Key result
Prior aortic root surgery and omitting beta blockers linked to higher TBAD risk in Marfan patients.
Why the study?
Type B aortic dissection is a growing problem in patients with Marfan syndrome, motivating an evaluation of its incidence and predictors in patients aged 30-50 years.
Are prior elective aortic root surgery and lack of betablocker treatment associated with an increased risk of type B aortic dissection in patients with Marfan syndrome?
Cohort (n=741)
Yes
Are prior elective aortic root surgery and lack of betablocker treatment associated with an increased risk of type B aortic dissection in patients with Marfan syndrome?
Absolute Event Rate: 10% vs 6%
p-value: p=0.049
In patients with Marfan syndrome, prior elective aortic root repair and lack of beta-blocker use are independent predictors of type B aortic dissection.
May warrant closer post-root surgery surveillance without beta-blockers; leaves open causal effects in Marfan syndrome.
Background/Introduction Occurrence of type B aortic dissection (TBAD) is a growing problem in patients with Marfan syndrome (MFS). Purpose To evaluate the incidence and predictors of TBAD in patients with MFS aged 30-50y in the last three decades in Europe. Methods In this retrospective study, patients from seven MFS referral centers in four countries (Netherlands, Belgium, France, and Spain) were included. Electronic patient records were reviewed to collect data on medication use and hospital admissions. Patients with MFS with a pathogenic FBN1 gene variant aged 30-50y and at least three years of follow-up were eligible. Chi-square tests, Wilcoxon rank sum tests and multivariate logistic regression were used to evaluate predictors of TBAD. Results We included 741 patients with MFS, 404 (55%) females, median age 33 (IQR: 30-37) with a median follow-up duration of 2922 days (IQR: 1826 – 4383) and found 51 TBAD in 51 patients (7%), at a median age of 38 years (IQR: 35–42). TBAD happened before MFS diagnosis in 10 patients. TBAD occurred more often in patients with prior elective aortic root surgery compared to unoperated patients (22/222 (10%) vs 29/519 (6%), respectively, p=0.049). Additionally, TBAD occurred more often in patients who were not treated with betablockers (BB) than in those with BB (19/175 (11%) vs 32/566 (6%), respectively, p=0.027) (Figure 1). No differences in the incidence of TBAD were found between: (1) patients with and without angiotensin II receptor blocker treatment (ARB), (2) patients with and without BB and ARB combination treatment, (3) men and women, (4) patients with and without a family history of aortic dissection, and (6) patients with and without a haploinsufficient FBN1 gene variant. Multivariable logistic regression revealed that prior elective aortic root surgery, and a lack of BB treatment were predictors of TBAD, independent of follow-up duration, while lack of ARB treatment was not. (Table 1). Conclusion(s) The occurrence of type B aortic dissection in patients with MFS is associated with prior elective aortic root repair and lack of BB use.
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Bosshardt et al. (2025) conducted a cohort in Marfan syndrome (n=741). Prior elective aortic root surgery vs. No prior elective aortic root surgery was evaluated on Type B aortic dissection (TBAD) (p=0.049). Prior elective aortic root surgery (10% vs 6%, p=0.049) and lack of betablocker treatment (11% vs 6%, p=0.027) were associated with an increased risk of type B aortic dissection in Marfan syndrome.
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