J Thorac Cardiovasc Surg 2006;132:1465-1467
© 2006 The American Association for Thoracic Surgery
A case of mitral valve plasty for Libman–Sacks endocarditis mimicking a cardiac tumor
Shingo Taguchi, MD,
Kazuhiro Hashimoto, MD*,
Yoshimasa Sakamoto, MD,
Hiroshi Okuyama, MD,
Shinichi Ishii, MD
Department of Cardiovascular Surgery, Jikei University School of Medicine, Tokyo, Japan.
Received for publication May 27, 2006; revisions received June 27, 2006; accepted for publication July 12, 2006.
* Address for reprints: Kazuhiro Hashimoto, MD, Department of Cardiovascular Surgery, Jikei University School of Medicine, 3-25-8, Nishishinbashi, Minato-ku, Tokyo, Japan, 105-8461. (Email: kaz-hashi{at}jikei.ac.jp).
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Drs Sakamoto, Hashimoto, and Taguchi (left to right).
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We report a patient with multiple cerebral infarcts in whom 2-dimensional echocardiography showed a large mass attached to the anterior mitral leaflet and chordae. An intracardiac tumor was diagnosed, and surgical intervention was performed. The resected mass proved to be a Libman–Sacks vegetation on microscopy. This is frequently found in patients with systemic lupus erythematosus (SLE). Unique echocardiographic and intracardiac findings are reported here with the technique of mitral valve plasty.
Clinical Summary
A 34-year-old woman was admitted after the sudden onset of aphasia associated with mild hemiplegia of her left arm and leg. She had been receiving treatment for SLE for 9 years, but the disease had been inactive, and she had been well, except for numbness in her left hand, at 7 months before this episode. Her medications were an H2 blocker and an oral steroid (5 mg/d prednisolone). Admission laboratory tests revealed normal serum chemistry, normal C-reactive protein (CRP) levels, no anemia, and mild thrombocytopenia (the platelet count was 55 x 103/µL). Antinuclear antibody was positive (titer 1:80), but other autoantibodies were normal. Magnetic resonance imaging revealed multiple focal infarcts in the left cerebral hemisphere, pons, and P4 area corresponding to the territory of the right posterior communicating artery. Two-dimensional transthoracic echocardiography revealed a mobile mass like bunches of grapes (1.5 cm in diameter) attached to the A3 area of the anterior mitral leaflet and the chordae of the mitral valve adjacent to the anterior papillary muscle (Figure 1, A). The mitral valve demonstrated normal movement, with no evidence of stenosis or chordal rupture, although trivial regurgitation was detected at the center of the orifice. On the basis of the size and mobility of the tumor, its potential for further systemic embolization was considered to be high, and the patient underwent surgical intervention. At the time of the operation, all intracardiac manipulations were performed through an aortotomy. The mass was first observed by means of endoscopy (Figure 2) and was found to be multifocal in origin, arising from the anterior mitral leaflet, the chordal apparatus, and the septum of the left ventricular outflow tract. The mass was excised as completely as possible, and a few chordae were also removed with it. Intraoperative pathology revealed the typical appearance of a Libman–Sacks vegetation, and it was distinguished from other lesions such as bacterial vegetations, myxoma, or papillary fibroelastoma. The surface of the anterior mitral leaflet that had been attached to the mass was rubbed thoroughly with a sponge, and the involved chordae were resected further. Then the anterior leaflet was supported with 4 artificial polytetrafluoroethylene chordae,*
which were placed with the loop technique.1
The patient’s postoperative course was good (Figure 1, B), and she was discharged 14 days after the operation. The antinuclear antibody titer has been well controlled by means of steroid therapy, and CRP levels remain negative.
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Figure 1. Parasternal long-axis view of echocardiography. A, Vegetation attached to the anterior mitral leaflet and chorda. B, The mitral valve is competent without mitral regurgitation after the operation. Ao, Aorta; LA, left atrium; LV, left ventricle.
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Discussion
Intracardiac masses attached to the mitral valve and chordae are most frequently seen in infective endocarditis. When other features of infective endocarditis, such as fever, an increased white blood cell count, or increase of CRP levels, are not observed at present or in the past, a clinical diagnosis of cardiac tumor is usually made. Tumors arising from valvular tissue are usually considered to be myxomas or papillary fibroelastomas because of their frequency and site of origin. In 20 years’ experience of papillary fibroelastomas at the Mayo Clinic, there were 10 lesions arising from the anterior mitral leaflet, papillary muscles, or both in a series of 88 cases.2 In patients with SLE, however, Libman–Sacks endocarditis might be strongly suspected, although the frequency of detection by means of echocardiography is lower than the frequency observed at autopsy. Galve and colleagues3 reported 7 cases of vegetations detected by means of echocardiography and compatible with Libman–Sacks endocarditis in a series of 74 patients.
Libman–Sacks endocarditis occurs on the aortic and mitral valves or submitral apparatus in most cases, and the affected chordae sometimes undergo rupture.4,5 Valve replacement has been the technique of first choice because resection of the mass and valve tissue en bloc will definitely prevent recurrence. In fact, all reports of surgical intervention for Libman–Sacks endocarditis involved valve replacement, as far as we could determine by review.5 Libman–Sacks endocarditis is not a neoplasm but represents degeneration caused by fibrinoid or inflammatory changes, and therefore appropriate steroid therapy to control SLE is quite important to prevent its recurrence. In other words, mitral valve plasty (particularly in patients without mitral regurgitation) is thought to be an appropriate option if good control of SLE is possible. At 6 months after the operation, our patient showed no evidence of recurrence on echocardiography.
Footnotes
* Gore-Tex chordae, registered trademark of W. L. Gore & Associates, Inc, Newark, Del. 
References
- von Oppell UO, Mohr FW. Chordal replacement for both minimally invasive and conventional mitral valve surgery using premeasured Gore-tex loops. Ann Thorac Surg 2000;70:2166-2168.[Abstract/Free Full Text]
- Ngaage DL, Mullany CJ, Daly RC, Dearani JA, Edwards WD, Orzulak TA, et al. Surgical treatment of cardiac papillary fibroelastoma: a single center experience with eighty-eight patients. Ann Thorac Surg 2005;80:1712-1718.[Abstract/Free Full Text]
- Galve E, Candell-Riera J, Pigrau C, Permanyer-Miralda G, Garcia-Del-Castillo H, Soler-Soler J. Prevalence, morphologic types and evolution of cardiac valvular disease in systemic lupus erythematosus. N Engl J Med 1988;319:817-823.[Medline]
- Roldan CA. Valvular disease associated with systemic illness. Cardiol Clin 1998;16:531-550.[Medline]
- Sasahashi N, Aono N, Kuji T, Shinoka T, Sueshiro M, Tomino T. A case of mitral replacement for Libman–Sacks endocarditis. Nipponn Kyobu Geka Gakkai Zasshi 1992;40:155-160.
