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Andersons pediatric cardiology 1458

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2 In at least one view jet length is ≥1 cmb Peak velocity ≥3 m/s Pandiastolic jet in at least one envelope Morphologic Features of RHD MV AMVL thickening ≥3 mmc Chordal thickening Restricted leaflet motion Excessive leaflet tip motion during systole AV Irregular or focal thickening Coaptation defect Restricted leaflet motion Prolapse Borderline RHD category only applies to individuals younger than 21 years AR, Aortic regurgitation; AMVL, aortic mitral valve leaflet; AV, aortic valve; MR, mitral regurgitation; MS, mitral stenosis; MV, mitral valve; RHD, rheumatic heart disease aCongenital anomalies must be excluded bA regurgitant jet length should be measured from the vena contracta to the last pixel of regurgitant colour (blue or red) on nonmagnified (nonzoomed) images cAMVL thickness should be measured during diastole at full excursion Measurement should be taken at the thickest portion of the leaflet and should be performed on a frame with maximal separation of chordae from the leaflet tissue Diagnosis of RHD Without a Previous History of ARF The majority of patients with RHD worldwide fit into the category of having RHD with no prior history of ARF The reason for cardiology referral or assessment may include evaluation of cardiac murmur, symptomatic status, complications of RHD, or abnormality detected on echocardiographic screening The 2012 World Heart Federation echocardiographic criteria were established to facilitate early diagnosis of RHD in individuals without a previous history of ARF.22 Box 55.1 details the minimum diagnostic criteria In this setting, pathologic regurgitation is insufficient to diagnose RHD Both morphologic features of RHD and pathologic regurgitation must be present or, alternatively, there must be multivalve disease of both the MVs and AVs If echocardiography is not available, then the diagnosis relies on auscultatory findings of valvar dysfunction Naturally auscultation alone does not determine the etiology of disease In this setting, the pretest probability of RHD will determine diagnostic and management strategies Disease Progression The outcome and progression of RHD relate to the severity at diagnosis, exposure to ARF recurrences, and access to tertiary medical care.11,23 Those with mild RHD at diagnosis and good adherence to secondary prophylaxis have excellent long-term outcomes.8,11 Those with severe disease at diagnosis have a bleak prognosis and will likely require cardiosurgical intervention within 2 years of diagnosis or will succumb.23,24 In the United States, before the introduction of secondary prophylaxis, 20-year mortality due to RHD was as high as 30% to 80%, with most affected individuals dying before the age of 30 years.25,26 Similar findings are still observed in many low- and middle-income countries, with annual mortality rates of 3.0% to 12.5% In Nigeria and Ethiopia, the mean age at death is below 25 years.27,28 In New Zealand, a high-income country, the outcome of RHD is more favorable, with a median life expectancy of 56 years.29 The New Zealand data may reflect the tail of RHD in European adults who had milder forms of RHD from earlier decades, when ARF affected adults of European ethnicity, or it may reflect better access to medical care Finally, there are limitations to using International Classification of Diseases (ICD) discharge data when nonspecified valvar disease is attributed a rheumatic etiology Overall, it can be said that the disease progression and mortality due to RHD is greatest in low-income countries, and within those countries it is the most disadvantaged groups that have the worst outcomes ... The majority of patients with RHD worldwide fit into the category of having RHD with no prior history of ARF The reason for cardiology referral or assessment may include evaluation of cardiac murmur, symptomatic status,

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