BACKGROUND: Rheumatic heart disease (RHD) continues to pose a significant burden on global health, particularly in socioeconomically disadvantaged populations. We present the case of a 38-year-old woman with severe multivalvular RHD and giant atria, highlighting the challenges and complexities of managing this condition.
METHODS: The patient presented with progressively worsening dyspnoea, signs of right heart failure, and severe valvular abnormalities. Diagnostic evaluations revealed severe mixed mitral valve disease in the form of mitral stenosis and regurgitation, along with involvement of the aortic and tricuspid valves, leading to significant enlargement of both atria. Despite facing socioeconomic constraints and poor adherence to treatment, the patient underwent successful surgical intervention, resulting in remarkable symptomatic improvement.
CONCLUSIONS: Through this case, we emphasise the importance of early detection, comprehensive management strategies, and multidisciplinary care in addressing the complexities of RHD. Despite the challenges posed by socioeconomic disparities, positive outcomes can be achieved with timely diagnosis and appropriate intervention. This case underscores the need for targeted efforts to improve access to healthcare resources and reduce the global burden of RHD.

BACKGROUND: Net atrioventricular compliance (Cn) can affect the accuracy of mitral valve area (MVA) assessment. We assessed how different methods of MVA assessment are affected by Cn, and if patients with abnormal Cn may be identified by clinical and/or echocardiographic parameters.
METHODS: We studied 244 patients with rheumatic MS. The concordance between mitral valve area (MVA) by 2D planimetry, pressure half-time (PHT), continuity equation (CE), Yeo\'s index, and 3-dimensional mitral valve area assessed by transesophageal echocardiography (TEE 3DMVA) in patients with normal and abnormal Cn (Cn ≤ 4 mL/mmHg) were evaluated in the 110 patients with both transesophageal echocardiogram (TEE) and transthoracic echocardiogram (TTE). Variables that were associated with abnormal Cn were validated in the remaining 134 patients with only TTE.
RESULTS: Except for MVA by CE, concordance with TEE 3DMVA was poorer for all other methods of MVA assessment in patients with abnormal Cn. But, the difference in concordance was only statistically significant for MVA by PHT. Patients with MVA ≤ 1.5 cm2 by 2D planimetry and PHT ≤ 130 ms were likely to have an abnormal Cn. (specificity 98.5%). This finding was validated in the remaining 134 patients (specificity 93%).
CONCLUSIONS: MVA assessment by PHT is significantly affected by Cn. Abnormal Cn should be suspected when 2D planimetry MVA is ≤1.5 cm2 together with an inappropriately short PHT that is ≤130 ms. In this scenario, MVA by PHT is inaccurate.

UNASSIGNED: Blood culture-negative endocarditis (BCNE) is a diagnostic challenge, therefore our objective was to pinpoint high-risk cohorts for BCNE.
UNASSIGNED: The study included adult patients with definite endocarditis. Data were collected via the Infectious Diseases International Research Initiative (ID-IRI). The study analysing one of the largest case series ever reported was conducted across 41 centers in 13 countries. We analysed the database to determine the predictors of BCNE using univariate and logistic regression analyses.
UNASSIGNED: Blood cultures were negative in 101 (11.65 %) of 867 patients. We disclosed that as patients age, the likelihood of a negative blood culture significantly decreases (OR 0.975, 95 % CI 0.963-0.987, p < 0.001). Additionally, factors such as rheumatic heart disease (OR 2.036, 95 % CI 0.970-4.276, p = 0.049), aortic stenosis (OR 3.066, 95 % CI 1.564-6.010, p = 0.001), mitral regurgitation (OR 1.693, 95 % CI 1.012-2.833, p = 0.045), and prosthetic valves (OR 2.539, 95 % CI 1.599-4.031, p < 0.001) are associated with higher likelihoods of negative blood cultures. Our model can predict whether a patient falls into the culture-negative or culture-positive groups with a threshold of 0.104 (AUC±SE = 0.707 ± 0.027). The final model demonstrates a sensitivity of 70.3 % and a specificity of 57.0 %.
UNASSIGNED: Caution should be exercised when diagnosing endocarditis in patients with concurrent cardiac disorders, particularly in younger cases.

Human fibrinogen (FIB) has been clinically proven to be considerably effective for the treatment of postoperative bleeding, with reported cases of allergic reactions to human FIB being rare. Here, we report a case of an anaphylactic shock in 27-year-old patients with rheumatic heart valve disease who received a human FIB infusion during mitral valve replacement, aortic valve replacement, and tricuspid valve-shaping surgery. The patients showed generalised profuse sweating, a barely noticeable skin rash, faint pulse, systolic pressure < 50 mmHg, and a heart rate of 71 beats/min. We share insights from a case of severe allergy to human FIB infusion during cardiac surgery, through which we have gained experience in the processes of diagnosing and treating. This report aims to provide a preliminary summary of the characteristics of this case to serve as a reference for fellow clinicians.

BACKGROUND: Rheumatic heart disease is a global health concern with a persistently high incidence in developing countries, including Africa. It has a significant economic, morbidity, and mortality burden, particularly for children and young adults during their most productive years. However, in the last ten years, the extent of its impact in Africa has remained unclear. Limited studies conducted on the continent have reported diverse prevalence rates of rheumatic heart disease. As a result of these, the study aimed to aggregate and synthesize findings from population-based studies to offer a comprehensive and updated overview of rheumatic heart disease prevalence and pattern at the African level.
METHODS: The studies were identified through a comprehensive literature search of the electronic databases, including PubMed, Google Scholar, Web searches, and manual searches. The descriptive information for the study is presented in the table, and the quantitative results are presented in forest plots. The Cochrane Q test and I2 test statistic were used to test heterogeneity across studies. The pooled estimate of the prevalence of rheumatic heart disease was computed by a random effects model.
RESULTS: Out of 22 population-based studies analyzed using random-effects, the pooled magnitude of rheumatic heart disease was found to be 18.41/1000 (95% CI: 14.08-22.73/1000). This comprised definite cases of rheumatic heart disease at a prevalence rate of 8.91/1000 (95% CI: 6.50-11.33/1000) and borderline cases at a prevalence rate of 10.69/1000 (95% CI: 7.74-13.65/1000). The combined prevalence of rheumatic heart disease in males was almost equivalent to that in females. Mitral valve regurgitation was the predominant valve affected by rheumatic heart disease, accounting for approximately 73%.
CONCLUSIONS: This study analysis found the prevalence of rheumatic heart disease in Africa is high. Because of this, policies and interventions should give attention to prioritize continuous population based active surveillance for early detection of cases to the reduction of rheumatic heart disease sequel, especially in the children and adolescent population.

BACKGROUND: Rheumatic mitral stenosis (MS) remains a common and concerning health problem in Asia. Percutaneous balloon mitral valvuloplasty (PBMV) is the standard treatment for patients with symptomatic severe MS and favorable valve morphology. However, studies on the incidence and predictors of adverse cardiac outcomes following PBMV in Asia have been limited. This study aims to evaluate the incidence and predictors of adverse outcomes in patients with rheumatic MS following PBMV.
METHODS: A retrospective cohort study was conducted on patients with symptomatic severe MS who underwent successful PBMV between 2002 and 2020 at a tertiary academic institute in Thailand. Patients were followed up to assess adverse outcomes, defined as a composite of cardiac death, heart failure hospitalization, repeat PBMV, or mitral valve surgery. Univariable and multivariable analyses were performed to identify predictors of adverse outcomes. A p-value of < 0.05 was considered statistically significant.
RESULTS: A total of 379 patients were included in the study (mean age 43 ± 11 years, 80% female). During a median follow-up of 5.9 years (IQR 1.7-11.7), 74 patients (19.5%) experienced adverse outcomes, with an annualized event rate of 2.7%. Multivariable analysis showed that age (hazard ratio [HR] 1.03, 95% confidence interval [CI] 1.008-1.05, p = 0.006), significant tricuspid regurgitation (HR 2.17, 95% CI 1.33-3.56, p = 0.002), immediate post-PBMV mitral valve area (HR 0.39, 95% CI 0.25-0.64, p = 0.01), and immediate post-PBMV mitral regurgitation (HR 1.91, 95% CI 1.18-3.07, p = 0.008) were independent predictors of adverse outcomes.
CONCLUSIONS: In patients with symptomatic severe rheumatic MS, the incidence of adverse outcomes following PBMV was 2.7% per year. Age, significant tricuspid regurgitation, immediate post-PBMV mitral valve area, and immediate post-PBMV mitral regurgitation were identified as independent predictors of these adverse outcomes.

Rheumatic heart disease (RHD) caused by group A streptococcus infection is one of the most important reasons of cardiovascular morbidity and mortality in low- and middle-income countries. Aberrant host immune response modulated by polymorphisms in inflammatory response genes plays an important role in RHD pathogenesis. This study aimed to determine risk-associated polymorphic variants in inflammatory response genes in Caucasian RHD patients. A total of 251 Caucasian RHD patients and 300 healthy donors were recruited for this study, and 27 polymorphic sites in 12 genes (TLR1, TLR2, TLR4, TLR6, IL1B, IL6R, IL6, IL10, IL12RB1, IL12B, TNF and CRP) were analyzed using allele-specific PCR. It was demonstrated that the polymorphic variants rs1800871 and rs1800872 in the IL10 gene, rs 1130864, rs3093077 and rs1205 in the CRP gene, rs375947 in the IL12RB1 gene, rs 5743551 and rs5743611 in the TLR1 gene, and rs3775073 in the TLR6 gene can modify RHD risk in a gender- and age-dependent manner. The obtained results can be used to determine the personalized risk of RHD in healthy donors during medical examination or screening, as well as to develop appropriate early prevention strategies targeting RHD in the risk groups.

OBJECTIVE: To describe the clinical profile of acute rheumatic fever (ARF) presentations to paediatric cardiology tertiary services in Western Australia (WA).
METHODS: A retrospective clinical audit of individuals with confirmed ARF referred to the only paediatric tertiary cardiac service in WA (1 January 1987 to 31 December 2020). Comparisons between inpatient, outpatient, remote and non-remote groups were assessed.
RESULTS: Four hundred seventy-one episodes of ARF in 457 individuals (235 male; median age = 8 years) met clinical criteria. The majority were Aboriginal and Torres Strait Islander children (91.2%), with 62.1% living in remote areas. The number of ARF and rheumatic heart disease (RHD) diagnoses per year increased from 1987 to 2017 with notable peaks in 2013 and 2017. The average annual incidence of tertiary-referred ARF in WA of 4-15-year-olds from 1987 to 2020 was 4.96 per 100 000. ARF features included carditis (59.9%), chorea (31%), polyarthritis (30%) and polyarthralgia (24.2%). RHD was evident in 61.8% of cases and predominantly manifested as mitral regurgitation (55.7%). Thirty-four children (7.4%) with severe RHD underwent valvular surgery. 12% had at least one recurrent ARF episode. Remote individuals had more than double the rate of recurrence compared to non-remote individuals (P = 0.0058). Compared to non-remote episodes, remote presentations had less polyarthritis (P = 0.0022) but greater proportions of raised ESR (P = 0.01), ASOT titres (P = 0.0073), erythema marginatum (P = 0.0218) and severe RHD (P = 0.0133).
CONCLUSIONS: The high proportion of Aboriginal and Torres Strait Islander Australians affected by ARF/RHD in WA reflects the significant burden of disease within this population. Children from remote communities were more likely to present with concurrent severe RHD. Our study reinforces the persisting need to improve primary and secondary ARF initiatives in rural and remote communities.

UNASSIGNED: Various techniques have been proposed for the preservation of the subvalvular apparatus (SVA) in mitral valve replacement. This study aimed to compare the midterm results of posterior leaflet preservation with the results of selective preservation of the SVA involving artificial chordae implantation in terms of left ventricular performance in patients undergoing mitral valve replacement.
UNASSIGNED: In total, 127 patients were included in this study. Patients were allocated to 1 of 2 groups according to the techniques used to preserve the SVA. Patients in group 1 underwent posterior leaflet preservation: The anterior leaflet was completely resected, and the posterior leaflet was preserved. In group 2, which comprised patients with severe leaflet extension and subvalvular fusion, the mitral valve was excised completely and substituted with artificial chordae. All relevant preoperative, intraoperative, and postoperative data were recorded.
UNASSIGNED: Mean (SD) ages in groups 1 and 2 were 63.1 (9.65) and 57.1 (12.3) years, respectively (P = .003). Mean (SD) follow-up time was 59.97 (23.63) months (range, 6-99 months). Left ventricular end-diastolic diameter decreased significantly after artificial chordae implantation (P < .001), while the decrease after posterior leaflet preservation was not statistically significant (P = .20). In both groups, there were statistically significant reductions (P < .001) in left ventricular end-systolic diameter and left atrium diameter in the postoperative period compared with respective preoperative levels. During follow-up, left ventricular ejection fraction was found to have increased beyond the preoperative levels in both groups, but the differences were not statistically significant (P > .05).
UNASSIGNED: Results of echocardiographic observations regarding the preservation of the SVA via artificial chordae implantation for mitral valve disease in this sample were satisfactory. Findings suggest that artificial chordae implantation should be considered when posterior leaflet preservation is not suitable.

OBJECTIVE: Rheumatic heart disease (RHD) is the most common cause of acquired heart disease in developing countries and remains a serious public health problem. In the subclinical course of carditis, the absence of typical symptoms and the normal range of classical echocardiographic measurements used to evaluate cardiac functions have required new echocardiographic methods and parameters. Previous studies regarding rheumatic heart disease in children and adults have shown that strain patterns obtained by speckle tracking echocardiography, are in fact affected although left ventricular systolic functions are preserved, yet some studies have suggested otherwise. The aim of our study is to compare the use of speckle tracking echocardiography with conventional methods in the evaluation of cardiac functions and myocardial involvement in children with subclinical RHD.
METHODS: The study group consisted of 24 patients with asymptomatic cardiovascular who had no history of acute rheumatic fever, but had definite or probable rheumatic valve disease. This study group was determined according to the World Heart Federation guidelines by an echocardiographic examination performed for different reasons, as well as the control group of 22 healthy children. In order to evaluate the left ventricular regional myocardial functions of the patients, tissue Doppler echocardiography (TDE) and speckle tracking echocardiographic parameters were compared with the control group.
RESULTS: The mean ages of the patient and control groups were 14.1±2.7 years and 13.9±2.3 years, respectively. There was no statistically significant difference between the two groups in terms of conventional methods (p>0.05) but global longitudinal strain and strain rate values were found to be significantly lower in the patient group (p<0.01). These changes appeared to be relevant throughout the duration of the illness.
CONCLUSIONS: In patients with subclinical rheumatic heart disease, conventional echocardiographic evaluations are likely negative, whereas two-dimensional speckle tracking echocardiography reveal systolic and diastolic dysfunctions of the disease.