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

Pediatric Heart Network Investigators

Research output: Contribution to journalArticle

2 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)1-9
Number of pages9
JournalPediatric Cardiology
DOIs
StateAccepted/In press - Jun 11 2018

Fingerprint

Marfan Syndrome
Dilatation
Referral and Consultation
Atenolol
Sensitivity and Specificity
Aorta
Losartan
Linear Models
Logistic Models
Clinical Trials
Pediatrics

Keywords

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

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

Cite this

Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome. / Pediatric Heart Network Investigators.

In: Pediatric Cardiology, 11.06.2018, p. 1-9.

Research output: Contribution to journalArticle

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title = "Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome",
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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author = "{Pediatric Heart Network Investigators} and Arvind Hoskoppal and Shaji Menon and Felicia Trachtenberg and Burns, {Kristin M.} and {de Backer}, Julie and Gelb, {Bruce D.} and Marie Gleason and Jeanne James and Lai, {Wyman W.} and Aimee Liou and Lynn Mahony and Olson, {Aaron K.} and Pyeritz, {Reed E.} and Sharkey, {Angela M.} and Mario Stylianou and Wechsler, {Stephanie Burns} and Luciana Young and Levine, {Jami C.} and Tierney, {Elif Seda Selamet} and Lacro, {Ronald V.} and Bradley, {Timothy J.} and Gail Pearson and Mario Stylianou and Victoria Pemberton and Lynn Mahony and Lynn Sleeper and Sharon Tennstedt and Steven Colan and Gloria Klein and Lin Guey and Lisa Wruck and Thomas Travison and Shan Chen and Eric Gerstenberger and Tanya Olesker and Teitel, {David F.} and Jane Newburger and Lacro, {Ronald V.} and Martha King and Carolyn Dunbar-Masterson and Jill Handisides and Hsu, {Daphne T.} and Quincy Nang and Cara Hass and Daphne Hsu and Wyman Lai and William Hellenbrand and Beth Printz and Roman, {Mary J.} and Richard Devereux",
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AU - Pediatric Heart Network Investigators

AU - Hoskoppal, Arvind

AU - Menon, Shaji

AU - Trachtenberg, Felicia

AU - Burns, Kristin M.

AU - de Backer, Julie

AU - Gelb, Bruce D.

AU - Gleason, Marie

AU - James, Jeanne

AU - Lai, Wyman W.

AU - Liou, Aimee

AU - Mahony, Lynn

AU - Olson, Aaron K.

AU - Pyeritz, Reed E.

AU - Sharkey, Angela M.

AU - Stylianou, Mario

AU - Wechsler, Stephanie Burns

AU - Young, Luciana

AU - Levine, Jami C.

AU - Tierney, Elif Seda Selamet

AU - Lacro, Ronald V.

AU - Bradley, Timothy J.

AU - Pearson, Gail

AU - Stylianou, Mario

AU - Pemberton, Victoria

AU - Mahony, Lynn

AU - Sleeper, Lynn

AU - Tennstedt, Sharon

AU - Colan, Steven

AU - Klein, Gloria

AU - Guey, Lin

AU - Wruck, Lisa

AU - Travison, Thomas

AU - Chen, Shan

AU - Gerstenberger, Eric

AU - Olesker, Tanya

AU - Teitel, David F.

AU - Newburger, Jane

AU - Lacro, Ronald V.

AU - King, Martha

AU - Dunbar-Masterson, Carolyn

AU - Handisides, Jill

AU - Hsu, Daphne T.

AU - Nang, Quincy

AU - Hass, Cara

AU - Hsu, Daphne

AU - Lai, Wyman

AU - Hellenbrand, William

AU - Printz, Beth

AU - Roman, Mary J.

AU - Devereux, Richard

PY - 2018/6/11

Y1 - 2018/6/11

N2 - 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.

AB - 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.

KW - Aortic root dilation

KW - Marfan syndrome

KW - Predictors

KW - Referral for aortic surgery

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