Latent Rheumatic Heart Disease

Abstract

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 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 (IQR 2.0–2.9). Penicillin prophylaxis was prescribed in 49.3% with overall adherence of 84.7%. Of children with moderate/severe definite RHD, 47.6% had echo progression (including 2 deaths), and 9.5% echo regression. Children with mild definite and borderline RHD showed 26% and 9.8% echo progression and 45.2% and 46.3% echo 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. While long-term outcome remains unclear, the initial change in latent RHD may be evident during the first 1–2 years following diagnosis. Natural history data is inherently limited and a randomized clinical trial is needed to definitively determine the impact of penicillin prophylaxis on the trajectory of latent RHD.

Cost-Effectiveness of Rheumatic Heart Disease Echocardiographic Screening in Brazil: Data from the PROVAR+ Study: Cost-effectiveness of RHD screening in Brazil

“…Only recently (2012) the WHF published an expert consensus for the echocardiographic diagnosis of asymptomatic RHD. For this project, most of the transition probabilities of RHD itself is taken from primary data of a study conducted in Uganda and secondary data from a study conducted in Australia [25,26].…”

“…The question is in how applicable this data is for the Brazilian context, because environmental factors are reported to have a big impact on the disease and its course [5]. The main study from which transition probabilities from Asymptomatic Borderline RHD to other states derived was conducted on a sample of children from the cities Kampala and Gulu in Uganda [26]. These areas are believed not to differ too much from the metropolitan context in Brazil.…”

Rheumatic fever – new diagnostic criteria

“…Authors suggests that ventricular dyssynchrony as a marker of latent carditis in children with acute rheumatic fever. Beaton et al [ 13 ] examined using echocardiography 227 patients with RF and with latent rheumatic heart disease and assessed risk of poor outcome in this group. The worse prognosis concerned children with moderate to severe intensity of LRHD.…”

Inter-scallop separations of the posterior leaflet of the mitral valve: an important cause of ‘pathological’ mitral regurgitation in rheumatic heart disease screening

“…Consequently, if not recognized and excluded from analysis, could falsely increase the prevalence of ‘borderline RHD’ detected in any RHD screening program. In addition, failure to exclude these cases could further distort the findings of long-term studies documenting the outcome of ‘borderline RHD’ as progression of MR from this entity remains unexplored ( 22 , 23 , 24 ).…”