Effect of long-term β-blockade on aortic root compliance in patients with Marfan syndrome☆,☆☆
Section snippets
Methods
We included patients from the Marfan’s Clinic at Michael Reese Hospital and Medical Center who gave informed consent. The exclusion criteria were (1) age less than 18 years or more than 45 years, (2) ongoing treatment with β-blockers, (3) aortic dissection or previous cardiac surgery, (4) significant decrease in left ventricular systolic performance, (5) moderate or severe mitral regurgitation or aortic insufficiency determined by 2-dimensional echocardiography, (6) resting bradycardia <50
Results
Twenty-three patients met the inclusion criteria, 12 women and 11 men. All the patients were asymptomatic at the time of selection. The age of the patients at the beginning of the study was 31 ± 14.2 years; other characteristics at baseline and after taking atenolol after an average of 26 months are shown in Table I.
Empty Cell Baseline Follow-up P value SBP (mm Hg) 124 ± 13 113 ± 26 <.001
Discussion
Despite the therapeutic success of β-blockers in delaying3, 14 and sometimes preventing3, 15 vascular complications associated with Marfan syndrome, the exact mechanism remains undefined. It was initially attributed to a negative inotropic effect with consequent reduction in the repeated strain on the aortic root.15, 16
The acute effects of β-blockade on the aortic root elastic properties was assessed noninvasively by Haouzi et al 10 comparing normal controls with patients with Marfan syndrome
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Cited by (73)
Preventive effect of beta-blockers in the development of aortic dilation in giant cell arteritis-related aortitis
2022, Seminars in Arthritis and RheumatismCitation Excerpt :BBs reduce cardiac frequency, blood pressure, and left ventricular dp/dt and contribute to reducing wall shear stress and the aortic growth rate. Other assumptions for the beneficial effects of BBs in Marfan syndrome include a direct impact on the extracellular matrix and possibly improvement of aortic stiffness and elasticity [23–25]. Despite no strong evidence to affirm that BBs reduce mortality and the risk of aortic dissection occurrence, they currently remain the “standard of care" in Marfan syndrome.
Precision Medicine Approaches to Vascular Disease: JACC Focus Seminar 2/5
2021, Journal of the American College of CardiologyCitation Excerpt :Beta-blockers have long been advocated in MFS; however, their only randomized controlled trial in MFS was performed in 1994, which found that propranolol decreased the rate of aortic root dilatation (7). Subsequent nonrandomized trials and case-control studies have been inconsistent in their findings (8,9), but a large retrospective review showed higher probability of survival in patients with MFS treated with beta-blockers (10). Based on these data, the use of beta-blockers in patients with TAA due to MFS carries a Class Ib recommendation in the American College of Cardiology/American Heart Association guidelines (11), predicated on reduction of aortic dilatation progression.
Influence of Aortic Stiffness on Aortic-Root Growth Rate and Outcome in Patients With the Marfan Syndrome
2018, American Journal of CardiologyCitation Excerpt :There were no treatment differences for aortic-root EM, ascending-aorta EM, or heart rate–corrected ascending-aorta SI. Previous studies using a variety of methods and assessing a number of different measures of aortic stiffness have shown inconsistent responses to β blockers and angiotensin receptor blockers, but these studies suggest that these medications might have unique and complex mechanisms of action in modifying vascular properties in patients with the Marfan syndrome.9–14 Few previous studies have examined the relation between aortic stiffness and aortic-root growth rate or risk of adverse clinical outcomes in the Marfan syndrome, and their results have been contradictory.
Marfan Syndrome
2017, Aneurysms-Osteoarthritis Syndrome: SMAD3 Gene MutationsMarfan Syndrome and Related Disorders
2013, Emery and Rimoin's Principles and Practice of Medical GeneticsA systematic review of the pharmacological management of aortic root dilation in Marfan syndrome
2013, Cardiology in the Young
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Reprint requests: Pablo Denes, MD, Cardiovascular Institute (4CVI), Michael Reese Hospital and Medical Center, 2929 S Ellis, Chicago, IL 60616.
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