Faculty Collaboration Database

Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy. Am Heart J 2013 May;165(5):828-835.e3

Date

04/30/2013

Pubmed ID

23622922

Pubmed Central ID

PMC4131445

DOI

10.1016/j.ahj.2013.02.019

Scopus ID

2-s2.0-84876815256 (requires institutional sign-in at Scopus site)   54 Citations

Abstract

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.

Author List

Lacro RV, Guey LT, Dietz HC, Pearson GD, Yetman AT, Gelb BD, Loeys BL, Benson DW, Bradley TJ, De Backer J, Forbus GA, Klein GL, Lai WW, Levine JC, Lewin MB, Markham LW, Paridon SM, Pierpont ME, Radojewski E, Selamet Tierney ES, Sharkey AM, Wechsler SB, Mahony L, Pediatric Heart Network Investigators

MESH terms used to index this publication - Major topics in bold

Adolescent
Adrenergic beta-1 Receptor Antagonists
Adult
Angiotensin II Type 1 Receptor Blockers
Aortic Aneurysm, Thoracic
Atenolol
Child
Child, Preschool
Female
Follow-Up Studies
Humans
Infant
Losartan
Male
Marfan Syndrome
Retrospective Studies
Treatment Outcome
Young Adult