clear zone. The rough zone is the thickest part; it is irregular, sometimes nodular, and corresponds to the area where first- and second-order cords are attached to the underside of this area. In our case the rough zone was totally covered by the lesions. Operatively, the biggest part of the mass looked like an "active volcano," and the small lesions had a "lava flow" shape (Figure 2). Given this unusual and unexpected diagnosis and the young age of the patient, we opted for exclusive tumor ablation without mitral valve replacement. Although the infiltration of valvular tissue by the PFE is impossible to prove inasmuch as the mitral valve was not extracted, the natural history of this tumor is unknown and preserving the native mitral valve could have been the optimal option. To the best of our knowledge this is the first case of invading PFE on the anterior leaflet of the mitral valve reported in the literature. Clinicians should be aware of this PFE presentation to decide how to manage such patients surgically.
| See related editorial on page 1256.
|
References
- Grandmougin D, Fayad G, Moukassa D, Decoene C, Abolmaali K, Bodart JC, et al. Cardiac valve papillary fibroelastomas: clinical, histological and immunohistochemical studies and a physiopathogenic hypothesis. J Heart Valve Dis 2000;9:832-841.[Medline]
- 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;103:2687-2693.
Related Article
-
"Invading papillary fibroelastoma"
- Robert T. Ownbey
J. Thorac. Cardiovasc. Surg. 2006 132: 1256.
[Extract]
[Full Text]
[PDF]
