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

التفاصيل البيبلوغرافية
العنوان: Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome
المؤلفون: Stephanie Burns Wechsler, Arvind Hoskoppal, Wyman W. Lai, Luciana Young, Reed E. Pyeritz, Ronald V. Lacro, Timothy J. Bradley, Kristin M. Burns, Jami C. Levine, Aimee Liou, Bruce D. Gelb, Julie De Backer, Felicia Trachtenberg, Angela M. Sharkey, Marie L. Gleason, Elif Seda Selamet Tierney, Lynn Mahony, Aaron K Olson, Jeanne James, Mario Stylianou, Shaji C. Menon
المساهمون: Amsterdam Neuroscience - Complex Trait Genetics, Paediatric Genetics, Amsterdam Neuroscience - Cellular & Molecular Mechanisms, General Paediatrics
المصدر: Pediatric cardiology, 39(7), 1453-1461. Springer New York
بيانات النشر: Springer Science and Business Media LLC, 2018.
سنة النشر: 2018
مصطلحات موضوعية: Adult, Male, Marfan syndrome, medicine.medical_specialty, Adolescent, Referral, Aortic Diseases, 030204 cardiovascular system & hematology, Risk Assessment, Article, Losartan, Marfan Syndrome, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Risk Factors, Internal medicine, medicine.artery, Ascending aorta, medicine, Humans, 030212 general & internal medicine, Child, Referral and Consultation, Antihypertensive Agents, Aorta, Receiver operating characteristic, business.industry, Sinotubular Junction, Infant, Repeated measures design, Vascular surgery, medicine.disease, Dilatation, Cardiac surgery, Atenolol, ROC Curve, Echocardiography, Child, Preschool, Pediatrics, Perinatology and Child Health, Cardiology, Female, Cardiology and Cardiovascular Medicine, business, Angiotensin II Type 1 Receptor Blockers, Vascular Surgical Procedures
الوصف: 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.
تدمد: 1432-1971
0172-0643
URL الوصول: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b012d50b5dd9450015ec7c49c4fd1afa
https://doi.org/10.1007/s00246-018-1916-6
حقوق: OPEN
رقم الأكسشن: edsair.doi.dedup.....b012d50b5dd9450015ec7c49c4fd1afa
قاعدة البيانات: OpenAIRE