World Heart Federation criteria for echocardiographic diagnosis of rheumatic heart disease--an evidence-based guideline

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.

Figure 1

Schematic images of the MV in systole. a | A normal MV. b | RHD with excessive leaflet tip motion, which results in abnormal coaptation and regurgitation, but usually does not meet the echocardiographic definition of ‘MV prolapse’. c | Echocardiographic MV prolapse, defined by >2 mm billowing of the leaflet tissue into the left atrium. In echocardiographic MV prolapse (c), coaptation of leaflets often remains normal, as the free edges of the leaflet stay in apposition below the plane of the MV annulus. Abbreviation: AMVL, anterior MV leaflet; MV, mitral valve; PMVL, posterior MV leaflet; RHD, rheumatic heart disease.

Figure 2

Schematic images of the MV in diastole. a | A normal MV. b | A rheumatic MV with thickened and restricted anterior and posterior leaflets. Abbreviations: AMVL, anterior MV leaflet; MV, mitral valve; PMVL, posterior MV leaflet.