Validation of a simplified score for predicting latent rheumatic heart disease progression using a prospective cohort of Brazilian schoolchildren

Abstract

ObjectivesEchocardiographic (echo) screening is an important tool to estimate rheumatic heart disease (RHD) prevalence, but the natural history of screen-detected RHD remains unclear. The PROVAR+ (Programa de RastreamentO da VAlvopatia Reumática) study, which uses non-experts, telemedicine and portable echo, pioneered RHD screening in Brazil. We aimed to assess the mid-term evolution of Brazilian schoolchildren (5–18 years) with echocardiography-detected subclinical RHD and to assess the performance of a simplified score consisting of five components of the World Heart Federation criteria, as a predictor of unfavourable echo outcomes.SettingPublic schools of underserved areas and private schools in Minas Gerais, southeast Brazil.ParticipantsA total of 197 patients (170 borderline and 27 definite RHD) with follow-up of 29±9 months were included. Median age was 14 (12–16) years, and 130 (66%) were woman. Only four patients in the definite group were regularly receiving penicillin.Primary and secondary outcome measuresUnfavourable outcome was based on the 2-year follow-up echo, defined as worsening diagnostic category, remaining with mild definite RHD or development/worsening of valve regurgitation/stenosis.ResultsAmong patients with borderline RHD, 29 (17.1%) progressed to definite, 49 (28.8%) remained stable, 86 (50.6%) regressed to normal and 6 (3.5%) were reclassified as other heart diseases. Among those with definite RHD, 13 (48.1%) remained in the category, while 5 (18.5%) regressed to borderline, 5 (18.5%) regressed to normal and 4 (14.8%) were reclassified as other heart diseases. The simplified echo score was a significant predictor of RHD unfavourable outcome (HR 1.197, 95% CI 1.098 to 1.305, p<0.001).ConclusionThe simple risk score provided an accurate prediction of RHD status at 2-year follow-up, showing a good performance in Brazilian schoolchildren, with a potential value for risk stratification and monitoring of echocardiography-detected RHD.

Outcomes of Echocardiography‐Detected Rheumatic Heart Disease: Validating a Simplified Score in Cohorts From Different Countries

“…Subsequently, an external validation was performed in a Brazilian cohort, with good – although more modest – discrimination of those at risk of echo progression. 14 Now, for the first time, the use of multinational longitudinal data, managed by different research groups, has allowed for a more robust assessment of the power of the score to predict outcomes.…”

“…There were major differences in baseline characteristics between cohorts, such as the higher proportions of definite cases in New Zealand 16 and Malawi, 15 while the cohort from Australia 13 demonstrated worse risk and progression profiles in sharp contrast to the milder phenotypes observed in the Brazilian RHD cohort. 14 There are, in addition, known epidemiological differences as well as screening approaches in these different settings. In Oceania, for example, there is a disproportionally high prevalence of RHD with a trend to severe phenotypes in indigenous and rural populations where screening programs are focused.…”

“…Lead investigators from different research groups were contacted and invited to participate in the pooled analysis. After acceptance, patient‐level baseline and follow‐up data of children (aged 5 to 18 years) diagnosed with latent RHD during screening studies conducted in 4 countries (Australia, 13 n=62; Brazil [sample not included in the original derivation study], 14 n=197; Malawi, 15 n=40; New Zealand, 16 n=94) were provided. The data incuded echocardiographic findings, derived from studies with portable or handheld devices, for each of the variables used in making a diagnosis under the WHF criteria.…”

Single-View Echocardiography by Nonexpert Practitioners to Detect Rheumatic Heart Disease: A Prospective Study of Diagnostic Accuracy

“…Secondary analyses were conducted to determine the accuracy of the index test for different severity of definite RHD, and to identify changes in sensitivity and specificity using different cutoff values for MR jet length. Borderline and definite RHD cases were classified as low-, intermediate-, or high-risk of disease progression based on echocardiographic features, 32 , 33 and diagnostic accuracy calculated for these categories (Table II in the Data Supplement ).…”

Echocardiography Screening for Latent Rheumatic Heart Disease: What Can We Do in Indonesia?

“…Another prospective cohort study validated this scoring system in subclinical RHD schoolchildren as part of an RHD screening program in Brazil. They reported that the simplified echo study was accurate in predicting an early outcome with 29 ± 9 (range 11–48) months' follow-up ( 17 ).…”