Peter S Lwabi | Makerere University (original) (raw)

Papers by Peter S Lwabi

Circulation: Cardiovascular Imaging, 2019

Background: The 2012 World Heart Federation Criteria are the current gold standard for the diagno... more Background: The 2012 World Heart Federation Criteria are the current gold standard for the diagnosis of latent rheumatic heart disease (RHD). Because data and experience using these criteria have grown, there is opportunity to simplify and develop outcome prediction tools. We aimed to develop a simple echocardiographic score applicable for RHD screening with potential to predict disease progression. Methods: This study included 3 cohorts used for score derivation (n=9501), score validation (n=7312), and assessment of outcomes prediction (n=227). In the derivation cohort, variables independently associated with definite RHD were assigned point values proportional to their regression coefficients. The sum of these values was stratified into low (0–6), intermediate (7–9), and high (≥10) risk. Results: Five components were selected for score development, including mitral valve anterior leaflet thickening, excessive leaflet tip motion, and regurgitation jet length ≥2 cm, and aortic valve...

Journal of the American College of Cardiology

Journal of the American College of Cardiology, Mar 1, 2022

Journal of the American College of Cardiology

Circulation, Jan 4, 2016

Background There are few contemporary data on the mortality and morbidity associated with rheumat... more Background There are few contemporary data on the mortality and morbidity associated with rheumatic heart disease (RHD) or information on their predictors. We report the two year follow-up of individuals with RHD from 14 low and middle income countries in Africa and Asia.. Methods Between January 2010 and November 2012, we enrolled 3343 patients from 25 centers in 14 countries and followed them for two years to assess mortality, congestive heart failure (CHF), stroke or transient ischemic attack (TIA), recurrent acute rheumatic fever (ARF), and infective endocarditis (IE). Results Vital status at 24 months was known for 2960 (88.5%) patients. Two thirds were female. Although patients were young (median age 28 years, interquartile range 18 to 40), the two year case fatality rate was high (500 deaths, 16.9%). Mortality rate was 116.3/1000 patient-years in the first year and 65.4/1000 patient-years in the second year. Median age at death was 28.7 years. Independent predictors of death were severe valve disease (hazard ratio (HR) 2.36, 95%

Cardiovascular Journal Of Africa, 2013

Background: Complications of rheumatic heart disease are associated with severe morbidity and mor... more Background: Complications of rheumatic heart disease are associated with severe morbidity and mortality in developing countries where the disease prevalence remains high. Due to lack of screening services, many patients present late, with severe valve disease. In Uganda, the disease and its complications are still not well studied. Objective: To profile and describe cardiovascular complications in newly diagnosed rheumatic heart disease patients attending the Mulago National Referral Hospital in Uganda. Methods: This was a cross-sectional study where consecutive, newly diagnosed rheumatic heart disease patients were assessed and followed up for complications, such as heart failure, pulmonary hypertension, atrial fibrillation, recurrence of acute rheumatic fever, and stroke. Results: A total of 309 (115 males and 196 females) definite rheumatic heart disease patients aged 15-60 years were enrolled in the study and analysed. Complications occurred in 49% (152/309) of the newly diagnosed rheumatic heart disease cases, with heart failure (46.9%) the most common complication, followed by pulmonary arterial hypertension (32.7%), atrial fibrillation (13.9%), recurrence of acute rheumatic fever (11.4%), infective endocarditis (4.5%) and stroke (1.3%). Atrial fibrillation and acute rheumatic fever were the most common complications associated with heart failure. Conclusion: In this study we found that about 50% of newly diagnosed rheumatic heart disease patients in Uganda presented with complications. Heart failure and pulmonary arterial hypertension were the most commonly observed complications.

Pediatric Cardiology, 2014

Screening with portable echocardiography has uncovered a large burden of latent rheumatic heart d... more 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.

JAIDS Journal of Acquired Immune Deficiency Syndromes, 2014

The expansion of antiretroviral therapy (ART) in resource-limited settings (RLS) has dramatically... more The expansion of antiretroviral therapy (ART) in resource-limited settings (RLS) has dramatically changed the face of the AIDS epidemic in Sub-Saharan Africa over the past decade 1. The progress in HIV/AIDS care is in large part due to successful bilateral and multilateral collaborations of governments and non-governmental AIDS organizations. In Uganda, for example, with funding support from the National Institutes of Health (NIH) and the President's Emergency Plan for AIDS Relief, Case Western Reserve University (CWRU) and others have partnered with Ugandan institutions to develop a comprehensive HIV/AIDS infrastructure. Because of this concerted effort, the country has seen a fall in HIV prevalence from a peak of 25-30% in major urban areas to now less than 7% nationally, and nearly half of those who qualify for treatment are currently receiving it 2 .

Circulation, 2012

Background— Historically, sub-Saharan Africa has had the highest prevalence rates of clinically d... more Background— Historically, sub-Saharan Africa has had the highest prevalence rates of clinically detected rheumatic heart disease (RHD). Echocardiography-based screening improves detection of RHD in endemic regions. The newest screening guidelines (2006 World Health Organization/National Institutes of Health) have been tested across India and the Pacific Islands, but application in sub-Saharan Africa has, thus far, been limited to Mozambique. We used these guidelines to determine RHD prevalence in a large cohort of Ugandan school children, to identify risk factors for occult disease, and to assess the value of laboratory testing. Methods and Results— Auscultation and portable echocardiography were used to screen randomly selected schoolchildren, 5 to 16 years of age, in Kampala, Uganda. Disease likelihood was defined as definite, probable, or possible in accordance with the 2006 National Institutes of Health/World Health Organization guidelines. Ninety-seven percent of eligible stude...

European Heart Journal, 2014

Aims Rheumatic heart disease (RHD) accounts for over a million premature deaths annually; however... more Aims Rheumatic heart disease (RHD) accounts for over a million premature deaths annually; however, there is little contemporary information on presentation, complications, and treatment. Methods and results This prospective registry enrolled 3343 patients (median age 28 years, 66.2% female) presenting with RHD at 25 hospitals in 12 African countries, India, and Yemen between January 2010 and November 2012. The majority (63.9%) had moderate-to-severe multivalvular disease complicated by congestive heart failure (33.4%), pulmonary hypertension (28.8%), atrial fibrillation (AF) (21.8%), stroke (7.1%), infective endocarditis (4%), and major bleeding (2.7%).

Vascular Diseases and Therapeutics, 2017

We share the story of a 36 year old black man who was previously healthy and worked for an electr... more We share the story of a 36 year old black man who was previously healthy and worked for an electricity distribution company as a meter reader. This required him to ride long distances on a motor cycle which he did comfortably. In early October 2016, he developed 'abdominal pain' which required admission and was treated as peptic ulcer disease. There were no other associated symptoms. The pain resolved and he returned to work feeling a perfect health condition. 1 week later while carrying out his field work he felt very dizzy and decided to ride to the nearest health centre. On the way, he felt sudden severe burning pain in both his legs below the knees. He persisted riding his bike and reached the health centre approximately 30 minutes from the onset of this burning pain, from where he was referred to a regional referral hospital, where he arrived 4 hours later. At this hospital he was initially given pain killers as the medical team carried out investigations to find out the cause of his 'burning legs'. On the 2 nd day in this hospital, the patient noted that both legs were getting cold and turning dark, were losing sensation in the toes and the pain was still ongoing in the calves. An arterial doppler study was performed which suggested arterial occlusion in both legs. The patient was referred to a private hospital in Kampala, Uganda's capital where he arrived in the early morning hours of the following day.

Heart (British Cardiac Society), 2018

Estimates of the prevalence of rheumatic heart disease (RHD) in many endemic countries are limite... more Estimates of the prevalence of rheumatic heart disease (RHD) in many endemic countries are limited to samples of children attending schools, which generate an incomplete picture of disease burden in communities. The present study conducted household-based RHD screening in a representative community in Gulu district, Uganda. Members of households identified through a two-stage cluster-sampling approach between the ages of 5 years and 50 years were invited to undergo limited cardiac testing with a handheld echocardiogram to assess for the presence of RHD. Suspicious cases underwent confirmatory echocardiogram with a fully functional machine. Of the 2453 community members screened, 2.45% (95% CI 1.87% to 3.14%) showed echocardiographic evidence of RHD with 1.26% (95% CI 0.860% to 1.79%) having definite RHD. The overall prevalence of RHD among participants <20 years was 2.52% (95% CI 1.78% to 3.45%), with a borderline prevalence of 1.97% (95% CI 1.33% to 2.82%) and a definite prevale...

Heart Asia, 2018

Rheumatic heart disease (RHD) remains a major driver of cardiovascular morbidity and mortality in... more Rheumatic heart disease (RHD) remains a major driver of cardiovascular morbidity and mortality in low-resource settings. Registry-based care for RHD has been advocated as a powerful tool to improve clinical care and track quality metrics. Data collected through an RHD registry may also reveal epidemiological and geospatial trends, as well as insight into care utilisation. Uganda established a central RHD registry at the country's only tertiary cardiac centre in 2010. In 2014 RHD care and registry enrolment expanded to the Western region and in 2015 to the North. Here, we examine the geographical distribution of RHD cases in Uganda and the impact of registry expansion. A retrospective search of the Ugandan national RHD registry was preformed to capture all cases of acute rheumatic fever or clinical RHD from January 2010 through July 2016. A geospatial analysis revealed that the density of detected cases (cases/100 000 district residents) reflected proximity to an RHD registry enr...

Global heart, Jan 20, 2018

In this case study, we describe an ongoing approach to develop sustainable acute and chronic card... more In this case study, we describe an ongoing approach to develop sustainable acute and chronic cardiovascular care infrastructure in Uganda that involves patient and provider participation. Leveraging strong infrastructure for HIV/AIDS care delivery, University Hospitals Harrington Heart and Vascular Institute and Case Western Reserve University have partnered with U.S. and Ugandan collaborators to improve cardiovascular capabilities. The collaboration has solicited innovative solutions from patients and providers focusing on education and advanced training, penicillin supply, diagnostic strategy (e.g., hand-held ultrasound), maternal health, and community awareness. Key outcomes of this approach have been the completion of formal training of the first interventional cardiologists and heart failure specialists in the country, establishment of 4 integrated regional centers of excellence in rheumatic heart disease care with a national rheumatic heart disease registry, a penicillin distr...

Circulation, Jan 27, 2017

Background -Screening echocardiography (echo) has emerged as a potentially powerful tool for earl... more Background -Screening echocardiography (echo) has emerged as a potentially powerful tool for early diagnosis of rheumatic heart disease (RHD). The utility of screening echo 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 echos were re-reviewed by experts (2012 WHF criteria) for inclusion and evidence of change. Bi- and multi- variable logistic regression, Kaplan-Meier analysis, as well as Cox proportional hazard models 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 i...

Circulation. Cardiovascular quality and outcomes, 2017

Rheumatic heart disease (RHD) is a leading cause of premature death and disability in low-income ... more Rheumatic heart disease (RHD) is a leading cause of premature death and disability in low-income countries; however, few receive optimal benzathine penicillin G (BPG) therapy to prevent disease progression. We aimed to comprehensively describe the treatment cascade for RHD in Uganda to identify appropriate targets for intervention. Using data from the Uganda RHD Registry (n=1504), we identified the proportion of patients in the following care categories: (1) diagnosed and alive as of June 1, 2016; (2) retained in care; (3) appropriately prescribed BPG; and (4) optimally adherent to BPG (>80% of prescribed doses). We used logistic regression to investigate factors associated with retention and optimal adherence. Overall, median (interquartile range) age was 23 (15-38) years, 69% were women, and 82% had clinical RHD. Median follow-up time was 2.4 (0.9-4.0) years. Retention in care was the most significant barrier to achieving optimal BPG adherence with only 56.9% (95% confidence in...

Patient Education and Counseling

To assess the impact of a peer-support group on knowledge, quality of life, and social support fo... more To assess the impact of a peer-support group on knowledge, quality of life, and social support for children with rheumatic heart disease (RHD). Children diagnosed with RHD and receiving 4-weekly injectable penicillin were invited to participate in a monthly support group for 6 months. Pre- and post-intervention assessments included a baseline RHD knowledge assessment, a measure of health related quality of life (HRQOL) (PedsQLTM4.0), and a measure of social support (Hawthorne Friendship Scale). Groups incorporated elements of cooperative play and team building, RHD education, and emotional support. 42 participants attended ≥3 groups and were included in the analyses. Attending support groups resulted in increased total HRQOL scores (60.3v 70.2, p&amp;amp;lt;0.001), as well as the following HRQOL sub-scores (physical functioning 55.3v 68.6 (p&amp;amp;lt;0.001), social functioning 64.2v 75.8 (p&amp;amp;lt;0.001) and school functioning 59.2v 69.1 (p=0.001)). Significant increases in Friendship Scale scores (15.4v 19.7, p&amp;amp;lt;0.001) and RHD Knowledge scores were observed (3.6v 6.4, p&amp;amp;lt;0.001). Peer-support groups may be effective at normalizing decreased HRQOL scores and increasing RHD disease knowledge and social support. Peer-support groups implemented in conjunction with RHD screening can minimize the negative psychosocial effects associated with early RHD detection.

American journal of medical genetics. Part A, 2017

The cover image, by Paul Kruszka et al., is based on the Original Article Noonan Syndrome in Dive... more The cover image, by Paul Kruszka et al., is based on the Original Article Noonan Syndrome in Diverse Populations, DOI: 10.1002/ajmg.a.38362. Design Credit: Darryl Leja.

American journal of medical genetics. Part A, Jan 27, 2017

Noonan syndrome (NS) is a common genetic syndrome associated with gain of function variants in ge... more 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 stenos...

Circulation: Cardiovascular Imaging, 2019

Background: The 2012 World Heart Federation Criteria are the current gold standard for the diagno... more Background: The 2012 World Heart Federation Criteria are the current gold standard for the diagnosis of latent rheumatic heart disease (RHD). Because data and experience using these criteria have grown, there is opportunity to simplify and develop outcome prediction tools. We aimed to develop a simple echocardiographic score applicable for RHD screening with potential to predict disease progression. Methods: This study included 3 cohorts used for score derivation (n=9501), score validation (n=7312), and assessment of outcomes prediction (n=227). In the derivation cohort, variables independently associated with definite RHD were assigned point values proportional to their regression coefficients. The sum of these values was stratified into low (0–6), intermediate (7–9), and high (≥10) risk. Results: Five components were selected for score development, including mitral valve anterior leaflet thickening, excessive leaflet tip motion, and regurgitation jet length ≥2 cm, and aortic valve...

Journal of the American College of Cardiology

Journal of the American College of Cardiology, Mar 1, 2022

Journal of the American College of Cardiology

Circulation, Jan 4, 2016

Background There are few contemporary data on the mortality and morbidity associated with rheumat... more Background There are few contemporary data on the mortality and morbidity associated with rheumatic heart disease (RHD) or information on their predictors. We report the two year follow-up of individuals with RHD from 14 low and middle income countries in Africa and Asia.. Methods Between January 2010 and November 2012, we enrolled 3343 patients from 25 centers in 14 countries and followed them for two years to assess mortality, congestive heart failure (CHF), stroke or transient ischemic attack (TIA), recurrent acute rheumatic fever (ARF), and infective endocarditis (IE). Results Vital status at 24 months was known for 2960 (88.5%) patients. Two thirds were female. Although patients were young (median age 28 years, interquartile range 18 to 40), the two year case fatality rate was high (500 deaths, 16.9%). Mortality rate was 116.3/1000 patient-years in the first year and 65.4/1000 patient-years in the second year. Median age at death was 28.7 years. Independent predictors of death were severe valve disease (hazard ratio (HR) 2.36, 95%

Cardiovascular Journal Of Africa, 2013

Background: Complications of rheumatic heart disease are associated with severe morbidity and mor... more Background: Complications of rheumatic heart disease are associated with severe morbidity and mortality in developing countries where the disease prevalence remains high. Due to lack of screening services, many patients present late, with severe valve disease. In Uganda, the disease and its complications are still not well studied. Objective: To profile and describe cardiovascular complications in newly diagnosed rheumatic heart disease patients attending the Mulago National Referral Hospital in Uganda. Methods: This was a cross-sectional study where consecutive, newly diagnosed rheumatic heart disease patients were assessed and followed up for complications, such as heart failure, pulmonary hypertension, atrial fibrillation, recurrence of acute rheumatic fever, and stroke. Results: A total of 309 (115 males and 196 females) definite rheumatic heart disease patients aged 15-60 years were enrolled in the study and analysed. Complications occurred in 49% (152/309) of the newly diagnosed rheumatic heart disease cases, with heart failure (46.9%) the most common complication, followed by pulmonary arterial hypertension (32.7%), atrial fibrillation (13.9%), recurrence of acute rheumatic fever (11.4%), infective endocarditis (4.5%) and stroke (1.3%). Atrial fibrillation and acute rheumatic fever were the most common complications associated with heart failure. Conclusion: In this study we found that about 50% of newly diagnosed rheumatic heart disease patients in Uganda presented with complications. Heart failure and pulmonary arterial hypertension were the most commonly observed complications.

Pediatric Cardiology, 2014

Screening with portable echocardiography has uncovered a large burden of latent rheumatic heart d... more 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.

JAIDS Journal of Acquired Immune Deficiency Syndromes, 2014

The expansion of antiretroviral therapy (ART) in resource-limited settings (RLS) has dramatically... more The expansion of antiretroviral therapy (ART) in resource-limited settings (RLS) has dramatically changed the face of the AIDS epidemic in Sub-Saharan Africa over the past decade 1. The progress in HIV/AIDS care is in large part due to successful bilateral and multilateral collaborations of governments and non-governmental AIDS organizations. In Uganda, for example, with funding support from the National Institutes of Health (NIH) and the President's Emergency Plan for AIDS Relief, Case Western Reserve University (CWRU) and others have partnered with Ugandan institutions to develop a comprehensive HIV/AIDS infrastructure. Because of this concerted effort, the country has seen a fall in HIV prevalence from a peak of 25-30% in major urban areas to now less than 7% nationally, and nearly half of those who qualify for treatment are currently receiving it 2 .

Circulation, 2012

Background— Historically, sub-Saharan Africa has had the highest prevalence rates of clinically d... more Background— Historically, sub-Saharan Africa has had the highest prevalence rates of clinically detected rheumatic heart disease (RHD). Echocardiography-based screening improves detection of RHD in endemic regions. The newest screening guidelines (2006 World Health Organization/National Institutes of Health) have been tested across India and the Pacific Islands, but application in sub-Saharan Africa has, thus far, been limited to Mozambique. We used these guidelines to determine RHD prevalence in a large cohort of Ugandan school children, to identify risk factors for occult disease, and to assess the value of laboratory testing. Methods and Results— Auscultation and portable echocardiography were used to screen randomly selected schoolchildren, 5 to 16 years of age, in Kampala, Uganda. Disease likelihood was defined as definite, probable, or possible in accordance with the 2006 National Institutes of Health/World Health Organization guidelines. Ninety-seven percent of eligible stude...

European Heart Journal, 2014

Aims Rheumatic heart disease (RHD) accounts for over a million premature deaths annually; however... more Aims Rheumatic heart disease (RHD) accounts for over a million premature deaths annually; however, there is little contemporary information on presentation, complications, and treatment. Methods and results This prospective registry enrolled 3343 patients (median age 28 years, 66.2% female) presenting with RHD at 25 hospitals in 12 African countries, India, and Yemen between January 2010 and November 2012. The majority (63.9%) had moderate-to-severe multivalvular disease complicated by congestive heart failure (33.4%), pulmonary hypertension (28.8%), atrial fibrillation (AF) (21.8%), stroke (7.1%), infective endocarditis (4%), and major bleeding (2.7%).

Vascular Diseases and Therapeutics, 2017

We share the story of a 36 year old black man who was previously healthy and worked for an electr... more We share the story of a 36 year old black man who was previously healthy and worked for an electricity distribution company as a meter reader. This required him to ride long distances on a motor cycle which he did comfortably. In early October 2016, he developed 'abdominal pain' which required admission and was treated as peptic ulcer disease. There were no other associated symptoms. The pain resolved and he returned to work feeling a perfect health condition. 1 week later while carrying out his field work he felt very dizzy and decided to ride to the nearest health centre. On the way, he felt sudden severe burning pain in both his legs below the knees. He persisted riding his bike and reached the health centre approximately 30 minutes from the onset of this burning pain, from where he was referred to a regional referral hospital, where he arrived 4 hours later. At this hospital he was initially given pain killers as the medical team carried out investigations to find out the cause of his 'burning legs'. On the 2 nd day in this hospital, the patient noted that both legs were getting cold and turning dark, were losing sensation in the toes and the pain was still ongoing in the calves. An arterial doppler study was performed which suggested arterial occlusion in both legs. The patient was referred to a private hospital in Kampala, Uganda's capital where he arrived in the early morning hours of the following day.

Heart (British Cardiac Society), 2018

Estimates of the prevalence of rheumatic heart disease (RHD) in many endemic countries are limite... more Estimates of the prevalence of rheumatic heart disease (RHD) in many endemic countries are limited to samples of children attending schools, which generate an incomplete picture of disease burden in communities. The present study conducted household-based RHD screening in a representative community in Gulu district, Uganda. Members of households identified through a two-stage cluster-sampling approach between the ages of 5 years and 50 years were invited to undergo limited cardiac testing with a handheld echocardiogram to assess for the presence of RHD. Suspicious cases underwent confirmatory echocardiogram with a fully functional machine. Of the 2453 community members screened, 2.45% (95% CI 1.87% to 3.14%) showed echocardiographic evidence of RHD with 1.26% (95% CI 0.860% to 1.79%) having definite RHD. The overall prevalence of RHD among participants <20 years was 2.52% (95% CI 1.78% to 3.45%), with a borderline prevalence of 1.97% (95% CI 1.33% to 2.82%) and a definite prevale...

Heart Asia, 2018

Rheumatic heart disease (RHD) remains a major driver of cardiovascular morbidity and mortality in... more Rheumatic heart disease (RHD) remains a major driver of cardiovascular morbidity and mortality in low-resource settings. Registry-based care for RHD has been advocated as a powerful tool to improve clinical care and track quality metrics. Data collected through an RHD registry may also reveal epidemiological and geospatial trends, as well as insight into care utilisation. Uganda established a central RHD registry at the country's only tertiary cardiac centre in 2010. In 2014 RHD care and registry enrolment expanded to the Western region and in 2015 to the North. Here, we examine the geographical distribution of RHD cases in Uganda and the impact of registry expansion. A retrospective search of the Ugandan national RHD registry was preformed to capture all cases of acute rheumatic fever or clinical RHD from January 2010 through July 2016. A geospatial analysis revealed that the density of detected cases (cases/100 000 district residents) reflected proximity to an RHD registry enr...

Global heart, Jan 20, 2018

In this case study, we describe an ongoing approach to develop sustainable acute and chronic card... more In this case study, we describe an ongoing approach to develop sustainable acute and chronic cardiovascular care infrastructure in Uganda that involves patient and provider participation. Leveraging strong infrastructure for HIV/AIDS care delivery, University Hospitals Harrington Heart and Vascular Institute and Case Western Reserve University have partnered with U.S. and Ugandan collaborators to improve cardiovascular capabilities. The collaboration has solicited innovative solutions from patients and providers focusing on education and advanced training, penicillin supply, diagnostic strategy (e.g., hand-held ultrasound), maternal health, and community awareness. Key outcomes of this approach have been the completion of formal training of the first interventional cardiologists and heart failure specialists in the country, establishment of 4 integrated regional centers of excellence in rheumatic heart disease care with a national rheumatic heart disease registry, a penicillin distr...

Circulation, Jan 27, 2017

Background -Screening echocardiography (echo) has emerged as a potentially powerful tool for earl... more Background -Screening echocardiography (echo) has emerged as a potentially powerful tool for early diagnosis of rheumatic heart disease (RHD). The utility of screening echo 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 echos were re-reviewed by experts (2012 WHF criteria) for inclusion and evidence of change. Bi- and multi- variable logistic regression, Kaplan-Meier analysis, as well as Cox proportional hazard models 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 i...

Circulation. Cardiovascular quality and outcomes, 2017

Rheumatic heart disease (RHD) is a leading cause of premature death and disability in low-income ... more Rheumatic heart disease (RHD) is a leading cause of premature death and disability in low-income countries; however, few receive optimal benzathine penicillin G (BPG) therapy to prevent disease progression. We aimed to comprehensively describe the treatment cascade for RHD in Uganda to identify appropriate targets for intervention. Using data from the Uganda RHD Registry (n=1504), we identified the proportion of patients in the following care categories: (1) diagnosed and alive as of June 1, 2016; (2) retained in care; (3) appropriately prescribed BPG; and (4) optimally adherent to BPG (>80% of prescribed doses). We used logistic regression to investigate factors associated with retention and optimal adherence. Overall, median (interquartile range) age was 23 (15-38) years, 69% were women, and 82% had clinical RHD. Median follow-up time was 2.4 (0.9-4.0) years. Retention in care was the most significant barrier to achieving optimal BPG adherence with only 56.9% (95% confidence in...

Patient Education and Counseling

To assess the impact of a peer-support group on knowledge, quality of life, and social support fo... more To assess the impact of a peer-support group on knowledge, quality of life, and social support for children with rheumatic heart disease (RHD). Children diagnosed with RHD and receiving 4-weekly injectable penicillin were invited to participate in a monthly support group for 6 months. Pre- and post-intervention assessments included a baseline RHD knowledge assessment, a measure of health related quality of life (HRQOL) (PedsQLTM4.0), and a measure of social support (Hawthorne Friendship Scale). Groups incorporated elements of cooperative play and team building, RHD education, and emotional support. 42 participants attended ≥3 groups and were included in the analyses. Attending support groups resulted in increased total HRQOL scores (60.3v 70.2, p&amp;amp;lt;0.001), as well as the following HRQOL sub-scores (physical functioning 55.3v 68.6 (p&amp;amp;lt;0.001), social functioning 64.2v 75.8 (p&amp;amp;lt;0.001) and school functioning 59.2v 69.1 (p=0.001)). Significant increases in Friendship Scale scores (15.4v 19.7, p&amp;amp;lt;0.001) and RHD Knowledge scores were observed (3.6v 6.4, p&amp;amp;lt;0.001). Peer-support groups may be effective at normalizing decreased HRQOL scores and increasing RHD disease knowledge and social support. Peer-support groups implemented in conjunction with RHD screening can minimize the negative psychosocial effects associated with early RHD detection.

American journal of medical genetics. Part A, 2017

The cover image, by Paul Kruszka et al., is based on the Original Article Noonan Syndrome in Dive... more The cover image, by Paul Kruszka et al., is based on the Original Article Noonan Syndrome in Diverse Populations, DOI: 10.1002/ajmg.a.38362. Design Credit: Darryl Leja.

American journal of medical genetics. Part A, Jan 27, 2017

Noonan syndrome (NS) is a common genetic syndrome associated with gain of function variants in ge... more 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 stenos...