Comparison of the newer proposed diagnostic score with the World Heart Federation criteria for echocardiographic detection of rheumatic heart disease.

INTRODUCTION: Echocardiography has been found to be a much better screening tool compared to clinical examination for the detection of rheumatic heart disease (RHD) in asymptomatic school children living in the RHD endemic areas. Recently, World Heart Federation (WHF) published echocardiographic criteria for the diagnosis of RHD. The present study was done to compare the performance of the newer proposed, quantitative diagnostic score against the qualitative WHF criteria in a field survey of asymptomatic school children belonging to the district having high prevalence of RHD. METHODS: 3000 asymptomatic school children studying in rural and urban schools of Bikaner district were screened both by clinical examination and echocardiography performed in parallel. The WHF criteria and the proposed diagnostic score were applied simultaneously for the diagnosis of RHD. RESULTS: A high prevalence of subclinical RHD was found. There was complete agreement between the two sets of criteria for the diagnosis of RHD. However, there was discrepancy in grading the severity of disease. The diagnostic score proved superior to the WHF criteria in grading the disease severity accurately. CONCLUSIONS: Diagnostic score captures the disease spectrum of RHD better than WHF criteria and reduces the subjectivity in the diagnosis of RHD.

The World Heart Federation criteria raise the threshold of diagnosis for mild rheumatic heart disease: Three reviewers are better than one.

BACKGROUND: The World Heart Federation (WHF) criteria, published in 2012, provided an evidence-based guideline for the minimal diagnosis of echocardiographically-detected RHD. Primary aim of the study was to determine whether use of the WHF criteria altered the threshold for the diagnosis of echocardiographically-detected RHD compared with the previous WHO/NIH criteria. A secondary aim was to explore the utility of a three reviewer reporting system compared to a single or two reviewer reporting structure. METHODS: 144 de-identified echocardiograms (RHD, congenital valvar abnormality, physiological valvar regurgitation) were independently reported using the WHF criteria by two reviewers blinded to the previous WHO/NIH diagnosis. If there was discordance between the two reviewers, a third cardiologist independently performed a tie-breaker review. RESULTS: There was a 21% reduction of cases classified as RHD using the WHF criteria compared to the modified WHO/NIH criteria (68 cases compared to 86, p = 0.04). There was a 60% consensus across the different diagnostic categories with 2 reviewers, 89% majority agreement with 3 reviewers. 11% required an open label discussion. There was moderate agreement between 2 reviewers for any RHD, kappa 0.57 (CI 0.44-0.70), with no significant difference in agreement between the different categories. CONCLUSION: The WHF criteria have raised the threshold for the diagnosis of RHD compared to the WHO/NIH criteria. However, inter-reporter variability of the WHF criteria is high. A three reviewer system is likely more accurate than a single or two reporter system for the diagnosis of mild RHD. This has resource implications for echocardiographic screening programmes.

Low positive predictive value of International Classification of Diseases, 10th Revision codes in relation to rheumatic heart disease: a challenge for global surveillance.

International Classification of Diseases, 10th Revision codes for rheumatic heart disease (RHD) include valvular heart disease of unspecified origin, limiting their usefulness for estimating RHD burden. A cross-disciplinary national consultation developed an algorithm to improve RHD identification in hospital data. The algorithm has been operationalised and piloted. The algorithm developed categorised 32% of RHD-coded patients as probable/possible RHD. We outline a series of research initiatives to improve identification of RHD in administrative data thereby contributing to monitoring the RHD burden globally.

Stroke, thromboembolism and bleeding in patients with atrial fibrillation according to the EHRA valvular heart disease classification.

AIMS: We compared thromboembolic (TE) and bleeding risks in patients with atrial fibrillation (AF) according to the new 'Evaluated Heartvalves, Rheumatic or Artificial' (EHRA) valve classification. METHODS: Patients were divided into 3 categories: (i) EHRA type 1 corresponds to the previous 'valvular' AF patients, with either rheumatic mitral valve stenosis or mechanical prosthetic heart valves; (ii) EHRA type 2 includes AF patients with other valvular heart disease (VHD) and valve bioprosthesis or repair; and (iii) 'non-VHD controls' i.e. all AF patients with neither VHD nor post-surgical valve disease. RESULTS: Among 8962 AF patients seen between 2000 and 2010, 357 (4%) were EHRA type 1, 1754 (20%) were EHRA type 2 and 6851 (76%) non-VHD controls. EHRA type 2 patients were older and had a higher CHA2DS2-VASc and HAS-BLED scores than either type 1 and non-VHD patients. After a mean follow-up of 1264 ±â€¯1160 days, the occurrence of TE events was higher in EHRA type 2 than non-VHD patients (HR (95%CI): 1.30 1.09-1.54), p = 0.003; also, p = 0.31 for type 1 vs 2, p = 0.68 for type 1 vs non-VHD controls). The rate of major BARC bleeding events for AF patients was higher in either EHRA type 1 (HR (95%CI): 3.16(2.11-4.72), p < 0.0001) or type 2 (HR (95%CI): 2.19(1.69-2.84), p < 0.0001) compared to non-VHD controls. CONCLUSION: The EHRA valve classification of AF patients with VHD appears useful in categorizing these patients, in terms of TE and bleeding risks. This classification can be used in clinical practice for appropriate choices of oral anticoagulation therapy and follow-up.

Prevalence of rheumatic valvular heart disease in Rwandan school children: echocardiographic evaluation using the World Heart Federation criteria.

BACKGROUND: Rheumatic fever (RF) and rheumatic valvular heart disease (RHD) remain important medical, surgical and public health concerns in many parts of the world, especially in sub-Saharan Africa. However, there are no published data from Rwanda. We performed a RHD prevalence study in a randomly selected sample of Rwandan school children using the 2012 World Heart Federation (WHF) criteria. METHODS: Echocardiographic assessment of 2 501 Rwandan school children from 10 schools in the Gasabo district near Kigali was carried out. Resulting data were evaluated by four experienced echocardiographers. Statistical analyses were carried out by statisticians. RESULTS: RHD prevalence was 6.8/1 000 children examined (95% CI: 4.2/1 000-10.9/1 000). Seventeen met WHF criteria for RHD, 13 fulfilled criteria for 'borderline' RHD and four were 'definite' RHD. None of these 17 had been previously identified. CONCLUSION: These data indicate a significant burden of RHD in Rwanda and support a need for defined public health RF control programmes in children there.

[Evaluation of the cardiovascular function in patients with chronic rheumatic heart disease in the practice of medical and social expertise].

Chronic rheumatic heart disease (HRHD) for a long time occupied a prominent position in the structure of the disability population in Ukraine. Aim of the study is to define clinical signs of cardiovascular function in patients with HRHD for disability evaluation in the practice of medical and social expertise. We examined 180 patients with HRHD aged 40-65 years. There were significant differences in patient groups, which allowed us to formulate clinical and functional diagnostic markers of dysfunction of circulation for the objective assessment of the limitations of their life, the definition of disability groups and the development of rehabilitation programs.

Myocardial dysfunction in rheumatic carditis--does it really exist?

OBJECTIVES: Acute rheumatic fever (ARF) continues to affect millions of children in developing countries. Aim of the present study was to evaluate the role of myocardial dysfunction in the genesis of heart failure in patients with rheumatic carditis. There are limited studies on this subject. METHODS AND RESULTS: In this prospective study, 108 consecutive patients of ARF were evaluated by echocardiography and assay of cardiac troponin I blood levels. The patients were divided into three groups. Group A (n = 30): patients with no evidence of carditis; Group B (n = 45): patients with first attack of carditis; and group C (n = 33): patients with recurrent attacks of carditis. Left ventricular dimensions tended to be larger in Group B and C patients. Left ventricular ejection fraction did not differ between the groups (Group A: 63 +/- 8.1%, Group B: 58 +/- 7.9%, Group C: 61.2 +/- 9%, p = ns). Heart failure was present in 37.7% patients of Group B, and in 60.6% patients of Group C (p = < 0.05). Ejection fraction was normal in majority of heart failure patients (75.7%). It was reduced in 29.4% of patients in Group B and in 20% of Group C patients with heart failure (p = ns). All patients with low ejection fraction had hemodynamically significant regurgitant valvular lesions. Mean cardiac troponin I values, an index of myocardial damage, did not differ between the three groups (Group A: 0.062 +/- 0.027 ng/ml, Group B: 0.068 +/- 0.019 ng/ml, Group C: 0.071 +/- 0.031 ng/ml, p = ns). CONCLUSION: The present study did not demonstrate any echocardiographic abnormalities or cardiac troponin I elevation suggesting significant myocardial involvement during acute rheumatic fever. This lends credence to the view that myocardial involvement does not play any significant role in the genesis of heart failure in patients with rheumatic carditis.

[Present-day classification and nomenclature of rheumatic fever]. / Sovremennaia klassifikatsiia i nomenklatura revmaticheskoi likhoradki.

The paper presents a new classification of rheumatic fever, which has been adopted by the Plenary Session of the Association of Rheumatologists of Russia in May 2003. This classification envisages the abandonment of the term "rheumatism" in its former interpretation. The term "acute rheumatic fever" (ARF) appears to be more justified since it makes a physician elucidate its association with Group A streptococcal infection of the throat and prescribe antibiotics for eradication of this infection in its acute period (primary prophylaxis) and for prevention of repeated attacks (secondary prophylaxis). The current interpretation of the clinical syndromes/symptoms and laboratory parameters that enter into diagnostic criteria for ARF is presented. There are 2 types of outcome of the disease. In case of recovery, we are dealing with the complete regression of the clinical symptomatology of ARF, normalized laboratory parameters and no residual changes. Chronic rheumatic diseases of the heart are considered to mean a disease characterized by a cardiac valvular lesion as postinflammatory marginal fibrosis of valvular leaflets or cardiac abnormality (failure and/or stenosis) developed after prior ARF. Emphasis is laid on the significance of evidence of Group A streptococcal infection that precedes the development of the disease. Examples of clinical diagnosis in accordance with the terminology of the International Classification of Diseases, the 10th edition, are given in the paper.

Association between transforming growth factor-beta1 gene C-509T and T869C polymorphisms and rheumatic heart disease.

BACKGROUND: Scarring and collagen deposition in the valves and destruction of myocytes may result from the combined effects of a smoldering rheumatic process and a constant trauma to the mitral valve or aortic valve by the turbulent flow in rheumatic heart disease (RHD). Transforming growth factor-beta1 (TGF-beta1) may be responsible for the increased valvular fibrosis and calcification in the pathogenesis of RHD. However, the role of TGF-beta1 genetic variant in RHD has not been studied. This case-controlled study was carried out to investigate the possible relationship between the TGF-beta1 gene C-509T and T869C polymorphisms and RHD among the Chinese population in Taiwan. METHODS: A group of 115 patients with RHD documented by using echocardiography and 100 age- and sex-matched healthy control patients were studied. TGF-beta1 gene C-509T and T869C polymorphisms were identified with polymerase chain reaction-based restriction analysis. RESULTS: A significant difference was seen in the distribution of genotypes between patients with RHD and control patients for either TGF-beta1 C-509T polymorphism (P <.0001) or T869C polymorphism (P <.0001). The frequency of TGF-beta1 C-509T CC genotype was lower in the RHD group than in the control group (chi2 = 19.05, P <.0001), which suggests that this genotype may confer protective effects against RHD. A significant difference was seen in the distribution of allelic frequency between patients with RHD and control patients for TGF-beta1 T869C polymorphism (P =.04). The odds ratio (OR) for risk of RHD associated with TGF-beta1 T869C T allele was 1.49 (95% CI, 1.02-2.19). Further categorization of patients with RHD into mitral valve disease and combined valve disease subgroups revealed no statistical difference in these gene polymorphisms when compared with the 2 subgroups. CONCLUSIONS: Patients with RHD have a lower frequency of TGF-beta1 C-509T CC genotype and a higher frequency of T869C T allele, which supports a role for the TGF-beta1 gene C-509T and T869C polymorphisms in determining the risk/protection of RHD in Taiwan Chinese patients.

Impact of tissue harmonic imaging on the assessment of rheumatic mitral stenosis.

We examined the effect of tissue harmonic imaging on the echocardiographic splitability score and valve area measurement of 40 patients with rheumatic mitral stenosis. Planimetered valve areas were unaffected by the use of harmonic imaging, but valve scores were increased, particularly in patients with scores <10.

Advanced atrioventricular block in a 39-year-old man with acute rheumatic fever.

This report describes the development of advanced (2:1 and 3:1) AV block in a 39-year-old man with acute rheumatic fever. AV block progressed from first-degree to type I second-degree and finally to advanced AV block. The latter lasted 5 days. AV block regressed in a stepwise fashion via sustained type I second-degree AV block and eventually first-degree AV block. The PR interval returned to normal several weeks after resolution of second-degree AV block. In view of the resurgence of acute rheumatic fever, the diagnosis of acute rheumatic fever should now be considered in young adults or middle-aged patients presenting with second-degree or third-degree AV block of undetermined etiology.

[Diagnostic criteria of infectious endocarditis]. / Critères diagnostiques des endocardites infectieuses.

Accurate diagnostic criteria for infective endocarditis are essential to epidemiological studies. The von Reyn's criteria have been widely used for more than a decade after they were published in 1981. In 1994, the Duke's criteria for the clinical diagnosis of infective endocarditis were published, incorporating echocardiographic findings. They are modeled after the Jones criteria for the identification of cases of rheumatic fever and include 2 major and 6 minor diagnostic criteria. They are about twice as specific as the former von Reyn's criteria, without loss of specificity, and should become a standard reference for diagnosing infective endocarditis.

Role of an operative score in mitral reconstruction in dominantly stenotic lesions.

OBJECTIVE: The role of an operative score in selection and results of mitral valve reconstruction in dominantly stenotic mitral lesions was assessed. METHODS: A total of 136 patients consecutively underwent reconstruction for rheumatic mitral stenosis with or without regurgitation from December 1989 through December 1994. Mitral valve structure was scored for cuspal pliability and thickness, cuspal area loss, chordal length, papillary muscle length, annular dilatation and degree of associated regurgitation and calcification. A score of 0 indicated normal valve while a score of 24 indicated a grossly deformed valve. Age, sex, functional class, cardiac rhythm, mitral valve orifice area, left ventricular and left atrial dimensions, transmitral gradients, pulmonary artery pressures and thoroughness of repair were evaluated. RESULTS: Mitral valve architecture was disorganised moderately in 50 (37%) and severely in 69 (52%) patients. Extent of thoroughness in different operative techniques correlated with good (n = 30) or adequate (n = 60) outcome. Mean mitral valve orifice area increased from 0.77 +/- 0.2 to 2.56 +/- 0.6 cm2, peak gradient dropped from 20.3 +/- 6.2 to 8.6 +/- 3.5 torr, mean gradient from 13.5 +/- 4.9 to 4.76 +/- 2.2 torr and end-diastolic gradient from 10.1 +/- 5.0 to 3.2 +/- 1.9 torr. Lack of recognition of anatomical details and of all necessary componental measures constituted the learning curve of different operators. CONCLUSIONS: Recurrent disease, learning curve, inadequate repair and higher operative mitral valve score were the factors for poorer results in the intermediate term follow-up to 64 months. Thoroughness of repair was the most important correlate of outcome and indicative of expertise.

Demanda hospitalaria, manifestaciones clínicas y prevención de fiebre reumática y cardiopatía en el Instituto de Salud del Niño 1989-1993 / Hospital demand, clinical sign and prevention of rheumatic fever and rheumatic cardiopathy in the Instituto de Salud del Niño 1989-1993

Se estudiaron retrospectivamente 66 pacientes hospitalizados en el Servicio de Cardiología del Instituto de Salud del Niño-entre 1989 y 1993- con el diagnóstico de fiebre reumática y cardiopatía reumática. Se encontró un aumento significativo en los 3 últimos años. El promedio en días de hospitalización se duplicó en 1993 respecto a 1989. El grupo etáreo más afectado fue entre los 11-15 años, con 39 pacientes (59 por ciento). El 74.5 por ciento tuvieron clase funcional III o IV, el 98 por ciento compromiso cardíaco; la lesión valvular más encontrada fue de insuficiencia aórtica (41 por ciento); la complicación más frecuente fue endocarditis infecciosa en 15 pacientes (23 por ciento). Fueron operados 8 pacientes sin mortalidad quirúrgica, 4 pacientes (6 por ciento) fallecieron de complicaciones clínicas. El severo compromiso cardiovascular mostrado en nuestra casuística se debe a una recurrencia elevada de carditis reumática por una ineficaz prevención. Ello hace imprescindible la implementación de un programa ampliado de prevención que considere la administración de fármacos y las condiciones sociales del enfermo para mejorar sus condiciones de vida.

[Echocardiography and Jones criteria in the diagnosis of rheumatic heart diseases: 138 cases reviewed at the Cardiology Institute of Abidjan]. / Echocardiographie et critères de Jones dans le diagnostic des cardiopathies rhumatismales : à propos de 138 cas colligés à l'Institut de Cardiologie d'Abidjan.

The files of 138 patients suffering from rheumatic heart disease were studied in order to confirm the value of modified Jones' criteria and heart damage and to evaluate the contribution of echocardiography findings. Only files containing complete clinical and laboratory findings were studied. Of the 138 cases reviewed there were 51 cases of carditis (36.6 percent) and 87 cases of heart damage (63.4 percent). Carditis was associated with arthritis in 35.3 percent of cases. Antistreptolysin O levels were elevated in 74.5% percent of cases. Regarding Jones' criteria, the presence of a minor clinical criteria such as fever, polyarthritis, and history of acute rheumatic joint inflammation and laboratory findings such as elevated erythrosedimentation and antistreptolysin O rates were associated with carditis significantly more often than with heart damage. Echocardiography played a determinant role in the diagnosis between rheumatic heart disease in 35.3 percent of cases. Only echocardiography allowed differential diagnosis between rheumatic heart damage and other tropical cardiovascular diseases.

[Jaccoud's arthropathy]. / Ob artropatii Zhakku.

Jaccoud's arthropathy is a variant of deforming arthropathy affecting primarily hands. The author presents literature data and analysis of 28 original cases of valvular disease combined with chronic arthritis. Though these cases were diagnosed as Jaccoud's arthropathy, only one of them met Bywaters' criteria. This female patient is described. Jaccoud's arthropathy is suggested to arise due to connective tissue dysplasia resulting in soft tissue impairment in different rheumatic and nonrheumatic diseases. A working classification and additional criteria for postrheumatic Jaccoud's arthropathy are proposed.

Profile of cardiac disease in an out-patient cardiac clinic.

The pattern of cardiac disease differs from one region of the world to that of another. This paper aims at providing the profile of heart disease as seen in a Children's Hospital. A total of 468 patients were seen in a follow-up conducted between August 1989 and February 29, 1992. Among these 168 (35.9%) had congenital heart disease (CHD); 278 (59.4%) had rheumatic heart disease (RHD); while 14 (3%) had acquired heart disease of non-rheumatic origin. In patients having RHD, the mitral valve was commonly affected. Ventricular septal defect was the commonest CHD seen followed by patent ductus arteriosus. The results show that RHD is the commonest heart disease seen in a Children's Hospital in Addis Ababa. It is recommended that the issues of school surveillance, better organized secondary prophylaxis need to be addressed.

Hemoptysis in rheumatic heart disease.

107 Consecutive patients with rheumatic valvular heart disease (41 males, 66 females, average age 24.2 years) being followed at an Ethiopian cardiology referral clinic were examined and questioned about their experience of hemoptysis. 51 Patients (48%) gave a history of hemoptysis; 11 described their usual hemoptysis as frank blood, 40 as blood-streaked sputum. 29 patients had frequent hemoptysis, 13 had several episodes, and 9 only 1 occurrence. Prevalence of hemoptysis did not vary significantly by sex, physical findings, New York Heart Association classification, or valvular lesion, including the presence or absence of mitral stenosis. A majority (64) had been tested for tuberculosis, with 5 having been treated for active disease. Hemoptysis is a common manifestation of rheumatic heart disease, and is usually well tolerated.