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Fulminating active rheumatic carditis has been observed for over 3 decades in this environment with no recent alteration in either the incidence or the pattern of presentation. Patients are black, seldom older than 20 years and are usually in their early teens but may occasionally be as young as five years. Heart failure is prevalent but occurs only when a haemodynamically important left-sided valve lesion supervenes. Regurgitation is the predominant valve lesion and involves principally the mitral valve. Mitral annular dilatation is the initial pathology and predisposes to lengthening--or rupture--of chordae tendineae and prolapse of the anterior leaflet. The resultant cardiac work-overload apparently perpetuates the rheumatic activity. Heart failure, whether caused by or associated with active rheumatic carditis, makes surgical management of the valve lesion mandatory as a life-saving measure. Mitral valve repair, rather than replacement, is the surgical procedure of choice but is not always practicable when the rheumatic activity is fulminant, significant aortic regurgitation associated or the surgeon relatively inexperienced. Aggressive medical therapy for heart failure, which should include vasodilator drugs, provides temporary improvement only. Contrary to ongoing doctrine, treatment with steroid drugs is neither life-saving nor beneficial. Varying degrees of left ventricular dysfunction are encountered pre-operatively and may be a sequel of the severe regurgitant valve lesion rather than of a rheumatic 'myocardial factor'.
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PMID:Aspects of active rheumatic carditis. 144 46

Fulminating active rheumatic carditis has been observed for over three decades in this environment with no recent alteration in either the incidence or the pattern of presentation. Heart failure (in this context defined as 'an inadequate circulation at rest together with a raised pulmonary venous pressure, with or without an associated high systemic venous pressure in the absence of haemodynamically significant tricuspid valve disease or pericardial effusion') is prevalent but occurs only when a haemodynamically important left-sided valve lesion supervenes. Regurgitation is the predominant valve lesion and involves principally the mitral valve. Mitral annular dilatation is marked and predisposes to lengthening--or rupture--of chordae tendineae and prolapse of the anterior leaflet. The resultant cardiac work-overload apparently perpetuates or aggravates the rheumatic activity. Heart failure, as defined, whether caused by or associated with active rheumatic carditis, makes surgical management of the valve lesion mandatory as a life-saving measure. Aggressive medical therapy for heart failure, which should include vasodilator drugs and especially angiotensin-converting enzyme inhibitors, provides temporary improvement only. Contrary to ongoing doctrine, treatment with steroid drugs in this context is neither life-saving nor beneficial.
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PMID:Mechanisms and management of heart failure in active rheumatic carditis. 220 Jan 47

A case of the rare juvenile form of Kearns-Shy syndrome with progressive external ophthalmoplegia and lid ptosis, carditis, skeletal muscle weakness, seizures, mental subnormality, short stature, EEG abnormality and deafness is presented. Electromyography revealed a myopathic pattern. Histochemical studies on quadriceps biopsy specimens showed atrophy of type II fibers and "ragged-red fibers." On electron microscopy these muscle cells were seen to contain an increased amount of glycogen particles and abnormal mitochondria were increased in number and size. It is of interest that abrupt deterioration of neurological findings such as seizures, mental subnormality, speech disturbance and deafness was present in our case. Computed tomographic scanning showed progressive changes of cerebral atrophy, low density of cerebral white matter and basal ganglia calcification, which were well associated with the clinical deterioration. A review of the literature also indicated that some patients with this syndrome showed abrupt neurological deterioration in childhood. Involvement of the central nervous system in this syndrome has to be considered as the cause of sudden deterioration and death in childhood.
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PMID:Neurologic deterioration with progressive CT changes in a child with Kearns-Shy syndrome. 648 80

The epidemiology of rheumatic fever and rheumatic heart disease in a rural community (total population 114,610) in northern India was studied by setting up a registry based on primary health care centres. Health workers and schoolteachers were trained to identify suspected patients in school and village surveys (121 villages). Medical specialists screened 5-15-year-olds (n = 31,200). The population was followed up for 3 years (from March 1988 to March 1991). All suspected and registered cases were investigated by serial echocardiography and Doppler ultrasonography at a tertiary care centre. A total of 102 cases were confirmed to have rheumatic fever/rheumatic heart disease (prevalence, 0.09%); 66 were aged 5-15 years (prevalence, 0.21%). A total of 48 patients (24 males, 24 females; mean age, 12.11 +/- 3.7 years) were diagnosed to have a possible first attack of rheumatic fever (incidence, 0.54 per 1000 per year). Arthritis was observed in 36 (75%) and carditis in 18 (37.5%) of cases. Prolapse of the anterior mitral leaflet into the left atrium occurred in 5 (22%) cases with carditis. Mitral regurgitation was observed in all 18 cases of carditis; over the period of observation it disappeared in three cases and progressed to mitral stenosis in a further three. A total of 22 patients (11 males, 11 females; mean age, 19.41 +/- 8.1 years) were registered as rheumatic fever recurrences, and 32 patients (18 females, 14 males; mean age, 22.1 +/- 10.1 years) had chronic rheumatic heart disease. Of those with recurrences, 9 (41%) had carditis and 11 (50%) had arthritis.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Epidemiology of rheumatic fever and rheumatic heart disease in a rural community in northern India. 844 39

The original Jones Criteria as proposed by Dr. T. Duckett Jones have been modified four times and the updated revised criteria were published in 1992. According to this latest publication major manifestations are carditis, polyarthritis, chorea, erythema marginatum and subcutaneous nodules. Minor manifestations include fever, arthralgia and laboratory findings of elevated erythrocyte sedimentation rate, C-reactive protein and prolonged PR interval on ECG. For making a diagnosis of acute rheumatic fever, two major, or one major and two minor manifestations must be accompanied by supporting evidence of antecedent group A streptococcal infection in the form of positive throat culture or elevated or rising anti-streptolysin titre. The updated guidelines also highlighted a subgroup of "exceptions to Jones Criteria" for patients with chorea, indolent carditis and previous history of rheumatic fever or "rheumatic heart disease". Role of echocardiography has not been defined in these modifications but may be important, as clinical detection of soft murmurs may be difficult due to tachycardia. Doppler and color flow mapping is more sensitive in picking up minor digress of valvular regurgitation. Several studies have confirmed that the yield of carditis with valvular regurgitation increased with use of echocardiography in patients with acute rheumatic fever. Also echocardiography is of great help in mixed valve lesions to determine the severity of each lesion. Other abnormalities detected on echocardiography in acute carditis include prolapse of the valve, focal nodular thickening of leaflets and pericardial effusion. Jones Criteria are guidelines to assist the physician and should not be substituted for clinical judgement as strictly following them may result in underdiagnosis of this disease in our country.
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PMID:Diagnosis of rheumatic fever: current status of Jones Criteria and role of echocardiography. 1087 70

The original Jones Criteria as proposed by Dr. T. Duckett Jones have been modified four times and the updated revised criteria were published in 1992. According to this latest publication major manifestations are carditis, polyarthritis, chorea, erythema marginatum and subcutaneous nodules. Minor manifestations include fever, arthralgia and laboratory findings of elevated erythrocyte sedimentation rate, C-reactive protein and prolonged PR interval on ECG. For making a diagnosis of acute rheumatic fever, two major, or one major and two minor manifestations must be accompanied by supporting evidence of antecedent group A streptococcal infection in the form of positive throat culture or elevated or rising anti-streptolysin titre. The updated guidelines also highlighted a subgroup of "exceptions to Jones Criteria" for patients with chorea, indolent carditis and previous history of rheumatic fever or "rheumatic heart disease". Role of echocardiography has not been defined in these modifications but may be important, as clinical detection of soft murmurs may be difficult due to tachycardia. Doppler and color flow mapping is more sensitive in picking up minor digress of valvular regurgitation. Several studies have confirmed that the yield of carditis with valvular regurgitation increased with use of echocardiography in patients with acute rheumatic fever. Also echocardiography is of great help in mixed valve lesions to determine the severity of each lesion. Other abnormalities detected on echocardiography in acute carditis include prolapse of the valve, focal nodular thickening of leaflets and pericardial effusion. Jones Criteria are guidelines to assist the physician and should not be substituted for clinical judgement as strictly following them may result in underdiagnosis of this disease in our country.
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PMID:Diagnosis of rheumatic fever: current status of Jones Criteria and role of echocardiography. 1112 13

Detailed echocardiographic analysis was performed in 10 children with first episode of acute rheumatic fever who presented with acute rheumatic polyarthritis or rheumatic chorea and had no clinically detectable evidence of active carditis. Significant changes were observed in the form of mitral valve prolapse with regurgitation in 3, aortic valve prolapse with regurgitation in 1 and mitral valve billowing without regurgitation in 1 patient each. A significant (p < 0.001) anterior mitral chordal elongation was observed in both the groups--rheumatic polyarthritis and chorea when compared with age and sex matched control subjects. Mitral annular diameter was found to be increased (p < 0.001) in patients presenting with polyarthritis alone. These observations of clinically silent but echocardiographically detectable element of carditis forms the basis of how patients of acute rheumatic fever develop permanent valvular deformities in their latter lives without revealing any cardiac affection earlier.
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PMID:Usefulness of echocardiography in detection of subclinical carditis in acute rheumatic polyarthritis and rheumatic chorea. 1122 18