β-Blockers and Angiotensin Converting Enzyme Inhibitors: Comparison of Effects on Aortic Growth in Pediatric Patients with Marfan Syndrome

Overview

Journal J Pediatr

Specialty Pediatrics

Date 2014 Aug 12

PMID 25109242

Citations 7

Authors

Venusa Phomakay

Wilson G Huett

Jeffrey M Gossett

Xinyu Tang

Renee A Bornemeier

R Thomas Collins 2nd

Affiliations

Soon will be listed here.

Abstract

Objectives: Angiotensin converting enzyme inhibitors (ACEI) have been shown to decrease aortic growth velocity (AGV) in Marfan syndrome (MFS). We sought to compare the effect of β-blockers and ACEI on AGV in MFS.

Study Design: We retrospectively reviewed all data from all patients with MFS seen at Arkansas Children's Hospital between January 1, 1976 and January 1, 2013. Generalized least squares were used to evaluate AGV over time as a function of age, medication group, and the interaction between the 2. A mixed model was used to compare AGV between medication groups as a function of age, medication group (none, β-blocker, ACEI), and the interaction between the 2.

Results: A total of 67 patients with confirmed MFS were identified (34/67, 51% female). Mean age at first encounter was 13 ± 10 years, with mean follow-up of 7.6 ± 5.8 years. There were 839 patient encounters with a median of 10 (range 2-42) encounters per patient. AGV was nearly normal in the β-blocker group, and was less than either the ACEI or untreated groups. The AGV was higher than normal in ACEI and untreated groups (P < .001 for both).

Conclusions: β-blocker therapy results in near-normalization of AGV in MFS. ACEI did not decrease AGV in a clinically significant manner.

Citing Articles

Marfan Syndrome: Enhanced Diagnostic Tools and Follow-up Management Strategies.

Marelli S, Micaglio E, Taurino J, Salvi P, Rurali E, Perrucci G Diagnostics (Basel). 2023; 13(13).

PMID: 37443678 PMC: 10340634. DOI: 10.3390/diagnostics13132284.

An update of medical care in Marfan syndrome.

Chiu H Tzu Chi Med J. 2022; 34(1):44-48.

PMID: 35233355 PMC: 8830539. DOI: 10.4103/tcmj.tcmj_95_20.

An Overview of Investigational and Experimental Drug Treatment Strategies for Marfan Syndrome.

Deleeuw V, De Clercq A, De Backer J, Sips P J Exp Pharmacol. 2021; 13:755-779.

PMID: 34408505 PMC: 8366784. DOI: 10.2147/JEP.S265271.

Aortic dilatation in complex congenital heart disease.

Niwa K Cardiovasc Diagn Ther. 2019; 8(6):725-738.

PMID: 30740320 PMC: 6331370. DOI: 10.21037/cdt.2018.12.05.

Beta-blockers for preventing aortic dissection in Marfan syndrome.

Koo H, Lawrence K, Musini V Cochrane Database Syst Rev. 2017; 11:CD011103.

PMID: 29110304 PMC: 6486285. DOI: 10.1002/14651858.CD011103.pub2.

References

Tahernia A . Cardiovascular anomalies in Marfan's syndrome: the role of echocardiography and beta-blockers. South Med J. 1993; 86(3):305-10. View

Henry W, Gardin J, Ware J . Echocardiographic measurements in normal subjects from infancy to old age. Circulation. 1980; 62(5):1054-61. DOI: 10.1161/01.cir.62.5.1054. View

Halpern B, Char F, Murdoch J, Horton W, MCKUSICK V . A prospectus on the prevention of aortic rupture in the Marfan syndrome with data on survivorship without treatment. Johns Hopkins Med J. 1971; 129(3):123-9. View

Lang R, Bierig M, Devereux R, Flachskampf F, Foster E, Pellikka P . Recommendations for chamber quantification: a report from the American Society of Echocardiography's Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of.... J Am Soc Echocardiogr. 2005; 18(12):1440-63. DOI: 10.1016/j.echo.2005.10.005. View

Yetman A, Bornemeier R, McCrindle B . Usefulness of enalapril versus propranolol or atenolol for prevention of aortic dilation in patients with the Marfan syndrome. Am J Cardiol. 2005; 95(9):1125-7. DOI: 10.1016/j.amjcard.2005.01.032. View

Salim M, Alpert B, Ward J, Pyeritz R . Effect of beta-adrenergic blockade on aortic root rate of dilation in the Marfan syndrome. Am J Cardiol. 1994; 74(6):629-33. DOI: 10.1016/0002-9149(94)90762-5. View

WHEAT Jr M, Palmer R, BARTLEY T, SEELMAN R . TREATMENT OF DISSECTING ANEURYSMS OF THE AORTA WITHOUT SURGERY. J Thorac Cardiovasc Surg. 1965; 50:364-73. View

Roman M, Devereux R, OLoughlin J . Two-dimensional echocardiographic aortic root dimensions in normal children and adults. Am J Cardiol. 1989; 64(8):507-12. DOI: 10.1016/0002-9149(89)90430-x. View

Legget M, Unger T, OSullivan C, Zwink T, Bennett R, Byers P . Aortic root complications in Marfan's syndrome: identification of a lower risk group. Heart. 1996; 75(4):389-95. PMC: 484317. DOI: 10.1136/hrt.75.4.389. View

10.

Tierney E, Feingold B, Printz B, Park S, Graham D, Kleinman C . Beta-blocker therapy does not alter the rate of aortic root dilation in pediatric patients with Marfan syndrome. J Pediatr. 2006; 150(1):77-82. DOI: 10.1016/j.jpeds.2006.09.003. View

11.

Danyi P, Elefteriades J, Jovin I . Medical therapy of thoracic aortic aneurysms: are we there yet?. Circulation. 2011; 124(13):1469-76. DOI: 10.1161/CIRCULATIONAHA.110.006486. View

12.

Gersony D, McClaughlin M, Jin Z, Gersony W . The effect of beta-blocker therapy on clinical outcome in patients with Marfan's syndrome: a meta-analysis. Int J Cardiol. 2006; 114(3):303-8. DOI: 10.1016/j.ijcard.2005.11.116. View

13.

Keane M, Pyeritz R . Medical management of Marfan syndrome. Circulation. 2008; 117(21):2802-13. DOI: 10.1161/CIRCULATIONAHA.107.693523. View

14.

Mueller G, Stark V, Steiner K, von Kodolitsch Y, Rybczynski M, Weil J . Impact of age and gender on cardiac pathology in children and adolescents with Marfan syndrome. Pediatr Cardiol. 2012; 34(4):991-8. DOI: 10.1007/s00246-012-0593-0. View

15.

Prokop E, Palmer R, WHEAT Jr M . Hydrodynamic forces in dissecting aneurysms. In-vitro studies in a Tygon model and in dog aortas. Circ Res. 1970; 27(1):121-7. DOI: 10.1161/01.res.27.1.121. View

16.

Shores J, Berger K, Murphy E, Pyeritz R . Progression of aortic dilatation and the benefit of long-term beta-adrenergic blockade in Marfan's syndrome. N Engl J Med. 1994; 330(19):1335-41. DOI: 10.1056/NEJM199405123301902. View

17.

Silverman D, Burton K, Gray J, Bosner M, Kouchoukos N, Roman M . Life expectancy in the Marfan syndrome. Am J Cardiol. 1995; 75(2):157-60. DOI: 10.1016/s0002-9149(00)80066-1. View

18.

Ladouceur M, Fermanian C, Lupoglazoff J, Edouard T, Dulac Y, Acar P . Effect of beta-blockade on ascending aortic dilatation in children with the Marfan syndrome. Am J Cardiol. 2007; 99(3):406-9. DOI: 10.1016/j.amjcard.2006.08.048. View

19.

Ose L, MCKUSICK V . Prophylactiv use of propranolol in the Marfan syndrome to prevent aortic dissection. Birth Defects Orig Artic Ser. 1977; 13(3C):163-9. View

20.

Murdoch J, Walker B, Halpern B, KUZMA J, MCKUSICK V . Life expectancy and causes of death in the Marfan syndrome. N Engl J Med. 1972; 286(15):804-8. DOI: 10.1056/NEJM197204132861502. View