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

Ronald V. Lacro, Lin T. Guey, Harry C. Dietz, Gail D. Pearson, Angela T Yetman, Bruce D. Gelb, Bart L. Loeys, D. Woodrow Benson, Timothy J. Bradley, Julie De Backer, Geoffrey A. Forbus, Gloria L. Klein, Wyman W. Lai, Jami C. Levine, Mark B. Lewin, Larry W. Markham, Stephen M. Paridon, Mary Ella Pierpont, Elizabeth Radojewski, Elif Seda Selamet Tierney & 3 others Angela M. Sharkey, Stephanie Burns Wechsler, Lynn Mahony

Research output: Contribution to journalArticle

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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.

Original languageEnglish (US)
JournalAmerican Heart Journal
Volume165
Issue number5
DOIs
StatePublished - Jan 1 2013

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Marfan Syndrome
Atenolol
Losartan
Dilatation
Young Adult
Mitral Valve Insufficiency
Dissection
Patient Advocacy
Mitral Valve Prolapse
Body Surface Area
Population Characteristics
Therapeutics
Demography
Clinical Trials
Pediatrics
Growth
Population

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

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, Angela T; Gelb, Bruce D.; Loeys, Bart L.; Benson, D. Woodrow; Bradley, Timothy J.; De Backer, Julie; Forbus, Geoffrey A.; Klein, Gloria L.; Lai, Wyman W.; Levine, Jami C.; Lewin, Mark B.; Markham, Larry W.; Paridon, Stephen M.; Pierpont, Mary Ella; Radojewski, Elizabeth; Selamet Tierney, Elif Seda; Sharkey, Angela M.; Wechsler, Stephanie Burns; Mahony, Lynn.

In: American Heart Journal, Vol. 165, No. 5, 01.01.2013.

Research output: Contribution to journalArticle

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 2013, 'Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy', American Heart Journal, vol. 165, no. 5. https://doi.org/10.1016/j.ahj.2013.02.019
Lacro, Ronald V. ; Guey, Lin T. ; Dietz, Harry C. ; Pearson, Gail D. ; Yetman, Angela T ; Gelb, Bruce D. ; Loeys, Bart L. ; Benson, D. Woodrow ; Bradley, Timothy J. ; De Backer, Julie ; Forbus, Geoffrey A. ; Klein, Gloria L. ; Lai, Wyman W. ; Levine, Jami C. ; Lewin, Mark B. ; Markham, Larry W. ; Paridon, Stephen M. ; Pierpont, Mary Ella ; Radojewski, Elizabeth ; Selamet Tierney, Elif Seda ; Sharkey, Angela M. ; Wechsler, Stephanie Burns ; Mahony, Lynn. / Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy. In: American Heart Journal. 2013 ; Vol. 165, No. 5.
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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.",
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T1 - Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy

AU - Lacro, Ronald V.

AU - Guey, Lin T.

AU - Dietz, Harry C.

AU - Pearson, Gail D.

AU - Yetman, Angela T

AU - Gelb, Bruce D.

AU - Loeys, Bart L.

AU - Benson, D. Woodrow

AU - Bradley, Timothy J.

AU - De Backer, Julie

AU - Forbus, Geoffrey A.

AU - Klein, Gloria L.

AU - Lai, Wyman W.

AU - Levine, Jami C.

AU - Lewin, Mark B.

AU - Markham, Larry W.

AU - Paridon, Stephen M.

AU - Pierpont, Mary Ella

AU - Radojewski, Elizabeth

AU - Selamet Tierney, Elif Seda

AU - Sharkey, Angela M.

AU - Wechsler, Stephanie Burns

AU - Mahony, Lynn

PY - 2013/1/1

Y1 - 2013/1/1

N2 - 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.

AB - 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.

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