Hostname: page-component-5db58dd55d-lqwgf Total loading time: 0 Render date: 2026-05-31T12:18:05.800Z Has data issue: false hasContentIssue false
  • English
  • Français

Racial and ethnic differences in response to treatment for Marfan syndrome

Published online by Cambridge University Press:  13 April 2021

Rachel Ayers Open the ORCID record for Rachel Ayers [Opens in a new window] ,
Michael Kelleman ,
Glen Iannucci ,
Courtney McCracken  and
Matthew E. Oster
Rachel Ayers*
Affiliation:
Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA
Michael Kelleman
Affiliation:
Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA
Glen Iannucci
Affiliation:
Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA Department of Pediatrics, Children’s Healthcare of Atlanta, Atlanta, GA, USA
Courtney McCracken
Affiliation:
Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA
Matthew E. Oster
Affiliation:
Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA Department of Pediatrics, Children’s Healthcare of Atlanta, Atlanta, GA, USA
*
Author for correspondence: Rachel Ayers, MD, Department of Pediatric Cardiology, Children’s Healthcare of Atlanta, Atlanta, GA 30341 USA. Tel: +1404-256-2593; Fax: 770- 488- 9477. E-mail: rachelayers02@gmail.com
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective:

To determine whether racial/ethnic differences exist for the treatment of Marfan syndrome aortopathy. The 2014 Pediatric Heart Network randomised trial of losartan versus atenolol in Marfan syndrome paediatric and young adult patients showed no treatment differences in the rate of aortic root growth over 3 years; however, they did not examine racial/ethnic differences, and recent data suggest that angiotensin receptor blockers may have different pharmacologic effects in different racial/ethnic populations.

Methods:

We performed a secondary analysis of public-use data from the Pediatric Heart Network randomised trial comparing the differences by race/ethnicity (non-Hispanic White, non-Hispanic Black, and Hispanic patients) amongst the treatment groups for the primary outcome of rate of aortic root enlargement by z score and secondary outcome of rate of change of absolute diameter of aortic root, z score and absolute diameter of ascending aorta, and blood pressure changes.

Results:

For aortic root enlargement by z score amongst non-Hispanic White patients, patients on losartan exhibited an annual z score change of –0.090 ± 0.016, compared to –0.146 ± 0.015 for those on atenolol (p = 0.01), favouring atenolol. For Hispanic and non-Hispanic Black patients, there was no difference in primary or secondary outcomes between treatment groups.

Conclusion:

Non-Hispanic White patients had a small, but statistically significantly greater decrease in aortic root z score favouring atenolol over losartan. There were no significant differences amongst Hispanic or non-Hispanic Black patients, which may be due to relatively small size numbers. These findings may have important implications for medication selection by race/ethnicity in Marfan syndrome patients, which has not previously been evaluated in studies.

Keywords

Marfanaortopathytreatmentracial/ethnic differences

Information

Type
Original Article
Information
Cardiology in the Young , Volume 31 , Issue 12 , December 2021 , pp. 1991 - 1998
Check for updates
Copyright
© The Author(s), 2021. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Article purchase

Temporarily unavailable

Footnotes

The online version of this article has been updated since original publication. A notice detailing the changes has also been published.

References

Chiu, H, Wu, M, Chen, H, et al. Epidemiological profile of Marfan syndrome in a general population: a national database study. Mayo Clin Proc 2014; 89: 34–42.CrossRefGoogle Scholar
Judge, D, Dietz, H. Marfan’s syndrome. Lancet 2005; 366: 1965–1976.CrossRefGoogle ScholarPubMed
Groth, K, Stochholm, K, Hove, H, et al. Causes of mortality in the Marfan syndrome (from a Nationwide Register Study). Am J Cardiol 2018; 122: 1231–1235.CrossRefGoogle Scholar
Radke, R, Baumgartner, H. Diagnosis and treatment of Marfan syndrome: an update. Heart 2014; 100: 1382–1391.CrossRefGoogle ScholarPubMed
Milleron, O, Arnoult, F, Delorme, G, et al. Pathogenic FBN1 genetic variation and aortic dissection in patients with Marfan syndrome. J Am Coll Cardiol 2020; 75: 843–852.CrossRefGoogle ScholarPubMed
Gao, L, Chen, L, Gao, D, et al. The effect of losartan on progressive aortic dilatation in patients with Marfan’s syndrome: a meta-analysis of prospective randomized clinical trials. Int J Cardiol 2016; 217: 190–194.CrossRefGoogle ScholarPubMed
Lacro, R, Dietz, H, Sleeper, A, et al Atenolol versus Losartan in children and young adults with Marfan’s syndrome. NEJM 2014; 371: 2061–2071.CrossRefGoogle Scholar
Lacro, R, Dietz, H, Sleeper, A, et al For the Pediatric Heart Network Investigators Trial of Beta Blocker Therapy (Atenolol) Vs. Angiotensin II Receptor Blocker Therapy (Losartan) in Individuals With Marfan Syndrome Public Use Dataset. Retrieved November 15, 2018, from http://pediatricheartnetwork.org/ForResearchers/PHNPublicUseDatasets.aspx Google Scholar
Teixido-Tura, G, Forteza, A, Rodriguez- Palomares, J, et al. Losartan versus atenolol for prevention of aortic dilation in patients with Marfan syndrome. J Am Coll Cardiol 2018; 72: 14.CrossRefGoogle ScholarPubMed
Malik, A, Yandrapalli, S, Pemmasani, G, et al. Pharmacotherapeutics for prevention of aortic root enlargement in Marfan Syndrome – a network meta-analysis of randomized controlled trials. Eur J Prev Cardiol 2019; 1–4.Google ScholarPubMed
Mullen, M, Jin, X, Child, A, et al. Irbesartan in Marfan syndrome (AIMS): a double-blind, placebo-controlled randomised trial. Lancet 2019; 394: 2263–2270.CrossRefGoogle ScholarPubMed
Tierney, E, Levine, J, Sleeper, L, et al. Influence of aortic stiffness on aortic- root growth rate and outcome in patients with the Marfan syndrome. Am J Cardiol 2018; 121: 1094–1101.CrossRefGoogle Scholar
Helmer, A, Slater, N, Smithgall, S. A review of ace inhibitors and ARBs in Black patients with hypertension. Ann Pharmacother 2018; 52: 1143–1151.CrossRefGoogle ScholarPubMed
Lacro, R, Dietz, H, Wruck, LM, et al. Rationale and design of a randomized clinical trial of beta-blocker therapy (atenolol) versus angiotensin II receptor blocker therapy (losartan) in individuals with Marfan syndrome. Am Heart J 2007; 154: 624–631.CrossRefGoogle ScholarPubMed
De Paepe, A, Devereux, R, Deitz, H, et al. Revised diagnostic criteria for the Marfan syndrome. Am J Med Genet 1996; 62: 417–426.3.0.CO;2-R>CrossRefGoogle ScholarPubMed
Van Driest, S, Sleeper, L, Gelb, B, et al. Variants in ADRB1 and CYP2C9: association with response to atenolol and losartan in Marfan syndrome. J Pediatr 2020; 222: 213–220.e5.CrossRefGoogle ScholarPubMed
Franken, R, den Hartog, AW, van de Ret, L, et al. Clinical features differ substantially between Caucasian and Asian populations of Marfan syndrome. Circulation 2013; 77: 2793.CrossRefGoogle ScholarPubMed
Yoo, E, Woo, H, Ki, C, et al. Clinical and genetic analysis of Korean patients with Marfan syndrome: possible ethnic differences in clinical manifestation. Clin Genetics 2010; 77: 177–182.CrossRefGoogle ScholarPubMed