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Background: There is an unmet surgical burden among people living with rheumatic heart disease (RHD) in Uganda. Nevertheless, risk factors associated with time to first intervention and preoperative mortality are poorly understood., Methods: Individuals with RHD who met indications for valve surgery were identified using the Uganda National RHD Registry (January 2010-August 2022). Kaplan-Meier estimates and multivariable Cox proportional hazard models were used., Results: Of the cohort with clinical RHD, 64% (1452 of 2269) met criteria for an index operation. Of those, 13.5% obtained a surgical intervention, whereas 30.6% died before the operation. The estimated likelihood of first operation was 50% at 9.3 years of follow-up (95% CI, 8.1-upper limit not reached). Intervention was more likely in men vs women (hazard ratio [HR], 1.78; 95% CI, 1.21-2.64), those with postsecondary education vs primary school or less (HR, 3.60; 95% CI, 1.88-6.89), and those with a history of atrial fibrillation (HR, 2.78; 95% CI, 1.63-4.76). Surgical intervention was less likely for adults vs those aged <18 years (HR, 0.49; 95% CI, 0.32-0.77) and those with New York Heart Association Functional Class III/IV vs I/II (HR, 0.51; 95% CI, 0.32-0.83). The median preoperative survival time among those awaiting first operation was 4.6 years (95% CI, 3.9-5.7 years). History of infective endocarditis, right ventricular dysfunction, pericardial effusion, atrial fibrillation, and having surgical indications for multiple valves were associated with increased probability of death., Conclusions: Our analysis revealed a prolonged time to first surgical intervention and high preintervention death for RHD in Uganda, with factors such as age, sex, and education level remaining barriers to obtaining surgery., Competing Interests: Disclosures The authors have no conflicts of interest to disclose., (Copyright © 2024 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A 19-month-old boy presented with a murmur and was found to have an unusual etiology of subvalvar aortic stenosis with discrete subaortic membrane and anomalous attachment of the anterior mitral valve papillary muscle to the interventricular septum. Preoperative suspicion for mitral valve involvement impacted surgical planning., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Background: Health-related quality of life (HRQOL) is a critical issue for patients undergoing surgery for congenital heart disease (CHD) but has never been assessed in a low-income country. We conducted a cross-sectional mixed methods study with age-matched healthy siblings serving as controls at the Uganda Heart Institute., Methods: One-hundred fifteen CHD pediatric and young adult patients and sibling control participants were recruited. Health-related quality of life was assessed using the Pediatric Quality of Life Inventory Version 4.0 in participants ages 5-17 and 36-Item Short Form Survey for young adults aged 18-25. A subset of 27 participants completed face-to-face interviews to supplement quantitative findings., Results: Eighty-six pediatric (age 5-17) sibling and parent pairs completed Peds QOL surveys, and 29 young adult (age 18-25) sibling pairs completed SF-36 surveys. One third of patients had surgery in Uganda. Ventricular septal defects and tetralogy of Fallot were the most common diagnoses. Health-related quality of life scores in patients were lower across all domains compared to control participants in children. Reductions in physical and emotional domains of HRQOL were also statistically significant for young adults. Variables associated with lower HRQOL score on multivariate analysis in pediatric patients were younger age in the physical and emotional domains, greater number of surgeries in the physical domain and surgery outside Uganda in the school domain. The only predictor of lower HRQOL score in young adults was surgery outside Uganda in the social domain. Qualitative interviews identified a number of themes that correlated with survey results including abandonment by family, isolation from peers and community, financial hardship and social stigmatization., Conclusion: Health-related quality of life was lower in Ugandan patients after CHD surgery than siblings. Younger patients and those who had surgery outside of Uganda had lower HRQOL. These data have important implications for patients undergoing CHD surgery in LMIC and have potential to inform interventions., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2024 The Author(s).)

Background: Secondary antibiotic prophylaxis reduces progression of latent rheumatic heart disease (RHD) but not all children benefit. Improved risk stratification could refine recommendations following positive screening. We aimed to evaluate the performance of a previously developed echocardiographic risk score to predict mid-term outcomes among children with latent RHD., Methods: We included children who completed the GOAL, a randomized trial of secondary antibiotic prophylaxis among children with latent RHD in Uganda. Outcomes were determined by a 4-member adjudication panel. We applied the point-based score, consisting of 5 variables (mitral valve (MV) anterior leaflet thickening (3 points), MV excessive leaflet tip motion (3 points), MV regurgitation jet length ≥ 2 cm (6 points), aortic valve focal thickening (4 points) and any aortic regurgitation (5 points)), to panel results. Unfavorable outcome was defined as progression of diagnostic category (borderline to definite, mild definite to moderate/severe definite), worsening valve involvement or remaining with mild definite RHD., Results: 799 patients (625 borderline and 174 definite RHD) were included, with median follow-up of 24 months. At total 116 patients (14.5%) had unfavorable outcome per study criteria, 57.8% not under prophylaxis. The score was strongly associated with unfavorable outcome (HR = 1.26, 95% CI 1.16-1.37, p < 0.001). Unfavorable outcome rates in low (≤6 points), intermediate (7-9 points) and high-risk (≥10 points) children at follow-up were 11.8%, 30.4%, and 42.2%, (p < 0.001) respectively (C-statistic = 0.64 (95% CI 0.59-0.69))., Conclusions: The simple risk score provided an accurate prediction of RHD status at 2-years, showing a good performance in a population with milder RHD phenotypes., Competing Interests: Declaration of Competing Interest The authors of the manuscript have no relevant conflicts of interest to disclose regarding this study., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Identification of children with latent rheumatic heart disease (RHD) by echocardiography, before onset of symptoms, provides an opportunity to initiate secondary prophylaxis and prevent disease progression. There have been limited artificial intelligence studies published assessing the potential of machine learning to detect and analyze mitral regurgitation or to detect the presence of RHD on standard portable echocardiograms., Methods and Results: We used 511 echocardiograms in children, focusing on color Doppler images of the mitral valve. Echocardiograms were independently reviewed by an expert adjudication panel. Among 511 cases, 229 were normal, and 282 had RHD. Our automated method included harmonization of echocardiograms to localize the left atrium during systole using convolutional neural networks and RHD detection using mitral regurgitation jet analysis and deep learning models with an attention mechanism. We identified the correct view with an average accuracy of 0.99 and the correct systolic frame with an average accuracy of 0.94 (apical) and 0.93 (parasternal long axis). It localized the left atrium with an average Dice coefficient of 0.88 (apical) and 0.9 (parasternal long axis). Maximum mitral regurgitation jet measurements were similar to expert manual measurements ( P value=0.83) and a 9-feature mitral regurgitation analysis showed an area under the receiver operating characteristics curve of 0.93, precision of 0.83, recall of 0.92, and F1 score of 0.87. Our deep learning model showed an area under the receiver operating characteristics curve of 0.84, precision of 0.78, recall of 0.98, and F1 score of 0.87., Conclusions: Artificial intelligence has the potential to detect RHD as accurately as expert cardiologists and to improve with more data. These innovative approaches hold promise to scale echocardiography screening for RHD.

There is limited information on the human immune response following infection with group A Streptococcus (Strep A). Animal studies have shown, in addition to the M protein, that shared Strep A antigens elicit protective immunity. This study aimed to investigate the kinetics of antibody responses against a panel of Strep A antigens in a cohort of school-aged children in Cape Town, South Africa. Participants provided serial throat cultures and serum samples at two-monthly follow-up visits. Strep A recovered were emm -typed, and serum samples were analyzed by enzyme-linked immunosorbent assay (ELISA) to assess immune responses to thirty-five Strep A antigens (10-shared and 25-M peptides). Serologic evaluations were performed on serial serum samples from 42 selected participants (from 256 enrolled) based on the number of follow-up visits, the frequency of visits, and throat culture results. Among these, there were 44 Strep A acquisitions, 36 of which were successfully emm -typed. Participants were grouped into three clinical event groups based on culture results and immune responses. A preceding infection was most convincingly represented by a Strep A-positive culture with an immune response to at least one shared antigen and M peptide (11 events) or a Strep A-negative culture with antibody responses to shared antigens and M peptides (9 events). More than a third of participants demonstrated no immune response despite a positive culture. This study provided important information regarding the complexity and variability of human immune responses following pharyngeal acquisition of Strep A, as well as demonstrating the immunogenicity of Strep A antigens currently under consideration as potential vaccine candidates. IMPORTANCE There is currently limited information regarding the human immune response to group A streptococcal throat infection. An understanding of the kinetics and specificity of antibody responses against a panel of Group A Streptococcus (GAS) antigens will serve to refine diagnostic approaches and contribute to vaccine efforts, which together will serve to reduce the burden of rheumatic heart disease, a major source of morbidity and mortality especially in the developing world. This study, utilizing an antibody-specific assay, uncovered three patterns of response profiles following GAS infection, among 256 children presenting with sore throat to local clinics. Overall, the response profiles were complex and variable. Of note, a preceding infection was most convincingly represented by a GAS-positive culture with an immune response to at least one shared antigen and M peptide. Also, more than a third of participants demonstrated no immune response despite a positive culture. All antigens tested were immunogenic, providing guidance for future vaccine development., Competing Interests: The authors declare a conflict of interest. James B. Dale is the inventor of certain technologies related to the development of group A streptococcal vaccines. The intellectual property is held by the University of Tennessee Research Foundation.

We aimed to evaluate the reasons for disagreement between screening echocardiography (echo), acquired by nonexperts, and standard echo in the Brazilian primary care (PC). Over 20 months, 22 PC workers were trained on simplified handheld (GE VSCAN) echo protocols. Screening groups, consisting of patients aged 17-20, 35-40 and 60-65 years, and patients referred for clinical indications underwent focused echo. Studies were remotelyinterpreted in US and Brazil, and those diagnosed with major or severe HD were referred for standard echoperformed by an expert. Major HD was defined as moderate to severe valve disease, ventriculardysfunction/hypertrophy, pericardial effusion or wall-motion abnormalities. A random sample of exams wasselected for evaluation of variables accounting for disagreement. A sample of 768 patients was analyzed, 651(85%) in the referred group. Quality issues were reported in 5.8%, and the random Kappa for major HD between screening and standard echo was 0.51. The most frequent reasons for disagreement were: overestimation of mitral regurgitation (MR) (17.9%, N=138), left ventricular (LV) dysfunction (15.7%, N=121), aortic regurgitation (AR) (15.2%, N=117), LV hypertrophy (13.5%, N=104) and tricuspid regurgitation (12.7%, N=98). Misdiagnosis of mitral and aortic morphological abnormalities was observed in 12.4% and 3.0%, and underestimation of AR and MR occurred in 4.6% and 11.1%. Among 257 patients with suspected mild/moderate MR, 129 were reclassified to normal. In conclusion, although screening echo with task-shifting in PC is a promising tool in low-income areas, estimation of valve regurgitation and LV function and size account for considerable disagreement with standard exams., (© 2023. The Author(s), under exclusive licence to Springer Nature B.V.)

Background: A novel paediatric disease, multi-system inflammatory syndrome in children, has emerged during the 2019 coronavirus disease pandemic., Objectives: To describe the short-term evolution of cardiac complications and associated risk factors in patients with multi-system inflammatory syndrome in children., Methods: Retrospective single-centre study of confirmed multi-system inflammatory syndrome in children treated from 29 March, 2020 to 1 September, 2020. Cardiac complications during the acute phase were defined as decreased systolic function, coronary artery abnormalities, pericardial effusion, or mitral and/or tricuspid valve regurgitation. Patients with or without cardiac complications were compared with chi-square, Fisher's exact, and Wilcoxon rank sum., Results: Thirty-nine children with median (interquartile range) age 7.8 (3.6-12.7) years were included. Nineteen (49%) patients developed cardiac complications including systolic dysfunction (33%), valvular regurgitation (31%), coronary artery abnormalities (18%), and pericardial effusion (5%). At the time of the most recent follow-up, at a median (interquartile range) of 49 (26-61) days, cardiac complications resolved in 16/19 (84%) patients. Two patients had persistent mild systolic dysfunction and one patient had persistent coronary artery abnormality. Children with cardiac complications were more likely to have higher N-terminal B-type natriuretic peptide (p = 0.01), higher white blood cell count (p = 0.01), higher neutrophil count (p = 0.02), severe lymphopenia (p = 0.05), use of milrinone (p = 0.03), and intensive care requirement (p = 0.04)., Conclusion: Patients with multi-system inflammatory syndrome in children had a high rate of cardiac complications in the acute phase, with associated inflammatory markers. Although cardiac complications resolved in 84% of patients, further long-term studies are needed to assess if the cardiac abnormalities (transient or persistent) are associated with major cardiac events.

Secondary antibiotic prophylaxis with regular intramuscular benzathine penicillin G (BPG) is the cornerstone of rheumatic heart disease management. However, there is a growing body of evidence that patients with rheumatic heart disease who have severe valvular heart disease with or without reduced ventricular function may be dying from cardiovascular compromise following BPG injections. This advisory responds to these concerns and is intended to: (1) raise awareness, (2) provide risk stratification, and (3) provide strategies for risk reduction. Based on available evidence and expert opinion, we have divided patients into low- and elevated-risk groups, based on symptoms and the severity of underlying heart disease. Patients with elevated risk include those with severe mitral stenosis, aortic stenosis, and aortic insuffiency; those with decreased left ventricular systolic dysfunction; and those with no symptoms. For these patients, we believe the risk of adverse reaction to BPG, specifically cardiovascular compromise, may outweigh its theoretical benefit. For patients with elevated risk, we newly advise that oral prophylaxis should be strongly considered. In addition, we advocate for a multifaceted strategy for vasovagal risk reduction in all patients with rheumatic heart disease receiving BPG. As current guidelines recommend, all low-risk patients without a history of penicillin allergy or anaphylaxis should continue to be prescribed BPG for secondary antibiotic prophylaxis. We publish this advisory in the hopes of saving lives and avoiding events that can have devastating effects on patient and clinician confidence in BPG.

We aimed to use echocardiographic (echo) screening to evaluate the risk of Rheumatic Heart Disease (RHD) among the relatives of patients with advanced RHD, who were enrolled in the University Hospital's outpatient clinics from February 2020 to September 2021. Consenting first-degree relatives were invited for echo screening using handheld devices (GE VSCAN) by non-physicians, with remote interpretation. Matched controls (spouses, neighbors) living in the same household were enrolled in a 1:5 fashion. A standard echo (GE Vivid-IQ) was scheduled if abnormalities were observed. In 16 months, 226 relatives and 47 controls of 121 patients were screened, including 129 children, 77 siblings and 20 parents. The mean age was 40 ± 17 years, 67% of the patients were women, and 239 (88%) lived with the index case for >10 years. Echo findings suggestive of RHD were confirmed in zero controls and 14 (7.5%) relatives ( p = 0.05): 11 patients had mild/moderate mitral regurgitation, and four were associated with mitral stenosis and abnormal morphology. Two patients had mild aortic regurgitation and abnormal morphology, which were associated with mild aortic and mitral stenosis, and two patients with advanced RHD had bioprostheses in the mitral (2) and aortic (1) positions. In conclusion, first-degree relatives of individuals with clinical RHD are at greater risk of having RHD, on top of socioeconomic conditions.

Background: Rheumatic heart disease affects more than 40.5 million people worldwide and results in 306,000 deaths annually. Echocardiographic screening detects rheumatic heart disease at an early, latent stage. Whether secondary antibiotic prophylaxis is effective in preventing progression of latent rheumatic heart disease is unknown., Methods: We conducted a randomized, controlled trial of secondary antibiotic prophylaxis in Ugandan children and adolescents 5 to 17 years of age with latent rheumatic heart disease. Participants were randomly assigned to receive either injections of penicillin G benzathine (also known as benzathine benzylpenicillin) every 4 weeks for 2 years or no prophylaxis. All the participants underwent echocardiography at baseline and at 2 years after randomization. Changes from baseline were adjudicated by a panel whose members were unaware of the trial-group assignments. The primary outcome was echocardiographic progression of latent rheumatic heart disease at 2 years., Results: Among 102,200 children and adolescents who had screening echocardiograms, 3327 were initially assessed as having latent rheumatic heart disease, and 926 of the 3327 subsequently received a definitive diagnosis on the basis of confirmatory echocardiography and were determined to be eligible for the trial. Consent or assent for participation was provided for 916 persons, and all underwent randomization; 818 participants were included in the modified intention-to-treat analysis, and 799 (97.7%) completed the trial. A total of 3 participants (0.8%) in the prophylaxis group had echocardiographic progression at 2 years, as compared with 33 (8.2%) in the control group (risk difference, -7.5 percentage points; 95% confidence interval, -10.2 to -4.7; P<0.001). Two participants in the prophylaxis group had serious adverse events that were attributable to receipt of prophylaxis, including one episode of a mild anaphylactic reaction (representing <0.1% of all administered doses of prophylaxis)., Conclusions: Among children and adolescents 5 to 17 years of age with latent rheumatic heart disease, secondary antibiotic prophylaxis reduced the risk of disease progression at 2 years. Further research is needed before the implementation of population-level screening can be recommended. (Funded by the Thrasher Research Fund and others; GOAL ClinicalTrials.gov number, NCT03346525.)., (Copyright © 2021 Massachusetts Medical Society.)

Background: Congenital heart disease (CHD) is the leading cause of morbidity and mortality from birth defects worldwide. We report an overview of trends in CHD mortality in 204 countries and territories over the past 30 years and associations with age, period, and birth cohort., Methods: Cause-specific CHD mortality estimates were derived from the Global Burden of Disease 2019 study. We utilised an age-period-cohort model to estimate overall annual percentage changes in mortality (net drifts), annual percentage changes from 0 to 4 to 65-69 years (local drifts), period and cohort relative risks (period/cohort effects) between 1990 and 2019. This approach allows for the examination and differentiation of age, period, and cohort effects in the mortality trends, with the potential to identify disparities and treatment gaps in cardiac care., Findings: CHD is the leading cause of deaths from non-communicable diseases (NCDs) in those under 20 years. Global CHD deaths in 2019 were 217,000 (95% uncertainty interval 177,000-262,000). There were 129 countries with at least 50 deaths. India, China, Pakistan, and Nigeria had the highest mortality, accounting for 39.7% of deaths globally. Between 1990 and 2019, the net drift of CHD mortality ranged from -2.41% per year (95% confidence interval [CI] -2.55, -2.67) in high Socio-demographic Index (SDI) countries to -0.62% per year (95% CI: -0.82, -0.42) in low-SDI countries. Globally, there was an emerging transition in the age distribution of deaths from paediatric to adult populations, except for an increasing trend of mortality in those aged 10-34 years in Mexico and Pakistan. During the past 30 years, favourable mortality reductions were generally found in most high-SDI countries like South Korea (net drift = -4.0% [95% CI -4.8 to -3.1] per year) and the United States (-2.3% [-2.5 to -2.0]), and also in many middle-SDI countries like Brazil (-2.7% [-3.1 to 2.4]) and South Africa (-2.5% [-3.2 to -1.8]). However, 52 of 129 countries had either increasing trends (net drifts ≥0.0%) or stagnated reductions (≥-0.5%) in mortality. The relative risk of mortality generally showed improving trends over time and in successively younger birth cohorts amongst high- and high-middle-SDI countries, with the exceptions of Saudi Arabia and Kazakhstan. 14 middle-SDI countries such as Ecuador and Mexico, and 16 low-middle-SDI countries including India and 20 low-SDI countries including Pakistan, had unfavourable or worsening risks for recent periods and birth cohorts., Interpretation: CHD mortality is a useful and accessible indicator of trends in the provision of congenital cardiac care both in early childhood and across later life. Improvements in the treatment of CHD should reduce the risk for successively younger cohorts and shift the risk for all age groups over time. Although there were gains in CHD mortality globally over the past three decades, unfavourable period and cohort effects were found in many countries, raising questions about adequacy of their health care for CHD patients across all age groups. These failings carry significant implications for the likelihood of achieving the Sustainable Development Goal targets for under-5 years and NCD mortality., Funding: Supported by the National Natural Science Foundation of China (81525002, 31971048, 82073573 to ZZ and HZ), Shanghai Outstanding Medical Academic Leader program (2019LJ22 to HZ), and Collaborative Innovation Program of Shanghai Municipal Health Commission (2020CXJQ01 to HZ), the Bill & Melinda Gates Foundation for the Global Burden of Disease Project (to NJK) and NHMRC fellowship administered through the University of Melbourne (to GCP)., Competing Interests: NJK reports consulting fees from Merck as a one-day consultation on a potential public visualization tool. All the other authors declare no competing interests., (© 2022 Published by Elsevier Ltd.)

Background: This study aims to describe one center's experience in expanding a fetal telecardiology program through collaborative work with maternal fetal medicine (MFM) clinics with the goal of safely reaching mothers during the COVID-19 pandemic. We sought to define the extent of fetal telehealth conversion at a large fetal cardiac care center and evaluate the diagnostic accuracy for studies performed. Methods: At our center, fetal telemedicine expanded from one MFM site before the pandemic to four additional sites by May 2020. A retrospective review of fetal telecardiology visits between March 15 and July 15, 2020, was performed. The chart was reviewed for confirmation of diagnosis postnatally. Results: With pandemic onset, there was a large increase in the number of telemedicine visits with a total of 122 mothers seen between five MFM clinics. Fourteen mothers (11.5%) had abnormal fetal echocardiograms requiring additional follow-up, and seven mothers (5.8%) had a fetal echocardiogram suspicious for a critical congenital heart disease (CCHD). All the fetal echocardiograms suspicious for CCHD were confirmed on postnatal echocardiogram. To our knowledge, none of the normal fetal echocardiograms were found to have congenital heart disease postnatally. Conclusions: In response to the COVID-19 pandemic, we rapidly transitioned to fetal telecardiology using a variety of formats. This has reduced potential infectious exposure for pregnant mothers and minimized contact between physicians without compromising diagnostic accuracy. In our experience, the expansion of a telemedicine program requires strong initial infrastructure, prior relationships with MFM providers, and appropriate training among obstetric sonographers.

We performed a retrospective study of cardiology telemedicine visits at a large academic pediatric center between 2016 and 2019 (pre COVID-19). Telemedicine patient visits were matched to data from their previous in-person visits, to evaluate any significant differences in total charge, insurance compensation, patient payment, percent reimbursement and zero reimbursement. Miles were measured between patient's home and the address of previous visit. We found statistically significant differences in mean charges of telemedicine versus in-person visits (2019US$) (172.95 vs 218.27, p=0.0046), patient payment for telemedicine visits versus in-person visits (2019US$) (11.13 vs 62.83, p≤0.001), insurance reimbursement (2019US$) (65.18 vs 110.85, p≤0.001) and insurance reimbursement rate (43% vs 61%, p=0.0029). Rate of zero reimbursement was not different. Mean distance from cardiology clinic was 35 miles. No adverse outcomes were detected. This small retrospective study showed cost reduction and a decrease in travel time for families participating in telemedicine visits. Future work is needed to enhance compensation for telemedicine visits., Competing Interests: Competing interests: None declared., (© American Federation for Medical Research 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Acute rheumatic fever is infrequently diagnosed in sub-Saharan African countries despite the high prevalence of rheumatic heart disease. We aimed to determine the incidence of acute rheumatic fever in northern and western Uganda., Methods: For our prospective epidemiological study, we established acute rheumatic fever clinics at two regional hospitals in the north (Lira district) and west (Mbarara district) of Uganda and instituted a comprehensive acute rheumatic fever health messaging campaign. Communities and health-care workers were encouraged to refer children aged 3-17 years, with suspected acute rheumatic fever, for a definitive diagnosis using the Jones Criteria. Children were referred if they presented with any of the following: (1) history of fever within the past 48 h in combination with any joint complaint, (2) suspicion of acute rheumatic carditis, or (3) suspicion of chorea. We excluded children with a confirmed alternative diagnosis. We estimated incidence rates among children aged 5-14 years and characterised clinical features of definite and possible acute rheumatic fever cases., Findings: Data were collected between Jan 17, 2018, and Dec 30, 2018, in Lira district and between June 5, 2019, and Feb 28, 2020, in Mbarara district. Of 1075 children referred for evaluation, 410 (38%) met the inclusion criteria; of these, 90 (22%) had definite acute rheumatic fever, 82 (20·0%) had possible acute rheumatic fever, and 24 (6%) had rheumatic heart disease without evidence of acute rheumatic fever. Additionally, 108 (26%) children had confirmed alternative diagnoses and 106 (26%) had an unknown alternative diagnosis. We estimated the incidence of definite acute rheumatic fever among children aged 5-14 years as 25 cases (95% CI 13·7-30·3) per 100 000 person-years in Lira district (north) and 13 cases (7·1-21·0) per 100 000 person-years in Mbarara district (west)., Interpretation: To the best of our knowledge, this is the first population-based study to estimate the incidence of acute rheumatic fever in sub-Saharan Africa. Given the known rheumatic heart disease burden, it is likely that only a proportion of children with acute rheumatic fever were diagnosed. These data dispel the long-held hypothesis that the condition does not exist in sub-Saharan Africa and compel investment in improving prevention, recognition, and diagnosis of acute rheumatic fever., Funding: American Heart Association Children's Strategically Focused Research Network Grant, THRiVE-2, General Electric, and Cincinnati Children's Heart Institute Research Core., Competing Interests: Declaration of interests TP reports grant funding from the National Institute for Health Research (ACF-2016-20-001). All other authors declare no competing interests., (Copyright © 2021 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY-NC-ND 4.0 license. Published by Elsevier Ltd.. All rights reserved.)

Objective: Rheumatic heart disease (RHD) affects an estimated 39 million people worldwide and is the most common acquired heart disease in children and young adults. Echocardiograms are the gold standard for diagnosis of RHD, but there is a shortage of skilled experts to allow widespread screenings for early detection and prevention of the disease progress. We propose an automated RHD diagnosis system that can help bridge this gap., Materials and Methods: Experiments were conducted on a dataset with 11 646 echocardiography videos from 912 exams, obtained during screenings in underdeveloped areas of Brazil and Uganda. We address the challenges of RHD identification with a 3D convolutional neural network (C3D), comparing its performance with a 2D convolutional neural network (VGG16) that is commonly used in the echocardiogram literature. We also propose a supervised aggregation technique to combine video predictions into a single exam diagnosis., Results: The proposed approach obtained an accuracy of 72.77% for exam diagnosis. The results for the C3D were significantly better than the ones obtained by the VGG16 network for videos, showing the importance of considering the temporal information during the diagnostic. The proposed aggregation model showed significantly better accuracy than the majority voting strategy and also appears to be capable of capturing underlying biases in the neural network output distribution, balancing them for a more correct diagnosis., Conclusion: Automatic diagnosis of echo-detected RHD is feasible and, with further research, has the potential to reduce the workload of experts, enabling the implementation of more widespread screening programs worldwide., (© The Author(s) 2021. Published by Oxford University Press on behalf of the American Medical Informatics Association. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Introduction: Access to public subspecialty healthcare is limited in underserved areas of Brazil, including echocardiography (echo). Long waiting lines and lack of a prioritisation system lead to diagnostic lag and may contribute to poor outcomes. We developed a prioritisation tool for use in primary care, aimed at improving resource utilisation, by predicting those at highest risk of having an abnormal echo, and thus in highest need of referral., Methods: All patients in the existing primary care waiting list for echo were invited for participation and underwent a clinical questionnaire, simplified 7-view echo screening by non-physicians with handheld devices, and standard echo by experts. Two derivation models were developed, one including only clinical variables and a second including clinical variables and findings of major heart disease (HD) on echo screening (cut point for high/low-risk). For validation, patients were risk-classified according to the clinical score. High-risk patients and a sample of low-risk underwent standard echo. Intermediate-risk patients first had screening echo, with a standard echo if HD was suspected. Discrimination and calibration of the two models were assessed to predict HD in standard echo., Results: In derivation (N = 603), clinical variables associated with HD were female gender, body mass index, Chagas disease, prior cardiac surgery, coronary disease, valve disease, hypertension and heart failure, and this model was well calibrated with C-statistic = 0.781. Performance was improved with the addition of echo screening, with C-statistic = 0.871 after cross-validation. For validation (N = 1526), 227 (14.9%) patients were classified as low risk, 1082 (70.9%) as intermediate risk and 217 (14.2%) as high risk by the clinical model. The final model with two categories had high sensitivity (99%) and negative predictive value (97%) for HD in standard echo. Model performance was good with C-statistic = 0.720., Conclusion: The addition of screening echo to clinical variables significantly improves the performance of a score to predict major HD., (© 2020 John Wiley & Sons Ltd.)

Introduction: Inflammation associated with rheumatic heart disease (RHD) is influenced by gene polymorphisms and inflammatory cytokines. There are currently no immunologic and genetic markers to discriminate latent versus clinical patients, critical to predict disease evolution. Employing machine-learning, we searched for predictors that could discriminate latent versus clinical RHD, and eventually identify latent patients that may progress to clinical disease., Methods: A total of 212 individuals were included, 77 with latent, 100 with clinical RHD, and 35 healthy controls. Circulating levels of 27 soluble factors were evaluated using Bio-Plex ProTM® Human Cytokine Standard 27-plex assay. Gene polymorphism analyses were performed using RT-PCR for the following genes: IL2, IL4, IL6, IL10, IL17A, TNF and IL23., Results: Serum levels of all cytokines were higher in clinical as compared to latent RHD patients, and in those groups than in controls. IL-4, IL-8, IL-1RA, IL-9, CCL5 and PDGF emerged in the final multivariate model as predictive factors for clinical, compared with latent RHD. IL-4, IL-8 and IL1RA had the greater power to predict clinical RHD. In univariate analysis, polymorphisms in IL2 and IL4 were associated with clinical RHD and in the logistic analysis, IL6 (GG + CG), IL10 (CT + TT), IL2 (CA + AA) and IL4 (CC) genotypes were associated with RHD., Conclusion: Despite higher levels of all cytokines in clinical RHD patients, IL-4, IL-8 and IL-1RA were the best predictors of clinical disease. An association of polymorphisms in IL2, IL4, IL6 and IL10 genes and clinical RHD was observed. Gene polymorphism and phenotypic expression of IL-4 accurately discriminate latent versus clinical RHD, potentially instructing clinical management., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Cardiac damage from chemotherapy is a known phenomenon leading to significant morbidity and mortality in the cancer surviving population, and identifying high-risk pediatric patients early is challenging. The purpose of this pilot study was to evaluate whether echo strain, cardiac MRI (CMR), and serum biomarkers are more sensitive methods for detecting cardiac toxicity than standard echo and to examine the relationship between biomarkers in patients without decreased systolic function as determined by standard echo. In this pilot study, we prospectively enrolled pediatric subjects after completion of anthracycline inclusive chemotherapy. Each subject underwent a post-treatment echocardiogram (standard with strain), serum biomarkers (N-terminal brain natriuretic peptide (NT-pro-BNP) and interleukin 1 receptor-like 1 protein (ST2)), and CMR (standard and extracellular volumes (ECVs)). We correlated the markers using Pearson correlation. We enrolled 30 subjects, 11F/19M, aged 8-21 years. Cumulative anthracycline dose (CAD) correlated with BNP (p=0.06), CMR ECV 4-chamber (p=0.05) and sagittal (p=0.01), and mitral valve E/A (p=0.02). BNP correlated with CMR ECV 4-chamber (p=0.001) and sagittal (p=0.001) and with echo average longitudinal strain (ALS) (p=0.05). This study demonstrated a significant correlation of CAD with BNP and CMR ECV. There was also a significant correlation of NT-pro-BNP with CMR ECV and ALS. Combining these parameters with standard echo has the potential to identify high-risk patients early. Further studies are needed for long-term follow-up and management in this vulnerable population., Competing Interests: Competing interests: None declared., (© American Federation for Medical Research 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Congenital heart disease (CHD) is the most common congenital malformation. Diagnosis of critical congenital heart disease (CCHD), the most severe type of congenital heart disease, in a newborn may be difficult. The addition of CCHD screening, using pulse oximetry, to clinical assessment significantly improves the rate of detection. We conducted a pilot study in Morocco on screening neonates for critical congenital heart disease. This study was conducted in the maternity ward of Mohammed VI University Hospital of Marrakesh, Morocco, and included asymptomatic newborns delivered between March 2019 and January 2020. The screening of CCHD was performed by pulse oximetry measuring the pre- and post-ductal saturation. Screening was performed on 8013/10,451 (76.7%) asymptomatic newborns. According to the algorithm, 7998 cases passed the screening test (99.82%), including one inconclusive test that was repeated an hour later and was normal. Fifteen newborns failed the screening test (0.18%): five CCHD, five false positives, and five CHD but non-critical. One false negative case was diagnosed at 2 months of age. Our results encourage us to strengthen screening for CCHD by adding pulse oximetry to the routine newborn screening panel., Competing Interests: Conflicts of InterestOne of the pulse oximetry equipment (the Rad 97) was a donation from Masimo Company. The authors declare no other conflict of interest., (© 2020 by the authors.)

Introduction: The burden of rheumatic heart disease (RHD) is still high in Brazil. Lack of population awareness about the disease limits the efficacy of prevention programs. We aimed to evaluate the effectiveness of education on RHD in schools, comparing the conventional expository teaching method with tablet-based worked examples., Method: A prospective, cluster randomized trial was conducted over eight months in six randomly selected low-income Brazilian public schools. Each class was considered a cluster (total: 90), being randomized 1:1 to receive one of the educational methods. Pre-test evaluated students' prior knowledge on RHD. Post-tests, 10 days, and three months later, evaluated retention of knowledge., Results: At total 1,301 students (52% female) completed the study, being 63% from high school. Baseline knowledge about RHD was universally low (average score expository classes [G1] 33.9% vs. worked examples [G2] 32.5%, p = 0.23). A significant but similar improvement was observed in both groups in the immediate post-test (pre- vs. post: p < 0.001): G1 57.5% vs. G2 56.7%, p = 0.69. In the late post-test, a significant 20% worsening was observed in both groups and the final scores were again similar: G1 45.0% vs. G2 45.9%, p = 0.87. Highschool students had higher scores (p < 0.001), and girls had better overall performances than boys (p < 0.001)., Conclusion: The novel technology of tablet-based worked examples had similar results compared with expository classes for RHD education in schools. Both educational processes resulted in modest gains in knowledge, with low retention. More studies are needed to determine if increased knowledge leads to behavioral changes that could reduce RHD burden., Highlights: In a cluster-randomized trial, two different educational strategies about Rheumatic Heart Disease (RHD) for children - standard expository classes and worked examples based on interactive modules in tablet computers - were compared in public schools of underserved Brazilian neighborhoods.Baseline knowledge was low, and the novel tablet-based technology had similar results compared with traditional teaching for RHD education in schools, with no differences in acquisition and mid-term retention of knowledge.Both educational interventions resulted in similar 71% improvement in the immediate post-test, although with suboptimal retention, with over 20% worsening in three months in both groups.Our data suggests that the optimal strategy for RHD education is yet to be determined, and future studies should be warranted to determine if increased knowledge leads to behavioral changes that could reduce disease burden in endemic areas., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2020 The Author(s).)

Background: In asymptomatic children, screening echocardiography has been used to attempt to diagnose rheumatic heart disease (RHD) at an early stage (latent RHD). World Heart Federation guidelines have standardized categorization of "definite," "borderline," or no RHD by echo findings. The progression of RHD diagnosed through echo screening is not known. In 2014, we screened 1450 schoolchildren in Malawi., Objective: Our objective was to evaluate 2-year RHD evolution among those diagnosed through screening., Methods: Two-year follow-up echocardiograms of those diagnosed with latent RHD were read by a primary, secondary, then third reader if there was disagreement. Progression or regression of both definite and borderline groups were tabulated. Penicillin adherence, age, gender, number in home, and household income were compared between those with definite RHD who regressed to borderline and those that stayed definite. We utilized the local system used to track HIV defaulters in order to bring participants back into care. Comparisons were made using Fisher's exact and Wilcoxon rank-sum tests., Results: Of the 39 with borderline RHD, 1 was lost to follow-up (2.6%), 1 progressed to definite (2.6%), 19 remained borderline (48.7%), 17 (43.6%) regressed to normal, and 1 was reclassified as mitral valve prolapse (2.6%). Of the 11 with definite RHD, 6 (54.5%) remained definite, 4 regressed to borderline (36.4%), and 1 regressed to normal (9.1%). Two of 11 with definite RHD had penicillin adherence above 80% for the 2-year follow-up period. There were no differences in adherence, gender, age, household income, or number in household between those with definite RHD that regressed to borderline and those who did not (P > .19)., Conclusions: Borderlines had a very low progression rate to definite RHD. A strength of our study was a high retention rate (98%). Longer follow-up is needed to determine expected disease evolution., (© 2019 Wiley Periodicals, Inc.)

Background: There is geographical overlap between areas endemic for rheumatic heart disease (RHD) and those endemic for HIV. A recent pilot study demonstrated that children living with HIV might be at less risk for RHD development; however, the sample size was too small to make definitive conclusions. Our objective was to determine the prevalence of RHD among HIV-positive children in Uganda., Methods: We conducted a prospective, cross-sectional study of HIV-positive children (5-15 years of age) receiving care at the Baylor Uganda HIV Clinic, Kampala, Uganda. A focused echocardiogram and chart review was performed. A sample size of 988 children was needed to provide 80% power to detect a difference in population prevalence between HIV-positive children and the general population, 2.97% [95% confidence interval (CI): 2.70-3.24%], based on previous reports., Results: Screening echocardiography of 993 HIV-positive children found 15 individuals (1.5%; 95% CI: 0.88%-2.54%) with RHD. Of these 15, 2 were classified as definite RHD and 13 as borderline RHD. The majority of children had isolated mitral valve disease (93%). Children found to have RHD were older than those without RHD, 12 versus 10 years of age (P = 0.004). When separated based on geographic location, the prevalence of RHD among HIV-positive children from Kampala was 1.28% (95% CI: 0.63%-2.51%) compared with 2.1% (95% CI: 0.89%-4.89%) in those from outside Kampala., Conclusions: Children living with HIV have a lower prevalence of RHD than the general pediatric population. Further studies are needed to explore this protective association.

Background: Rheumatic heart disease remains an important preventable cause of cardiovascular death and disability, particularly in low-income and middle-income countries. We estimated global, regional, and national trends in the prevalence of and mortality due to rheumatic heart disease as part of the 2015 Global Burden of Disease study., Methods: We systematically reviewed data on fatal and nonfatal rheumatic heart disease for the period from 1990 through 2015. Two Global Burden of Disease analytic tools, the Cause of Death Ensemble model and DisMod-MR 2.1, were used to produce estimates of mortality and prevalence, including estimates of uncertainty., Results: We estimated that there were 319,400 (95% uncertainty interval, 297,300 to 337,300) deaths due to rheumatic heart disease in 2015. Global age-standardized mortality due to rheumatic heart disease decreased by 47.8% (95% uncertainty interval, 44.7 to 50.9) from 1990 to 2015, but large differences were observed across regions. In 2015, the highest age-standardized mortality due to and prevalence of rheumatic heart disease were observed in Oceania, South Asia, and central sub-Saharan Africa. We estimated that in 2015 there were 33.4 million (95% uncertainty interval, 29.7 million to 43.1 million) cases of rheumatic heart disease and 10.5 million (95% uncertainty interval, 9.6 million to 11.5 million) disability-adjusted life-years due to rheumatic heart disease globally., Conclusions: We estimated the global disease prevalence of and mortality due to rheumatic heart disease over a 25-year period. The health-related burden of rheumatic heart disease has declined worldwide, but high rates of disease persist in some of the poorest regions in the world. (Funded by the Bill and Melinda Gates Foundation and the Medtronic Foundation.).

Background: Echocardiographic screening for rheumatic heart disease in asymptomatic children may result in early diagnosis and prevent progression. Physician-led screening is not feasible in Malawi. Task shifting to mid-level providers such as clinical officers may enable more widespread screening. Hypothesis With short-course training, clinical officers can accurately screen for rheumatic heart disease using focussed echocardiography., Methods: A total of eight clinical officers completed three half-days of didactics and 2 days of hands-on echocardiography training. Clinical officers were evaluated by performing screening echocardiograms on 20 children with known rheumatic heart disease status. They indicated whether children should be referred for follow-up. Referral was indicated if mitral regurgitation measured more than 1.5 cm or there was any measurable aortic regurgitation. The κ statistic was calculated to measure referral agreement with a paediatric cardiologist. Sensitivity and specificity were estimated using a generalised linear mixed model, and were calculated on the basis of World Heart Federation diagnostic criteria., Results: The mean κ statistic comparing clinical officer referrals with the paediatric cardiologist was 0.72 (95% confidence interval: 0.62, 0.82). The κ value ranged from a minimum of 0.57 to a maximum of 0.90. For rheumatic heart disease diagnosis, sensitivity was 0.91 (95% confidence interval: 0.86, 0.95) and specificity was 0.65 (95% confidence interval: 0.57, 0.72)., Conclusion: There was substantial agreement between clinical officers and paediatric cardiologists on whether to refer. Clinical officers had a high sensitivity in detecting rheumatic heart disease. With short-course training, clinical officer-led echo screening for rheumatic heart disease is a viable alternative to physician-led screening in resource-limited settings.

Aim: The aims of this study were to 1) determine if cardiac disease can be detected in HIV-infected children by strain imaging and 2) to evaluate differences in exercise performance between HIV-infected children on antiretroviral therapy (ART) and HIV-infected children not yet on ART and in HIV-uninfected children by 6-minute walk tests (6MWTs)., Methods: This cross-sectional study evaluated cardiac function by echocardiogram and exercise performance by 6MWT in HIV-infected and HIV-uninfected children 4-18 years of age in Lilongwe, Malawi. Analyses compared HIV uninfected, HIV infected not yet on ART, and HIV infected on ART. Comparisons used χ(2) test, t test, analysis of variance and multiple linear regression., Results: No differences were found in ejection fraction, shortening fraction or strain in 73 children not yet on ART, 149 on ART and 77 HIV-uninfected controls. As viral load increased, children had worse circumferential strain. In addition, children receiving ART had better circumferential strain than those not yet on ART. Increased CD4 percentage was associated with better longitudinal strain and farther 6MWT distance. As longitudinal strain worsened, the 6MWT distance decreased. HIV-infected children not yet on ART walked a mean of 25.8 m less than HIV-uninfected children, and HIV-infected children on ART walked 25.9 m less (P = 0.015 comparing 3 groups)., Conclusions: HIV-uninfected children performed better on the 6MWT than HIV-infected children. Lower viral load, being on ART, and higher CD4 percentage were associated with better strain measures. Better longitudinal strain was associated with a farther 6MWT distance. Overall, ejection fraction, shortening fraction and strain measures between groups were similar, so cardiac strain did not detect cardiac dysfunction in this young population.

Rheumatic heart disease (RHD) is the largest cardiac cause of morbidity and mortality in the world's youth. Early detection of RHD through echocardiographic screening in asymptomatic children may identify an early stage of disease, when secondary prophylaxis has the greatest chance of stopping disease progression. Latent RHD signifies echocardiographic evidence of RHD with no known history of acute rheumatic fever and no clinical symptoms., Objective: Determine the prevalence of latent RHD among children ages 5-16 in Lilongwe, Malawi., Design: This is a cross-sectional study in which children ages 5 through 16 were screened for RHD using echocardiography., Setting: Screening was conducted in 3 schools and surrounding communities in the Lilongwe district of Malawi between February and April 2014., Outcome Measures: Children were diagnosed as having no, borderline, or definite RHD as defined by World Heart Federation criteria. The primary reader completed offline reads of all studies. A second reader reviewed all of the studies diagnosed as RHD, plus a selection of normal studies. A third reader served as tiebreaker for discordant diagnoses. The distribution of results was compared between gender, location, and age categories using Fisher's exact test., Results: The prevalence of latent RHD was 3.4% (95% CI = 2.45, 4.31), with 0.7% definite RHD and 2.7% borderline RHD. There was no significant differences in prevalence between gender (P = .44), site (P = .6), urban vs. peri-urban (P = .75), or age (P = .79). Of those with definite RHD, all were diagnosed because of pathologic mitral regurgitation (MR) and 2 morphologic features of the mitral valve. Of those with borderline RHD, most met the criteria by having pathological MR (92.3%)., Conclusion: Malawi has a high rate of latent RHD, which is consistent with other results from sub-Saharan Africa. This study strongly supports the need for a RHD prevention and control program in Malawi., (© 2016 Wiley Periodicals, Inc.)

Background: Clinicians rely on age- and size-specific measures of cardiac structures to diagnose cardiac disease. No universally accepted normative data exist for fetal cardiac structures, and most fetal cardiac centers do not use the same standards. The aim of this study was to derive predictive models for Z scores for 13 commonly evaluated fetal cardiac structures using a large heterogeneous population of fetuses without structural cardiac defects., Methods: The study used archived normal fetal echocardiograms in representative fetuses aged 12 to 39 weeks. Thirteen cardiac dimensions were remeasured by a blinded echocardiographer from digitally stored clips. Studies with inadequate imaging views were excluded. Regression models were developed to relate each dimension to estimated gestational age (EGA) by dates, biparietal diameter, femur length, and estimated fetal weight by the Hadlock formula. Dimension outcomes were transformed (e.g., using the logarithm or square root) as necessary to meet the normality assumption. Higher order terms, quadratic or cubic, were added as needed to improve model fit. Information criteria and adjusted R 2 values were used to guide final model selection., Results: Each Z-score equation is based on measurements derived from 296 to 414 unique fetuses. EGA yielded the best predictive model for the majority of dimensions; adjusted R 2 values ranged from 0.72 to 0.893. However, each of the other highly correlated (r > 0.94) biometric parameters was an acceptable surrogate for EGA. In most cases, the best fitting model included squared and cubic terms to introduce curvilinearity., Conclusions: For each dimension, models based on EGA provided the best fit for determining normal measurements of fetal cardiac structures. Nevertheless, other biometric parameters, including femur length, biparietal diameter, and estimated fetal weight provided results that were nearly as good. Comprehensive Z-score results are available on the basis of highly predictive models derived from gestational age or other biometrics as preferable for clinical reasons. These results supplant current equations and will provide a foundation for future multicenter collaborations., (Copyright © 2016 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Background: Advances in fetal echocardiography have improved recognition of congenital heart disease (CHD). Imaging protocols have been developed that predict delivery room (DR) risk and anticipated postnatal level of care (LOC). The aim of this study was to determine the utility of fetal echocardiography in the perinatal management of CHD., Methods: A retrospective analysis of fetal and postnatal records was conducted. The anticipated LOC was assigned by fetal echocardiography (LOC 1, nursery consult/outpatient follow-up; LOC 2, stable in DR with transfer to cardiac hospital; LOC 3 or 4, DR instability/urgent intervention needed). Prenatal diagnoses and LOC assignment were compared with postnatal diagnoses, treatment, and short-term outcomes., Results: From 2004 to 2012, 8,101 fetuses were evaluated; 7,405 were normal. Of 696 with CHD, 101 terminated, 40 died in utero, and 37 received palliative care. LOC was assigned in the remaining 518. Of 219 LOC 1, 195 (89%) had postnatal follow-up. Only two required transfer for intervention (LOC 1 sensitivity, 0.9; LOC 1 positive predictive value, 0.99). Of 260 assigned LOC 2, 229 (88%) had follow-up. Of these, 200 (87%) were transferred for surgery or intervention. The median time to admission was 195 min. Twenty-two patients (10%) assigned LOC 2 did not require intervention; however, seven (all with D-transposition of the great arteries) required catheter intervention before surgery. Hospital survival was 86% (LOC 2 sensitivity, 0.97; LOC 2 positive predictive value, 0.87). All LOC 3 and 4 patients had follow-up. Thirty-four (87%) needed urgent intervention, with 100% DR and 87% hospital survival (LOC 3 and 4 sensitivity, 0.83; LOC 3 and 4 positive predictive value, 0.87)., Conclusions: Fetal echocardiography enables accurate postnatal risk stratification in CHD, with the exception of D-transposition of the great arteries. LOC 1 assignment facilitated outpatient follow-up; LOC 2 assignment facilitated transfer for intervention. LOC 3 and 4 patients underwent stabilizing intervention or surgery with good short-term outcomes. Given the inability to predict need for intervention in D-transposition of the great arteries, all such patients should be assigned as LOC 3 or 4. Fetal echocardiography with LOC assignment should be used in the planning of postnatal care in CHD., (Copyright © 2015 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

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., (© 2015 American Heart Association, Inc.)

Background: With the advent of three-dimensional (3D) printers and high-resolution cardiac imaging, rapid prototype constructions of congenital cardiac defects are now possible. Typically, source images for these models derive from higher resolution, cross-sectional cardiac imaging, such as cardiac magnetic resonance imaging or computed tomography. These imaging methods may involve intravenous contrast, sedation, and ionizing radiation. New echocardiographic transducers and advanced software and hardware have optimized 3D echocardiographic images for this purpose. Thus, the objectives of this study were to confirm the feasibility of creating cardiac models from 3D echocardiographic data and to assess accuracy by comparing 3D model measurements with conventional two-dimensional (2D) echocardiographic measurements of cardiac defects., Methods: Nine patients undergoing 3D echocardiography were identified (eight with ventricular septal defects, one with three periprosthetic aortic valve leaks). Raw echocardiographic image data were exported anonymously and converted to Digital Imaging and Communications in Medicine format. The image data were filtered for noise reduction, imported into segmentation software to create a 3D digital model, and printed. Measurements of the defects from the 3D model were compared with defect measurements from conventional 2D echocardiographic data. Meticulous care was taken to ensure identical measurement planes., Results: Long- and short-axis measurements of eight ventricular septal defects and three perivalvar leaks were obtained. Mean ± SD values for the 3D model measurements and conventional 2D echocardiographic measurements were 7.5 ± 6.3 and 7.1 ± 6.2 mm respectively (P = .20), indicating no significant differences between the standard 2D and 3D model measurements. The two groups were highly correlated, with a Pearson correlation coefficient of 0.988. The mean absolute error (2D - 3D) for each measurement was 0.4 ± 0.9 mm, indicating accuracy of the 3D model of <1 mm., Conclusions: Three-dimensional printed models of echocardiographic data are technically feasible and may accurately reflect ventricular septal defect anatomy. Three-dimensional models derived from 3D echocardiographic data sets represent a new tool in procedural planning for children with congenital heart disease., (Copyright © 2015 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Background: Patients with Kawasaki disease (KD) are at risk for developing coronary artery lesions, but the association of noncoronary changes such as mitral regurgitation (MR) and/or pericardial effusion (PE) with cardiac mechanics in the acute phase of KD has not been previously described. The aim of this study was to test the hypothesis that these noncoronary markers for carditis are associated with abnormalities in strain (ε) and strain rate (SR) in patients with MR or PE not appreciated by conventional echocardiography., Methods: Longitudinal and circumferential ε and SR analyses were retrospectively performed on patients with KD. Patients with and without MR or PE were compared. Strain values were also compared between patients with and without coronary artery lesions. Values for ejection fraction, shortening fraction, and clinical laboratory parameters were correlated with MR or PE. Follow-up echocardiographic outcomes were recorded at the first encounter after initial diagnosis. Follow-up ε and SR data were also obtained in the group with MR or PE and altered ventricular mechanics at diagnosis., Results: Of the 110 patients reviewed, 92 had appropriate image quality for either longitudinal ε and SR or circumferential ε and SR analysis. Twenty-eight patients (30%) had either MR or PE. Longitudinal ε and SR were significantly decreased in patients with MR or PE compared with patients without MR or PE (ε: -16.4 ± 4.0% vs -19.0 ± 3.7%, P = .004; SR: -1.3 ± 0.7 vs -1.6 ± 0.4 sec(-1), P = .03). No significant difference in longitudinal ε or SR was noted between patients with and without coronary artery lesions (ε: -17.9 ± 4.1% vs -17.8 ± 3.8%, P = .50; SR: -1.5 ± 0.3 vs -1.6 ± 0.8 sec(-1), P = .50). In the group with abnormal coronary arteries, presence of MR or PE was correlated with decreased longitudinal ε (-16.1 ± 3.6% vs -18.9 ± 3.4%, P = .02), without a significant difference in longitudinal SR (-1.6 ± 0.4 vs -1.5 ± 0.4 sec(-1), P = .20). At approximately 3-week follow-up (21.3 ± 15.8 days), longitudinal ε and SR for the group with MR or PE had increased significantly compared with diagnosis (ε: -16.4 ± 4.3% vs -18.6 ± 0.5%, P = .03; SR: -1.3 ± 0.6 vs -1.8 ± 0.4 sec(-1), P = .008), coincident with resolution of MR or PE. In both groups, erythrocyte sedimentation rate and C-reactive protein were elevated (85.3 ± 36.2 mm/h vs 75.1 ± 33.1 mm/h [P = .34] and 12.3 ± 6.7 vs 11.7 ± 8.2 mg/dL [P = .83]), but only modest correlations were noted between longitudinal ε and elevated erythrocyte sedimentation rate (r = 0.52, P = .01; confidence interval, 0.10-0.80) and C-reactive protein (r = 0.50, P = .02; confidence interval, 0.10-0.80) in patients with MR or PE. Shortening fraction and ejection fraction were within the normal range in both groups., Conclusions: Patients presenting with KD with MR or PE at diagnosis are likely to have altered ventricular mechanics compared with patients with KD without MR or PE despite normal conventional echocardiographic measures of function. There is no significant difference in ventricular mechanics when comparing patients with KD with coronary ectasia or aneurysms and those without coronary lesions. Presence of abnormal ε in patients with KD with altered ventricular mechanics correlates modestly with laboratory inflammatory markers. Peak systolic longitudinal ε and SR increased significantly at 3-week follow-up compared with initial diagnosis, coincident with resolution of MR or PE., (Copyright © 2014 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Background: Transesophageal echocardiographic imaging of small infants has been limited because of equipment size. A newer miniaturized transducer has allowed for the imaging of even the smallest patients but has been limited by intermittent poor image quality because of hardware durability. A second generation of the transducer was developed to address this problem. The aim of this study was to evaluate the performance and durability of the second-generation transducer over multiple uses and to compare the image quality from the modified transducer and new software preset to the original transducer., Methods: Patients undergoing transesophageal echocardiography (TEE) based on clinical indications with the original or second-generation transducers were included in the study. All studies were reviewed for image quality and unacceptable image degradation, which was defined as imaging quality inadequate for clinical use. Study review was performed by two echocardiographers., Results: Using the original transducer, 37 studies were performed with a mean patient weight of 3.46±0.89 kg (range 2.1-5.7). Using the second-generation transducer, 65 studies were performed with a mean patient weight of 4.87±2.05 kg (range 2.2-13.8). Image quality was clinically inadequate in 8 of the 35 studies with the original transducer and in zero with the second generation (P<.0001)., Conclusions: The second-generation transducer allows for effective use of TEE even in the smallest infants and avoids the mechanical failures associated with the first-generation release., (© The Author(s) 2014.)