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Background: Although rheumatic heart disease remains the leading cause of valve heart disease (VHD) in developing countries, other forms of valve disease have been over shadowed and not regarded as a public health problem. However, several facts suggest that the role of non-rheumatic VHD as a significant cardiovascular disease should be reconsidered. We aimed to assess the prevalence and characteristics of different forms of primary left sided valve diseases from a series of 15,009 echocardiographic studies., Methods: This was a retrospective review of echocardiographic reports for studies performed between January 2012 and December 2013 (24 months) at Uganda Heart Institute. All patients with primary left-sided valve disease were classified into one of five major diagnostic categories and in each diagnostic category; patients were sub-classified into stages A-D of primary valve disease as defined by the American College of Cardiology., Results: Three thousand five hundred eighty-two echocardiography reports qualified for final data analysis. The "sclerotic valve changes with normal valve function", a Stage A sub-class of "degenerative valve disease" overwhelmingly overshadowed all the other diagnostic categories in this stage. "Rheumatic Heart Disease", "Degenerative Valve Disease", "Bicuspid Aortic Valve", "Mitral Valve Prolapse" and "Endomyocardial Fibrosis" diagnostic categories accounted for 53.0%, 41.8%, 2.2%, 1.4% and 1.7% respectively in stages B-D of primary VHD. Rheumatic heart disease disproportionately affected the young, productive age groups. It was the major risk factor for infective endocarditis; and was the indication for valve surgery in 44 of 50 patients who had undergone valve replacement procedures., Conclusions: We acknowledge that rheumatic heart disease remains a leading cause of progressive and severe primary left-sided valve disease among young adults in Uganda. But we bring to light the contemporary footprints of other forms of primary valve disease that require coordinated multidisciplinary approach to research, education and clinical management to ensure improved patient outcomes.

Background: Screening echocardiography has emerged as a potentially powerful tool for early diagnosis of rheumatic heart disease (RHD). The utility of screening echocardiography 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 echocardiograms were re-reviewed by experts (2012 World Heart Federation criteria) for inclusion and evidence of change. Bi- and multivariable logistic regression, Kaplan-Meier analysis, and Cox proportional hazards models, as well, 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-to-severe definite RHD, 47.6% had echocardiographic progression (including 2 deaths), and 9.5% had echocardiographic regression. Children with mild definite and borderline RHD showed 26% and 9.8% echocardiographic progression and 45.2% and 46.3% echocardiographic 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. Although long-term outcome remains unclear, the initial change in latent RHD may be evident during the first 1 to 2 years following diagnosis. Natural history data are inherently limited, and a randomized clinical trial is needed to definitively determine the impact of penicillin prophylaxis on the trajectory of latent RHD., (© 2017 American Heart Association, Inc.)