Key result
Marfan syndrome is linked to ~36% lower risk of obstructive coronary sclerosis versus tricuspid aortic valves.
Why the study?
Patients with Marfan syndrome and bicuspid aortic valve share pathophysiological mechanisms for aortopathy, but while bicuspidy carries a low risk for acquired coronary artery sclerosis, the risk in Marfan syndrome was unknown.
Do patients with Marfan syndrome have a lower risk of coronary artery disease compared to patients with bicuspid or tricuspid aortic valves?
Observational (n=90)
Yes
Do patients with Marfan syndrome have a lower risk of coronary artery disease compared to patients with bicuspid or tricuspid aortic valves?
Effect estimate: OR 0.64 (95% CI 0.41-0.99)
p-value: p=0.049
Patients with Marfan syndrome and bicuspid aortic valves have a significantly lower prevalence of coronary artery disease compared to those with tricuspid aortic valves, despite similar age and sex.
MFS associated with lower obstructive coronary sclerosis risk vs TAV; hypothesis-generating and should not yet change practice.
Objective Patients with Marfan syndrome (MFS) and patients with a bicuspid aortic valve (BAV) have a significantly increased risk to develop thoracic aortopathy. Both conditions share many pathophysiological mechanisms leading to aortic complications. Bicuspidy is known to have a low risk for acquired coronary artery sclerosis. The aim of this study is to determine the risk of coronary sclerosis in MFS patients. Methods Marfan syndrome patients with an aortic root dilatation, which were surgically treated between 1999 and 2017, were included and matched with BAV and tricuspid aortic valves (TAV) patients based on sex and age. Cardiovascular risk profiles were determined in all three groups. Coronary sclerosis was graded in all patients on coronary imaging (coronary angiography or computed tomography) using a coronary artery scoring method, which divides the coronaries in 28 segments and scores non-obstructive (20–49% sclerosis) and obstructive coronary sclerosis (>49% sclerosis) in each segment. Results A total of 90 matched patients (30 within each group) were included. MFS patients showed less cardiovascular risk factors compared to BAV and TAV patients. TAV patients had higher amounts of obstructive coronary sclerosis as compared to BAV patients ( p = 0.039) and MFS patients ( p = 0.032). No difference in non- and obstructive coronary artery disease (CAD) was found between the MFS and BAV population. Conclusion Marfan syndrome and bicuspid aortic valve patients have a significantly lower risk for, and prevalence of CAD as compared to TAV individuals.
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Dolmaci et al. (2023) conducted an observational in Thoracic aortopathy (n=90). Marfan syndrome (MFS) vs. Tricuspid aortic valve (TAV) was evaluated on Obstructive coronary sclerosis (CAGE ≥ 50) (OR 0.64, 95% CI 0.41-0.99, p=0.049). Patients with Marfan syndrome had a significantly lower risk of obstructive coronary sclerosis compared to patients with tricuspid aortic valves (OR 0.64).
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