Advanced

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

, ; Lacro, Ronald V; Guey, Lin T; Dietz, Harry C; Pearson, Gail D; Yetman, Anji T; Gelb, Bruce D; Loeys, Bart L; Benson, D Woodrow and Bradley, Timothy J, et al. (2013) In American Heart Journal 165(5). p.3-835
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... (More)

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.

(Less)
Please use this url to cite or link to this publication:
@article{6556fc18-031b-44a8-9c9d-b374ce6c04ef,
  abstract     = {<p>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.</p><p>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 &gt;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.</p><p>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.</p>},
  author       = {,  and Lacro, Ronald V and Guey, Lin T and Dietz, Harry C and Pearson, Gail D and Yetman, Anji T and Gelb, Bruce D and Loeys, Bart L and Benson, D Woodrow and Bradley, Timothy J and De Backer, Julie and Forbus, Geoffrey A and Klein, Gloria L and Lai, Wyman W and Levine, Jami C and Lewin, Mark B and Markham, Larry W and Paridon, Stephen M and Pierpont, Mary Ella and Radojewski, Elizabeth and Selamet Tierney, Elif Seda and Sharkey, Angela M and Wechsler, Stephanie Burns and Mahony, Lynn},
  issn         = {1097-6744},
  keyword      = {Adolescent,Adrenergic beta-1 Receptor Antagonists/therapeutic use,Adult,Angiotensin II Type 1 Receptor Blockers/therapeutic use,Aortic Aneurysm, Thoracic/complications,Atenolol/therapeutic use,Child,Child, Preschool,Female,Follow-Up Studies,Humans,Infant,Losartan/therapeutic use,Male,Marfan Syndrome/complications,Retrospective Studies,Treatment Outcome,Young Adult},
  language     = {eng},
  number       = {5},
  pages        = {3--835},
  publisher    = {Mosby},
  series       = {American Heart Journal},
  title        = {Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy},
  url          = {http://dx.doi.org/10.1016/j.ahj.2013.02.019},
  volume       = {165},
  year         = {2013},
}