Primary surgical repair of tetralogy of fallot at the Uganda Heart Institute: a ten-year review of 30day mortality and morbidity.

BACKGROUND: Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease (CHD) worldwide. It accounts for 7% of CHD cases in Uganda and leads to fatal outcomes in the long term without surgery. Surgery is often delayed in developing countries like Uganda due to limited resources. OBJECTIVE: This study aimed to determine the early surgical outcomes of patients with TOF who underwent primary intracardiac repair at the Uganda Heart Institute (UHI) and to identify factors associated. METHODOLOGY: This retrospective chart review evaluated outcomes of primary TOF repair patients at UHI from February 2012 to October 2022. Patient outcomes were assessed from surgery until 30 days post-operation. RESULTS: Out of the 104 patients who underwent primary TOF repair at UHI, records of 88 patients (84.6%) were available for review. Males accounted for 48.9% (n = 43). The median age at the time of operation was 4 years (with an interquartile range of 2.5-8.0 years), ranging from 9 months to 16 years. Genetic syndromes were present in 5/88 (5.7%). These included 2 patients with trisomy 21, 2 with Noonan's, and 1 with 22q11.2 deletion syndrome. Early postoperative outcomes for patients included: residual ventricular septal defects in 35/88 (39.8%), right ventricular dysfunction in 33/88 (37.5%), residual pulmonary regurgitation in 27/88 (30.7%), residual right ventricular outflow tract obstruction in 27/88 (30.0%), pleural effusion in 24/88 (27.3%), arrhythmias in 24/88(27.3%), post-operative infections in 23/88(26.1%) and left ventricular systolic dysfunction in 9/88 (10.2%). Out of the children who underwent surgery after one year of age, 8% (7 children) died within the first 30 days. There was a correlation between mortality and post-operative ventilation time, cardiopulmonary bypass (CPB) time, aortic cross-clamp time, preoperative oxygen saturations, RV and LV dysfunction and the operating team. CONCLUSION: The most frequent outcomes after surgery were residual ventricular septal defects and right ventricular failure. In our study, the 30-day mortality rate following TOF repair was 8%. Deceased patients had lower pre-operative oxygen levels, longer CPB and cross-clamp times, longer post-operative ventilation, RV/LV dysfunction, and were more likely operated by the local team.

Echocardiography in Indigenous Populations and Resource Poor Settings.

The majority of global cardiovascular disease burden occurs in low- and middle-income countries (LMIC) and indigenous populations. Although common diseases, such as ischaemic heart disease, cause significant burden, there are also neglected diseases. Forgotten by many, these diseases-including rheumatic heart disease, endomyocardial fibrosis and Chagas cardiomyopathy-continue to take a tremendous toll on a large proportion of the world's population. Whilst the technology of echocardiography continues to evolve in many high-income countries, low resource countries are working out how to make this vital tool available and affordable for the most remote and poorest populations. This paper aims to highlight the neglected cardiovascular diseases and their echocardiographic features. It also highlights the latest research in relation to portable echocardiography, task shifting and disease screening. The authors make recommendations in relation to future directions, including making echocardiography an affordable and accessible tool for all.

Latent Rheumatic Heart Disease: Identifying the Children at Highest Risk of Unfavorable Outcome.

BACKGROUND: Screening echocardiography has emerged as a potentially powerful tool for early diagnosis of rheumatic heart disease (RHD). The utility of screening echocardiography hinges on the rate of RHD progression and the ability of penicillin prophylaxis to improve outcome. We report the longitudinal outcomes of a cohort of children with latent RHD and identify risk factors for unfavorable outcomes. METHODS: This was a prospective natural history study conducted under the Ugandan RHD registry. Children with latent RHD and ≥1 year of follow-up were included. All echocardiograms were re-reviewed by experts (2012 World Heart Federation criteria) for inclusion and evidence of change. Bi- and multivariable logistic regression, Kaplan-Meier analysis, and Cox proportional hazards models, as well, were developed to search for risk factors for unfavorable outcome and compare progression-free survival between those treated and not treated with penicillin. Propensity and other matching methods with sensitivity analysis were implemented for the evaluation of the penicillin effect. RESULTS: Blinded review confirmed 227 cases of latent RHD: 164 borderline and 63 definite (42 mild, 21 moderate/severe). Median age at diagnosis was 12 years and median follow-up was 2.3 years (interquartile range, 2.0-2.9). Penicillin prophylaxis was prescribed in 49.3% with overall adherence of 84.7%. Of children with moderate-to-severe definite RHD, 47.6% had echocardiographic progression (including 2 deaths), and 9.5% had echocardiographic regression. Children with mild definite and borderline RHD showed 26% and 9.8% echocardiographic progression and 45.2% and 46.3% echocardiographic improvement, respectively. Of those with mild definite RHD or borderline RHD, more advanced disease category, younger age, and morphological mitral valve features were risk factors for an unfavorable outcome. CONCLUSIONS: Latent RHD is a heterogeneous diagnosis with variable disease outcomes. Children with moderate to severe latent RHD have poor outcomes. Children with both borderline and mild definite RHD are at substantial risk of progression. Although long-term outcome remains unclear, the initial change in latent RHD may be evident during the first 1 to 2 years following diagnosis. Natural history data are inherently limited, and a randomized clinical trial is needed to definitively determine the impact of penicillin prophylaxis on the trajectory of latent RHD.

A focussed single-view hand-held echocardiography protocol for the detection of rheumatic heart disease.

BACKGROUND: Echocardiographic screening represents an opportunity for reduction in the global burden of rheumatic heart disease. A focussed single-view screening protocol could allow for the rapid training of healthcare providers and screening of patients. OBJECTIVE: The aim of this study was to determine the sensitivity and specificity of a focussed single-view hand-held echocardiographic protocol for the diagnosis of rheumatic heart disease in children. METHODS: A total of nine readers were divided into three reading groups; each interpreted 200 hand-held echocardiography studies retrospectively as screen-positive, if mitral regurgitation ⩾1.5 cm and/or any aortic insufficiency were observed, or screen-negative from a pooled study library. The performance of experts receiving focussed hand-held protocols, non-experts receiving focussed hand-held protocols, and experts receiving complete hand-held protocols were determined in comparison with consensus interpretations on fully functional echocardiography machines. RESULTS: In all, 587 studies including 76 on definite rheumatic heart disease, 122 on borderline rheumatic heart disease, and 389 on normal cases were available for analysis. The focussed single-view protocol had a sensitivity of 81.1%, specificity of 75.5%, negative predictive value of 88.5%, and a positive predictive value of 63.2%; expert readers had higher specificity (86.1 versus 64.8%, p<0.01) but equal sensitivity. Sensitivity - experts, 96% and non-experts, 95% - and negative predictive value - experts, 99% and non-experts, 98% - were better for definite rheumatic heart disease. False-positive screening studies resulting from erroneous identification of mitral regurgitation and aortic insufficiency colour jets increased with shortened protocols and less experience (p<0.01). CONCLUSION: Our data support a focussed screening protocol limited to parasternal long-axis images. This holds promise in making echocardiographic screening more practical in regions where rheumatic heart disease remains endemic.

Noonan syndrome in diverse populations.

Noonan syndrome (NS) is a common genetic syndrome associated with gain of function variants in genes in the Ras/MAPK pathway. The phenotype of NS has been well characterized in populations of European descent with less attention given to other groups. In this study, individuals from diverse populations with NS were evaluated clinically and by facial analysis technology. Clinical data and images from 125 individuals with NS were obtained from 20 countries with an average age of 8 years and female composition of 46%. Individuals were grouped into categories of African descent (African), Asian, Latin American, and additional/other. Across these different population groups, NS was phenotypically similar with only 2 of 21 clinical elements showing a statistically significant difference. The most common clinical characteristics found in all population groups included widely spaced eyes and low-set ears in 80% or greater of participants, short stature in more than 70%, and pulmonary stenosis in roughly half of study individuals. Using facial analysis technology, we compared 161 Caucasian, African, Asian, and Latin American individuals with NS with 161 gender and age matched controls and found that sensitivity was equal to or greater than 94% for all groups, and specificity was equal to or greater than 90%. In summary, we present consistent clinical findings from global populations with NS and additionally demonstrate how facial analysis technology can support clinicians in making accurate NS diagnoses. This work will assist in earlier detection and in increasing recognition of NS throughout the world.

Cardiac strain findings in children with latent rheumatic heart disease detected by echocardiographic screening.

BACKGROUND: Identification of patients with latent rheumatic heart disease by echocardiography presents a unique opportunity to prevent disease progression. Myocardial strain is a more sensitive indicator of cardiac performance than traditional measures of systolic function. OBJECTIVE: The objective of this study was to test the hypothesis that abnormalities in myocardial strain may be present in children with latent rheumatic heart disease. METHODS: Standard echocardiography images with electrocardiogram gating were obtained from Ugandan children found to have latent rheumatic heart disease as well as control subjects. Traditional echocardiography measures of systolic function were obtained, and offline global longitudinal strain analysis was performed. Comparison between groups was performed using strain as a continuous (Mann-Whitney U-test) and categorical (cut-off 5th percentile for age) variable. RESULTS: Our study included 14 subjects with definite rheumatic heart disease, 13 with borderline rheumatic heart disease, and 112 control subjects. None of the subjects had abnormal left ventricular size or ejection fraction. Global longitudinal strain was lower than the 5th percentile in 44% of the subjects with any rheumatic heart disease (p=0.002 versus controls) and 57% of the subjects with definite rheumatic heart disease (p=0.03). The mean absolute strain values were significantly lower when comparing subjects with any rheumatic heart disease with controls (20.4±3.95 versus 22.4±4.35, p=0.025) and subjects with definite rheumatic heart disease with controls (19.9±4.25 versus 22.4±4.35, p=0.033). CONCLUSION: Global longitudinal strain is decreased in subjects with rheumatic heart disease in the absence of abnormal systolic function. Larger studies with longer-term follow-up are required to determine whether there is a role for strain to help better understand the pathophysiology of latent rheumatic heart disease.

Child and teacher acceptability of school-based echocardiographic screening for rheumatic heart disease in Uganda.

Introduction Rheumatic heart disease causes substantial morbidity in children in low-income countries. School-based echocardiographic screening has been suggested as a means to identify children with latent disease; however, little is known about the experience of children and teachers participating in screenings. The aim of our study was to assess students' and teachers' experience of school-based echocardiographic screening and identify areas for improvement. Materials and methods A school-based echocardiographic screening programme was conducted in five schools in Northern Uganda in 2013. After 8 months, an age- and gender-stratified population that included 5% of the participating students and teachers completed a questionnaire via an in-person interview. Responses were reviewed by question and coded to identify key themes. RESULTS: A total of 255 students (mean 10.7 years; 48% male) and 35 teachers participated in our study. In total, 95% of the students and 100% of the teachers were happy to have participated in the screening; however, students reported feeling scared (35%) and nervous (48%) during the screening process. Programmatic strengths included the following: knowing one's health status, opportunity to receive treatment, and staff interactions. Although 43% of the patients did not suggest a change with open-ended questioning, concerns regarding privacy, fear of the screening process, and a desire to include others in the community were noted. Discussion School-based echocardiographic rheumatic heart disease screening was well received by students and teachers. Future programmes would likely benefit from improved pre-screening education regarding the screening process and diagnosis of rheumatic heart disease. Furthermore, education of teachers and students could improve screening perception and establish realistic expectations regarding the scope of screening.

The Impact of Echocardiographic Screening for Rheumatic Heart Disease on Patient Quality of Life.

OBJECTIVE: To assess the impact of rheumatic heart disease (RHD) on child-reported health-related quality of life (HRQOL) in the context of a Ugandan school-based echocardiographic screening program. Echocardiography-based screening has emerged as a tool for the early detection of RHD, but little is known about its impact on those screened. STUDY DESIGN: Participants included 358 children from Gulu and 28 children from Kampala Uganda. The Pediatric Quality of Life Inventory Version 4.0 was used to assess HRQOL in 4 groups of children: Gulu prescreen, Gulu postscreen, Gulu previously linked to care, and Kampala previously linked to care. Children in the pre- and postscreen groups were selected from a single school before and after screening occurred and matched by age and sex. Children previously linked to care were recruited from previous screening studies. RESULTS: When the echocardiogram was normal, there was no difference in HRQOL in the prescreen and postscreen groups. In the postscreen group, identification of latent RHD resulted in lower physical (75.3 vs 68.3, P = .03) and emotional (71.7 vs 63.4, P < .01) HRQOL, despite a lack of symptoms. The Kampala group had longer linkage to care (42 months vs 6 months, P < .01) and demonstrated greater HRQOL scores compared with the Gulu-linked group (70.7 vs 77.8, P < .01) and the combined Gulu cohort (77.8 vs 69.4, P = .02). CONCLUSIONS: Echocardiography-based screening for RHD does not diminish HRQOL in Ugandan children; rather, a diminished HRQOL score may be associated with being identified as RHD positive. Further investigation is needed to understand if longer linkage to care may ultimately normalize or improve HRQOL.

Latent rheumatic heart disease: outcomes 2 years after echocardiographic detection.

Screening with portable echocardiography has uncovered a large burden of latent rheumatic heart disease (RHD) among asymptomatic children in endemic regions, the significance of which remains unclear. This study aimed to determine the 2-year outcomes for children with latent RHD diagnosed by echocardiographic screening. Children identified with latent RHD enrolled in a biannual follow-up program. Risk factors for disease persistence and progression were examined. Of 62 children, 51 (82 %) with latent RHD had a median follow-up period of 25 months. Of these 51 children, 17 (33.3 %) reported an interval sore throat or symptoms consistent with acute rheumatic fever (ARF). Of 43 children initially classified as having borderline RHD, 21 (49 %) remained stable, 18 (42 %) improved (to no RHD) and 4 (10 %) worsened to definite RHD. Of the 8 children initially classified as having definite RHD, 6 (75 %) remained stable, and 2 (25 %) improved to borderline RHD. Two children had confirmed episodes of recurrent ARF, one of which represented the sole case of clinical worsening. The risk factors for disease persistence or progression included younger age (p = 0.05), higher antistreptolysin O titers at diagnosis (p = 0.05), and more morphologic valve abnormalities (p = 0.01). After 2 years, most of the children had a benign course, with 91 % remaining stable or showing improvement. Education may improve recognition of streptococcal sore throat. Longer-term follow-up evaluation, however, is warranted to confirm disease progression and risk factor profile. This could help tailor screening protocols for those at highest risk.

Health Related Quality of Life of Ugandan Children Following Valve Replacement Surgery for Rheumatic Heart Disease.

Background: Valve replacement surgery (VRS) improves clinical outcomes in patients with severe rheumatic heart disease (RHD). However, lifelong anticoagulation and frequent monitoring are required, which potentially impacts health-related quality of life (HRQoL). In this study, we assessed the HRQoL of people with RHD in Uganda following VRS. Methods: This was a hospital-based, cross-sectional study conducted between March and August 2021. Eligible participants were individuals who had VRS before the age of 18 years. The Pediatric Quality of Life Inventory-Cardiac Module (PedsQL-Cardiac module) was used to evaluate HRQoL. A total mean score of ≥ 80% was considered as optimal HRQoL. Results: Of the 83 eligible participants, 52 (60.5%) were female, with a median age of 18 (interquartile range: 14-22) years. Most participants had NYHA I functional status (n = 79, 92%). Most (n = 73, 92.4%) surgeries were performed outside of Uganda, and 61 (72.6%) were single mechanical valve replacement. Almost half (n = 45, 54%) expressed no concern about being on life-long warfarin therapy. However, 24 (29.3%) feared bleeding. The optimal mean score of cardiac-specific HRQoL was achieved in 50 (60.2%) of participants. Factors associated with optimal HRQoL were body mass index (BMI) (adjusted odds ratio (aOR), 1.2, 95% Confidence Interval: 1.1-1.3, p = 0.006), being afraid of bleeding or bruising (aOR: 1.5, 95% CI: 1.21-2.47, p = 0.004), acceptance of having an artificial valve (aOR: 2.7, 95% CI; 1.64-3.81, p < 0.001). Conclusion: HRQoL was optimal in about three in five participants following VRS. Increasing BMI and acceptance of artificial valve were significantly associated with optimal HRQoL.

Trends in Annual Incidence Rates of Newly Diagnosed Endomyocardial Fibrosis Cases at the Uganda Heart Institute: A 14-Year Review.

Background: First described in Uganda over seven decades ago, Endomyocardial fibrosis (EMF) is a rare form of restrictive cardiomyopathy found in the tropics. EMF occurs mainly in two phenotypes; biventricular involvement and right ventricular (RV) form. Previously endemic in several countries, there are reports suggesting that the disease is on the decline. Objectives: To describe trends in annual incidence rates of newly diagnosed EMF cases at the Uganda Heart Institute (UHI). Methods: This was a retrospective chart review of all newly diagnosed EMF cases at UHI from January 2007 to December 2020. Cases were divided into two groups A (2007-2013) and B (2014-2020). Results: A total of 155 cases were diagnosed during the period (Group A, n = 124; Group B, n = 31). There were no significant differences between the two groups A and B regarding median age at diagnosis (14 vs. 12 years, p = 0.0940), gender (48.4% female vs. 35.5%, p = 0.1987), and EMF type (66.9% RV EMF vs. 71.0%, p = 0.6634), respectively. The presence of complications such as intracardiac thrombus (5.6 vs. 32.2%, p = 0.0002) and pericardial effusion (57.3% vs. 80.6, p = 0.0172) were more frequent in group B than A, respectively. Pulmonary hypertension (PHT) was predominantly seen in cases with biventricular EMF compared to those with RV EMF (26 vs. 3.8%, p = 0.0001). The number of new cases diagnosed per year remained largely stable in the period 2007-2011, ranging 14-21 per year, peaked in 2012 (26 new cases), and thereafter declined from 10 cases seen in 2013 to 1-5 cases seen per year in the period 2017-2020. Similarly, the annual incidence rates of new EMF diagnosis remained relatively stable in the period 2007-2012, ranging between 22.7 and 29.7 per 10,000 patients seen in the echo labs, and then dramatically declined after 2012 to range between 1.0 and 4.5 new cases per 10,000 patients in the period between 2017 and 2020. Conclusion: There has been a steady decline in the number of new cases of EMF seen at the UHI. However, there were no significant differences in the gender, age at diagnosis and EMF subtype of cases during the period under review. Complication rates were more frequent in the later cohort.

Cardiovascular diseases in Africa in the twenty-first century: Gaps and priorities going forward.

In 2015, the United Nations set important targets to reduce premature cardiovascular disease (CVD) deaths by 33% by 2030. Africa disproportionately bears the brunt of CVD burden and has one of the highest risks of dying from non-communicable diseases (NCDs) worldwide. There is currently an epidemiological transition on the continent, where NCDs is projected to outpace communicable diseases within the current decade. Unchecked increases in CVD risk factors have contributed to the growing burden of three major CVDs-hypertension, cardiomyopathies, and atherosclerotic diseases- leading to devastating rates of stroke and heart failure. The highest age standardized disability-adjusted life years (DALYs) due to hypertensive heart disease (HHD) were recorded in Africa. The contributory causes of heart failure are changing-whilst HHD and cardiomyopathies still dominate, ischemic heart disease is rapidly becoming a significant contributor, whilst rheumatic heart disease (RHD) has shown a gradual decline. In a continent where health systems are traditionally geared toward addressing communicable diseases, several gaps exist to adequately meet the growing demand imposed by CVDs. Among these, high-quality research to inform interventions, underfunded health systems with high out-of-pocket costs, limited accessibility and affordability of essential medicines, CVD preventive services, and skill shortages. Overall, the African continent progress toward a third reduction in premature mortality come 2030 is lagging behind. More can be done in the arena of effective policy implementation for risk factor reduction and CVD prevention, increasing health financing and focusing on strengthening primary health care services for prevention and treatment of CVDs, whilst ensuring availability and affordability of quality medicines. Further, investing in systematic country data collection and research outputs will improve the accuracy of the burden of disease data and inform policy adoption on interventions. This review summarizes the current CVD burden, important gaps in cardiovascular medicine in Africa, and further highlights priority areas where efforts could be intensified in the next decade with potential to improve the current rate of progress toward achieving a 33% reduction in CVD mortality.

Recent Advances in the Rheumatic Fever and Rheumatic Heart Disease Continuum.

Nearly a century after rheumatic fever (RF) and rheumatic heart disease (RHD) was eradicated from the developed world, the disease remains endemic in many low- and middle-income countries (LMICs), with grim health and socioeconomic impacts. The neglect of RHD which persisted for a semi-centennial was further driven by competing infectious diseases, particularly the human immunodeficiency virus (HIV) pandemic. However, over the last two-decades, slowly at first but with building momentum, there has been a resurgence of interest in RF/RHD. In this narrative review, we present the advances that have been made in the RF/RHD continuum over the past two decades since the re-awakening of interest, with a more concise focus on the last decade's achievements. Such primary advances include understanding the genetic predisposition to RHD, group A Streptococcus (GAS) vaccine development, and improved diagnostic strategies for GAS pharyngitis. Echocardiographic screening for RHD has been a major advance which has unearthed the prevailing high burden of RHD and the recent demonstration of benefit of secondary antibiotic prophylaxis on halting progression of latent RHD is a major step forward. Multiple befitting advances in tertiary management of RHD have also been realized. Finally, we summarize the research gaps and provide illumination on profitable future directions towards global eradication of RHD.

Establishment of a cardiac telehealth program to support cardiovascular diagnosis and care in a remote, resource-poor setting in Uganda.

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Starting and Operating a Public Cardiac Catheterization Laboratory in a Low Resource Setting: The Eight-Year Story of the Uganda Heart Institute Catheter Laboratory.

Background: Low- and-middle-income-countries (LMICs) currently bear 80% of the world's cardiovascular disease (CVD) mortality burden. The same countries are underequipped to handle the disease burden due to critical shortage of resources. Functional cardiac catheterization laboratories (cath labs) are central in the diagnosis and management of CVDs. Yet, most LMICs, including Uganda, fall remarkably below the minimum recommended standards of cath lab:population ratio due to a host of factors including the start-up and recurring costs. Objectives: To review the performance, challenges and solutions employed, lessons learned, and projections for the future for a single cath lab that has been serving the Ugandan population of 40 million people in the past eight years. Methods: A retrospective review of the Uganda Heart Institute cath lab clinical database from 15 February 2012 to 31 December 2019 was performed. Results: In the initial two years, this cath lab was dependent on skills transfer camps by visiting expert teams, but currently, Ugandan resident specialists independently operate this lab. 3,542 adult and pediatric procedures were conducted in 8 years, including coronary angiograms and percutaneous coronary interventions, device implantations, valvuloplasties, and cardiac defect closures, among others. There was a consistent expansion of the spectrum of procedures conducted in this cath lab each year. The initial lack of technical expertise and sourcing for equipment, as well as the continual need for sundries present(ed) major roadblocks. Government support and leveraging existing multi-level collaborations has provided a platform for several solutions. Sustainability of cath lab services remains a significant challenge especially in relation to the high cost of sundries and other consumables amidst a limited budget. Conclusion: A practical example of how centers in LMIC can set up and sustain a public cardiac catheterization laboratory is presented. Government support, research, and training collaborations, if present, become invaluable leverage opportunities.

Pattern of congenital heart disease among children presenting to the Uganda Heart Institute, Mulago Hospital: a 7-year review.

BACKGROUND: Congenital heart disease (CHD) is the most common congenital anomaly in children. Over half of the deaths due to CHD occur in the neonatal period. Most children with unrepaired complex heart lesions do not live to celebrate their first birthday. We describe the spectrum of congenital heart disease in Uganda. METHODS: We retrospectively reviewed the data of children with CHD who presented to the Uganda Heart Institute (UHI), Mulago Hospital Complex from 2007 to 2014. RESULTS: A total of 4621 children were seen at the UHI during the study period. Of these, 3526 (76.3%) had CHD; 1941(55%) were females. Isolated ventricular septal defect (VSD) was the most common CHD seen in 923 (27.2%) children followed by Patent ductus arteriosus (PDA) 760 (22%) and atrial septal defects (ASD) 332 (9.4%). Tetralogy of Fallot (TOF) and Truncus arteriosus were the most common cyanotic heart defects (7% and 5% respectively). Dysmorphic features were diagnosed in 185 children, of which 61 underwent genetic testing (Down syndrome=24, 22q11.2 deletion syndrome n=10). Children with confirmed 22q11.2 deletion had conotruncal abnormalities. CONCLUSION: Isolated VSD and Tetralogy of Fallot are the most common acyanotic and cyanotic congenital heart defects. We report an unusually high occurrence of Truncus arteriosus.

Prevalence of group A ß-hemolytic streptococcal throat carriage and prospective pilot surveillance of streptococcal sore throat in Ugandan school children.

OBJECTIVES: Group A ß-hemolytic Streptococcus (GAS), also known as Streptococcus pyogenes, is responsible for an annual 600 million cases of acute pharyngitis globally, with 92% of those infections occurring in low-resource settings. Further knowledge of the acute streptococcal pharyngitis burden in low-resource settings is essential if serious post-streptococcal complications - rheumatic fever (RF) and its long-term sequel rheumatic heart disease (RHD) - are to be prevented. METHODS: Two studies were conducted in school-aged children (5-16 years): a cross-sectional study of streptococcal pharyngeal carriage followed by a prospective cohort study of streptococcal sore throat over 4 weeks from March to April 2017. RESULTS: The cross-sectional study revealed an overall prevalence of GAS carriage of 15.9% (79/496, 95% confidence interval 12.8-19.5%). Among 532 children enrolled in the prospective cohort study, 358 (67%) reported 528 sore throats, with 221 (41.1%) experiencing at least one GAS-positive sore throat. The overall GAS-positive rate for sore throat was 41.8% (221/528). CONCLUSIONS: The GAS pharyngeal carriage rates seen in Uganda (15.9%, 95% confidence interval 12.8-19.5%) are higher than the most recent pooled results globally, at 12% (range 6-28%). Additionally, pilot data suggest a substantially higher percentage of sore throat that was GAS-positive (41.8%) compared to pooled global rates when active recruitment is employed.

Prevalence, Clinical Features and Antibiotic Susceptibility of Group A Streptococcal Skin Infections in School Children in Urban Western and Northern Uganda.

BACKGROUND: Group A Streptococcus (GAS) skin infections can lead to invasive sepsis, poststreptococcal glomerulonephritis, and potentially rheumatic heart disease (RHD). Within a study to identify predisposing factors of RHD in Ugandan schoolchildren, we determined the prevalence of skin infections and assessed the clinical features and antibiotic susceptibility of GAS skin infection. METHODS: Cross-sectional study conducted at 3 urban primary schools in Western and Northern Uganda in March 2017. A dermatologist rendered clinical diagnoses and obtained a skin swab specimen from lesions with signs of bacterial infection. Beta-hemolytic colonies underwent Lancefield grouping, species identification by polymerase chain reaction and antimicrobial susceptibility testing. RESULTS: From 3265 schoolchildren, we observed 32% with ≥1 fungal, 1.8% with ≥1 bacterial, 0.9% with ≥1 viral, and 0.2% with ≥1 ectoparasitic infection. Of 79, 25 (32%) specimens were GAS-positive, of which one-third demonstrated tetracycline resistance. Of 17 impetigo cases, 13 (76%) were located on the leg/foot and 3 (18%) on the head/neck. Prevalence of GAS skin infection was 0.8% (25 of 3265). In Northern Uganda, where subclinical definite RHD prevalence is 1.1%, GAS skin infection prevalence was 1.2% (4 of 343) and 0.9% (3 of 352). CONCLUSION: This study identifies tetracycline-resistant GAS in Ugandan communities, suggests modified skin examination of exposed anatomic locations may be appropriate for population-based GAS skin infection studies, and underscores need for clear case definitions of GAS skin infection. Future studies are needed to evaluate the role of GAS skin infection in development of RHD in Ugandan communities.

Improving the accuracy of heart failure diagnosis in low-resource settings through task sharing and decentralization.

Background: Task sharing of TTE may improve capacity for heart failure diagnosis and management in patients in remote, low-resource settings but the impact on diagnostic accuracy and patient outcomes has not been studied.Objectives: Determine feasibility and impact of non-expert training in transthoracic echocardiography (TTE) to improve the diagnosis and outcomes of patients with suspected heart failure in Uganda.Methods: This two-part study examined an innovative training program to develop TTE competency among non-experts and used a pre-post design to determine the impact of decentralized TTE. Four of 8 non-experts (50%) passed a three-part training course. The training comprised of distance learning through a web-based curriculum, a 2-day hands-on workshop with cardiologists, and independent practice with remote mentorship. Continuous measures were compared (pre- vs. post-TTE) using t-tests or Wilcoxon rank-sum tests as distributionally appropriate and categorical variables assessed through chi-square testing. Sensitivity and specificity were calculated according to standard methodology comparing diagnosis pre- and post-TTE during phase 2.Results: Performance in the post-training phase showed good agreement with expert categorization (κ = 0.80) with diagnostic concordance in 421 of 454 studies (92.7%). TTE changed the preliminary diagnosis in 81% of patients, showing low specificity of clinical decision-making alone (14.2%; 95% CI 10.1-19.2%). Dilated cardiomyopathy, hypertensive heart disease with preserved systolic function, and right heart failure were the most underdiagnosed conditions prior to TTE while hypertensive heart disease with decreased systolic function was the most over-diagnosed condition.Conclusions: In conclusion, non-expert providers can achieve a high level of proficiency for the categorization of heart failure using handheld TTE in low-resource settings and use of telemedicine and remote mentorship may improve performance and feasibility. The addition of TTE resulted in substantial improvement in etiological specificity. Further study is needed to understand implications of this strategy on healthcare utilization, long-term patient outcomes, and cost.