Abstract:
Over the past 5 years, the advent of echocardiographic screening for rheumatic heart disease
(RHD) has revealed a higher RHD burden than previously thought. In light of this global experience, the
development of new international echocardiographic guidelines that address the full spectrum of the
rheumatic disease process is opportune. Systematic differences in the reporting of and diagnostic approach
to RHD exist, reflecting differences in local experience and disease patterns. The World Heart Federation
echocardiographic criteria for RHD have, therefore, been developed and are formulated on the basis of the
best available evidence. Three categories are defined on the basis of assessment by 2D, continuous-wave,
and color-Doppler echocardiography: ‘definite RHD’, ‘borderline RHD’, and ‘normal’. Four subcategories of
‘definite RHD’ and three subcategories of ‘borderline RHD’ exist, to reflect the various disease patterns.
The morphological features of RHD and the criteria for pathological mitral and aortic regurgitation are also
defined. The criteria are modified for those aged over 20 years on the basis of the available evidence. The
standardized criteria aim to permit rapid and consistent identification of individuals with RHD without a clear
history of acute rheumatic fever and hence allow enrollment into secondary prophylaxis programs. However,
important unanswered questions remain about the importance of subclinical disease (borderline or definite
RHD on echocardiography without a clinical pathological murmur), and about the practicalities of implementing
screening programs. These standardized criteria will help enable new studies to be designed to evaluate the
role of echocardiographic screening in RHD control.
