Silent Acute Rheumatic Fever Unmasked by Using Handheld Echocardiography for Febrile Children Presenting in a Rheumatic Heart Disease-Endemic Area.

OBJECTIVE: To understand the burden of acute rheumatic fever (ARF) among children living in low-income countries who present to the hospital with febrile illness and to determine the role of handheld echocardiography (HHE) in uncovering subclinical carditis as a major manifestation of ARF. STUDY DESIGN: This was a cross-sectional study carried at the Pediatric Hospital in Al Obeid, North Kordofan, Sudan, from September 2022 to January 2023 and including febrile children 3 through 18 years of age with or without clinical features of ARF and without another cause for their fever (not excluding malaria). History, examination, blood investigations, and HHE were done. ARF was diagnosed according to the Jones criteria. Clinical ARF was diagnosed if there was a major clinical Jones criterion and silent ARF if the only major Jones criteria was subclinical carditis. RESULTS: The study cohort included 400 children with a mean age of 9 years. Clinical ARF was diagnosed in 95 patients (95/400, 24%), most of whom presented with a joint major manifestation (88/95, 93%). Among the 281 children who did not present with a clinical manifestation of ARF, HHE revealed rheumatic heart disease (RHD) in 44 patients (44/281, 16%); 31 of them fulfilled criteria for silent ARF (31/281, 11%). HHE increased the detection of ARF by 24%. HHE revealed mild RHD in 41 of 66 (62%) and moderate or severe RHD in 25 of 66 (38%) patients. Both sensitivity and specificity of HHE compared with standard echocardiography were 88%. CONCLUSIONS: There is a significant burden of ARF among febrile children in Sudan. HHE increased the sensitivity of diagnosis, with 11% of children having subclinical carditis as their only major manifestation (ie, silent ARF). RHD-prevention policies need to prioritize decentralization of echocardiography to improve ARF detection.

Hyperendemic rheumatic heart disease in a remote Australian town identified by echocardiographic screening.

OBJECTIVES: Using echocardiographic screening, to estimate the prevalence of rheumatic heart disease (RHD) in a remote Northern Territory town. DESIGN: Prospective, cross-sectional echocardiographic screening study; results compared with data from the NT rheumatic heart disease register. SETTING, PARTICIPANTS: People aged 5-20 years living in Maningrida, West Arnhem Land (population, 2610, including 2366 Indigenous Australians), March 2018 and November 2018. INTERVENTION: Echocardiographic screening for RHD by an expert cardiologist or cardiac sonographer. MAIN OUTCOME MEASURES: Definite or borderline RHD, based on World Heart Federation criteria; history of acute rheumatic fever (ARF), based on Australian guidelines for diagnosing ARF. RESULTS: The screening participation rate was 72%. The median age of the 613 participants was 11 years (interquartile range, 8-14 years); 298 (49%) were girls or women, and 592 (97%) were Aboriginal Australians. Definite RHD was detected in 32 screened participants (5.2%), including 20 not previously diagnosed with RHD; in five new cases, RHD was classified as severe, and three of the participants involved required cardiac surgery. Borderline RHD was diagnosed in 17 participants (2.8%). According to NT RHD register data at the end of the study period, 88 of 849 people in Maningrida and the surrounding homelands aged 5-20 years (10%) were receiving secondary prophylaxis following diagnoses of definite RHD or definite or probable ARF. CONCLUSION: Passive case finding for ARF and RHD is inadequate in some remote Australian communities with a very high burden of RHD, placing children and young people with undetected RHD at great risk of poor health outcomes. Active case finding by regular echocardiographic screening is required in such areas.

Quantitative Imaging in Inflammatory Arthritis: Between Tradition and Innovation.

Radiologic imaging is crucial for diagnosing and monitoring rheumatic inflammatory diseases. Particularly the emerging approach of precision medicine has increased the interest in quantitative imaging. Extensive research has shown that ultrasound allows a quantification of direct signs such as bone erosions and synovial thickness. Dual-energy X-ray absorptiometry and high-resolution peripheral quantitative computed tomography (CT) contribute to the quantitative assessment of secondary signs such as osteoporosis or lean mass loss. Magnetic resonance imaging (MRI), using different techniques and sequences, permits in-depth evaluations. For instance, the perfusion of the inflamed synovium can be quantified by dynamic contrast-enhanced imaging or diffusion-weighted imaging, and cartilage injury can be assessed by mapping (T1ρ, T2). Furthermore, the increased metabolic activity characterizing the inflammatory response can be reliably assessed by hybrid imaging (positron emission tomography [PET]/CT, PET/MRI). Finally, advances in intelligent systems are pushing forward quantitative imaging. Complex mathematical algorithms of lesions' segmentation and advanced pattern recognition are showing promising results.

Changes in valvular regurgitation in mid-term follow-up of children with first attack acute rheumatic fever: first evaluation after the updated Jones criteria.

AIM: In present study, we aimed to evaluate the changes in valvular regurgitations in mid-term follow-up of children with first attack acute rheumatic fever diagnosed after updated Jones criteria. MATERIALS AND METHODS: The medical records of the children diagnosed with acute rheumatic fever between June 2015 and November 2018 were evaluated retrospectively. When compared to the findings during diagnosis, the changes in the degree of valvular regurgitation in the last visit were coded as same, regressed, or disappeared. RESULTS: A total of 50 children were diagnosed with the first attack of acute rheumatic fever between the noted dates. Nine patients (18%) could be diagnosed depending on the new criteria. Eight patients did not have carditis, and 35 patients (49 valves) could be followed for a median follow-up period of 11.7 ± 3.3 months. In our study, the valvar lesions continued in 82% of patients with clinical carditis at the end of the first year and the degree of valvular regurgitation decreased in 39% of them. Despite this, in a significantly higher (p = 0.031) ratio of patients with silent carditis (41%), valvar lesions disappeared in the same follow-up period. In 18.4% of the involved valves, regurgitation regressed to physiological level. CONCLUSION: Updated Jones criteria make it possible to diagnose a significant number of patients, and the ratio of complete recovery among patients with silent carditis is significantly higher. Also, it can be speculated that the normal children in whom a physiological mitral regurgitation is detected should be followed in terms of rheumatic heart disease.

Poststreptococcal reactive arthritis in Japan.

Reactive arthritis after Group A streptococcal infection (poststreptococcal reactive arthritis: PSRA) that does not meet the Jones criteria for acute rheumatic fever (ARF) has been reported as a new entity for over a decade. In Japan there are few reports of PSRA. We encountered four children with arthritis accompanied with Group A streptococcal infection in our department. We investigated our cases and the recent Japanese literature. Japanese cases of PSRA are frequently accompanied with uveitis and erythema nodosum, and tonsillectomy resolved their symptoms in some cases. There were overlap cases between ARF, juvenile idiopathic arthritis, and PSRA.

Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association.

BACKGROUND: Acute rheumatic fever remains a serious healthcare concern for the majority of the world's population despite its decline in incidence in Europe and North America. The goal of this statement was to review the historic Jones criteria used to diagnose acute rheumatic fever in the context of the current epidemiology of the disease and to update those criteria to also take into account recent evidence supporting the use of Doppler echocardiography in the diagnosis of carditis as a major manifestation of acute rheumatic fever. METHODS AND RESULTS: To achieve this goal, the American Heart Association's Council on Cardiovascular Disease in the Young and its Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee organized a writing group to comprehensively review and evaluate the impact of population-specific differences in acute rheumatic fever presentation and changes in presentation that can result from the now worldwide availability of nonsteroidal anti-inflammatory drugs. In addition, a methodological assessment of the numerous published studies that support the use of Doppler echocardiography as a means to diagnose cardiac involvement in acute rheumatic fever, even when overt clinical findings are not apparent, was undertaken to determine the evidence basis for defining subclinical carditis and including it as a major criterion of the Jones criteria. This effort has resulted in the first substantial revision to the Jones criteria by the American Heart Association since 1992 and the first application of the Classification of Recommendations and Levels of Evidence categories developed by the American College of Cardiology/American Heart Association to the Jones criteria. CONCLUSIONS: This revision of the Jones criteria now brings them into closer alignment with other international guidelines for the diagnosis of acute rheumatic fever by defining high-risk populations, recognizing variability in clinical presentation in these high-risk populations, and including Doppler echocardiography as a tool to diagnose cardiac involvement.

Plasma adiponectin levels and relations with cytokines in children with acute rheumatic fever.

AIM: We aimed to investigate the role of adiponectin in acute rheumatic fever by evaluating correlations with cytokines and acute-phase reactants. METHODS: Patients were divided into three groups by clinical findings. Group 1 included 8 patients with only chorea, Group 2 included 13 patients with arthritis and carditis, and Group 3 included 12 patients with only carditis. A total of 54 age- and gender-matched children were enrolled in the control group. Blood samples were drawn for analysing acute-phase reactants, adiponectin, tumour necrosis factor-α, interleukin-6, and interleukin-8 levels at baseline on Days 2, 5, 10, and 15, and at 8 weeks. RESULTS: There was no statistically significant difference between baseline age, gender, body mass index, serum triglyceride, total cholesterol, and low-density lipoprotein levels of the study and control groups (p>0.05). No correlation was found between baseline plasma adiponectin levels, age, body mass index, follicle-stimulating hormone, luteinising hormone, oestradiol, total testosterone, and blood lipid levels of the study and control groups (p>0.05). We found that adiponectin and interleukin-6 levels increased, tumour necrosis factor-α levels decreased, and interleukin-8 levels remained unchanged in acute rheumatic fever, which is an inflammatory disease. Moreover, adiponectin level was higher and tumour necrosis factor-α level was lower in the improvement period in comparison with the acute period, particularly in the carditis group. CONCLUSION: It was considered that, increasing throughout the treatment period, adiponectin may have anti-inflammatory effects in acute rheumatic fever. In addition, adiponectin levels are associated with a decline in inflammatory mediators in rheumatic fever.

FALSE AND TRUE ACUTE RHEUMATIC FEVER: A Lebanese Experience.

OBJECTIVES: The main aim of this study is to evaluate the significance of several clinical and laboratory findings presented by a large number of pediatric patients who were suspected to have acute rheumatic fever (ARF) by physicians who ordered echocardiogram for confirmation and for avoiding overuse of unnecessary antibiotics. These suspecting reasons include elevated ASO titers and recurrent tonsillitis. Another aim is to stress the role of Doppler echocardiogram in confirming the diagnosis of ARF by detecting rheumatic heart disease (RHD) in suspected cases. MATERIALS AND METHODS: 401 patients were registered in the National Registry of the Pediatric and Congenital Heart Disease (RNCPC) from April 1, 2002 until the end of March 2013 as suspected cases of ARF. The clinical reasons that brought suspicion are discussed and evaluated in accordance to modified Jones criteria. The patients are distributed into two groups: 1) confirmed ARF and 2) different diagnosis. All patients underwent echocardiogram. Patients came from different geographic areas and were referred by a variety of physicians. RESULTS: 48 patients out of 401 suspected cases turned to have confirmed ARF (12%). In this group, the predominant suspecting reasons are reported as heart murmur in 29/48 (60.4%), arthritis in 28/48 (58.3%), arthralgia with reduced physical activity in 15/48 (31%) and chorea in 5/48 (10%). In the different diagnosis group with a high ASO titer 174/353 (49.3%), isolated arthralgia 91/353 (25.8%) and combined elevated ASO with arthralgia in 44/353 (12.5%) were the predominant suspecting reasons. Doppler echocardiography could detect 38/48 cases of active carditis and subclinical carditis (SCC). CONCLUSION: Heart murmur, arthritis, arthralgia with reduced physical activity and chorea are the main clinical reasons that lead to suspicion in the ARF group. High ASO titers, arthralgia, and combination of high ASO titers with arthralgia are the main presenting symptoms in the group with a different diagnosis. We concluded that elevated ASO titer and normal ESR with arthralgia not reducing physical activity can exclude ARF. Doppler echocardiography is found to be a gold standard key to confirm acute rheumatic heart disease and ARF.

Assessment of atrial electromechanical delay in children with acute rheumatic fever.

PURPOSE: There may be an increase in the risk of atrial arrhythmia due to left atrial enlargement and the influence on conduction system in acute rheumatic fever. The aim of this study is to investigate atrial electromechanical delay and P-wave dispersion in patients with acute rheumatic fever. PATIENTS: A total of 48 patients diagnosed with acute rheumatic fever and 40 volunteers of similar age, sex, and body mass index were included in the study. The study groups were compared for M-mode echocardiographic parameters, interatrial electromechanical delay, intra-atrial electromechanical delay, and P-wave dispersion. RESULTS: Maximum P-wave duration, P-wave dispersion, and interatrial electromechanical delay were significantly higher in patients with acute rheumatic fever compared with the control group (p < 0.001). However, there was no difference in terms of intra-atrial electromechanical delay (p > 0.05). For patients with acute rheumatic fever, a positive correlation was identified between the left atrium diameter and the P-wave dispersion and interatrial electromechanical delay (r = 0.524 and p < 0.001, and r = 0.351 and p = 0.014, respectively). Furthermore, an important correlation was also identified between the P-wave dispersion and the interatrial electromechanical delay (r = 0.494 and p < 0.001). CONCLUSION: This study shows the prolongation of P-wave dispersion and interatrial electromechanical delay in acute rheumatic fever. Left atrial enlargement can be one of the underlying reasons for the increase in P-wave dispersion and interatrial electromechanical delay.

[Arthrodesis for patients with rheumatic arthritis of the ankle and hindfoot. A reasonable option?]. / Arthrodesen bei Patienten mit rheumatisch bedingten Sprunggelenks- und Rückfußarthrosen. Eine sinnvolle Option?

BACKGROUND: Ankle and hindfoot deformities as well as degenerative changes are often found in patients with rheumatological diseases. They often suffer from severe pain and complain of increasing immobility. Corrective procedures with ankle or hindfoot arthrodesis are promising options. OBJECTIVES: This article presents epidemiological data and describes the clinical aspects, diagnostics and treatment options for patients with ankle and hindfoot osteoarthritis. MATERIALS AND METHODS: The retrospective results of 56 patients after ankle or hindfoot arthrodesis are presented. RESULTS: After an average follow-up of 52 months the majority of results were good or excellent with relief of pain and reconstruction of the function of the foot. CONCLUSION: Ankle or hindfoot arthrodesis represents a promising option for patients with severe osteoarthritis and can safeguard patients from increasing immobility.

The influence of preoperative deformity on valgus correction angle: an analysis of 503 total knee arthroplasties.

We prospectively studied variations in valgus correction angle (VCA) and the influence of preoperative limb deformity on VCA in 503 consecutive total knee arthroplasties done in 393 patients. The percentage of limbs that had VCA values less than 5° was 10.9%, and that with VCA values greater than 7° was 44.9%. The percentage of limbs with VCA greater than 7° was significantly more in varus knees, and that with VCA less than 5° was significantly more in valgus knees; preoperative deformity showed a significant correlation with VCA. Choosing a fixed-routine VCA of 5° to 7° may cause an unacceptable planning error that may be minimized by individualizing VCA or using computer navigation.

[Joint sonography in rheumatology]. / Gelenksonographie in der Rheumatologie.

Sonographic examination of joints has become a decisive component in rheumatological diagnostics and is now indispensible in the clinical routine. The exceptional representation of acute inflammatory soft tissue processes, very early recognition of bony destruction and the ubiquitous availability of the method have been major contributors to this success. In recent years there have been new developments in technology and in examination methods. The substantial importance of sonography for early detection of arthritis, differential diagnostics, therapy monitoring and estimation of prognosis is underlined by the continuously increasing number of international publications. Several scoring systems have been developed for small and large joints and have been proven not only under study conditions but also in practice. Subclinical inflammatory processes which are held responsible for the so-called silent progression can be detected using sonography.

Assessment of Myocardial Mechanics in Acute Rheumatic Fever Using Speckle-Tracking Echocardiography.

BACKGROUND: This study aims to evaluate the role of speckle-tracking echocardiography to identify myocardial deformation in acute rheumatic fever. METHODS: Twenty-seven patients and 27 healthy children were prospectively evaluated. The patient group was divided into 2 subgroups based on echocardiographic findings, with or without carditis. The left ventricular global longitudinal strain and strain rate, left ventricular global circumferential strain and strain rate, and right ventricular global lon-gitudinal strain and strain rate were assessed by speckle-tracking echocardiography. RESULTS: In the acute phase of the disease, all values except the right ventricular global longitudinal strain were found to be significantly below the control group in the patient cohort. No significant difference was found between the patients grouped as carditis and non-carditis in the acute period. Comparison of the acute period with the post-treatment period revealed a significant increase in all strain values of the patients with carditis and significant increases observed in all values except left ventricular global longitudinal strain rate, left ventricular global circumferential strain rate, and right ventricular global longitudinal strain rate values in patients without carditis. Apart from the right ventricular global longitudinal strain rate, which was significantly lower in the non-carditis group compared to the control group, there was no significant difference in strain values between the patient and control groups following treatment. CONCLUSION: In the present study, we found that all patients, including patients in whom no valvular involvement was detected by echocardiography in the acute phase of acute rheumatic fever, had a lower right and left ventricular strain and strain rate measurements and that these findings improved after treatment, suggesting that strain echocardiography may be a helpful diagnostic method, especially in patients without valvular involvement.

Rheumatic Fever Follow-Up Study (RhFFUS) protocol: a cohort study investigating the significance of minor echocardiographic abnormalities in Aboriginal Australian and Torres Strait Islander children.

BACKGROUND: In Australia, rheumatic heart disease (RHD) is almost exclusively restricted to Aboriginal Australian and Torres Strait Islander people with children being at highest risk. International criteria for echocardiographic diagnosis of RHD have been developed but the significance of minor heart valve abnormalities which do not reach these criteria remains unclear. The Rheumatic Fever Follow-Up Study (RhFFUS) aims to clarify this question in children and adolescents at high risk of RHD. METHODS/DESIGN: RhFFUS is a cohort study of Aboriginal and/or Torres Strait Islander children and adolescents aged 8-17 years residing in 32 remote Australian communities. Cases are people with non-specific heart valve abnormalities detected on prior screening echocardiography. Controls (two per case) are age, gender, community and ethnicity-matched to cases and had a prior normal screening echocardiogram. Participants will have echocardiography about 3 years after initial screening echocardiogram and enhanced surveillance for any history suggestive of acute rheumatic fever (ARF). It will then be determined if cases are at higher risk of (1) ARF or (2) developing progressive echocardiography-detected valve changes consistent with RHD.The occurrence and timing of episodes of ARF will be assessed retrospectively for 5 years from the time of the RhFFUS echocardiogram. Episodes of ARF will be identified through regional surveillance and notification databases, carer/subject interviews, primary healthcare history reviews, and hospital separation diagnoses.Progression of valvular abnormalities will be assessed prospectively using transthoracic echocardiography and standardized operating and reporting procedures. Progression of valve lesions will be determined by specialist cardiologist readers who will assess the initial screening and subsequent RhFFUS screening echocardiogram for each participant. The readers will be blinded to the initial assessment and temporal order of the two echocardiograms. DISCUSSION: RhFFUS will determine if subtle changes on echocardiography represent the earliest changes of RHD or mere variations of normal heart anatomy. In turn it will inform criteria to be used in determining whether secondary antibiotic prophylaxis should be utilized in individuals with no clear history of ARF and minor abnormalities on echocardiography. RhFFUS will also inform the ongoing debate regarding the potential role of screening echocardiography for the detection of RHD in this setting.

[Musculoskeletal ultrasound in rheumatology--current status and future perspectives]. / Muskuloskeletni ultrazvuk u reumatologiji--sadasnje stanje i potencijalna primjena u buducnosti.

In the last ten years we are whitnesses of fast development of ultrasound in rheumatology clinical practice and research. The main problem is general perception that US can be highly subjective. Since 2004 an international collaborative group of ultrasound experts known as the OMERACT Ultrasound Task Force has worked to address the metric qualities of ultrasound in RA and other arthritides, according to criteria specified by the OMERACT filter. The same group has worked since 2000 in the EULAR setting for promoting training and educational issues. Part of this group is now promoting the standardized use of ultrasound at a practical international level: the TUI initiative (Targeted ultrasound initiative). One of the main goals of TUI initiative is standardisation and formal assessment of musculoskeletal ultrasound.

The resurgence of rheumatic fever in a developed country area: the role of echocardiography.

OBJECTIVES: The annual incidence of ARF ranges from 5 to 51/100, 000 population worldwide in the 5- to 15-year age group. In the past, there was a decline in the incidence of ARF; however, focal outbreaks have been reported. This study evaluated the incidence of ARF in 2007-08 in a region of a developed country compared with the previous decade. METHODS: A retrospective review of all admission records for ARF in Trieste between January 2007 and December 2008 was undertaken. The diagnosis of ARF was established by the Jones criteria according to the 1992 revision. RESULTS: Between January 2007 and December 2008: 13 cases of ARF were recorded, 11 females and 2 males. The estimated incidence was 23 and 27/100, 000 population new cases each year, respectively, in the 5- to 15-year age group. Migratory polyarthritis occurred in 6/13, chorea in 7/13 and clinical carditis in 5/13 cases. Five out of 13 patients had only echocardiographic abnormalities, with no clinical cardiac manifestations. Another two patients did not fulfil diagnostic criteria for ARF, presenting with only three minor criteria, but they revealed silent carditis at echocardiography evaluation. During the follow-up, in one case the carditis receded and in the other it significantly improved. CONCLUSIONS: Our experience underlines that ARF has not yet disappeared in industrialized countries. We observed a high incidence of chorea, always associated with mild carditis. Echocardiographic assessment should be routinely performed in all patients with suspected ARF in order to identify those subclinical cases of valvulitis that would otherwise pass undiagnosed without receiving proper prophylaxis.

Valvular heart disease in systemic lupus erythematosus and Jaccoud's arthropathy.

Jaccoud's arthropathy (JA) was initially described as a secondary complication to rheumatic fever (RF). However, most recently described cases are associated with systemic lupus erythematosus (SLE). At least in RF, this articular complication has been observed to occur in association with valvular heart disease. The aim of this work is to investigate the presence of valvulopathy in patients with SLE and JA, when compared to lupus patients without such complication. Patients with diagnosis of SLE based on the American College of Rheumatology criteria were enrolled in the study and divided into two groups: with or without JA and evaluated by transthoracic echocardiography. A total of 113 patients with SLE (25 with JA and 88 without JA) were assessed, of which 108 were females and five were males. Echocardiographic changes were found in 24 patients (21.2%) out of the entire population, including valvulopathy in 17 cases (15%), pulmonary hypertension in 7 cases (6.2%) and pericardial effusion in 2 cases (1.8%). In general, echocardiographic changes were more frequently seen in the JA group in comparison with the control group (p = 0.04). Additionally, in the JA group, valvulopathy was found in nine cases (36%) against eight cases (9%) in the control group (p = 0.001). This study reveals for the first time the association between the presence of valvular heart disease and JA in SLE patients, suggesting that the presence of JA may be a marker of such complication. Additional studies are required for clarification of the mechanisms involved in both complications.

[Silent enemy in acute rheumatic fever: subclinical carditis]. / Akut romatizmal ateste sessiz düsman: Subklinik kardit.

OBJECTIVES: Routine use of Doppler echocardiography for the initial diagnosis of acute rheumatic fever (ARF) might allow early detection and, hence, prevention of rheumatic recurrences. This study sought to determine the incidence of subclinical carditis in ARF patients. STUDY DESIGN: The study included 80 patients (64 migratory polyarthritis, 16 rheumatic chorea) who were diagnosed with ARF according to the Jones criteria, 1992 update. Rheumatic carditis was defined as the presence of the following findings on Doppler echocardiography: mitral and aortic regurgitant jets in at least two planes, the length of the jets >1 cm, and peak flow velocities of >2.5 m/sec for both mitral and aortic regurgitations persisting throughout systole and diastole, respectively. The patients diagnosed with subclinical carditis were re-evaluated at 1, 3, 6, and 12 months. RESULTS: Echocardiography revealed subclinical carditis in 25 patients (31.3%; 13 girls, 12 boys; mean age 11.4±2.5 years) with ARF. Of 64 patients with migratory polyarthritis, 34 (53.1%) had clinical carditis and 17 (26.6%) had subclinical carditis. The incidences of clinical and subclinical carditis among 16 patients with rheumatic chorea were 31.3% (n=5) and 50% (n=8), respectively. Of 20 patients who completed one-year follow-up, persistence of subclinical carditis was observed in 11 cases (55%). CONCLUSION: Clinicians should be attentive to the presence of cardiac involvement among patients with suspected ARF. Considering the high incidence of subclinical carditis, echocardiographic evidence of carditis should be used as a diagnostic criterion.

The spectrum of rheumatic mitral valve regurgitation presenting to Inkosi Albert Luthuli Central Hospital, KwaZulu-Natal, over a 10-year period.

BACKGROUND: Recent evidence suggests that there is a change in the profile of rheumatic mitral regurgitation (MR) in South Africa to a pattern of chronic fibrotic valvular disease. OBJECTIVE: This study describes the clinical profile of patients with rheumatic MR in the province of KwaZulu-Natal (KZN). METHODS: A retrospective chart review was performed on patients seven years and older with moderate to severe rheumatic MR referred to Inkosi Albert Luthuli Central Hospital from 2006 to 2015. RESULTS: There were 320 patients meeting the study criteria (mean age 22.2 ± 15.8 years, male:female 1:2). Severe dyspnoea was present in 45.9% of patients, heart failure in 117 (36.6%) and atrial fibrillation in 13.8%. Three patients were diagnosed with active carditis at initial presentation and a further 31 had evidence of carditis during follow up. Of the 216 patients who underwent surgery, over one-third (37%) had prolapse of the anterior mitral leaflet, which was due to chordal elongation (n = 63, 29.2%) and/or ruptured chordae (n = 41, 19%). There were 32 deaths (10%) and of these, 27 (8.4%) patients died prior to surgery. CONCLUSIONS: Rheumatic MR in KZN predominantly affects the young, with concomitant carditis resulting in high morbidity and mortality rates.

An evidence-based scoring system to diagnose acute rheumatic fever with carditis in children.

BACKGOUND: Acute rheumatic fever (ARF) with carditis and chronic rheumatic heart disease (RHD) may be difficult to differentiate and may lead to missed diagnosis of carditis. We aimed to determine differences between these groups and to develop a new scoring system for this purpose. METHODS: Children (N = 514; mean age 11.6 ± 2.8, range 5-18 years; 65% males) enrolled in a RHD registry at a tertiary care centre were studied. Clinical, laboratory and echocardiographic features of acute rheumatic carditis group (N = 126) were compared with chronic RHD group (N = 388). We performed multiple regression analysis and then developed a new scoring system using independent predictors. Accuracy of this scoring system was assessed using receiver operating characteristic (ROC) curve analysis. RESULTS: Patients with ARF and carditis were younger, more commonly had history of fever, arthritis/arthralgias and had worse NYHA class. On echocardiography, severity of mitral regurgitation, presence of mitral leaflet nodules, mitral valve prolapse and pericardial effusion were more common in ARF group. On multiple regression analysis, following features were found to be independently predictive of ARF with carditis: age -negative association, NYHA class, severity of mitral regurgitation, mitral leaflet nodules, erythrocyte sedimentation rate and antistreptolysin titer. Based on these 6 variables, a new scoring system (0-13) was developed. A score of 5 or more was found to be best threshold for diagnosis of carditis (ROC AUC 0.87, sensitivity 76%, specificity 79%). CONCLUSION: A new scoring system based on independent statistical associations appears promising for differentiating ARF with carditis from chronic RHD.