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Serum alpha-1-antitrypsin in ischemia and rheumatic heart diseases.

Tissue damage, inflammation and necrosis are hallmarks of myocardial infarction. In the present study significant elevations of serum alpha-1-antitrypsin were noted in coronary artery disease and angina cases. Interestingly chronic rheumatic heart disease which is also characterized by tissue injury. Inflammation revealed normal levels of serum alpha-1-antitrypsin. The level in chronic rheumatic heart disease was 3.37 +/- 0.57 mumol/mt/ml (control level was 3.37 +/- 0.54 mumol/mt/ml). The corollary of these observations is that in heart diseases acute phase response in terms of enhanced levels of alpha-1-antitrypsin differ depending on the causative factors. Except chronic rheumatic heart disease, in all other stressful states studied there is (to a certain degree) an altered systemic homeostasis and haemostasis. On the other hand chronic rheumatic heart disease encompass certain amount of acute phase status in terms of tissue damage and inflammation does exist unaccompanied by altered systemic homeostasis and haemostasis. However, bacteriological etiologies predominate the triggered immune responses. It is hypothesised that serum alpha-1-antitrypsin enhancement will not occur even though acute phase state exists if specific immune responses are also a part of the disease manifestation.

Adolescent↗

Comparative studies of mitral valves in rheumatic heart disease.

Mitral valve tissues from 195 patients with rheumatic heart disease removed at the time of cardiac surgery were studied by conventional histologic techniques. One hundred seven patients from a five-year period at All-India Institute for Medical Sciences in New Delhi were studied in conjunction with 88 patients undergoing cardiac surgery in Albuquerque during a similar period. In Indian patients a correlation was noted between degree of moderate or severe valvular calcification and extent of adjacent valvular mononuclear cellular infiltration often consisting of striking lymphocytic-plasma cell collections. Similar distinct correlations were not observed among the mitral valves from the Albuquerque patients. It is possible that mitral valve mononuclear cell infiltrates among Indian patients may reflect a chronic immune reaction influencing the natural course of rheumatic heart disease in this latter population.

Adolescent↗

Systematic review of rheumatic heart disease prevalence in children in developing countries: the role of environmental factors.

OBJECTIVES: To consider the worldwide prevalence of rheumatic heart disease in children in developing countries using surveys with uniform methodologies, and to consider the effect of environmental factors including socio-economic status, overcrowding, urbanization, nutrition and access to medical services on the distribution of rheumatic heart disease in developing countries. METHODS: Sixty-one surveys of the prevalence of rheumatic heart disease in developing countries were found using a systematic review of MEDLINE from January 1976 to July 1999. Twenty-four studies were selected for comparison based on their uniform methodological and diagnostic techniques. RESULTS: There is a high prevalence of rheumatic heart disease in the Pacific region with a lower prevalence in the Indian subcontinent, Asian, sub-Saharan African, Mediterranean, Latin American and Caribbean regions. However, aside from the Indian subcontinent, these regions have not been well studied, and it may be that the true prevalence is actually higher. CONCLUSIONS: There is a lack of good quality prevalence surveys of rheumatic heart disease in developing countries. It appears that a threshold level where higher socio-economic status is associated with reduced prevalence of rheumatic heart disease is not reached in developing countries. Therefore, differences in prevalence between socio-economic groups in the one area cannot be detected. A similar case can be made for overcrowding. Many regions need well-designed studies of rheumatic heart disease that incorporate assessment of environmental factors as well as the study of the microbiological epidemiology of rheumatic heart disease and group A streptococci.

Acute Disease↗

[A quantitative evaluation of rheumatism activity in chronic rheumatic heart disease].

The clinical data of 185 patients with rheumatic heart disease (RHD) proved by pathological biopsies were recorded before operations, and analysed using the stepwise regression analysis of multi-factors (SRAM). A mathematical formula to judge the activities of rheumatism in RHD was applied. The multi-factors were put in order according to their importance in effect: progressive reduction of cardiac function (age less than 30), sustained atrial fibrillation, recent changes in heart murmur or onset of a new murmur, paroxysmal atrial fibrillation, speed-up of erythrocyte sedimentation rate and acute pulmonary edema (age less than 30). 310 cases of RHD were tested and verified by this formula, 85.5% of them were diagnosed correctly in comparison with Aschoff's bodies observed by pathological biopsies in left cardiac atrial appendages. All cases were operated and confirmed by pathological examination. The presence of Aschoff's bodies in atrial appendages was considered the index of active rheumatism pathologically.

Adult↗

Pattern of rheumatic heart disease in Southern Yemen.

OBJECTIVE: To determine the pattern of rheumatic heart disease in Aden city and surrounding areas by assessing its frequency distribution, severity and complications. METHODS: We conducted this research in a sole regional echocardiographic department for Southern Yemen governorates at a referral hospital located in Aden city, from January 1999 to December 2003. It was a retrospective study focused on echocardiographic findings in 805 patients affected by rheumatic heart disease. RESULTS: Out of 805 patients, 383 (47.6%) were male and 422 (52.4%) were female (age range, 4-70 years; mean age, 28.6 +/- 14.5). Heart valve damage was distributed among patients as follow: mitral 459 (57.1%), aorta 70 (8.7%) and both valves 276 (34.2%); isolated lesions 55.2% and multiple 44.8%; mitral regurgitation 464 (57.6%), mitral stenosis 405 (50.3%), aortic regurgitation 327 (40.6%) and aortic stenosis 54 (6.7%). All children aged less than 10 years had regurgitation. Stenosis and multiple valve lesions predominated in adolescents and young adults. Complications were detected in 20.8% of cases. Pulmonary hypertension was the most common complication (80.4%). Lesions with moderate and severe degree were detected in 51% cases. Only 34.8% of patients at severe stage were operated. CONCLUSION: Rheumatic heart disease takes an aggressive course in Southern Yemen. Children, adolescents and young adults of both gender are the victims. Complications appear early with scanty opportunity to reach advanced age. There is a little chance for palliative treatment.

Adolescent↗

Echocardiographic patterns of juvenile rheumatic heart disease at the Kenyatta National Hospital, Nairobi.

OBJECTIVE: To describe the echocardiographic features of children with rheumatic heart disease seen at the Kenyatta National Hospital. DESIGN: A retrospective study. SETTING: The Kenyatta National Hospital Heart Unit. SUBJECTS: Patients aged 20 years and less with echocardiographic diagnosis of rheumatic heart disease. RESULTS: Two hundred and twenty four echocardiograms were analysed. Seventy six point four of cases were aged between 5 and 15 years, while on 3% were less than five years. The combinations mitral and aortic regurgitation was the most common lesion followed by isolated mitral regurgitation. Isolated aortic regurgitation was as rare as isolated mitral stenosis in the paediatric age group 1.8% and 2.7% respectively. When seen in the younger age group, the leaflet morphology in mitral regurgitation was predominantly thickening and clubbing of the leaflets while in the older children the pathology was of marked fibrosis of the sub-valvular apparatus. Leaflet prolapse and commissural fusion was the major pathology in aortic regurgitation. Pulmonary hypertension was the most common complication commonly seen in mitral valve disease. Calcification was a rare encounter in this age bracket. CONCLUSIONS: Rheumatic heart disease in the paediatric age commonly presents as isolated mitral regurgitation or in combinations of mitral and aortic regurgitation. The complications of pulmonary hypertension was predominant in mitral valve disease. Valvular calcification is rare in juvenile rheumatic heart disease.

Adolescent↗

Aschoff bodies at necropsy in valvular heart disease. Evidence from an analysis of 543 patients over 14 years of age that rheumatic heart disease, at least anatomically, is a disease of the mitral valve.

Among 543 necropsy patients over age 14 years with severe chronic valvular heart disease, Aschoff bodies were found in 11 patients (2%). The ages of the 11 patients ranged from 18 to 68 years (avg. 38), and nine had had a history of acute rhematic fever earlier in life. Clinically, nine of the 11 patients had mitral stenosis with or without dysfunction of one or more other cardiac valves, one had isolated aortic regurgitation, and one had both mitral and aortic regurgitation. All 11 patients had diffuse fibrous thickening of the mitral valve leaflets, and all but one had diffuse anatomic lesions of at least one other cardiac valve. No patient with anatomic lesions limited to the aortic valve had Aschoff bodies. Thus, among patients with chronic valvular heart disease, Aschoff bodies, the only anatomic lesion pathognomonic of rheumatic heart disease, indicate diffuse anatomic lesions of the mitral leaflets and usually also anatomic lesions of one or more other cardiac valves. The functional mitral lesion is usually stenosis.

Adolescent↗

Restrictive-type hemodynamics following valve surgery for rheumatic heart disease.

Over a six-year period three patients with rheumatic valvular disease presented with congestive heart failure due to abnormalities in myocardial diastolic function. Each patient previously had been operated for mitral stenosis; one patient had additional aortic valve replacement for aortic insufficiency. The mean time for the development of symptoms following surgery was 4.7 years. In all patients, left ventricular systolic function was normal (radionuclide or angiographic ejection fraction greater than 0.50). Abnormalities in diastolic function involved the left ventricle in all patients. Biopsy material from right (one patient) and left (one patient) ventricles was nonspecific in its histologic appearance. Other disease processes, such as constrictive pericarditis and diabetic cardiomyopathy were considered to be clinically unimportant in these patients. Restrictive-type hemodynamics in patients with postoperative rheumatic heart disease may comprise a newly recognized entity.

Aortic Valve Stenosis↗

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.

Adolescent↗

Comparative study on cerebral injury after open heart surgery in patients with congenital and rheumatic heart disease.

OBJECTIVE: To comparatively study the different effects of open heart surgery on brain tissues of patients with congenital and rheumatic heart disease. METHODS: Forty patients with congenital heart disease (CHD, CHD group, n = 20) or rheumatic heart disease (RHD, RHD group, n = 20) underwent on-pump (cardiopulmonary bypass, CPB) heart-beating open heart surgery. Blood samples before CPB, and 20 minutes, 1 hour, 24 hours and 7 days after CPB were collected, and the levels of neuron-specific enolase (NSE) and protein S-100b in the plasma were determined with enzyme-linked immunoadsorbent assay (ELISA), respectively. All the patients were examined with electroencephalogram (EEG) before and 1 week after operation. The changes of NSE, S-100b and EEG compared to verify the difference of postoperative cerebral injury between CHD cases and RHD cases. RESULTS: The plasma level of S-100b increased significantly 20 minutes after CPB and was still higher than the preoperative level at 24 hours after operation in both groups (P < 0.01). The plasma level of NSE increased more significantly in the CHD group than in the RHD group 20 minutes after CPB and it returned to the normal level 24 hours after CPB in the CHD group but remained at a high level in the RHD group (P < 0.01). The levels of NSE and S-100b returned to the normal levels on the 7th day after CPB. Abnormal EEG was found in 75% of the patients in the CHD group and 60% in the RHD group. CONCLUSIONS: On-pump heart-beating open heart surgery can cause certain cerebral injury in the patients with CHD or RHD. The injury was more severe and recovered more quickly in the CHD group than in the RHD group.

Cardiac Surgical Procedures↗

Analysis of costs of acute rheumatic fever and rheumatic heart disease in Auckland.

AIM: This analysis aims to identify the direct costs of rheumatic fever and its sequelae to the Auckland Area Health Board and to describe the indirect and intangible costs to patients. METHODS: The annual cost was estimated using primarily 1987 data costed in 1991 dollars. The cost analysis was undertaken in five sections: (1) acute rheumatic fever admissions; (2) surgical admissions; (3) rheumatic heart disease related admissions (nonsurgical); (4) outpatient clinic appointments; and (5) secondary prophylaxis programme. Non hospital board direct costs and indirect and intangible costs are described. Ethnic distribution and subsequent economic burden were analysed for each section. RESULTS: The total cost to the health board was estimated to be $3.60M. The management of chronic rheumatic heart disease accounts for 71% of the cost. Rheumatic heart disease related nonsurgical admissions cost $1,228,495 (34%), surgical admissions $846,235 (23%) and outpatient clinic appointments $490,060 (14%) respectively. Both Maori (30% of costs, $1.1M) and Pacific Island people (36% of costs, $1.3M) are disproportionately affected by this largely preventable disease. Of the total cost 13% is spent on coordinated secondary prevention programmes. CONCLUSION: An energetic secondary prevention programme over 10 years to prevent recurrences and the development of carditis has only partially reduced the rate of rheumatic heart disease. A targeted primary prevention pilot programme should be actively considered.

Acute Disease↗

Autoantibodies in the sera of patients with rheumatic heart disease: characterization of myocardial antigens by two-dimensional immunoblotting and N-terminal sequence analysis.

The concept of antigenic mimicry in autoimmune diseases such as rheumatic fever has been under investigation for decades and the range of cross-reactive tissue antigens for streptococcal-induced antibodies identified in rheumatic heart disease is still expanding. To identify heart tissue-reactive antigens which may be implicated in the secondary immunopathogenesis of rheumatic fever, sera from 56 patients with acute rheumatic heart disease were probed in two-dimensional Western blots for reactivity against heart tissue antigens. After two-dimensional immunoblot analysis, proteins were submitted to N-terminal amino acid sequence analysis. This analysis identified creatine kinase, two mitochondrial proteins and, at a low level, various stress proteins as cross-reactive myocardial antigens. Therefore, in addition to myosin, creatine kinase may represent another major antigen for autoreactive antibodies in rheumatic heart disease. Mitochondrial proteins have been implicated in the pathogenesis of inflammatory heart disease for some years, and in this study we have identified two mitochondrial proteins as relevant antigens in rheumatic heart disease.

Aconitate Hydratase↗