Mitral valve surgery for mitral regurgitation caused by Libman-Sacks endocarditis: a report of four cases and a systematic review of the literature

Libman-Sacks endocarditis of the mitral valve was first described by Libman and Sacks in 1924. Currently, the sterile verrucous vegetative lesions seen in Libman-Sacks endocarditis are regarded as a cardiac manifestation of both systemic lupus erythematosus (SLE) and the antiphospholipid syndrome (APS). Although typically mild and asymptomatic, complications of Libman-Sacks endocarditis may include superimposed bacterial endocarditis, thromboembolic events, and severe valvular regurgitation and/or stenosis requiring surgery. In this study we report two cases of mitral valve repair and two cases of mitral valve replacement for mitral regurgitation (MR) caused by Libman-Sacks endocarditis. In addition, we provide a systematic review of the English literature on mitral valve surgery for MR caused by Libman-Sacks endocarditis. This report shows that mitral valve repair is feasible and effective in young patients with relatively stable SLE and/or APS and only localized mitral valve abnormalities caused by Libman-Sacks endocarditis. Both clinical and echocardiographic follow-up after repair show excellent mid- and long-term results

Hypertrophic cardiomyopathy in systemic sclerosis. A report of two cases

We describe two patients with diffuse systemic sclerosis (SSc) where the echocardiogram revealed asymmetric hypertrophy of the interventricular septum with signs of subaortic obstruction consistent with hypertrophic obstructive cardiomyopathy. Hypertrophic cardiomyopathy is associated with the human lymphocyte antigen (HLA DR3), and this may provide a possible link with SSc, as this HLA phenotype is common in the latter condition. However, further studies should examine whether a true association exists

Right coronary ostium agenesis with absence of the right coronary artery: A rare case of non-ST elevation coronary syndrome

We present a case with right coronary ostium agenesis with anomalous origin of the right coronary artery from the left circumflex artery, which caused a non-ST elevation coronary syndrome. A review of the literature indicates this to be an extremely rare case

Obscure gastrointestinal bleeding and calcific aortic stenosis (Heyde's syndrome)

The coexistence of calcific aortic valve stenosis and obscure gastrointestinal bleeding secondary to intestinal angiodysplasias usually of the cecum and the ascending colon constitutes Heyde's syndrome. The pathophysiologic link between both entities has remained unclear so far but newer studies suggest that it is the result of subtle alterations in plasma coagulation factors. Cessation of the bleeding has followed replacement of the aortic valve. We describe a patient with recurrent obscure gastrointestinal bleeding, calcific aortic stenosis and intestinal angiodysplasias, and discuss the current literature. © 2007 Elsevier Ireland Ltd. All rights reserved

Severe aortic stenosis and mitral regurgitation in a woman with systemic lupus erythematosus

Libman-Sacks endocarditis complicating systemic lupus erythematosus has rarely been reported to cause hemodynamically significant valvular lesions. This report presents a case of severe aortic stenosis combined with severe mitral regurgitation associated with systemic lupus erythematosus in a young woman who died while on the quota list for surgery

Systolic and diastolic function in middle aged patients with sickle ß thalassaemia. An echocardiographic study

Objective: To evaluate the right and left ventricular systolic and diastolic function in middle aged patients with sickle ß thalassaemia. Methods: Forty three patients with sickle ß thalassaemia were recruited for echocardiographic study while 55 controls, matched for age and sex, served as the control group. Parameters measured included: dimensions and wall thickness of left (LV) and right (RV) ventricle and left atrium, LV mass, and cardiac index. LV and RV contractility variables—ejection fraction, circumferential fibre shortening velocity, end systolic stress, end systolic stress/volume index ratio, mitral and tricuspid annulus systolic excursion, and Tei index—were also calculated. The study also evaluated parameters of RV and LV diastolic function including early and late atrioventricular flow velocities (E and A wave respectively), E/A ratio, deceleration time (DT), isovolumic relaxation time (IVRT) as well as pulmonary and hepatic veins systolic to diastolic (S/D) ratio. Results: Chamber enlargement, greater LV mass index, cardiac index, and RV wall thickness were found in the anaemic group compared with controls. The LV and RV contractility variables of the patients were similar to controls. Conversely the LV and RV Tei index was significantly greater in the patient group. Diastolic dysfunction was present in the anaemic patients resulting from the increased LV and RV A-wave, the longer LVIVRT, RVIVRT, and RVDT, as well as the higher hepatic and pulmonary veins S/D ratio. Conclusions: The results show that in middle aged patients with sickle ß thalassaemia the diastolic function is abnormal in both ventricles but still more in RV, whereas the systolic function remains unchanged