© 2014, Wiley Periodicals, Inc. DOI: 10.1111/echo.12558
Echocardiography
CASE REPORTS Section Editor: Brian D. Hoit, M.D.
Isolated Tricuspid Valve Repair for Libman-Sacks Endocarditis Ali Kemal Gur, M.D., Dolunay Odabasi, M.D., Ayse Gul Kunt, M.D., and Alper Sami Kunt, M.D. Department of Cardiovascular Surgery, School of Medicine, Yuzuncu Yil University, Van, Turkey
Cardiac involvement is a well-known complication of systemic lupus erythematosus (SLE), which can involve most cardiac components, including pericardium, conduction system, myocardium, heart valves, and coronary arteries. Libman-Sacks (verrucous) endocarditis is the characteristic cardiac valvular manifestation. Although isolated tricuspid valve involvement is quite rare, we report a patient with SLE who had tricuspid stenosis caused by Libman-Sacks endocarditis. The patient underwent successful commisurotomy and Kay annuloplasty on the tricuspid valve under cardiopulmonary bypass. (Echocardiography 2014;31:E166–E168) Key words: inflammation, systemic, tricuspid valve
Case Report: Systemic lupus erythematosus (SLE) is a multisystem illness including cardiac involvement. Cardiac involvement involves most cardiac components, including the pericardium, conduction system, myocardium, heart valves, and coronaries.1 Libman-Sacks (verrucous) endocarditis is the characteristic cardiac valvular manifestation. These atypical, sterile, verrucous vegetations were first published by Libman and Sacks in 1924.2 Some patients in SLE need surgical intervention, which carries a high morbidity and mortality due to multisystem involvement. Although the most common valves involved in SLE are the mitral and aortic valves, isolated tricuspid valve involvement can be seen a quite rare manifestation. A 20-year-old woman had recently suffered from sweating, weakness, fatigue, malar rash, and photosensitivity. Results of investigations, including computed tomography and diffusion magnetic resonance imaging of the head, were reported as normal. Right kidney was detected as atrophic in abdominal ultrasonography (Grade III/IV renal disease); she had also renal proteinuria (12 g/day). Renal biopsy confirmed lupus nephritis on this basis and the presence of symptoms and the laboratory results. She was evaluated by a rheumatologist, who confirmed the diagnosis of SLE. The patient remained afebrile, and all culture results remained negative after 72 hours. Address for Correspondence and reprint requests: Ayse Gul Kunt, Department of Cardiovascular Surgery, School of Medicine, Yuzuncu Yıl University, Zeve Campus, 65080 Van, Turkey. Fax: +90 (432) 216 75 19; E-mail: [email protected]
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Meanwhile, she was evaluated for connective tissue disorders and was found to have elevated antinuclear antibody, double-stranded deoxyribonucleic acid, and anticardiolipin antibody. Laboratory results were as follows: C-reactive protein level was 3.3 mg/L, sedimentation rate was 34 mm/h, platelet count was 29 9 103/lL. She was in active phase of the SLE and Mesnex 800 mg/day plus methylprednisolone 48 mg/ day were started as initial medical therapy. An incidental mid-diastolic murmur was detected, and transthoracic echocardiography demonstrated tricuspid stenosis with preserved left ventricular systolic function (Fig. 1). A mobile mass (1.8 9 3.0 cm) both on the anterior and posterior leaflets of tricuspid valve which causes mean transvalvular gradient (minimum–maximum 5–10 mmHg) color-flow imaging (Fig. 2). The other 3 cardiac valves were free of vegetations by transthoracic echocardiography. Cardiac magnetic resonance imaging confirmed mobile tumor (19 9 13 mm) on the tricuspid valve. Coronary angiography was normal, and a right heart catheter showed normal pulmonary artery pressure. The patient underwent a successful tricuspid valve commisurotomy and Kay annuloplasty under the cardiopulmonary bypass. Myocardial protection was with intermittent antegrade cold blood cardioplegia. The tricuspid valve showed multiple verrucous vegetations as if being a mass on the anterior and posterior leaflets (Fig. 3). Histological findings of the tricuspid valve were consistent with Libman-Sacks endocarditis. Photomicrographs showed sections of the tricuspid
Valve Repair for Libman-Sacks Endocarditis
operative recovery and was discharged home on the day 11. In the follow-up period, transthoracic echocardiography was performed in 6th month after the operation and tricuspid valve showed 1+ insufficiency. She had no dyspnea and pretibial edema. The patient was rehospitalized 2 times after the operation in this period. Laboratory results were as follows: C-reactive protein level was 2.6 mg/L, sedimentation rate was 32 mm/h, platelet count was 369 9 103/lL. The proteinuria is 4 g/day now and she is still on the same medical therapy in addition to aspirin.
Figure 1. Spectral flow imaging of the tricuspid valve stenosis in transthoracic echocardiography.
Figure 2. Transthoracic echocardiographic view of the vegetation on the tricuspid valve.
valve with calcified extensive deposition of fibrin and other blood elements. The features are of bland vegetation consistent with Libman-Sacks endocarditis. The patient had an uneventful post-
Comment: Systemic lupus erythematosus is an inflammatory disease with multiple organ involvement. All SLE patients may have cardiac involvement at some stage during their illness.3 Cardiac manifestations show diversity and can involve many components of the heart, including the pericardium, conduction system, myocardium, heart valves, and coronary arteries.1 Valvular abnormalities are its most important cardiac manifestation. The prominent cardiac valvular manifestation in SLE is Libman-Sacks (verrucous) endocarditis. Incidence of Libman-Sacks endocarditis varies widely, because the use of steroids in the management of SLE seems to have decreased its prevalence. The prevalence of Libman-Sacks endocarditis in SLE patients was 59% before corticosteroids and 35% after their use 4 in a lager autopsy series. Although steroids may help in the healing of the vegetations, this will ultimately result in scarring, valve deformity, and dysfunction. The vegetations are not specific to the original illness and usually less than 0.5 cm in diameter. They may be flat and only visible microscopically or can be raised, rounded, or
Figure 3. Operative views of the tricuspid valve involved with vegetations related to the systemic lupus erythematosus.
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verrucous. Fibrin and thrombus with minimal inflammatory infiltrate can be found in the vegetations. Echocardiographic findings are independent from the SLE, and a definitive diagnosis can only be made on pathologic examination of the affected valves. The edges and the surfaces of the valve may be affected, the vegetations may occur in these areas. The atrial or ventricular endocardium or the chordae tendinea and papillary muscles are the other localizations may be involved. In present case, all vegetations were removed successfully from the leaflets of tricuspid valve so that there was no need for the prostheses. The prevalence of significant valve dysfunction in SLE varies. Moreover, valvular involvement in SLE is not parallel to the infective endocarditis. Data on surgical outcomes for valvular operations in patients with SLE are limited to case reports and small case series. Although mitral and aortic valvular involvement is the most commonly encountered echocardiographic findings, the mitral valve is the most frequently affected. On the other hand tricuspid valve involvement, either regurgitation or stenosis, is the least common form of valvular involvement in SLE.5–7 In one review of patients with SLE who underwent valve replacement, mortality rate was reported as 25%.8 This high surgical mortality rate may be partly due to multisystem involvement in SLE. Actually, valve repair therefore is often not feasible in the left-sided valvular heart operations, and valve replacement remains the only option, because of the extensive tissue destruction with significant tissue thickening and verrucous vegetations. On the other hand, right-
E168
sided valvular heart operations are more tolerable than the left-sided, however, the long-term outcome is not known in patients with SLE. Conclusion: Significant improvements in medical management of patients with SLE do not prevent cardiac valvular involvement or slow its progression. Importantly, isolated tricuspid stenosis in young patients should be treated surgically to relieve from symptoms and finally to increase quality of life. References 1. Moder KG, Miller TD, Tazelaar HD: Cardiac involvement in systemic lupus erythematosus. Mayo Clin Proc 1999;74: 275–284. 2. Libman E, Sacks B: A hitherto undescribed form of valvular and mural endocarditis. Arch Intern Med 1924;33:701– 737. 3. Bridgen W, Baywaters GL, Lessof MH, et al: The heart in systemic lupus erythematosus. Br Heart J 1960;22:1–16. 4. Doherty NE, Siegel RJ: Cardiovascular manifestations of systemic lupus erythematosus. Am Heart J 1985;110: 1257–1265. 5. Moyssakis I, Tektonidou MG, Vassilios VA, et al: LibmanSacks endocarditis in systemic lupus erythematosus: Prevalence, associations, and evolution. Am J Med 2007;120: 636–642. 6. Perez-Villa F, Font J, Azqueta M, et al: Severe valvular regurgitation and antiphospholipid antibodies in systemic lupus erythematosus: A prospective, long-term follow up study. Arthritis Rheum 2005;53:460–467. 7. Ong K, Yu G, Jue J: Prevalence and spectrum of conditions associated with severe tricuspid regurgitation. Echocardiography 2013; doi: 10.1111/echo.12420. 8. Dajee H, Hurley EJ, Szarnicki RJ: Cardiac valve replacement in systemic lupus erythematosus: A review. J Thorac Cardiovasc Surg 1983;85:718–726.
Echocardiography
CASE REPORTS Section Editor: Brian D. Hoit, M.D.
Isolated Tricuspid Valve Repair for Libman-Sacks Endocarditis Ali Kemal Gur, M.D., Dolunay Odabasi, M.D., Ayse Gul Kunt, M.D., and Alper Sami Kunt, M.D. Department of Cardiovascular Surgery, School of Medicine, Yuzuncu Yil University, Van, Turkey
Cardiac involvement is a well-known complication of systemic lupus erythematosus (SLE), which can involve most cardiac components, including pericardium, conduction system, myocardium, heart valves, and coronary arteries. Libman-Sacks (verrucous) endocarditis is the characteristic cardiac valvular manifestation. Although isolated tricuspid valve involvement is quite rare, we report a patient with SLE who had tricuspid stenosis caused by Libman-Sacks endocarditis. The patient underwent successful commisurotomy and Kay annuloplasty on the tricuspid valve under cardiopulmonary bypass. (Echocardiography 2014;31:E166–E168) Key words: inflammation, systemic, tricuspid valve
Case Report: Systemic lupus erythematosus (SLE) is a multisystem illness including cardiac involvement. Cardiac involvement involves most cardiac components, including the pericardium, conduction system, myocardium, heart valves, and coronaries.1 Libman-Sacks (verrucous) endocarditis is the characteristic cardiac valvular manifestation. These atypical, sterile, verrucous vegetations were first published by Libman and Sacks in 1924.2 Some patients in SLE need surgical intervention, which carries a high morbidity and mortality due to multisystem involvement. Although the most common valves involved in SLE are the mitral and aortic valves, isolated tricuspid valve involvement can be seen a quite rare manifestation. A 20-year-old woman had recently suffered from sweating, weakness, fatigue, malar rash, and photosensitivity. Results of investigations, including computed tomography and diffusion magnetic resonance imaging of the head, were reported as normal. Right kidney was detected as atrophic in abdominal ultrasonography (Grade III/IV renal disease); she had also renal proteinuria (12 g/day). Renal biopsy confirmed lupus nephritis on this basis and the presence of symptoms and the laboratory results. She was evaluated by a rheumatologist, who confirmed the diagnosis of SLE. The patient remained afebrile, and all culture results remained negative after 72 hours. Address for Correspondence and reprint requests: Ayse Gul Kunt, Department of Cardiovascular Surgery, School of Medicine, Yuzuncu Yıl University, Zeve Campus, 65080 Van, Turkey. Fax: +90 (432) 216 75 19; E-mail: [email protected]
E166
Meanwhile, she was evaluated for connective tissue disorders and was found to have elevated antinuclear antibody, double-stranded deoxyribonucleic acid, and anticardiolipin antibody. Laboratory results were as follows: C-reactive protein level was 3.3 mg/L, sedimentation rate was 34 mm/h, platelet count was 29 9 103/lL. She was in active phase of the SLE and Mesnex 800 mg/day plus methylprednisolone 48 mg/ day were started as initial medical therapy. An incidental mid-diastolic murmur was detected, and transthoracic echocardiography demonstrated tricuspid stenosis with preserved left ventricular systolic function (Fig. 1). A mobile mass (1.8 9 3.0 cm) both on the anterior and posterior leaflets of tricuspid valve which causes mean transvalvular gradient (minimum–maximum 5–10 mmHg) color-flow imaging (Fig. 2). The other 3 cardiac valves were free of vegetations by transthoracic echocardiography. Cardiac magnetic resonance imaging confirmed mobile tumor (19 9 13 mm) on the tricuspid valve. Coronary angiography was normal, and a right heart catheter showed normal pulmonary artery pressure. The patient underwent a successful tricuspid valve commisurotomy and Kay annuloplasty under the cardiopulmonary bypass. Myocardial protection was with intermittent antegrade cold blood cardioplegia. The tricuspid valve showed multiple verrucous vegetations as if being a mass on the anterior and posterior leaflets (Fig. 3). Histological findings of the tricuspid valve were consistent with Libman-Sacks endocarditis. Photomicrographs showed sections of the tricuspid
Valve Repair for Libman-Sacks Endocarditis
operative recovery and was discharged home on the day 11. In the follow-up period, transthoracic echocardiography was performed in 6th month after the operation and tricuspid valve showed 1+ insufficiency. She had no dyspnea and pretibial edema. The patient was rehospitalized 2 times after the operation in this period. Laboratory results were as follows: C-reactive protein level was 2.6 mg/L, sedimentation rate was 32 mm/h, platelet count was 369 9 103/lL. The proteinuria is 4 g/day now and she is still on the same medical therapy in addition to aspirin.
Figure 1. Spectral flow imaging of the tricuspid valve stenosis in transthoracic echocardiography.
Figure 2. Transthoracic echocardiographic view of the vegetation on the tricuspid valve.
valve with calcified extensive deposition of fibrin and other blood elements. The features are of bland vegetation consistent with Libman-Sacks endocarditis. The patient had an uneventful post-
Comment: Systemic lupus erythematosus is an inflammatory disease with multiple organ involvement. All SLE patients may have cardiac involvement at some stage during their illness.3 Cardiac manifestations show diversity and can involve many components of the heart, including the pericardium, conduction system, myocardium, heart valves, and coronary arteries.1 Valvular abnormalities are its most important cardiac manifestation. The prominent cardiac valvular manifestation in SLE is Libman-Sacks (verrucous) endocarditis. Incidence of Libman-Sacks endocarditis varies widely, because the use of steroids in the management of SLE seems to have decreased its prevalence. The prevalence of Libman-Sacks endocarditis in SLE patients was 59% before corticosteroids and 35% after their use 4 in a lager autopsy series. Although steroids may help in the healing of the vegetations, this will ultimately result in scarring, valve deformity, and dysfunction. The vegetations are not specific to the original illness and usually less than 0.5 cm in diameter. They may be flat and only visible microscopically or can be raised, rounded, or
Figure 3. Operative views of the tricuspid valve involved with vegetations related to the systemic lupus erythematosus.
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verrucous. Fibrin and thrombus with minimal inflammatory infiltrate can be found in the vegetations. Echocardiographic findings are independent from the SLE, and a definitive diagnosis can only be made on pathologic examination of the affected valves. The edges and the surfaces of the valve may be affected, the vegetations may occur in these areas. The atrial or ventricular endocardium or the chordae tendinea and papillary muscles are the other localizations may be involved. In present case, all vegetations were removed successfully from the leaflets of tricuspid valve so that there was no need for the prostheses. The prevalence of significant valve dysfunction in SLE varies. Moreover, valvular involvement in SLE is not parallel to the infective endocarditis. Data on surgical outcomes for valvular operations in patients with SLE are limited to case reports and small case series. Although mitral and aortic valvular involvement is the most commonly encountered echocardiographic findings, the mitral valve is the most frequently affected. On the other hand tricuspid valve involvement, either regurgitation or stenosis, is the least common form of valvular involvement in SLE.5–7 In one review of patients with SLE who underwent valve replacement, mortality rate was reported as 25%.8 This high surgical mortality rate may be partly due to multisystem involvement in SLE. Actually, valve repair therefore is often not feasible in the left-sided valvular heart operations, and valve replacement remains the only option, because of the extensive tissue destruction with significant tissue thickening and verrucous vegetations. On the other hand, right-
E168
sided valvular heart operations are more tolerable than the left-sided, however, the long-term outcome is not known in patients with SLE. Conclusion: Significant improvements in medical management of patients with SLE do not prevent cardiac valvular involvement or slow its progression. Importantly, isolated tricuspid stenosis in young patients should be treated surgically to relieve from symptoms and finally to increase quality of life. References 1. Moder KG, Miller TD, Tazelaar HD: Cardiac involvement in systemic lupus erythematosus. Mayo Clin Proc 1999;74: 275–284. 2. Libman E, Sacks B: A hitherto undescribed form of valvular and mural endocarditis. Arch Intern Med 1924;33:701– 737. 3. Bridgen W, Baywaters GL, Lessof MH, et al: The heart in systemic lupus erythematosus. Br Heart J 1960;22:1–16. 4. Doherty NE, Siegel RJ: Cardiovascular manifestations of systemic lupus erythematosus. Am Heart J 1985;110: 1257–1265. 5. Moyssakis I, Tektonidou MG, Vassilios VA, et al: LibmanSacks endocarditis in systemic lupus erythematosus: Prevalence, associations, and evolution. Am J Med 2007;120: 636–642. 6. Perez-Villa F, Font J, Azqueta M, et al: Severe valvular regurgitation and antiphospholipid antibodies in systemic lupus erythematosus: A prospective, long-term follow up study. Arthritis Rheum 2005;53:460–467. 7. Ong K, Yu G, Jue J: Prevalence and spectrum of conditions associated with severe tricuspid regurgitation. Echocardiography 2013; doi: 10.1111/echo.12420. 8. Dajee H, Hurley EJ, Szarnicki RJ: Cardiac valve replacement in systemic lupus erythematosus: A review. J Thorac Cardiovasc Surg 1983;85:718–726.
