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.     

Cardiac valvular disease in chronic inflammatory disorders of connective tissue. Factors influencing survival after surgery.

Three cases of cardiac valvular disease complicating chronic inflammatory disorders of connective tissue are described. Each underwent surgical valve replacement. The outcome in the first case, a patient with aortitis complicating Reiter's syndrome and in good health otherwise, has been excellent. The other two patients, one with rheumatoid arthritis and vasculitis, the other with systemic lupus erythematosus and nephritis, died after operation as the result of their systemic disease. The importance of systemic features in assessing this type of patient is emphasized.     

Systemic lupus erythematosus and Klinefelter''s syndrome.

A case of Klinefelter's syndrome presenting with systemic lupus erythematosus while receiving androgen replacement therapy is described. The association of systemic lupus erythematosus with Klinefelter's syndrome is discussed, particularly in terms of the effect of sex hormones.     

Left atrial dimension and risk of systemic embolism in patients with a prosthetic heart valve. Department of Veterans Affairs Cooperative Study on Valvular Heart Disease

The relation between left atrial dimension measured by M-mode echocardiography and systemic embolization after valve replacement was examined prospectively among 397 patients with a prosthetic valve enrolled in the Department of Veterans Affairs Cooperative Study on Valvular Heart Disease. Baseline characteristics including several measures of left atrial enlargement were compared for 31 patients who developed systemic embolism and 366 who did not develop embolism during a 5 year follow-up period. Variables that were significantly related to left atrial dimension or systemic embolization in univariate analyses were included with several others in a multiple logistic regression model. The incidence rate of systemic embolism was more than three times higher after mitral valve replacement than after aortic valve replacement (4.4 and 1.3 per 100 patient-years, respectively); this difference persisted after adjustment for other factors. Univariate analysis indicated a threefold higher incidence of systemic embolism in patients with a left atrial dimension greater than or equal to 4 cm compared with that in patients with a dimension less than 4 cm (3 versus 1 per 100 patient-years, respectively). However, when the effect of valve location (mitral versus aortic) was taken into account using either univariate or multivariate techniques, left atrial dimension was found not to be associated with systemic embolism. In multivariate analysis, atrial fibrillation, age, ejection fraction and location of the prosthetic valve were significantly associated with embolism. Results of this multicenter study suggest that left atrial dimension is not independently related to the development of systemic embolism in patients undergoing valve replacement.     

Acute aortitis and aortic incompetence due to systemic rheumatological disorders.

We report the clinical, laboratory and echocardiographic features of five cases of aortic incompetence associated with ankylosing spondylitis, rheumatoid arthritis and undefined connective tissue diseases. Immunosuppression with steroids and cytotoxic agents was used to suppress aortic root inflammation in four cases; in three the aortic root size stabilized and the patients remain well with no evidence of increasing aortic incompetence. In one case, control of the inflammatory process was never fully achieved for any length of time and the patient died shortly after aortic valve replacement. A fifth case required urgent valve replacement and remains well. A systemic rheumatological disorder should be considered in cases of apparent "lone" aortic incompetence and conversely aortic incompetence should not be overlooked in established systemic rheumatological disease. Immunosuppressive therapy may prevent or delay the need for aortic valve replacement in such cases.     

Male hypogonadism in systemic disease.

Hypogonadism is often observed in the presence of common acute and chronic illnesses in men. Low testosterone levels in these patients can be associated with loss of lean body mass and bone mass density, decline in mood, loss of energy, and sexual dysfunction. The mechanisms explaining hypogonadism and various systemic diseases are not completely understood, but these conditions are likely caused by a combination of stress, nonspecific weight loss, inflammation, and medication. Testosterone replacement can be considered in this population to improve lean body mass, bone mass density, and quality of life. More information is needed regarding the risk benefits of testosterone treatment on health outcomes in men who have systemic illness.     

Seasonal recruitment of hippocampal neurons in adult free-ranging black-capped chickadees.

Neurogenesis and neuronal replacement in a population of wild free-ranging animals are described. Our subjects were adult black-capped chickadees (Parus atricapillus). Neuronal birth dates were determined by a single systemic injection of [3H]thymidine, followed by release of the bird and its recapture 6 or more weeks later. Newly formed neurons appeared in the hippocampal complex during all times of year, but with a marked peak in the fall (October). New neurons were also added to the hippocampal complex of captive chickadees, but at levels only half as high as seen in the wild birds. Neurons born at different times of the year lived for a few months and then disappeared. We suggest that the neurons added are part of a process of neuronal replacement and that they are important for the acquisition of new spatial memories, a need that is particularly acute in the fall. Hormonal and experiential factors may determine the rate of neuronal replacement.     

Severe aortic regurgitation from systemic hypertension (without aortic dissection) requiring aortic valve replacement: Analysis of four patients

Clinical and morphologic observations are described in four patients who had severe aortic regurgitation from severe systemic hypertension un-associated with aortic dissection; each patient underwent aortic valve replacement. Although aortic regurgitation of minimal or mild degree is well recognized to occur in patients with systemic hypertension, severe degrees of aortic regurgitation are rare in such patients; aortic valve replacement in such patients has not previously been reported. Why these four patients had such severe aortic regurgitation was not determined. Although systemic hypertension is rarely a cause, it nevertheless must be added to the list of causes of severe pure aortic regurgitation.     

Multiple valve replacement with pericardial xenograft. Clinical and haemodynamic study.

Multiple valve replacement with pericardial xenografts (sizes 17 to 31) was undertaken in 76 patients. The incidence of early and late deaths was 10.5 and 3.9 per cent, respectively. ACtuarially it is predicted that 94.7 +/- 3.9 per cent of hospital survivors will be alive at three and a half years after valve replacement. Though long-term anticoagulants were not used, thrombotic valve obstruction was not seen in this series. A systemic embolus occurred early in one case only (0.95 episodes/100 patient years). Mechanical dysfunction of the pericardial xenograft has not been seen. Haemodynamic studies were performed in seven patients, eight to 21 months after valve replacement. The transvalvular gradients were negligible across the aortic pericardial xenografts. Gradients across mitral and tricuspid xenografts were small. The calculated surface areas ranged from 1.0 to 1.4 cm2 for aortic, 1.7 to 2.1 cm2 for mitral, and 2.0 to 2.4 cm2 for tricuspid valves. These results after multiple valve replacement are comparable to those reported with isolated mitral or aortic pericardial xenografts over a similar period of observation.     

Surgical techniques. Ascending aorta

Aneurysm and dissection are the most common diseases affecting the ascending aorta. Graft replacement of the ascending aorta is a straightforward cardiovascular procedure with excellent early and late results. When aneurysm or dissection extends into the aortic sinuses or arch, management becomes more complex and may entail replacement of the aortic root, aortic valve, or a portion of the aortic arch using hypothermic circulatory arrest. The optimal root prosthesis depends on several patient- and procedure-related variables. Valve-sparing procedures confer many long-term advantages and should be considered in all cases where the aortic valve leaflets are normal. The Ross procedure, although ideally suited for isolated aortic valve disease in young patients, may be applicable to some patients with combined aortic valve and ascending aortic disease, unless there is evidence of a systemic connective tissue disorder.     

Indications for surgical replacement of the mitral valve. With particular reference to common and uncommon causes of mitral regurgitation.

Mitral valve replacement is considered when there is severe mitral stenosis, severe mitral insufficiency or a combination of the two. Ordinarily, surgical replacement is considered only for patients who are in functional classes III or IV and do not respond to medical management. Patients with symptomatic mitral stenosis should be treated with mitral commissurotomy whenever possible. Patients selected for commissurotomy should have a pliable valve, no other major valve dysfunction, sinus rhythm, no systemic embolism and good left ventricular function. Early operation is not ordinarily required. Mitral insufficiency may require mitral valve replacement in six rather common settings: rheumatic disease, rupture of mitral chordae tendineae, postinfarction rupture of a papillary muscle, intractable infective endocarditis, floppy mitral valve and malfunction of a prosthetic valve. Rupture of mitral chordae tendineae can usually be recognized from the history, physical examination, echocardiogram and angiocardiogram. Severe left ventricular papillary muscle dysfunction is usually due to cardiac infarction, and occurs within the first 9 days of infarction. When only a papillary muscle tip is ruptured the patient may survive long enough for a mitral valve replacement. In infective endocarditis, operation is more often needed because of congestive heart failure than because of refractory infection. Evidence of mitral stenosis or insufficiency in a patient with a previously implanted prosthetic valve usually indicates an urgent need for study and early operation. Uncommon causes of mitral incompetence that may require valve replacement are endocardial fibroelastosis, Marfan's syndrome, calcified mitral anulus, osteogenesis imperfecta, methysergide-induced heart disease and carcinoid heart disease.     

Progressive systemic sclerosis of the internal anal sphincter leading to passive faecal incontinence.

Two female patients aged 62 and 44 years with progressive systemic sclerosis and passive faecal incontinence are described. Both had the typical gut motility disorders of dysphagia, heartburn, and constipation. Anorectal physiology tests showed a low resting pressure in both and an absent rectoanal inhibitory reflex in one. In both patients anal endosonography showed a thin internal anal sphincter with changed reflectivity suggestive of fibrosis. In both patients anorectal sensation and pudendal nerve function were normal. Histological examination of the rectum in one patient showed collagenous replacement of the rectal muscularis propria with prominent atrophy of the musculature. This study suggests that the internal sphincter may be selectively affected by progressive systemic sclerosis, which may lead to passive faecal incontinence.     

Takayasu's aortitis with dissection in systemic lupus erythematosus.

A forty-seven-year-old Japanese woman under treatment for systemic lupus erythematosus (SLE), complained of severe back pain. Chest X-ray and MRI showed an aneurysmal dilatation of the ascending aorta. Subsequently an aortic replacement was performed. Microscopically, the resected aorta showed Takayasu's aortitis with chronic dissection. Both aortitis and dissection are rare events in SLE patients. To our knowledge, this is the first report of Takayasu's aortitis with dissection in a patient with SLE.     

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.     

Clinical use of heparin-coated cardiopulmonary bypass in coronary artery bypass grafting.

Clinical handling, risk and benefit of a heparin-coated cardiopulmonary bypass system combined with reduced systemic heparinization in coronary bypass surgery was investigated in a prospective, randomized clinical study. 243 patients (Pts.) were divided into 3 groups: group A (n = 83) had a standard uncoated extracorporeal circulation (ECC) set, and systemic heparin was administered in an initial dose of 400 IE/kg body weight. During ECC activated clotting time (ACT) was kept > or = 480 sec. Group B (n = 77) had the same ECC set completely coated with low-molecular-weight heparin; i.v. heparin was given in the same dose as in group A, ACT was kept at the same level. Group C (n = 83) had the same coated ECC set as group B, but i.v. heparin was reduced to 150 IE/kg, and was set to be > or = 240 sec during ECC ACT. The same circulatory components were used in all 3 groups including roller pumps, coronary suction and an open cardiotomy reservoir. In the postoperative clinical course, recovery was not significantly different between groups, especially with respect to organ dysfunction; but there was significantly reduced postoperative bleeding where heparin-coated ECC and low-dose systemic heparinization were both used. This circulatory technique was also associated with a distinctly lower need for postoperative blood replacement. We conclude that heparin-coated extracorporeal circulation combined with either full-dose or reduced systemic heparinization can be used effectively with the same standard equipment and procedures as in uncoated technology. Combination with low-dose i.v. heparin leads to significantly decreased blood loss and less need for blood replacement.     

Valvular aortic stenosis: disease severity and timing of intervention.

Standard echocardiographic evaluation of aortic stenosis (AS) severity includes measurement of aortic velocity, mean transaortic pressure gradient, and continuity equation valve area. Although these measures are adequate for decision making in most patients, there is no single value that defines severe stenosis. Aortic stenosis affects not just the valve, but the entire vascular system, including the left ventricle (LV) and systemic vasculature. More sophisticated measures of disease severity might explain the apparent overlap in hemodynamic severity between symptomatic and asymptomatic patients and might better predict the optimal timing of valve replacement. There have been several approaches to evaluation of stenosis severity based on valve hemodynamics, the ventricular response to increased afterload, ventricular-vascular coupling, or the systemic functional consequences of valve obstruction, such as exercise testing and serum brain natriuretic peptide levels. Aortic valve replacement is indicated when symptoms due to severe AS are present. In most asymptomatic patients, the risk of surgery is greater than the risk of watchful waiting so that management includes patient education, periodic echocardiography, and cardiac risk factor modification. Many adults with AS have comorbid conditions that affect both the diagnosis and management of the valve disease, including aortic regurgitation, aortic root dilation, hypertension, coronary artery disease, LV dysfunction, and atrial fibrillation. Comorbid conditions should be evaluated and treated based on established guidelines, although awareness of the potential effects of therapy in the presence of valve obstruction is needed.     

Early diagnosis of osteonecrosis in systemic lupus erythematosus with magnetic resonance imaging. Failure of core decompression

A 42-year-old white male with systemic lupus erythematosus developed radiologically evident left femoral head osteonecrosis. Magnetic resonance imaging showed evidence of bilateral disease, including the asymptomatic right femoral head. Despite early surgical decompression of the latter lesion, there was rapid progression requiring total hip joint replacement.     

Mallory-Weiss syndrome. Characterization of 75 Mallory-weiss lacerations in 528 patients with upper gastrointestinal hemorrhage.

Seventy-five Mallory-Weiss lacerations were visualized endoscopically in 58 of 528 patients evaluated acutely for upper gastrointestinal bleeding. The Mallory-Weiss mucosal laceration is more common than generally recognized, is usually associated with hiatal hernia and a prodrome of retching or vomiting, and the ingestion of alcohol or acetylsalicyclic acid, or both. The lacerations are most commonly gastric and are associated with other mucosal lesions which may in fact be the instigating cause of the retching and vomiting. Although blood loss may be considerable, 90% or more patients with this lesion can be managed nonsurgically with appropriate blood component replacement and occasional use of systemic pitressin. There was one fatality in this series of 58 patients with Mallory-Weiss lacerations.     

Double valve replacement for lupus valvulitis: report of a case and review of the literature

Hemodynamically significant lupus valvulitis, requiring valve replacement, is rare: 21 cases have been reported so far in the literature, and only 2 of these have involved double valve replacement. We describe an additional case of double valve replacement in a patient with systemic lupus erythematosus. The histopathologic and clinical features of this case suggest that valvular involvement resulted from both acute and chronic disease processes. Medical success in the treatment of systemic lupus erythematosus, especially that achieved through prolonged or high-dose steroid therapy, may cause chronic valvular disease to become a more common surgical problem. A review of the literature supports this contention.     

Surgical repair of lesions associated with corrected transposition. Late results.

Between 1970 and 1980 19 patients aged 13 months to 47 years (mean 16 years) had surgical repair of lesions associated with corrected transposition. Four had had previous palliative surgery. Operations were performed for ventricular septal defects in 17 (multiple in two), and in addition 10 had relief of pulmonary stenosis, three had atrial septal defects closed, and three had valve replacement for left atrioventricular valve regurgitation. Two patients had annuloplasty for isolated left atrioventricular valve regurgitation. There was a high operative mortality (37%). Twelve survivors left hospital and were followed up for three to eight years. There is concern about the high incidence of left atrioventricular valve regurgitation and progressive postoperative left sided ventricular dysfunction. Heart block after surgical intervention contributes to this, and careful pacemaker policies are necessary as two late deaths were related to this. Only one patient is asymptomatic and without complications 40 months after operation. These disappointing late results are partly related to the onset of heart block, but it seems that independent systemic ventricular function may deteriorate in some patients. Thus surgical treatment of lesions associated with corrected transposition should be recommended only in those with important symptoms or signs of changing systemic ventricular dysfunction.