title: Using a Low-Risk Population to Estimate the Specificity of the World Heart Federation Criteria for the Diagnosis of Rheumatic Heart Disease.
abstract: BACKGROUND
  The 2012 World Heart Federation (WHF) criteria set the minimum standards for echocardiographic diagnosis of rheumatic heart disease (RHD) in a high-risk population without history of acute rheumatic fever. The high sensitivity of the 2012 WHF criteria is well accepted, but the specificity cannot be directly assessed without a confirmatory test. The objective of this study was to estimate the false-positive rate of the WHF criteria through their application in a population at very low risk for RHD.
  
  
  METHODS
  The Children's National Health System echocardiography database was searched for patients (6-15 years of age) with an International Classification of Diseases, Ninth Revision, code for "chest pain." Chart review was performed; children with congenital heart disease or systemic disease were excluded. Patients were classified according to WHF criteria as having normal, borderline, or definite RHD on the basis of mitral and aortic valve morphology and the presence of pathologic mitral and/or aortic regurgitation.
  
  
  RESULTS
  A total of 1,251 studies were identified, and 152 were excluded. Fifty studies were randomly chosen from each age, with equal gender distribution, for a total of 500 echocardiograms. No patients met the criteria for definite RHD and four (0.8%) for borderline RHD, both by pathologic mitral regurgitation. No patients met the criteria for borderline RHD on the basis of mitral valve morphology or pathologic aortic regurgitation.
  
  
  CONCLUSIONS
  In a US pediatric population, no children had findings of definite RHD, and 0.8% had findings of borderline RHD, most likely representing false-positive results. Although there appears to be some overlap between the findings of borderline RHD and those in the normal pediatric population, our data suggest acceptable specificity for all RHD, with excellent specificity for definite RHD.
authors.affiliation: Division of Cardiology, Children's National Health System, Washington, District of Columbia. Electronic address: brclark@childrensnational.org.
authors.forename: Bradley C
authors.initials: BC
authors.lastname: Clark
authors.affiliation: Division of Cardiology, Children's National Health System, Washington, District of Columbia.
authors.forename: Anita
authors.initials: A
authors.lastname: Krishnan
authors.affiliation: Department of Biostatistics and Informatics, Children's National Health System, Washington, District of Columbia.
authors.forename: Robert
authors.initials: R
authors.lastname: McCarter
authors.affiliation: Division of Cardiology, Children's National Health System, Washington, District of Columbia.
authors.forename: Janet
authors.initials: J
authors.lastname: Scheel
authors.affiliation: Division of Cardiology, Children's National Health System, Washington, District of Columbia.
authors.forename: Craig
authors.initials: C
authors.lastname: Sable
authors.affiliation: Division of Cardiology, Children's National Health System, Washington, District of Columbia.
authors.forename: Andrea
authors.initials: A
authors.lastname: Beaton
mesh_terms: D000293:Adolescent; D001022:Aortic Valve Insufficiency / Q000000981:diagnostic imaging / Q000453:epidemiology*; D002309:Cardiology / Q000592:standards; D002648:Child; D004452:Echocardiography / Q000592:standards* / Q000706:statistics & numerical data; D005189:False Positive Reactions; D005260:Female; D006801:Humans; D038622:Internationality; D008297:Male; D008944:Mitral Valve Insufficiency / Q000000981:diagnostic imaging / Q000453:epidemiology*; D015588:Observer Variation; D017410:Practice Guidelines as Topic; D015995:Prevalence; D015203:Reproducibility of Results; D012214:Rheumatic Heart Disease / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D012307:Risk Factors; D012680:Sensitivity and Specificity; D014481:United States / Q000453:epidemiology
keywords: Aortic regurgitation; Echocardiogram; Mitral regurgitation; Rheumatic heart disease; World Heart Federation criteria