Mitral valve replacement and repair. Report of 5 patients with systemic lupus erythematosus

Severe mitral valve regurgitation due to systemic lupus erythematosus is a rare cause of valvular heart disease, necessitating valve surgery. Currently, there are 36 case reports in the world medical literature of mitral valve replacement or repair in patients who have lupus. The current trend in mitral valve surgery is toward anatomic valve repair. In patients who have systemic lupus erythematosus, however, valve repair often leads to repeat surgery and valve replacement. We report the cases of 5 patients with lupus and severe mitral valve regurgitation who underwent mitral valve surgery. In 3 of these patients, replacement with a mechanical prosthetic mitral valve was performed with good long-term results. In the other 2 patients, mitral valve repair was performed, but only 1 of the repairs was successful. The 2nd patient required subsequent replacement with a mechanical valve. To our knowledge, this report of 5 patients is the largest series of mitral valve surgery in patients with lupus. These results, along with a review of the literature, suggest the superiority of mechanical prosthetic valve replacement to repair in patients who have systemic lupus erythematosus.     

Heart disease in systemic lupus erythematosus: diagnosis and management

Most patients suffering from systemic lupus erythematosus develop secondary heart disease at some time during the course of the primary illness. The most common forms of this type of heart disease are acute fibrinous pericarditis and hypertension. By means of echocardiography, an increased incidence of pericardial effusion has been demonstrated. Although commonly noted at autopsy, myocarditis is often clinically silent. However, endomyocardial biopsy may confirm its presence during life. Libman-Sacks endocarditis, although encountered in 40 to 50% of hearts at autopsy, is rarely diagnosed during life. When significant valve dysfunction such as aortic insufficiency or mitral regurgitation develops during the course of systemic lupus erythematosus, then Libman-Sacks endocarditis should be strongly suspected. Cardiac arrhythmias, first degree AV block, and acquired complete heart block may develop either de novo or in association with lupus pericarditis, myocarditis, vasculitis, etc. Complete congenital heart block has been reported in newborns of mothers with systemic lupus erythematosus, particularly those who have an antibody to a soluble tissue ribonucleoprotein antigen called RO(SS-A). Coronary arteritis and premature coronary atherosclerosis manifesting in either angina pectoris or myocardial infarction in young adults, particularly women suffering from systemic lupus erythematosus, have received attention recently. The development of hypertension and hyperlipidemia while such patients are receiving prolonged corticosteroid therapy has been incriminated as the significant risk factor in premature coronary atherosclerosis. Longstanding hypertension and congestive heart failure have unfavorable prognoses. This report is based on a cumulative review of 50 patients with acute and chronic systemic lupus erythematosus seen at our institution and in private practice during the last 10 years.     

Mitral valve prolapse in systemic lupus erythematosus

Despite a low incidence of clinical manifestations, autopsy data suggest endocardial and myocardial disease in about 50% of patients with systemic lupus erythematosus. To investigate whether mitral valve prolapse can be considered a clinical manifestation of cardiac involvement in systemic lupus erythematosus, we carried out an echocardiographic study in 51 affected subjects and 102 normals matched for age and sex. Prevalence of mitral valve prolapse was 25% in patients with systemic lupus erythematosus and 9% in healthy controls with a statistically significant difference (p less than 0.01). Neither pericardial effusion nor prolonged (more than 12 months) treatment with corticosteroids were associated with higher prevalence of mitral valve prolapse. Libman-Sacks verrucae on the mitral valve apparatus as well as focal myocardial scars affecting the papillary muscles and adjacent myocardium could be responsible for the development of the valvular dysfunction. We suggest that mitral valve prolapse can be considered a manifestation of cardiac involvement in patients with systemic lupus erythematosus.     

Endomyocardial biopsy in diagnosis and management of cardiovascular manifestations of systemic lupus erythematosus (SLE)

Summary In recent years, the cardiovascular manifestations of systemic lupus erythematosus have became more apparent as a consequence of both prolonged survival and improvement in diagnostic modalities. We report the case of a 16-year old woman with systemic lupus erythematosus in whom the presence of cardiomyopathy was characterized by endomyocardial biopsy. This diagnostic technique also showed that the histologic hall-mark of lupus myocardiopathy persisted despite corticosteroid and plasmapheresis, suggesting the need of a careful cardiac follow-up in these patients.     

Diagnosis by endomyocardial biopsy: angina pectoris as a manifestation of lupus erythematosus]

A 64-year-old female patient with a prosthetic mitral valve suffered from recurrent typical angina and dyspnea. Left heart catheterization excluded a dysfunction of the prosthesis and coronary artery disease (CAD). A stress thallium scan demonstrated an ischemic reaction. An antimyosin scintigram was positive, indicating myocytal membrane disruption. Serological tests were suspicious for systemic lupus erythematosus (SLE). Therefore, an endomyocardial biopsy (EMB) was performed and a severe alteration of an intramyocardial artery, comparable with chronic SLE, was diagnosed. EMB is a useful diagnostic tool in patients with typical chest pain, positive thallium and anti-myosin scintigrams, however exclusion of CAD.     

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.     

Rapid appearance of severe mitral regurgitation under high-dosage corticosteroid therapy in a patient with systemic lupus erythematosus

Survival of patients with systemic lupus erythematosus has increasedwith corticosteroid therapy. However, adverse effects of corticosteroidtherapy on cardiovascular structures, such as scarring and shrinkingof affected valves, are not well known. We report the case ofa 19-year-old patient who developed severe mitral insufficiencywithin a few weeks after high-dosage corticosteroid therapyhad been instituted for an acute relapse of systemic lupus erythematosus.The rapid development of severe mitral regurgitation was documentedby sequential colour Doppler echocardiography.     

Systemic lupus erythematosus in a patient with human immunodeficiency virus infection – challenges in diagnosis and management

Systemic lupus erythematosus (SLE) is mainly a disease of young women. The problem of coexistent human immunodeficiency virus (HIV) infection is a growing problem in sub-Saharan Africa. We describe a case of active life-threatening neuropsychiatric lupus in a patient with underlying HIV infection. The challenges of interpreting physical signs and laboratory features that are common to both conditions, and the dilemma of treating active SLE with cytotoxics in the immunocompromised patient, are discussed.Abbreviations AIDS Acquired immunodeficiency syndrome - ART antiretroviral therapy - DLE discoid lupus erythematosus - HIV Human immunodeficiency virus - SLE systemic lupus erythematosus     

Chronic intestinal pseudo-obstruction in systemic lupus erythematosus due to intestinal smooth muscle myopathy

We report the case of a woman with systemic lupus erythematosus initially manifesting with fever, rash and arthritis, and two years later with Class IV lupus nephritis. Following treatment with cyclophosphamide she developed symptoms and signs of chronic intestinal pseudo-obstruction (CIPO) that was initially thought to be due to a neutropenic enterocolitis. However, persistence of symptoms resulted in segmental resection of the ileum which showed widespread myocyte necrosis and active inflammation within the muscularis propria. A subsequent, more extensive ileocolic resection showed severe diffuse atrophy and fibrosis of the muscularis propria throughout the resected bowel. The absence of mesenteric vasculitis and the clinical response of the CIPO to the immunosupressive regimen of prednisolone and cyclosporin A suggest that the bowel muscle coat changes reflect an intestinal myopathy secondary to systemic lupus erythematosus, and may have an auto-immune etiology.     

Systemic lupus erythematosus complicated by tricuspid stenosis and regurgitation: successful treatment by valve transplantation.

Clinical tricuspid stenosis has not previously been reported in patients with systemic lupus erythematosus (SLE). A 25 year old woman with active SLE presented with signs of severe right ventricular failure. Cardiac catheterisation confirmed the diagnosis of tricuspid stenosis and regurgitation together with mitral regurgitation. This patient underwent successful tricuspid and mitral valve replacement.     

Cardiac abnormalities in patients with systemic lupus erythematosus.

OBJECTIVE: To determine the prevalence of cardiac abnormalities in patients with systemic lupus erythematosus. DESIGN: Prospective survey. SETTING: Rheumatic diseases unit of a university hospital. PATIENTS: Volunteer sample comprising 83% of patients with systemic lupus erythematosus followed annually in the rheumatic disease unit (93 patients; mean age 46 +/- 13 years; female 79, male 14). These patients were age-matched with 16 female control volunteers (mean age 43 +/- 5 years) recruited from hospital staff. INTERVENTIONS: Electrocardiograms, two-dimensional echocardiograms and radionuclide angiograms were performed in patients and controls. Anticardiolipin antibodies were measured by enzyme-linked immunosorbent assay in the systemic lupus erythematosus patients. MAIN RESULTS: At least one cardiac abnormality was detected in 44 of 93 systemic lupus erythematosus patients (47%). These abnormalities included: aortic valve thickening 12%; mitral valve thickening, prolapse, vegetations or stenosis 23%; left ventricular segmental dysfunction 4%; left ventricular global hypokinesis 4%; right ventricular hypokinesis 4%; left ventricular hypertrophy 14%; left ventricular diastolic dysfunction 16%; and pericardial effusion 2%. Three of the 16 controls (19%) had cardiac abnormalities consisting of mitral valve prolapse (one), right ventricular hypokinesis (one) and pericardial effusion (one). Cardiac abnormalities were more common in the systemic lupus erythematosus group compared with controls (47% versus 19%, P less than 0.05). Raised anticardiolipin antibodies were specific (88%) but not sensitive (33%) for the presence of cardiac abnormalities in systemic lupus erythematosus patients. Renal disease and prednisone therapy were more common in systemic lupus erythematosus patients with cardiac involvement than in such patients without evidence of cardiac disease (40% versus 16%, P = 0.03; and 81% versus 59%, P = 0.04, respectively). CONCLUSIONS: Cardiac abnormalities can be identified noninvasively in 47% of patients with systemic lupus erythematosus.     

Active idiopathic systemic lupus erythematosus in a 90-year-old woman requiring corticosteroid therapy.

A woman who developed multisystem systemic lupus erythematosus (SLE) at the age 80 improved with therapy, but after a hip fracture her disease flared necessitating corticosteroid treatment at age 90. This is the oldest case of active lupus ever reported, and points out the importance of not excluding active SLE as a cause of symptoms or signs even in advanced age.     

Hypertrophic cardiomyopathy in systemic lupus erythematosus and "lupus-like" disease. Chance association? A report of 2 cases.

Two patients, both women, one with "lupus-like" disease, age 51 years, the other a 45-year-old with systemic lupus erythematosus (SLE), developed symptoms and echocardiographic signs of hypertrophic cardiomyopathy. One patient had a family history of sudden maternal death. Neither patient had a history of sustained hypertension and there were no significant valvular lesions detectable to account for the septal and ventricular hypertrophy. The association of SLE or any related condition with hypertrophic cardiomyopathy has not been recorded. In one patient the question of a hereditary cardiomyopathy remains a possibility. The diagnosis of the condition was based on clinical and echocardiographic grounds alone. No endomyocardial biopsies were performed.     

SYSTEMIC LUPUS ERYTHEMATOSUS AND RIGHT RECURRENT LARYNGEAL NERVE PALSY

A 52-year-old woman presenting with systemic lupus erythematosus(SLE) and a right recurrent laryngeal nerve palsy is described.The symptoms and signs resolved within days of initiating steroidtherapy. This is the first reported case of recurrent laryngealnerve palsy associated with SLE in the absence of pulmonaryhypertension or laryngeal lupus. KEY WORDS: Laryngeal nerve palsy, Systemic lupus erythematosus     

Systemic lupus erythematosus as a cause of severe mitral regurgitation: New problem in an old disease

Clinical and morphologic observations in three patients with systemic lupus erythematosus and severe mitral regurgitation are described. Attention is called to the “healing” of Libman-Sacks endocarditis, an infrequent occurrence in patients with systemic lupus erythematosus in the era before steroid therapy. The mitral regurgitation in our patients appears to have resulted from “healing” of the Libman-Sacks vegetations by scarring and calcification. The healing is attributed to longterm corticosteroid therapy.     

Double-valve Libman-Sacks endocarditis causing ventricular fibrillation cardiac arrest

Libman-Sacks endocarditis is a well-known and rather common cardiac manifestation of systemic lupus erythematosus. Transesophageal and transthoracic echocardiography are the definitive imaging methods used to evaluate cardiac valvular involvement in this disease. Valvular masses (vegetations) and valvular thickening are 2 common morphologic echocardiographic patterns. Libman-Sacks lesions are typically characterized by single-valve involvement and their small size of 1 to 4 mm.Herein, we present the unusual case of a 22-year-old woman with newly diagnosed systemic lupus erythematosus who had large, sterile vegetations of Libman-Sacks endocarditis that involved the mitral and aortic valves. This compromised coronary blood flow and resulted in ventricular fibrillation cardiac arrest. The vegetations were surgically excised, and the patient's cardiac function recovered. We discuss the treatment of the patient and that of Libman-Sacks endocarditis.     

Cardiac tamponade as the first manifestation of systemic lupus erythematosus in the elderly

Although pericardial involvement is very frequent in systemic lupus erythematosus, cardiac tamponade is extremely rare as the first manifestation of the disease. On the other hand, systemic lupus erythematosus is a disease that predominantly affects young women, and it is a very uncommon condition in the elderly. We report a 91-year-old woman diagnosed with cardiac tamponade, which was the presenting clinical feature of a previously undiagnosed case of systemic lupus erythematosus.     

Severe mitral regurgitation in Libman-Sacks endocarditis. Conservative surgery

Libman-Sacks endocarditis is a classic but rarely symptomatic manifestation of systemic lupus erythematosus, and valvular surgery is needed in a few cases. We present a patient with systemic lupus erythematosus and Libman-Sacks endocarditis that progressed rapidly to severe mitral regurgitation that needed surgery; surgical valve repair was decided upon. The literature on this topic is reviewed.     

Mitral valve disease of systemic lupus erythematosus. A cause of severe congestive heart failure reversed by valve replacement.

Libman-Sacks endocarditis caused progressive life-threatening mitral regurgitation necessitating mitral valve replacement in an 18 year old woman with systemic lupus erythematosus (SLE). Although Libman-Sacks endocarditis is frequently seen at autopsy in patients with SLE, hemodynamically significant valvular disease due to that lesion is quite rare. We found no previous reports describing mitral regurgitation in a patient with SLE which has necessitated surgical intervention.     

Myocardial infarction due to coronary atherosclerosis in three young adults with systemic lupus erythematosus

Three patients, 24, 24 and 25 years of age, with systemic lupus erythematosus had signs of myocardial infarction. Two had serial electrocardiographic changes indicative of infarction without any cardiac symptoms. The third patient had clinical evidence of an acute massive myocardial infarction, which was proved at autopsy to be due to coronary atherosclerosis. This case is presented in detail and the association between systemic lupus erythematosus and myocardial infarction is reviewed. It is postulated that the relation between lupus erythematosus and coronary atherosclerosis is more than coincidental.