Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy

被引：42

作者：

Lacro, Ronald V. ^{[1
]}

Guey, Lin T. ^{[2
]}

Dietz, Harry C. ^{[3
]}

Pearson, Gail D. ^{[4
]}

Yetman, Anji T. ^{[5
,6
]}

Gelb, Bruce D. ^{[7
]}

Loeys, Bart L. ^{[8
,9
]}

Benson, D. Woodrow ^{[10
]}

Bradley, Timothy J. ^{[11
]}

De Backer, Julie ^{[12
]}

Forbus, Geoffrey A. ^{[13
]}

Klein, Gloria L. ^{[2
]}

Lai, Wyman W. ^{[14
]}

Levine, Jami C. ^{[1
]}

Lewin, Mark B. ^{[15
]}

Markham, Larry W. ^{[16
]}

Paridon, Stephen M. ^{[17
]}

Pierpont, Mary Ella ^{[18
]}

Radojewski, Elizabeth ^{[11
]}

Tierney, Elif Seda Selamet ^{[1
]}

Sharkey, Angela M. ^{[19
]}

Wechsler, Stephanie Burns ^{[20
]}

Mahony, Lynn ^{[21
]}

机构：

[1] Childrens Hosp Boston, Boston, MA 02115 USA

[2] New England Res Inst, Watertown, MA 02172 USA

[3] Johns Hopkins Univ, Sch Med, Baltimore, MD USA

[4] NHLBI, NIH, Bethesda, MD 20892 USA

[5] Primary Childrens Med Ctr, Salt Lake City, UT 84103 USA

[6] Univ Utah, Salt Lake City, UT USA

[7] Mt Sinai Sch Med, New York, NY USA

[8] Univ Antwerp Hosp, Antwerp, Belgium

[9] Univ Antwerp, B-2020 Antwerp, Belgium

[10] Cincinnati Childrens Hosp Med Ctr, Cincinnati, OH USA

[11] Hosp Sick Children, Toronto, ON M5G 1X8, Canada

[12] Ghent Univ Hosp, Ghent, Belgium

[13] Med Univ S Carolina, Charleston, SC 29425 USA

[14] Childrens Hosp New York, New York, NY USA

[15] Seattle Childrens Hosp, Seattle, WA USA

[16] Vanderbilt Univ, Sch Med, Nashville, TN 37212 USA

[17] Childrens Hosp Philadelphia, Philadelphia, PA 19104 USA

[18] Childrens Hosp & Clin Minnesota, St Paul, MN USA

[19] St Louis Univ, Sch Med, St Louis, MO USA

[20] Duke Univ, Med Ctr, Durham, NC USA

[21] Univ Texas SW Med Ctr Dallas, Dallas, TX 75390 USA

来源：

AMERICAN HEART JOURNAL | 2013年 / 165卷 / 05期

关键词：

BETA-BLOCKER THERAPY; MOUSE MODEL; FBN1; PATHOGENESIS; PROBANDS;

D O I：

10.1016/j.ahj.2013.02.019

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Background The Pediatric Heart Network designed a clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in children and young adults with Marfan syndrome randomized to receive atenolol or losartan. We report here the characteristics of the screened population and enrolled subjects. Methods and results Between 2007 and 2011, 21 clinical sites randomized 608 subjects, aged 6 months to 25 years who met the original Ghent criteria and had a body surface area-adjusted aortic root diameter z-score >3.0. The mean age at study entry was 11.2 years, 60% were male, and 25% were older teenagers and young adults. The median aortic root diameter z-score was 4.0. Aortic root diameter z-score did not vary with age. Mitral valve prolapse and mitral regurgitation were more common in females. Among those with a positive family history, 56% had a family member with aortic surgery, and 32% had a family member with a history of aortic dissection. Conclusions Baseline demographic, clinical, and anthropometric characteristics of the randomized cohort are representative of patients in this population with moderate to severe aortic root dilation. The high percentage of young subjects with relatives who have had aortic dissection or surgery illustrates the need for more definitive therapy; we expect that the results of the study and the wealth of systematic data collected will make an important contribution to the management of individuals with Marfan syndrome.

引用

页码：828 / +

页数：11

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