Clinical outcomes of children with rheumatic heart disease

Abstract

ObjectiveTo evaluate the long-term clinical outcomes of children with rheumatic heart disease (RHD) in Uganda, and determine characteristics that predict adverse outcomes.MethodsThis retrospective cohort study evaluated the risk of death in Ugandan children with clinical RHD from 2010 to 2018; enrolling children aged 5–18 years old from an existing registry. Demographic data and clinical data (baseline complications, RHD severity, cardiac interventions) were collected. The primary outcome was survival. Univariable and multivariable hazard ratios (HR) were obtained from Cox proportional hazards regression. Survival probabilities were developed using Kaplan-Meier curves; log-rank tests compared survival based on cardiac interventions, disease severity and time of enrolment.Results612 cases met inclusion criteria; median age 12.8 years (IQR 5.3), 37% were male. Thirty-one per cent (187 of 612) died during the study period; median time to death 7.8 months (IQR 18.3). In univariable analysis, older age (HR 1.26, 95% CI=1.0 to 1.58), presence of baseline complications (HR 2.06, 95% CI=1.53 to 2.78) and severe RHD (HR 5.21, 95% CI=2.15 to 12.65) were associated with mortality. Cardiac intervention was associated with a lower risk of mortality (HR 0.06, 95% CI=0.02 to 0.24). In multivariable models, baseline complications (HR 1.78, 95% CI=1.31 to 2.41), severe RHD (HR 4.58, 95% CI=1.87 to 11.23) and having an intervention (HR 0.05, 95% CI=0.01 to 0.21) remained statistically significant. Kaplan-Meier survival curves demonstrated >25% mortality in the first 30 months, with significant differences in mortality based on intervention status and severity of disease.ConclusionsThe mortality rate of children with clinical RHD in Uganda exceeds 30%, over an 8-year time frame, despite in-country access to cardiac interventions. Children at highest risk were those with cardiac complications at baseline and severe RHD.

Global threat of rheumatic heart disease: do not forget children!

“…When there is no local infrastructure, cardiac surgery can be performed by visiting non-governmental organisations, with good immediate results, but the long-term impact on patient outcome has not been evaluated. Consistently with established knowledge on heart valve disease in adults, the performance of valve surgery was associated with a major decrease in mortality, as illustrated by the adjusted HR of 0.05 (95% CI 0.01 to 0.21) in the paper by Zimmerman et al 2. Operative mortality was low, attesting for a particularly striking benefit of valvular interventions in young patients.…”

“…The paper by Zimmerman et al 2 further confirms the insufficient access to cardiac surgery in African countries, where the prevalence of RHD is particularly high. Although Uganda has a cardiac surgery programme, only 11 of the 73 cardiac interventions were performed in Uganda 2.…”

“…The paper by Zimmerman et al 2 further confirms the insufficient access to cardiac surgery in African countries, where the prevalence of RHD is particularly high. Although Uganda has a cardiac surgery programme, only 11 of the 73 cardiac interventions were performed in Uganda 2. The shortage in cardiac surgery resources in Africa has also been reported in the VALVular disease in AFRICa (VALVAFRIC) registry and contributes to high mortality related to RHD 4.…”

“…Mortality was lower in a series of 592 indigenous Australians aged between 5 and 24 years presenting with RHD 3. However, there were major differences with the series of Zimmerman et al 2 : only 16% of patients had severe heart valve disease in the Australian series vs 79% in the series from Uganda, and valvular intervention was more frequently performed in the Australian series (16% vs 12%; 50% of those with severe heart valve disease) despite less severe presentations. This emphasises the need for country-specific data taking into account local particularities in healthcare access.…”

“…The paper by Zimmerman et al 2 fills an important gap, not only because it reports data from a Sub-Saharan country, but also due to the specific study of RHD in 612 young patients aged ≤18 years, with a median age of 13 years. Although RHD typically affects young adults, early valvular remodelling following recurrent acute rheumatic fever episodes may occur in countries where there is a high burden of group A streptococcal infections.…”