title: Marfan syndrome in children and adolescents: an adjusted nomogram for screening aortic root dilatation.
abstract: OBJECTIVE
  To construct an adjusted nomogram for the echocardiographic screening of aortic root diameter in children with possible Marfan disease.
  
  
  METHODS
  In 91 children (42 boys, 49 girls, age range 3.2 to 18.4 years) undergoing Marfan screening from 1983 until 1996, the diagnosis Marfan syndrome and any other aortic pathology was definitely ruled out. These served as a control population to set appropriate reference standards.
  
  
  RESULTS
  Compared with a standard Dutch reference population, body surface area of the control subjects (mean (SD)) was above the 50th centile (boys 0.09 (0.20) m2, range -0.28 to 0.69 m2; girls 0.09 (0.17) m2, range -0.17 to 0.69 m2). Echocardiographically determined aortic root diameter and body surface area showed a linear relation and a greater variability of aortic root diameter in these relatively tall subjects (n = 91, R2 = 0.62) than in the standard nomogram (n = 56, R2 = 0.93). In 24% of cases (n = 22), the aortic root exceeded the upper limit of normal in the standard nomogram, by 2.2 (2.0) mm. An adjusted nomogram was constructed with a higher upper limit.
  
  
  CONCLUSIONS
  A Marfan screening population differs from the unselected population in body surface area and aortic root size variability. An adjusted nomogram should therefore be used to detect a truly enlarged aortic root.
authors.affiliation: Department of Cardiology, Academic Medical Centre, Amsterdam, The Netherlands.
authors.forename: L
authors.initials: L
authors.lastname: Rozendaal
authors.forename: M
authors.initials: M
authors.lastname: Groenink
authors.forename: M S
authors.initials: MS
authors.lastname: Naeff
authors.forename: R C
authors.initials: RC
authors.lastname: Hennekam
authors.forename: A A
authors.initials: AA
authors.lastname: Hart
authors.forename: E E
authors.initials: EE
authors.lastname: van der Wall
authors.forename: B J
authors.initials: BJ
authors.lastname: Mulder
mesh_terms: D000293:Adolescent; D001011:Aorta / Q000000981:diagnostic imaging / Q000473:pathology; D001830:Body Surface Area; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000000981:diagnostic imaging* / Q000473:pathology / Q000517:prevention & control; D008403:Mass Screening / Q000379:methods