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

Publication ,  Journal Article
Hoskoppal, A; Menon, S; Trachtenberg, F; Burns, KM; De Backer, J; Gelb, BD; Gleason, M; James, J; Lai, WW; Liou, A; Mahony, L; Olson, AK ...
Published in: Pediatr Cardiol
October 2018

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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Published In

Pediatr Cardiol

DOI

EISSN

1432-1971

Publication Date

October 2018

Volume

39

Issue

7

Start / End Page

1453 / 1461

Location

United States

Related Subject Headings

  • Young Adult
  • Vascular Surgical Procedures
  • Risk Factors
  • Risk Assessment
  • Referral and Consultation
  • ROC Curve
  • Marfan Syndrome
  • Male
  • Losartan
  • Infant
 

Citation

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Hoskoppal, A., Menon, S., Trachtenberg, F., Burns, K. M., De Backer, J., Gelb, B. D., … Pediatric Heart Network Investigators, . (2018). Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome. Pediatr Cardiol, 39(7), 1453–1461. https://doi.org/10.1007/s00246-018-1916-6
Hoskoppal, Arvind, Shaji Menon, Felicia Trachtenberg, Kristin M. Burns, Julie De Backer, Bruce D. Gelb, Marie Gleason, et al. “Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome.Pediatr Cardiol 39, no. 7 (October 2018): 1453–61. https://doi.org/10.1007/s00246-018-1916-6.
Hoskoppal A, Menon S, Trachtenberg F, Burns KM, De Backer J, Gelb BD, et al. Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome. Pediatr Cardiol. 2018 Oct;39(7):1453–61.
Hoskoppal, Arvind, et al. “Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome.Pediatr Cardiol, vol. 39, no. 7, Oct. 2018, pp. 1453–61. Pubmed, doi:10.1007/s00246-018-1916-6.
Hoskoppal A, Menon S, Trachtenberg F, Burns KM, De Backer J, Gelb BD, Gleason M, James J, Lai WW, Liou A, Mahony L, Olson AK, Pyeritz RE, Sharkey AM, Stylianou M, Wechsler SB, Young L, Levine JC, Tierney ESS, Lacro RV, Bradley TJ, Pediatric Heart Network Investigators. Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome. Pediatr Cardiol. 2018 Oct;39(7):1453–1461.
Journal cover image

Published In

Pediatr Cardiol

DOI

EISSN

1432-1971

Publication Date

October 2018

Volume

39

Issue

7

Start / End Page

1453 / 1461

Location

United States

Related Subject Headings

  • Young Adult
  • Vascular Surgical Procedures
  • Risk Factors
  • Risk Assessment
  • Referral and Consultation
  • ROC Curve
  • Marfan Syndrome
  • Male
  • Losartan
  • Infant