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Randomized Controlled Trial
. 2018 Oct;39(7):1453-1461.
doi: 10.1007/s00246-018-1916-6. Epub 2018 Jun 11.

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

Arvind Hoskoppal  1 , Shaji Menon  2 , Felicia Trachtenberg  3 , Kristin M Burns  4 , Julie De Backer  5 , Bruce D Gelb  6 , Marie Gleason  7 , Jeanne James  8 , Wyman W Lai  9 , Aimee Liou  10 , Lynn Mahony  11 , Aaron K Olson  12 , Reed E Pyeritz  13 , Angela M Sharkey  14 , Mario Stylianou  4 , Stephanie Burns Wechsler  15 , Luciana Young  16 , Jami C Levine  17 , Elif Seda Selamet Tierney  17 , Ronald V Lacro  17 , Timothy J Bradley  18 ; Pediatric Heart Network Investigators
Collaborators, Affiliations
Randomized Controlled Trial

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

Arvind Hoskoppal et al. Pediatr Cardiol. 2018 Oct.

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.

Keywords: Aortic root dilation; Marfan syndrome; Predictors; Referral for aortic surgery.

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Conflict of interest statement

Compliance with Ethical Standards

Conflict of interest/Relation to Industry The authors do not have any conflict of interest. The views expressed in this article are those of the authors and do not represent the official views of the National Heart, Lung, and Blood Institute (NHLBI) or the National Institutes of Health.

Figures

Fig. 1
Fig. 1
a, b The Loess (non-parametric) curves, that were used to assess the relationship of the annual changes in AoRz and AoRd with age, as solid red lines with the dotted red lines representing the 95% confidence intervals. c, d The ROC curves that were used to determine the AoR dilation cut-points in annual changes in AoRz and AoRd, that maximized the sensitivity and specificity for referral for aortic surgery
See this image and copyright information in PMC

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