Isolated Tricuspid Valve Libman-Sacks Endocarditis and Valvular Stenosis: Unusual Manifestations of Systemic Lupus Erythematosus




The most common valves involved in systemic lupus erythematosus are the mitral and aortic valves. Although isolated tricuspid valve involvement is quite rare, the authors report such a case. A 42-year-old woman presented with exertional dyspnea and was found to have a cardiac murmur. Echocardiography showed a stenotic tricuspid valve with vegetations on all 3 cusps. No other valvular vegetation could be detected. Concomitant tricuspid regurgitation was noted too. Blood culture results were negative. Clinical findings and serologic tests confirmed the diagnosis of systemic lupus erythematosus. The patient was successfully treated with prednisolone and hydroxychloroquine, and follow-up echocardiography showed the disappearance of the vegetations.


Case Report


A 42-year-old woman was seen by a cardiologist because of the recent onset of exertional dyspnea and chest discomfort. Following transthoracic echocardiography, which demonstrated tricuspid valvular vegetations, she was admitted to our university hospital with suspected endocarditis. She was pancultured, and although afebrile, she was started on empirical parenteral antibiotics.


Systemic review was positive for the presence of arthritic symptoms for the prior 4 years, for which the patient was once treated with oral corticosteroids.


On admission, the patient was found to be afebrile and normotensive. The jugular veins were distended. The crest of the external jugular vein was used to estimate the mean right atrial pressure with the patient in partially upright position (30°-40° above the horizontal). The height of the crest was measured to be about 9 cm above the angle of Louis. Cardiac examination showed a grade III/VI pansystolic murmur at the left lower sternal border. No splenomegaly or other peripheral stigmata of bacterial endocarditis were detected.


The patient’s erythrocyte sedimentation rate was 45 mm/h. Complete blood work showed a white blood cell count of 8000/μL, a hemoglobin level of 13.7 g/dL, and a platelet count of 53 × 10 3 /μl. The C-reactive protein level was 6 mg/L. Other initial workup results were normal.


Complete transesophageal echocardiography was subsequently performed, which showed the presence of a thickened, mildly stenotic tricuspid valve with nodules on all 3 cusps ( Figures 1 A and 1 B ; and Videos 1 and 2 ). The area of the largest vegetation was 0.8 cm 2 . The mean transvalvular gradient was 4.5 mm Hg ( Figure 1 C). The patient was found to have moderate tricuspid regurgitation by color-flow imaging. The vegetations were on the middle portion and the tips of the cusps and were seen on both the atrial and ventricular sides. The other 3 cardiac valves were free of vegetations ( Video 3 ).




Figure 1


All echocardiographic features of tricuspid valve involvement in a patient with systemic lupus erythematosus. (A) Apical 4-chamber view showing thickening of the tricuspid valve leaflets and the presence of Libman-Sacks vegetations on either side of the leaflets. (B) Transgastric right ventricular inflow view (40°) obtained by transesophageal echocardiography showing the presence of vegetations on all 3 cusps. (C) Continuous-wave Doppler recording showing the presence of tricuspid stenosis (mean gradient, 4.5 mm Hg) as well as concomitant tricuspid regurgitation. (D) Apical 4-chamber view 16 months after discharge showing the persistence of valvular thickening but no Libman-Sacks vegetations. Simultaneous Doppler recording (not shown here) showed the persistence of tricuspid stenosis and tricuspid regurgitation.


The patient remained afebrile, and all culture results remained negative after 48 hours. Meanwhile, she was evaluated for connective tissue disorders and was found to have elevated antinuclear antibody, double-stranded deoxyribonucleic acid, and anticardiolipin antibody and low levels of C 3 and C 4 . She was evaluated by a rheumatologist, who confirmed the diagnosis of systemic lupus erythematosus. Antibiotics were discontinued, and she was started on prednisolone and hydroxychloroquine. During the remainder of her hospital stay, she did well and had no cardiac or pulmonary symptoms. Her arthritic pains improved, and she was subsequently discharged in good condition. Repeat echocardiography showed the persistence of valvular thickening, stenosis, and regurgitation but the gradual disappearance of the Libman-Sacks vegetations. The same findings were present on her last echocardiogram 16 months later as well ( Figure 1 D).

Only gold members can continue reading. Log In or Register to continue

Stay updated, free articles. Join our Telegram channel

Jun 16, 2018 | Posted by in CARDIOLOGY | Comments Off on Isolated Tricuspid Valve Libman-Sacks Endocarditis and Valvular Stenosis: Unusual Manifestations of Systemic Lupus Erythematosus

Full access? Get Clinical Tree

Get Clinical Tree app for offline access