Latent Rheumatic Heart Disease

Study Questions:

What are the longitudinal outcomes of a cohort of children with latent rheumatic heart disease (RHD) and risk factors for unfavorable outcomes?

Methods:

The investigators conducted a prospective natural history study conducted under the Ugandan RHD registry. Children with latent RHD and >1-year follow-up were included. All echocardiograms were reviewed by experts (2012 World Heart Federation criteria) for inclusion and evidence of change. Bi- and multivariable 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 (interquartile range, 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 two deaths), and 9.5% had echo regression. Children with mild definite and borderline RHD showed 26% and 9.8% echo progression and 45.2% and 46.3% showed 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:

The authors concluded that latent RHD is a heterogeneous diagnosis with variable disease outcomes, and children with moderate to severe latent RHD have poor outcomes.

Perspective:

This study reports that latent RHD is a heterogeneous diagnosis with variable disease outcomes. While children with moderate-to-severe latent RHD found by echo screening have poor outcomes, children with both borderline and mild definite RHD have better outcomes but remain at substantial risk of progression, and at a minimum should be enrolled in close clinical follow-up. Furthermore, since natural history data are inherently limited by confounding variables, a randomized controlled clinical trial is needed to definitively assess the impact of penicillin prophylaxis on the trajectory of latent RHD.

Clinical Topics: Noninvasive Imaging, Prevention, Valvular Heart Disease, Echocardiography/Ultrasound

Keywords: Diagnostic Imaging, Disease Progression, Echocardiography, Heart Valve Diseases, Mitral Valve, Outcome Assessment (Health Care), Pediatrics, Penicillins, Primary Prevention, Rheumatic Heart Disease, Risk Factors


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