Mitral valve prolapse in patients with prior rheumatic fever

It is known that rheumatic heart disease frequently results in isolated mitral regurgitation without concomitant mitral stenosis, especially in countries with a high prevalence of rheumatic fever. However, more recent surgical pathologic data also have demonstrated a high incidence of mitral valve prolapse in cases of rheumatic heart disease, which suggests that rheumatic fever may be a cause of mitral valve prolapse. To determine whether this association of mitral valve prolapse and rheumatic heart disease is present in a stable clinic population, we studied 30 patients who had an apical systolic murmur and a well-documented history of rheumatic fever with dynamic auscultation, two-dimensional echocardiography, and pulsed Doppler examinations. Twenty of the 30 patients (67%) had findings on physical examination consistent with isolated mitral regurgitation and 25 patients (84%) had mitral regurgitation by Doppler examination. Echocardiography demonstrated mitral valve prolapse in 24 patients (80%), whereas only one of the total study group had echocardiographic findings consistent with mitral stenosis. We conclude that (1) the presence of an isolated systolic murmur in patients with a history of rheumatic fever frequently represents pure mitral regurgitation secondary to mitral valve prolapse and (2) postinflammatory changes in valvular tissue resulting from rheumatic fever may be the etiology of mitral valve prolapse in these patients.     

Prevalence of rheumatic fever and rheumatic heart disease in school children in a rural community of the hill region of Nepal

A survey of school children aged 5 to 16 years living in a rural community of the hill region of Nepal, situated about 15-22 km outside Kathmandu city, was conducted to determine the prevalence of rheumatic fever (RF) and rheumatic heart disease (RHD). Of the 4,816 eligible children enrolled in the selected schools, 4,452 (92.4%) were examined. WHO expert committee criteria (1966) was used for the diagnosis and classification of rheumatic fever. Chest x-ray, electro-cardiography, echocardiography and Doppler study were done in all suspected cases of rheumatic heart disease. Six cases of RHD (1 pure mitral stenosis, 3 mitral regurgitation and 2 combined mitral stenosis and regurgitation) were identified giving overall prevalence rate of 1.35 per thousand. No case with active rheumatic fever could be identified. This is the first study on prevalence of RF/RHD in Nepal. The prevalence rate is lower than that reported from neighbouring countries.     

Natural history of rheumatic carditis. A follow-up of more than 20 years

In this retrospective study of 43 patients of the National Institute of Cardiology of Mexico, 20 to 35 years after the first attack of rheumatic carditis shows that the prognosis of the heart valve disease is directly influenced by the number of attacks of rheumatic fever. In fact, when patients had only one rheumatic attack the secuelae was mild mitral regurgitation (19%), without hemodynamic significance, with less proportion of mitral stenosis (15%), or aortic regurgitation (7.6%), less plurivalvular lesions (16%), or required heart surgery (15%). In the other hand, when the patients suffered three rheumatic attacks had more proportion of mitral stenosis (33%), aortic regurgitation (41%), pluryvalvular lesions (38%) and required more heart (50%). We conclude that prophylactic treatment is important in patients with chronic rheumatic heart disease.     

Calcareous aortic valve stenosis: With particular reference to its etiology

Seventeen cases of calcareous aortic valve stenosis without mitral valve deformity are compared with twenty-two cases of calcareous aortic valve stenosis in which there was an associated lesion of the mitral valve of rheumatic origin. Patients under fifty years of age, women, histories of rheumatic fever, and instances of pericarditis were scarce in the former group and numerous in the latter.In the cases of pure aortic valve stenosis the calcification in the valves was most pronounced at the base of the cusps, whereas in some cases in the other group it was more marked at the free edges of the cusps.A slightly higher incidence of arteriosclerosis of the aorta and coronary arteries was found in the cases of uncomplicated aortic valve disease.Calcareous aortic valve stenosis is not always rheumatic in origin. In some cases the etiology of the lesion is probably of the nature of an arteriosclerotic degeneration.     

Juvenile rheumatic fever and rheumatic heart disease at Ramathibodi Hospital, Thailand.

One hundred consecutive cases of rheumatic fever and rheumatic heart disease who were seen at Department of Pediatrics. Ramathibodi Hospital were reviewed. Particular attention was given to the pattern and the outcome of the cardiac status of the patients. The high incidence of severe carditis and tight mitral stenosis was similar to most reports from other developing countries. There was a poor prognosis for the cardiac status of those who came late, had more than valvular lesions, were in congestive heart failure, or had preexisting heart disease and atrial fibrillation. In spite of this, 6 patients had no evidence of heart disease after being followed up for less than 5 years.     

A genetic marker for rheumatic heart disease

The frequency of antigen types (A, B, C, and DR) in an unselected group of 25 patients with chronic rheumatic heart disease and an unselected group of 15 patients with acute rheumatic fever was compared with that in a group of 100 healthy volunteers. All patients and controls were Arabs of Saudi origin. Only the frequency of HLA-DR4 was significantly different in the controls and the patient groups--controls 12%, chronic rheumatic heart disease 72%, acute rheumatic fever 53%, both patient groups together 65% (relative risk 13.6 with 95% confidence interval 10.5-16.7). Eighty three per cent of 12 patients with mitral stenosis and 70% of seven with aortic incompetence had HLA-DR4 antigen. In 17 non-Saudi Arab patients who had acute rheumatic fever or chronic rheumatic heart disease, the frequency of HLA-DR4 was identical (65%) to that in Saudi patients. These findings may have implications for the pathogenesis of rheumatic fever and rheumatic heart disease.     

A genetic marker for rheumatic heart disease.

The frequency of antigen types (A, B, C, and DR) in an unselected group of 25 patients with chronic rheumatic heart disease and an unselected group of 15 patients with acute rheumatic fever was compared with that in a group of 100 healthy volunteers. All patients and controls were Arabs of Saudi origin. Only the frequency of HLA-DR4 was significantly different in the controls and the patient groups--controls 12%, chronic rheumatic heart disease 72%, acute rheumatic fever 53%, both patient groups together 65% (relative risk 13.6 with 95% confidence interval 10.5-16.7). Eighty three per cent of 12 patients with mitral stenosis and 70% of seven with aortic incompetence had HLA-DR4 antigen. In 17 non-Saudi Arab patients who had acute rheumatic fever or chronic rheumatic heart disease, the frequency of HLA-DR4 was identical (65%) to that in Saudi patients. These findings may have implications for the pathogenesis of rheumatic fever and rheumatic heart disease.     

Non-rheumatic-acquired valvular disease in the elderly

The analysis of the clinical data permit some conclusions about the state of the art of valvular lesions. (i) Mitral stenosis is not the predominant lesion any more, particularly above 65 years of age, although is it still more frequent in females. The decreased appearance of new cases goes parallel with the reduced incidence of rheumatic fever, showing indirectly that the latter is the most common cause of the disease. (ii) Mitral insufficiency is the fourth most common lesion, without any age- and sex-dependent differences. Ischemic heart disease, floppy mitral valve and bacterial endocarditis are the usual causes above the age of 65 years. (iii) Aortic stenosis is becoming the predominant valvular lesion, especially in females over 65 years of age; two thirds of cases have good left ventricular function, emphasizing that surgical removal of the stenotic valve in persons above 75 years may be performed more frequently. (iv) The incidence of aortic regurgitation has not changed during the last 10 years; however, rheumatic fever and syphilis are not any more the main causes, but aortic annular ecstasy and bacterial endocarditis are the most common pathologies leading to the insufficiency of the aortic valve. Taking together these facts, and considering also the problems linked to the surgical treatment (or to the possibility of mitral angioplasty), the disease of the heart valves represent major problems even today.     

Displacement of the left nipple in mitral stenosis

In healthy children and in male adults both nipples are usually on the same level. In patients with mitral disease of the heart, who have had rheumatic fever in childhood, the dynamics of the valvular lesion cause a deformity of the chest in the region of the left half of the sternum and the adjacent second, third, and fourth costosternal junctions. Because of this there results an upward and outward displacement of the left nipple. This sign is not present in patients with isolated aortic insufficiency or congenital heart disease. It was present in over 90 per cent of 200 consecutive patients with mitral stenosis examined at teh Montefiore Hospital within the last two years.     

Mitral stenosis

The decrease in the incidence of acute rheumatic fever in western countries has led to a sharp decrease in the incidence of mitral stenosis. This decrease also modifies the clinical presentation and mitral stenosis is now encountered in older patients who have severe impairment of valve anatomy. In developing countries, mitral stenosis remains a frequent disease. The management of patients with mitral stenosis has been modified by the development of percutaneous mitral commissurotomy whose safety and efficacy have been demonstrated in a number of studies with a follow-up of up to ten years. Percutaneous mitral commissurotomy is now the reference treatment for mitral stenosis with pliable valves in young patients and its efficacy has been validated in randomised trials versus surgery. Mitral stenosis in older patients, as it is encountered in western countries, is a more heterogeneous group. Predictive analysis has shown that the predictions of immediate and late results are multifactorial. This has led to consider the indications for percutaneous mitral commissurotomy in patients who do not have ideal anatomic conditions, when their other characteristics are favourable. This is particularly the case in young patients who do not have a very tight mitral stenosis (1-1.5 cm2) and who do not have an advanced heart disease. When the conditions are favourable, percutaneous mitral commissurotomy can be considered in patients who have few symptoms, in particular in order to reduce the thromboembolic risk.     

Chorea in a pregnant woman with rheumatic mitral stenosis

Chorea gravidarum is a rare movement disorder of pregnancy with a broad differential diagnosis. Although often a benign condition, it may indicate underlying acute rheumatic fever, antiphospholipid antibody syndrome or a hypercoagulable state. However, now that rheumatic fever is rare in western countries, chorea gravidarum occurs most commonly in patients with chronic rheumatic heart disease. Heightened awareness of chorea gravidarum and the morbidity of the often associated rheumatic heart disease, particularly in immigrants from developing countries, is essential for early diagnosis and effective management. A case of chorea gravidarum in a woman with rheumatic mitral stenosis is described. The diagnostic approach, pathophysiology and management of this rare condition are discussed.     

Rheumatic aortic stenosis in young patients presenting with combined aortic and mitral stenosis.

This report describes 30 patients under the age of 30 years with rheumatic aortic stenosis, presenting with combined aortic and mitral stenosis. Three patients had additional tricuspid stenosis. Twenty-eight patients gave a history of rheumatic polyarthritis. The diagnosis was confirmed by right and left heart catheterisation in all. The murmur of aortic stenosis was not initially present in 8 out of 10 patients in congestive heart failure. Aortic valve calcification was not seen. Cineangiography showed a tricuspid aortic valve in all, unlike congenital aortic stenosis. A unique feature of this group was the raised pulmonary vascular resistance in 87 per cent of the patients. The present study shows that patients in India developing aortic stenosis after rheumatic fever do so early in the natural history of the disease.     

Influence of surgery on the natural history of rheumatic mitral and aortic valve disease

The rate of survival, the evolution of functional cardiac status and the incidence of major complications during a 5 year period were studied in 410 patients with rheumatic mitral or aortic valve disease, of whom 200 were treated medically and 210 by surgery. The 5 year survival rates in patients with various types of rheumatic mitral valve disease were similar (45 percent for those with mitral stenosis and 46 percent for those with mitral insufficiency or mixed mitral insufficiency and stenosis). The survival rate in patients with aortic valve disease was somewhat more favorable (64 percent).Mitral valvulotomy had the most positive influence on mortality. The 85 percent 5 year survival rate of patients who underwent this procedure was significantly higher than that of patients with medically treated mitral stenosis. In patients submitted to mitral and aortic valve replacement, the survival rate was also improved in comparison with data in the corresponding medically treated groups, but to a lesser degree (70 percent for aortic valve replacement and 60 percent for mitral valve replacement). In all surgically treated groups, initial operative mortality was the primary determinant of the rate of survival at the end of 5 years.Survivors of all surgical groups had appreciable improvement in cardiac functional classification and a remarkable reduction in the incidence of heart failure and atrial fibrillation. The incidence of infectious endocarditis was significantly reduced after mitral valvulotomy, as compared with the incidence in patients with medically treated mitral stenosis. Mitral and aortic valve replacement did not reduce the incidence of infectious endocarditis. The incidence of thromboembolic phenomena was favorably influenced by mitral valvulotomy and aortic valve replacement, but not by mitral valve replacement.     

邵阳地区心脏瓣膜病发病率及病因调查

目的 调查分析邵阳地区心脏瓣膜病的发病率、病因及其演变。方法 对单一中心的1010名18～74岁人群在自填调查表的基础上普查心脏彩色超声心动图（心脏彩超）、心电图、心脏听诊等。结果心脏瓣膜病总发病率为5．3％，男性发病率3．8％，女性6．9％（P〉0．05）。各瓣膜病发病率：单纯主动脉瓣关闭不全2．5％，单纯二尖瓣关闭不全1．5％，单纯二尖瓣狭窄0．1％，单纯三尖瓣关闭不全0．1％，二尖瓣脱垂0．2％，多瓣膜病1．0％，未检出单纯主动脉瓣、三尖瓣狭窄者，亦未检出肺动脉瓣病变者。各年龄段发病率：18～29岁为1．5％，30～39岁为2．7％，40～49岁为1．8％，50～59岁为4．8％，60～74岁为13．2％。原发病：老年退行性瓣膜病占31．5％，高血压病、冠心病各占16．7％，高血压病合并冠心病（高冠心病）占1．9％，风心病占13．0％，扩张型心肌病和甲状腺功能亢进症（甲亢）各占3．7％，心肌炎、先心病各占1．9％，糖尿病（可能原因）占3．7％，另有5．6％原因不明。结论 邵阳地区心脏瓣膜病总发病率5．3％，男女发病无显著差异，其原发病已由风心病为主逐渐演变为老年退行性瓣膜病为主。     

Endovascular Therapy for Rheumatic Mitral and Aortic Valve Disease: Review Article

Rheumatic heart disease remains a major cause of significant mortality and morbidity in the developing world. Rheumatic mitral and aortic stenosis are the two most common valvular sequelae of acute rheumatic fever. Open surgical procedures with either valve replacement or surgical commissurotomy have largely been replaced by percutaneous commissurotomy and balloon valvuloplasty for selected patients since the early 1980s. The purpose of our review is to examine the literature for any recent advancement in procedural techniques of balloon valvuloplasty for selected patients with rheumatic mitral and or aortic stenosis. Our search indicates that, in general, the volume of recent publications on this important topic is rather scanty with no major changes in the fundamentals of procedural techniques and or primary indications for balloon valvuloplasty of a rheumatic valve stenosis. Percutaneous balloon valvuloplasty/commissurotomy remains the main stay of therapy for rheumatic valvular stenosis in selected patients with favorable valve anatomy.     

Pattern of heart disease in Ethiopia as seen in a cardiology referral clinic

385 patients were seen in the cardiology clinic of Tikur Anbessa Hospital, Addis Ababa, Ethiopia over 20 months. Of 338 with defined pathology, 152 had rheumatic heart disease, 47 were hypertensive, 39 had cardiomyopathy, 36 had congenital heart disease and 24 arrhythmia. Average age of rheumatics was 25.5, 78% were less than or equal to 30, male:female = 58:94. The mitral valve was affected in 91%; 18% of rheumatics had pure mitral stenosis and 56% only mitral involvement. Average age of cardiomyopathy patients was 52, 90% had dilated cardiomyopathy. In congenital cases, mitral valve prolapse was most common (25%), followed by ventricular septal defect (19%), and patent ductus arteriosus (19%). Comparison is made with Ethiopian and other African data. Clearly, rheumatic fever is the main cause of cardiac pathology in Ethiopia, and deserves greatly increased attention.     

Quadrivalvular heart disease: transition from congenital pulmonary stenosis to rheumatic disease

The case is reported of a 36-year-old male patient suffering from congenital pulmonary stenosis who previously had undergone pulmonary balloon valvuloplasty. During the past nine years, he had experienced recurrent attacks of rheumatic fever that gradually damaged all four heart valves. The patient underwent aortic, mitral and pulmonary valve replacement with tricuspid valve annuloplasty and pulmonary artery reconstruction. Histologically, all heart valves--including the pulmonary--had similar changes that corresponded to chronic rheumatic disease.     

Mitral valve stenosis in the elderly

Forty-seven elderly patients (aged 70-95 years) with mitral stenosis (MS) were studied. The initial presentation in 77% of patients was in the postretirement age and 55% were diagnosed after the age of 70 years. A history of acute rheumatic fever (ARF) was given by 43% of all patients. One patient had mitral valve stenosis secondary to mitral annular calcification. MS due to rheumatic heart disease may be mild or the progress may be slow enabling patients to live a near normal life; increased longevity of the population in general, use of potent diuretics, limitation of physical activity due to medical complications and decreased number of pregnancies may have been contributory factors. In 23% of patients the diagnosis was initially unrecognized, underlining the possible occult nature of the valve lesion in some elderly patients. The advent of echocardiography has helped in case detection.     

Epidemiology of rheumatic heart disease among primary school children in western Kenya

A community survey was conducted to determine the prevalence of rheumatic heart disease in Kenyan children in all primary schools in 2 sublocations in Western Kenya. In total, 3631 children (97% school attendance) aged from 5 to 15 years were examined by one experienced physician and 2 cardiologists between September and December 1985. Six children were found to have evidence of rheumatic heart disease, giving a prevalence rate of 1.7/1000. As all cases occurred in the age group between 10 and 15 years, the rate was calculated for this group as 2.4/1000. None of these patients had previously been diagnosed as having rheumatic fever. There were 3 males and 3 females. Four patients had mitral regurgitation, one had aortic regurgitation and one had mitral stenosis. Three had electrocardiographic evidence of left atrial abnormality and one showed findings of left ventricular hypertrophy but two patients had normal tracings. Half were symptomatic with dyspnoea on exertion and two gave a history of fleeting polyarthritis. The socio-economic status (peasant), family size (mean = 6 siblings) and the number of children sleeping per room were the same in both the cases with rheumatic heart disease and their controls. The health workers achieved low scores (mean 26%) with regard to the clinical diagnosis, treatment and prevention of rheumatic fever at the level of primary health care. Rheumatic heart disease, nonetheless, was relatively insignificant in the overall picture of health care of school children in Western Kenya.     

Comparison of the echocardiographic and hemodynamic diagnosis of rheumatic tricuspid stenosis

Two-dimensional echocardiography has proved to be reliable in the diagnosis of mitral, aortic and pulmonary stenosis. Its role in the diagnosis of rheumatic tricuspid stenosis is still being defined; therefore, the tricuspid valve echograms of 147 patients with rheumatic heart disease were examined. Thirty-eight of these patients also underwent hemodynamic evaluation. Tricuspid stenosis was defined echocardiographically as diastolic anterior leaflet doming, thickening and restricted excursion of the other two tricuspid leaflets and decreased separation of the leaflet tips. Using these criteria, the sensitivity and specificity of the echocardiogram in detecting tricuspid stenosis were 69 and 96%, respectively, in the group of 38 patients who had both echocardiographic and hemodynamic evaluations. However, when the smaller group of 17 patients who had simultaneous right atrial and right ventricular pressure recordings were considered separately, there was complete agreement between the echocardiographic and hemodynamic data. Thus, the two-dimensional echocardiogram is a sensitive and specific test for diagnosing rheumatic tricuspid stenosis. In addition, these data provided an opportunity to determine the prevalence of tricuspid stenosis in this group of patients with chronic rheumatic valvular disease. Tricuspid stenosis was present in 14 (9.5%) of the total group of 147 patients who had two-dimensional echocardiograms, and in 10 (26.3%) of the 38 who had both echocardiographic and hemodynamic studies. In patients with rheumatic heart disease about to undergo cardiac catheterization, an echocardiographic study should prove useful in making the diagnosis of tricuspid stenosis.