Craig Sable

The island nations of the South Pacific are home to more than 3 million Polynesians and Melanesians. There is a high birthrate and unique susceptibility to rheumatic fever which together have led to a large and increasing population of children and young adults with both congenital and rheumatic heart disease. Wide geographic dispersal has resulted in major challenges in both diagnosis and management of these patients. Volunteer medical and surgical teams have carried much of the load until now, supported by generous donations from numerous countries and religious foundations. Expensive referral to centers in Australia and New Zealand has been needed for complex patients. However, the changing landscape of international aid and evolving geopolitical tensions threaten the traditional paradigm. In this setting there is an urgent need for a regionally funded, centrally located, locally administered cardiac center to coordinate training of healthcare professionals as well as providing the medical and surgical care of children and adults with congenital and rheumatic heart disease throughout the South Pacific.

Background: The GBD (Global Burden of Disease) study provides a unique opportunity to better understand the socioeconomic and geographical drivers of clinical outcomes and mortality in adult congenital heart disease (ACHD) in the United States. Methods: We performed a secondary data analysis using a population‐based observational study design that studied state specific data on mortality and disability‐adjusted life years from 1990 to 2021 for patients with ACHD aged 20 years and older in the United States. These data were acquired from the GBD study. We subsequently correlated these data with median household income and percentage of uninsured people in each state by using the US census bureau poverty and income data. Results: In 2021, the GBD study identified 292 624 (95% CI, 258 956–342 906) adults ≥20 years with CHD living in the United States with 1074 (95% CI, 964–1331) ACHD related deaths and 62 244 (95% CI, 51 820–76 301) disability‐adjusted life years. The state with the highest mortality and disability‐adjusted life years (West Virginia) also has one of the lowest median household incomes ($51 122). States with lower ACHD related mortality have higher median household incomes ( r ² =0.62, P < 0.001). There is a lower correlation between mortality and the percentage of patients uninsured in each state ( r ² =0.14, P =0.0068). Conclusions: The GBD study provides unique insight into ACHD mortality and disability in the United States. Although mortality and disability rates have decreased from 1990 to 2021, patients with ACHD living in states with lower median household income and higher percentage of uninsured people have higher rates of mortality and disability. Ensuring adequate access to specialized care is warranted for this population.

BACKGROUND: The World Health Organization recommends echocardiographic screening and secondary prophylaxis for rheumatic heart disease (RHD), but evidence on how to implement these interventions within healthcare systems is limited. We report early implementation and clinical outcomes of an RHD program in northern Uganda. METHODS: From May 2023, the District Health Office in Kitgum trained primary healthcare workers in RHD screening and implemented registry-based secondary antibiotic prophylaxis in public facilities. We analyzed program and registry data through December 31, 2024. Data were obtained from aggregated program reports and individual-level records from the national RHD registry. We developed an RHD care cascade to examine screening uptake and outcomes, including adherence to secondary antibiotic prophylaxis. Adherence was categorized as perfect (100%), near perfect (90%–99%), acceptable (80%–89%), suboptimal (60% to 79%), or poor (2 tests. Time-to-event analysis was conducted to assess the probability of receiving an injection over time since enrollment. RESULTS: Health workers screened about 1% of the district’s target population each month. Over 18 months, 25 358 people were screened, including 1213 (4.8%) positive screens and 333 (1.3%) confirmed RHD cases. Among those screened, 16 406 (64%) were female, and 10 746 (42%) and 7333 (28%) were children (aged 5 years–14 years) and adolescents (aged 15 years–24 years), respectively. Of those with positive screens, 972 (80%) received confirmatory echocardiograms. Confirmed cases were relatively young (mean age 28 years), and 224 (67%) were female. Overall, 220 (75%) individuals achieved at least acceptable adherence. Among those who defaulted, 79% did so within 6 months of enrollment. CONCLUSIONS: An integrated RHD program in Uganda achieved successful delivery of echocardiographic screening within primary healthcare and acceptable early outcomes. The care cascade framework can be used to monitor similar RHD programs and identify opportunities for improvement.

Background: Early echocardiographic detection improves rheumatic heart disease (RHD) outcomes, but screening in endemic, resource‐limited settings remains a challenge. The Accelerating Delivery of Rheumatic Heart Disease Preventive Services in Northern Uganda program was developed to integrate task‐shifted RHD screening into the existing health system in Kitgum, Uganda. We evaluated the program’s impact on RHD case detection and assessed screening performance during its first year. Methods: We conducted a programmatic evaluation of all individuals screened via the program from May 2023 to April 2024, describing demographic and echocardiographic findings. We assessed sensitivity, specificity, predictive values, and accuracy of screenings performed by health care workers who had completed the full training, using expert‐confirmed diagnoses as the gold standard. Results: Of 11 793 individuals screened, 806 (6.8%) screened positive, with 544 receiving confirmatory testing. A total of 126 new RHD cases were diagnosed (74 mild, 52 moderate/severe). Among 3243 screenings by fully trained health care workers, sensitivity was 55% (95% CI, 47%–63%), specificity 97% (95% CI, 97%–98%), positive predictive value 52% (95% CI, 45%–60%), negative predictive value 98% (95% CI, 97%–98%), and accuracy 95% (95% CI, 95%–96%). Accuracy varied with age, sex, and health care worker screening volume: older age (odds ratio [OR], 0.96 [95% CI, 0.93–0.98]) and female sex (OR, 0.46 [95% CI, 0.22–0.91]) were associated with decreased accuracy, whereas higher screening volume improved accuracy (OR, 5.24 [95% CI, 2.52–10.65]). Conclusions: Task‐shifted echocardiographic screening can be effectively integrated into low‐resource health systems. Accelerating Delivery of Rheumatic Heart Disease Preventive Services in Northern Uganda trained a local screening workforce and identified 126 new RHD cases in its first year. Moderate sensitivity and subgroup variability indicate opportunities for improvement through targeted training and supportive technologies.

The lack of specific diagnostic tests for acute rheumatic fever (ARF) and its consequence rheumatic heart disease (RHD) is a key barrier to effective control of these diseases in low resource settings. Prothymosin alpha (PTA) and immunoglobulin G3 (IgG3) have recently been proposed as ARF biomarkers, although studies of these markers have so far been limited to single populations. Consequently, we evaluated PTA and IgG3 in patients with ARF, RHD and controls from studies set in Pakistan and Uganda.

Congenital heart disease is a leading cause of preventable death in children, with a disproportionate impact on low‐ and middle‐income countries. Despite progress in treating congenital heart disease globally, significant challenges remain in accessing specialized cardiovascular care, particularly cardiac surgery, in low‐ and middle‐income countries. This review examines current models of assistance and proposes a novel global training program to address these inequities. Key challenges identified include building program infrastructure, training health care providers, ensuring financial sustainability, and promoting local engagement. The proposed program, structured under a new international organization, will leverage emerging technologies to deliver accessible and rigorously assessed training in pediatric and congenital cardiac care. By collaborating with local experts and global partners, the program will promote access to education for various health care personnel involved in congenital heart disease care, establish credentialing standards, and foster global collaboration. This unified, scalable approach aims to bridge the health equity gap and accelerate progress toward comprehensive and sustainable cardiac care programs worldwide.

Background: Rheumatic heart disease (RHD) is a preventable cause of premature death among young individuals in low- and middle-income countries. Education is a key strategy to alleviate the burden of this disease. We aimed to assess the acceptability and knowledge gain of a series of low-literacy education flipcharts, presented during screening in high-burden areas of Brazil. Methods: Four low-literacy flipcharts were developed over three years and taught over 36 months to patients, community, school children, and health and education professionals, mostly in the state of Minas Gerais. In-person training and education workshops were assessed through printed surveys. Post-education surveys (for patients and community members), and post-training surveys (for healthcare and education professionals) were conducted from January 2023 to December 2025. A knowledge test, delivered at pre-training, post-training and three-month follow-up, was incorporated from January 2024 to March 2025. Results: Flipchart training was delivered to 1,317 healthcare and education professionals, while 1,292 patients and community members and 2,585 school students received education using the flipcharts. There was a statistically significant (p < 0.01) improvement in knowledge about rheumatic fever (RF) and RHD among healthcare and education professionals participating in the pre- and post-training survey (n = 511): RF as the cause of RHD (64% vs 95%), use of benzathine penicillin G (43% vs 98%), and frequency of antibiotic prophylaxis (21% vs 77%). The improvement from baseline was sustained at follow-up. Over the entire study period, 98% of survey respondents (2,134) reported learning something new, and 94% (2,041) intended to share the learnings with their peers or community. Conclusion: Culturally adapted, low-literacy educational flipcharts were successfully integrated into an existing RHD screening program in Brazil. The tool was well accepted among people living with RHD, their providers, and at-risk communities, with significant knowledge gain for healthcare and education professionals.

Background: Rheumatic heart disease (RHD) remains a significant public health concern in middle- to low-income countries. Despite advancements in healthcare access and public health measures in Brazil, future projections of RHD burden are essential to guide policy-making. Thus, we projected the national and regional burden of RHD in Brazil through 2050. Methods: Annual prevalence counts and disability-adjusted life years (DALYs) for RHD from 2000 to 2021 were extracted from the 2021 Global Burden of Disease (GBD) dataset for 10-year age brackets (5–74 years). Age-standardized prevalence (asPR) and DALYs rates (asDALYs) per 100,000 were calculated nationally and regionally. Bayesian age-period-cohort models were used to project trends through 2050, with results reported as medians (25th, 75th percentiles) and estimated annual percentage changes (EAPCs). Results: From 2000 to 2021, Brazil’s population grew by 27%. Nationally, the asPR declined slightly from 1,503 to 1,495 per 100,000 [EAPC: –0.04% (95% CI: –0.05, –0.03)], with reductions observed in most regions. However, increases were noted in the North [EAPC: 0.14% (95% CI: 0.13, 0.15)] and Northeast [EAPC: 0.02% (95% CI: 0.01, 0.03)]. Males experienced greater reductions [EAPC: –0.16% (95% CI: –0.19, –0.13)] compared to females, who showed a slight increase [EAPC: 0.05% (95% CI: 0.03, 0.07)]. Projections indicate that asPR will decline nationally to 1,418 per 100,000 by 2050 [EAPC: –0.20% (95% CI: –0.20, –0.19)], with the South and Central West regions reducing the most. The asDALY rates declined from 142 to 104 per 100,000 [EAPC: –1.58% (95% CI: –1.69, –1.46)] during 2000–2021, with all regions showing decreases, particularly the Southeast [EAPC: –1.83% (95% CI: –1.98, –1.69)]. Nationally, projections suggest further reductions to 75 per 100,000 by 2050 [EAPC: –1.17% (95% CI: –1.22, –1.11)]. Conclusion: The burden of RHD in Brazil has decreased nationally and regionally over recent decades. Projections suggest that these trends will continue.