Comparability of different Z-score equations for aortic root dimensions in children with Marfan syndrome

Aortic root dilation is a major complication of Marfan syndrome and is one of the most important criteria in establishing the diagnosis. Currently, different echocardiographic nomograms are used to calculate aortic root Z-scores. The aim of the present study was to assess the potential differences i...

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Bibliographic Details
Published in:	Cardiology in the young 2021-12, Vol.31 (12), p.1962-1968
Main Authors:	Rutten, Dominiek W.E., Aarts-Janssen, Ilse J.H., Kempers, Marlies J.E., Reimer, Annette G., Udink ten Cate, Floris E.A., Loeys, Bart L., Slieker, Martijn G.
Format:	Article
Language:	eng
Subjects:	Aorta Aortic dissection Children Coronary vessels Diagnosis Diameters Doppler effect Echocardiography and Scans Marfan syndrome Marfan's syndrome Mathematical analysis Nomograms Original Article Patients Pediatrics Sinuses Ultrasonic imaging
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Summary:	Aortic root dilation is a major complication of Marfan syndrome and is one of the most important criteria in establishing the diagnosis. Currently, different echocardiographic nomograms are used to calculate aortic root Z-scores. The aim of the present study was to assess the potential differences in aortic root measurements when aortic root Z-scores were obtained in a cohort of paediatric Marfan patients using several published nomograms. In a cohort of 100 children with Marfan syndrome, Z-scores for aortic root dimensions were calculated according to the nomograms of Pettersen et al, Gautier et al, Colan et al, and Lopez et al. Bland-Altman plots were used to estimate mean differences in Z-scores and to establish limits of agreement. The mean Z-score of the sinus of Valsalva for Lopez et al was significantly higher compared to Gautier et al (p < 0.01) and Pettersen et al (p = 0.03). The nomogram of Lopez et al resulted in substantially higher Z-scores in patients with a large sinus of Valsalva diameter. Thirty-five percentage of the studied patients would have a Z-score ≥ 2 using Lopez et al compared to 20% for Pettersen et al, 21% for Gautier et al, and 33% for Colan et al. The currently available nomograms for calculating Z-scores of aortic dilation in children with Marfan syndrome lead to clinically relevant differences in Z-scores, especially in children with a relative large aortic root diameter. This could have impact on both the diagnosis and treatment of patients with Marfan syndrome.
ISSN:	1047-9511 1467-1107