A clinical appraisal of different Z-score equations for aortic root assessment in the diagnostic evaluation of Marfan syndrome
2013; Elsevier BV; Volume: 15; Issue: 7 Linguagem: Inglês
10.1038/gim.2012.172
ISSN1530-0366
AutoresRoland R.J. van Kimmenade, Marlies Kempers, Menko‐Jan de Boer, Bart Loeys, Janneke Timmermans,
Tópico(s)Aortic Disease and Treatment Approaches
ResumoPurposeAortic sinus diameter dilatation expressed as a Z-score >2.0 is diagnostic in Marfan syndrome. In addition to the classic equation (Z1) for calculating Z-scores, two new equations were recently introduced (Z2 and Z3).MethodsWe studied the effects of obesity, age, and the absolute cut point of 40mm on these three equations in 2,674 echocardiographic measurements of 260 patients with Marfan syndrome.ResultsDiameters ≥40mm were associated with Z1 scores <2.0 in 109 measurements (11.0%; 35 patients), Z2 scores <2.0 in 37 measurements (3.8%; 13 patients), and Z3 scores <2.0 in 24 measurements (2.4%; 11 patients). Mean diameters increased after the 40th birthday: 42.0 (37.3–44.8 mm interquartile range) to 42.5 (39.0–45.0 mm interquartile range; P = not significant) and mean Z1 scores decreased from 3.60 to 2.17 (P < 0.01), whereas Z2 and Z3 scores tended to increase (Z2: 3.04–3.27; Z3: 3.39–3.55; P = not significant for both). Comparing Z-scores between patients with body mass index <25kg/m² (group A) and those with body mass index ≥25kg/m² (group B), median Z1 scores differed between groups (Z1 = 3.00 in group A, Z1 = 1.78 in group B; P = 0.012), whereas Z2 (Z2 = 2.82 in group A, Z2 = 2.47 in group B; P = 0.52) and Z3 scores (Z3 = 2.72 in group A, Z2 = 3.12 in group B; P = 0.32) did not.ConclusionZ1 scores are inferior to Z2 and Z3 scores in Marfan syndrome. In particular, the Z3 score, correcting aortic sinus diameter for body height, seems excellent.Genet Med 2013:15(7):528–532
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