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Pediatr Cardiol. 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.

Collaborators (159)

Pearson G, Stylianou M, Pemberton V, Mahony L, Sleeper L, Tennstedt S, Colan S, Klein G, Guey L, Wruck L, Travison T, Chen S, Gerstenberger E, Olesker T, Teitel DF, Newburger J, Lacro RV, King M, Dunbar-Masterson C, Handisides J, Posa A, Nang Q, Hass C, Hsu D, Lai W, Hellenbrand W, Printz B, Roman MJ, Devereux R, Korsin R, Sherwood G, Vetter V, Paridon S, Gleason M, Pyeritz R, Mirarchi N, DiLullo S, Ejembi A, Morgan R, Morrison T, Benson DW, Border W, Cnota J, Heydarian H, James J, Hamstra M, Hogan K, Bogenschutz L, Anderson PAW, Li JS, Wechsler SB, Cook A, Sang C, Covitz W, Xu M, Sutton LJ, Crawford K, Roberts S, Palmer D, Saul JP, Atz A, Forbus G, Atz T, Infinger P, Choudhury A, Minich L, Williams R, Yetman A, Shearrow M, Robinson M, Porter J, McCrindle B, Bradley TJ, Russell J, Colman J, Radojewski E, Khaikin S, Slater N, Dietz HC, Ravekes WJ, Rykiel M, Sparks E, Oswald G, Leadroot J, Canter C, Sharkey A, Braverman A, Rainey C, Jefferies JL, Slesnick T, Liou A, Martinez H, Menesses A, Tenende T, Liang D, Merkel E, Loeys B, De Backer J, Cobben JM, Sluysmans T, De Paepe A, De Nobele S, Gelb B, Srivastava S, Mendiz-Ramdeen T, Weismann C, Lawrence E, Chin S, Ko H, Yau JL, Webber S, Drant S, Luce J, Stiegler K, Markham L, Kinnard C, Stewart C, Sommers S, Madison C, Young L, Domenico M, Waitzman K, Lozano C, Pierpont ME, Baker C, Zielinski E, Velden HV, Overman A, Lewin M, Olson A, Payne A, Rimoin D, Pariani M, Siegel R, Rafique A, Grossfeld P, Smith A, McLees-Palinkas T, Colan SD, Selamet Tierney ES, Levine J, Trevey S, Rivera M, Artman M, Austin E, Baldwin HS, Bernstein D, Feltes T, Johnson J, Klitzner T, Krischer J, Matherne GP, Zahka KG, Kugler J, Driscoll DJ, Galantowicz M, Hunsberger SA, Knight TJ, Taylor H.

Author information

University of Utah, Salt Lake City, UT, USA.
University of Utah, Salt Lake City, UT, USA.
New England Research Institutes Inc., Watertown, MA, USA.
National Heart, Lung, and Blood Institute, Bethesda, MD, USA.
Ghent University Hospital, Ghent, Belgium.
Icahn School of Medicine at Mount Sinai, New York, NY, USA.
Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA.
Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH, USA.
Columbia University, New York, NY, USA.
Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.
Southwestern Medical Center, University of Texas, Dallas, TX, USA.
University of Washington, Seattle, WA, USA.
The Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Washington University, St. Louis, MO, USA.
Duke University Medical Center, Duke University, Durham, NC, USA.
Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.


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.


Aortic root dilation; Marfan syndrome; Predictors; Referral for aortic surgery

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