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

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


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 number, NCT00429364.

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

Publication types

  • Randomized Controlled Trial

MeSH terms

  • Adolescent
  • Adult
  • Angiotensin II Type 1 Receptor Blockers
  • Antihypertensive Agents / therapeutic use
  • Aorta / pathology*
  • Aorta / surgery
  • Aortic Diseases / epidemiology
  • Aortic Diseases / etiology*
  • Aortic Diseases / surgery
  • Atenolol / therapeutic use
  • Child
  • Child, Preschool
  • Dilatation
  • Echocardiography / methods
  • Female
  • Humans
  • Infant
  • Losartan / therapeutic use
  • Male
  • Marfan Syndrome / complications*
  • Marfan Syndrome / drug therapy
  • Marfan Syndrome / surgery
  • ROC Curve
  • Referral and Consultation / statistics & numerical data
  • Risk Assessment / methods
  • Risk Factors
  • Vascular Surgical Procedures / statistics & numerical data*
  • Young Adult


  • Angiotensin II Type 1 Receptor Blockers
  • Antihypertensive Agents
  • Atenolol
  • Losartan

Associated data