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Data from the Global Burden of Disease study indicate that there were 319,400 deaths due to rheumatic heart disease and 33.4 million cases of rheumatic heart disease in 2015. The highest death and prevalence rates were found in Oceania, South Asia, and central sub-Saharan Africa.

During the 1920s, acute rheumatic fever (ARF) was the leading cause of mortality in children in the United States. By the 1980s, many felt ARF had all but disappeared from the US. However, although ARF and rheumatic heart disease (RHD) rates remain low in the US today, disease burden is unequal and tracks along other disparities of cardiovascular health. It is estimated that 1% to 3% of patients with untreated group A streptococcus (GAS) infection, most typically GAS pharyngitis, will develop ARF, and of these, up to 60% of cases will result in chronic RHD. This article reviews the epidemiology, pathogenesis, diagnosis, and management of ARF/RHD to increase awareness of ARF/RHD for clinicians based in the US. [Pediatr Ann. 2021;50(3):e98–e104.]

Echocardiography screening for rheumatic heart disease (RHD) has gained support as a public health approach, but scale up of RHD screening services is complex. We sought to evaluate the effectiveness of a novel training program to build non-expert competency for RHD echocardiography screening within the Uganda public health system and to describe the human and material resources required to support it. Guided by a logic model, we evaluated the Accelerating Delivery of Rheumatic Heart Disease Prevention in Northern Uganda (ADUNU) Program, a novel RHD control program, 15 months after its implementation within the Ugandan public health care system. Sixty-one healthcare workers (HCW) across 10 public health facilities started in training under the program, of which 58 (95%) advanced past the initial stage of training and earned conditional certification to screen for RHD with ongoing remote and in-person feedback and oversight. Of these, 17 (29%) completed all stages of training and earned full certification to independently screen for RHD with no ongoing oversight. A total of 17,927 community members were screened through ADUNU during the program's first 15 months. After receiving final certification, 14 HCWs (93%) continued to perform screening echocardiograms (≥20/month) at median follow-up of 8 months [IQR 8-10]. HCW sensitivity and specificity were 61% and 96%, respectively. Development and deployment of a large scale RHD screening echocardiography training program within an existing public health system is feasible. Future program iterations are needed to improve HCW screening sensitivity and decrease the reliance on human resources.

Background: Rheumatic heart disease (RHD) is a key contributor to maternal cardiovascular morbidity and mortality in sub-Saharan Africa (SSA). Though low- and middle-income countries (LMICs), particularly those in SSA, face a greater burden of RHD, existing systematic reviews have not specifically focused on cardiac and obstetric complications among affected women. We aimed to study cardiac and obstetric complications in pregnant and postpartum women with RHD in SSA and to evaluate the rate of valvular interventions in pregnant or postpartum women with severe disease. Methods: We performed a systematic search in MEDLINE and online sources for studies of women of childbearing age (15–49 years) with RHD published after 2000 in SSA. Included study types were randomized controlled trials, retrospective and prospective cohort studies, case-control studies, case reports, and case series. Two authors independently extracted data and critically appraised articles. PROSPERO registration number: CRD42024628121. Results: We identified 1,478 unique citations, and nine full-text studies met inclusion criteria. Included studies were case series (7), one cohort study, and one case-control study, including a total of 787 pregnant women with cardiac disease, of whom the majority had RHD. Mitral stenosis and regurgitation were the most common valve lesions. Heart failure and arrhythmia occurred in at least 12.9% and up to 36% of study participants, respectively. Eight studies reported deaths due to cardiac causes (median: six deaths due to cardiac disease; total number of deaths: 56). Preterm labor/delivery was the most reported obstetric event, with incidence ranging from 5.2–35.2%. Few pregnant patients received any valve intervention. Conclusions: Pregnant women with RHD in SSA are at risk for both adverse cardiac and obstetric outcomes in pregnancy, particularly heart failure and preterm labor. Future efforts may include registries focused on pregnant women with RHD and scaling cardiac interventional capacity to benefit pregnant women with RHD in SSA.   Unstructured Abstract We performed a systematic search in MEDLINE and online sources to study cardiac and obstetric complications and rates of valvular interventions in pregnant and postpartum women with rheumatic heart disease (RHD) in sub-Saharan Africa (SSA). Two authors independently extracted data and critically appraised articles. Nine full-text studies met inclusion criteria, capturing 787 pregnant women with cardiac disease, mostly RHD. Heart failure and arrhythmia occurred in at least 12.9% and up to 36% of study participants, respectively. Fifty-six deaths were reported from cardiac causes. Preterm labor/delivery was the most reported obstetric event, and few pregnant patients received any valve intervention. We found that women with RHD in SSA are at risk for adverse cardiac and obstetric outcomes in pregnancy, particularly heart failure and preterm labor. Future efforts may include registries focused on pregnant women with RHD and scaling cardiac interventional capacity to benefit pregnant women with RHD in SSA.

ObjectiveTo determine the ability to accurately diagnose acute rheumatic fever (ARF) given the resources available at three levels of the Ugandan healthcare system.MethodsUsing data obtained from a large epidemiological database on ARF conducted in three districts of Uganda, we selected variables that might positively or negatively predict rheumatic fever based on diagnostic capacity at three levels/tiers of the Ugandan healthcare system. Variables were put into three statistical models that were built sequentially. Multiple logistic regression was used to estimate ORs and 95% CI of predictors of ARF. Performance of the models was determined using Akaike information criterion, adjusted R2, concordance C statistic, Brier score and adequacy index.ResultsA model with clinical predictor variables available at a lower-level health centre (tier 1) predicted ARF with an optimism corrected area under the curve (AUC) (c-statistic) of 0.69. Adding tests available at the district level (tier 2, ECG, complete blood count and malaria testing) increased the AUC to 0.76. A model that additionally included diagnostic tests available at the national referral hospital (tier 3, echocardiography, anti-streptolysin O titres, erythrocyte sedimentation rate/C-reactive protein) had the best performance with an AUC of 0.91.ConclusionsReducing the burden of rheumatic heart disease in low and middle-income countries requires overcoming challenges of ARF diagnosis. Ensuring that possible cases can be evaluated using electrocardiography and relatively simple blood tests will improve diagnostic accuracy somewhat, but access to echocardiography and tests to confirm recent streptococcal infection will have the greatest impact.

To address workforce shortages and expand access to care, we developed a telemedicine program incorporating existing infrastructure for delivery of cardiovascular care in Gulu, Northern Uganda. Our study had three objectives: 1) assess feasibility and clinical impact 2) evaluate patient/parent satisfaction and 3) estimate costs. All cardiology clinic visits during a two-year study period were included. All patients received an electrocardiogram and echocardiogram performed by a local nurse in Gulu which were stored and transmitted to the Uganda Heart Institute in the capital of Kampala for remote consultation by a cardiologist. Results were relayed to patients/families following cardiologist interpretation. The following telemedicine process was utilized: 1) clinical intake by nurse in Gulu; 2) ECG and echocardiography acquisition in Gulu; 3) echocardiography transmission to the Uganda Heart Institute in Kampala, Uganda; 4) remote telemedicine consultation by cardiologists in Kampala; and 5) communication of results to patients/families in Gulu. Clinical care and technical aspects were tracked. Diagnoses and recommendations were analyzed by age groups (0-5 years, 6-21 years, 22-50 years and > 50 years). A mixed methods approach involving interviews and surveys was used to assess patient satisfaction. Healthcare sector costs of telemedicine-based cardiovascular care were estimated using time-driven activity-based costing. Normal studies made up 47%, 55%, 76% and 45% of 1,324 patients in the four age groups from youngest to oldest. Valvular heart disease (predominantly rheumatic heart disease) was the most common diagnosis in the older three age groups. Medications were prescribed to 31%, 31%, 24%, and 48% of patients in the four age groups. The median time for consultation was 7 days. A thematic analysis of focus group transcripts displayed an overall acceptance and appreciation for telemedicine, citing cost- and time-saving benefits. The cost of telemedicine was $29.48/visit. Our data show that transmission and interpretation of echocardiograms from a remote clinic in northern Uganda is feasible, serves a population with a high burden of heart disease, has a significant impact on patient care, is favorably received by patients, and can be delivered at low cost. Further study is needed to better assess the impact relative to existing standards of care and cost effectiveness.

In 2018, the World Health Assembly mandated Member States to take action on rheumatic heart disease (RHD), which persists in countries with weak health systems. We conducted an assessment of the current state of RHD-related healthcare in Uganda. This was a mixed-methods, deductive simultaneous design study conducted in four districts of Uganda. Using census sampling, we surveyed health facilities in each district using an RHD survey instrument that was modeled after the WHO SARA tool. We interviewed health workers with experience managing RHD, purposively sampling to ensure a range of qualification and geographic variation. Our final sample included 402 facilities and 36 health workers. We found major gaps in knowledge of clinical guidelines and availability of diagnostic tests. Antibiotics used in RHD prevention were widely available, but cardiovascular medications were scarce. Higher levels of service readiness were found among facilities in the western region (Mbarara district) and private facilities. Level III health centers were the most prepared for delivering secondary prevention. Health worker interviews revealed that limited awareness of RHD at the district level, lack of diagnostic tests and case management registries, and absence of clearly articulated RHD policies and budget prioritization were the main barriers to providing RHD-related healthcare. Uganda's readiness to implement the World Health Assembly RHD Resolution is low. The forthcoming national RHD strategy must focus on decentralizing RHD diagnosis and prevention to the district level, emphasizing specialized training of the primary healthcare workforce and strengthening supply chains of diagnostics and essential medicines.

Opinion statement Early recognition of group A streptococcal pharyngitis and appropriate management with benzathine penicillin using local clinical prediction rules together with validated rapi-strep testing when available should be incorporated in primary health care. A directed approach to the differential diagnosis of acute rheumatic fever now includes the concept of low-risk versus medium-to-high risk populations. Initiation of secondary prophylaxis and the establishment of early medium to long-term care plans is a key aspect of the management of ARF. It is a requirement to identify high-risk individuals with RHD such as those with heart failure, pregnant women, and those with severe disease and multiple valve involvement. As penicillin is the mainstay of primary and secondary prevention, further research into penicillin supply chains, alternate preparations and modes of delivery is required.