Screening-detected rheumatic heart disease can progress to severe disease

Abstract

Objectives Echocardiography is a sensitive test for rheumatic heart disease (RHD) screening; however the natural history of RHD detected on screening has not been established. We aimed to evaluate the progression of screening-detected RHD in Fiji. Methods All young people previously diagnosed with RHD through screening, with echocardiograms available for review, were eligible. All baseline echocardiograms were reported again. Participants underwent follow-up echocardiography. A paediatric cardiologist determined the diagnosis using the World Heart Federation criteria and assessed the severity of regurgitation and stenosis. Results Ninety-eight participants were recruited (mean age, 17 years; median duration of follow-up, 7.5 years). Two other children had died from severe RHD. Fourteen of 20 (70%) definite RHD cases persisted or progressed, including four (20%) requiring valve surgery. Four (20%) definite RHD cases improved to borderline RHD and two (10%) to normal. Four of 17 (24%) borderline cases progressed to definite RHD (moderate: 2; severe: 2) and two (12%) improved to normal. Four of the 55 cases reclassified as normal at baseline progressed to borderline RHD. Cases with a follow-up interval >5 years were more likely to improve (37% vs 6%, p=0.03). Conclusions The natural history of screening-detected RHD is not benign. Most definite RHD cases persist and others may require surgery or succumb. Progression of borderline cases to severe RHD demonstrates the need for monitoring and individualised consideration of prophylaxis. Robust health system structures are needed for follow-up and delivery of secondary prophylaxis if RHD screening is to be scaled up.

Latent Rheumatic Heart Disease

“…Progression is challenging to directly compare across cohorts, even in those using the 2012 WHF criteria, due to inconsistent definitions of progression, use of different binomial outcomes (stable + progression), and inclusion of children with advanced RHD. However the ratio of progression among those with mild definite RHD compared to those with borderline in our cohort (2.5:1) is similar to reports from Fiji (2.5:1) 24 . The progression seen among children with borderline RHD in our study (10%) is also similar to smaller recent cohorts from New Caledonia (5%) 28 and Fiji (12%) 24 , although lower than reported from South Africa (20%) 27 and Australia (24%) 25 .…”

“…In our cohort, only 2 such children (9.5%) showed improvement over the study period, and there were 2 deaths (9.5%). Similar findings were recently reported from Fiji, where more than 80% of children with moderate-to-severe RHD at time of screening showed persistence or progression 24 . When screening reveals moderate to severe RHD, children should be considered “missed clinical” cases, and treated as such, with referral to a specialist provider, given consideration of BPG prescription, and valvular intervention as appropriate and available.…”

“…It may be that we were underpowered to find differences, however the previous report had similarly small numbers (19 children with ≥ 5 years follow-up 24 ) and was underpowered to conduct a multivariate analysis.. It is also important to remember that in our study, we are referencing only initial progression.…”

“…Children with definite RHD were classified by severity of regurgitation at the most affected valve (mild, moderate, severe), or severe if any mitral stenosis was present at baseline. Children with non-specific valvular abnormalities not meeting criteria for RHD by the 2012 WHF criteria 9 were excluded, as outcomes for these children have been previously reported as benign 22, 24 .…”

“…Three of these were published prior to the publication of standardized criteria for latent RHD diagnosis 8, 23, 26 , making them difficult to compare. The remaining 5 studies 22, 24, 25, 27, 28 report on relatively small cohorts, ranging from 25–55 children limiting the power to detect features that predict progression or regression of disease.…”