2010 Revised Ghent Nosology for Marfan syndrome
Z-score calculator (≥ 15 years of age)
Z = (Measured aortic root diameter - predicted diameter)/ SD
Note: SD = 0.261 cm
Devereux (BSA-based)
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Predicted Aortic Root Diameter = 2.423 + (age x 0.009) + (BSA x 0.46) - (sex x0.267)
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Note: Use 1 for males and 2 for females for the 'sex' variable
Du Bois and Du Bois:
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BSA = (0.007184 x Hู^0.725 x W^0.425)
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Note: H is height in cm, and W is weight in kg.
A Z-score of 2.0 corresponds to the 97.7th percentile, meaning the value is more than two standard deviations above the mean.
0
Reduced Upper Segment / Lower Segment & Increased Arm span / Height (+1)
* At least three of the five typical facial characteristics including dolichocephaly, downward slanting palpebral fissures, enophthalmos, retrognathia and malar hypoplasia.
Result
Does not meet the diagnostic criteria for Marfan syndrome.
Reference
Devereux RB, de Simone G, Arnett DK, Best LG, Boerwinkle E, Howard BV, Kitzman D, Lee ET, Mosley TH Jr, Weder A, Roman MJ. Normal limits in relation to age, body size and gender of two-dimensional echocardiographic aortic root dimensions in persons ≥15 years of age. Am J Cardiol. 2012 Oct 15;110(8):1189-94. doi: 10.1016/j.amjcard.2012.05.063. Epub 2012 Jul 6. PMID: 22770936; PMCID: PMC3462295.
Loeys BL, Dietz HC, Braverman AC, Callewaert BL, De Backer J, Devereux RB, Hilhorst-Hofstee Y, Jondeau G, Faivre L, Milewicz DM, Pyeritz RE, Sponseller PD, Wordsworth P, De Paepe AM. The revised Ghent nosology for the Marfan syndrome. J Med Genet. 2010 Jul;47(7):476-85. doi: 10.1136/jmg.2009.072785. PMID: 20591885.