Comparability of different Z-score equations for aortic root dimensions in children with Marfan syndrome
| Autor: | Bart Loeys, Floris E A Udink Ten Cate, Martijn G. Slieker, Annette G Reimer, Marlies Kempers, Dominiek W E Rutten, Ilse J H Aarts-Janssen |
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| Rok vydání: | 2021 |
| Předmět: |
Marfan syndrome
medicine.medical_specialty Aortic root Vascular damage Radboud Institute for Health Sciences [Radboudumc 16] Aortic Diseases 030204 cardiovascular system & hematology Standard score Marfan Syndrome 03 medical and health sciences 0302 clinical medicine Internal medicine Medicine Humans In patient Major complication Child Aorta 030304 developmental biology 0303 health sciences Neurodevelopmental disorders Donders Center for Medical Neuroscience [Radboudumc 7] business.industry Other Research Radboud Institute for Health Sciences [Radboudumc 0] General Medicine Nomogram medicine.disease Echocardiography Pediatrics Perinatology and Child Health Cohort Cardiology Human medicine Cardiology and Cardiovascular Medicine business Aortic root dilation |
| Zdroj: | Cardiology in the Young, 31, 1962-1968 Cardiology in the Young, 31, 12, pp. 1962-1968 Cardiology in the young |
| ISSN: | 1467-1107 1047-9511 |
| Popis: | Background: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.Methods: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.Results: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.Conclusion: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. |
| Databáze: | OpenAIRE |
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