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FIG 55.3 Pattern of native rheumatic heart disease in 2475 children and adults with no percutaneous or surgical intervention in the international REMEDY registry for rheumatic heart disease AVD, Aortic valve disease; MAVD, mixed AVD; MMAVD, mixed mitral and AVD; MMVD, mixed mitral valve disease; MR, mitral regurgitation; MS, mitral stenosis Patients younger than 10 years predominantly have pure MR.15,18 By the second decade of life, RHD is characterized by mixed MV disease In the third decade of life, up to 5% of RHD patients will develop pure MS.15,18 In some regions of Sub-Saharan Africa and India, pure MS is more common and often occurs earlier in life.19 Concomitant AV disease increases with age and is present in over 50% of patients by the second decade (see Fig 55.3).15 Like MV disease, aortic disease in the young is characterized by regurgitation By the second decade of life, mixed aortic disease develops in some; however, pure aortic stenosis is generally not seen until the fourth or fifth decade of life.15 Diagnostic Criteria for Rheumatic Heart Disease The majority of patients with RHD are diagnosed late, when individuals present with complications of RHD including heart failure, infective endocarditis, tachyarrhythmias, stroke, pregnancy-related complications, or sudden death.15 Patients with RHD often have a long latent phase of asymptomatic valvar heart disease, often without any preceding symptoms of ARF The global registry of RHD, REMEDY, suggests that even in high-income populations, only 59% of patients with RHD had a preceding history of ARF, and this drops to 22% in low-income populations.15 The reason for this is likely multifactorial, and access to health care and public awareness are important factors The current ARF guidelines may not be sufficiently sensitive to detect ARF in high-risk populations Patients may not present to local health facilities with what might be seen as relatively minor symptoms or, alternatively, medical officers may not be equipped to make the diagnosis In contrast to the arthritis of ARF, acute carditis may not cause symptoms Consequently the early diagnosis of RHD remains challenging This is especially so in resource-poor settings Diagnosis of RHD in the Setting of a Documented Episode of ARF In individuals with a documented history of ARF, once acute inflammation subsides, the persistence of pathologic regurgitation of the mitral and/or AVs on echocardiography is sufficient to confirm chronic RHD The echocardiographic criteria to diagnose pathologic mitral and aortic regurgitation (AR) are detailed in Box 55.1.20 In circumstances where echocardiography is not available, the persistence of a mitral and/or aortic regurgitant murmur is sufficient, although auscultation has a low positive predictive value and hence low diagnostic utility in detecting mild regurgitation.21 Box 55.1 World Heart Federation Criteria for the Echocardiographic Diagnosis of Rheumatic Heart Disease Echocardiographic Criteria for RHDa Definite RHD (Either A, B, C, or D): A Pathologic MR and at least two morphologic features of RHD of the MV B MS mean gradient ≥4 mm Hg C Pathologic AR and at least two morphologic features of RHD of the AV D Borderline disease of both the AV and MV Borderline RHD (Either A, B, or C): A At least two morphologic features of RHD of the MV without pathologic MR or MS B Pathologic MR C Pathologic AR Echocardiographic Criteria for Pathologic Regurgitation (All Four Doppler Criteria Must Be Met) Pathologic MR Seen in two views In at least one view jet length is ≥2 cmb Peak velocity ≥3 m/s Pansystolic jet in at least one envelope Pathologic AR Seen in two views