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Erschienen in: Pediatric Cardiology 7/2018

11.06.2018 | Original Article

Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome

verfasst von: Arvind Hoskoppal, Shaji Menon, Felicia Trachtenberg, Kristin M. Burns, Julie De Backer, Bruce D. Gelb, Marie Gleason, Jeanne James, Wyman W. Lai, Aimee Liou, Lynn Mahony, Aaron K. Olson, Reed E. Pyeritz, Angela M. Sharkey, Mario Stylianou, Stephanie Burns Wechsler, Luciana Young, Jami C. Levine, Elif Seda Selamet Tierney, Ronald V. Lacro, Timothy J. Bradley, Pediatric Heart Network Investigators

Erschienen in: Pediatric Cardiology | Ausgabe 7/2018

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Abstract

Few data exist regarding predictors of rapid aortic root dilation and referral for aortic surgery in Marfan syndrome (MFS). To identify independent predictors of the rate of aortic root (AoR) dilation and referral for aortic surgery, we investigated the data from the Pediatric Heart Network randomized trial of atenolol versus losartan in young patients with MFS. Data were analyzed from the echocardiograms at 0, 12, 24, and 36 months read in the core laboratory of 608 trial subjects, aged 6 months to 25 years, who met original Ghent criteria and had an AoR z-score (AoRz) > 3. Repeated measures linear and logistic regressions were used to determine multivariable predictors of AoR dilation. Receiver operator characteristic curves were used to determine cut-points in AoR dilation predicting referral for aortic surgery. Multivariable analysis showed rapid AoR dilation as defined by change in AoRz/year > 90th percentile was associated with older age, higher sinotubular junction z-score, and atenolol use (R2 = 0.01) or by change in AoR diameter (AoRd)/year > 90th percentile with higher sinotubular junction z-score and non-white race (R2 = 0.02). Referral for aortic root surgery was associated with higher AoRd, higher ascending aorta z-score, and higher sinotubular junction diameter:ascending aorta diameter ratio (R2 = 0.17). Change in AoRz of 0.72 SD units/year had 42% sensitivity and 92% specificity and change in AoRd of 0.34 cm/year had 38% sensitivity and 95% specificity for predicting referral for aortic surgery. In this cohort of young patients with MFS, no new robust predictors of rapid AoR dilation or referral for aortic root surgery were identified. Further investigation may determine whether generalized proximal aortic dilation and effacement of the sinotubular junction will allow for better risk stratification. Rate of AoR dilation cut-points had high specificity, but low sensitivity for predicting referral for aortic surgery, limiting their clinical use. Clinical Trial Number ClinicalTrials.gov number, NCT00429364.
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Metadaten
Titel
Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome
verfasst von
Arvind Hoskoppal
Shaji Menon
Felicia Trachtenberg
Kristin M. Burns
Julie De Backer
Bruce D. Gelb
Marie Gleason
Jeanne James
Wyman W. Lai
Aimee Liou
Lynn Mahony
Aaron K. Olson
Reed E. Pyeritz
Angela M. Sharkey
Mario Stylianou
Stephanie Burns Wechsler
Luciana Young
Jami C. Levine
Elif Seda Selamet Tierney
Ronald V. Lacro
Timothy J. Bradley
Pediatric Heart Network Investigators
Publikationsdatum
11.06.2018
Verlag
Springer US
Erschienen in
Pediatric Cardiology / Ausgabe 7/2018
Print ISSN: 0172-0643
Elektronische ISSN: 1432-1971
DOI
https://doi.org/10.1007/s00246-018-1916-6

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