Comparative evaluation of coronary disease burden: bicuspid valve disease is not atheroprotective

Dolmaci, Onur B.; Driessen, Antoine H.G.; Klautz, Robert J.M.; Poelmann, Robert E.; Lindeman, J.; Grewal, Nimrat

doi:10.1136/openhrt-2021-001772

Cited by 12 publications

(26 citation statements)

References 8 publications

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“…For instance, BAV aortopathy is generally regarded as less/ non-inflammatory (42,46). These findings are comparable with the clinical observation of BAV ascending aortic aneurysms not commonly being associated with systemic atherosclerosis (47)(48)(49). Differences in medial elastic fibers have also been described between BAV and TAV.…”

Section: The Ascending Aortic Wall In Bavsupporting

confidence: 70%

Normal and abnormal development of the aortic valve and ascending aortic wall: a comprehensive overview of the embryology and pathology of the bicuspid aortic valve

Grewal¹,

Groot²,

Lindeman³

et al. 2022

Ann Cardiothorac Surg

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Section: The Ascending Aortic Wall In Bavsupporting

confidence: 70%

Normal and abnormal development of the aortic valve and ascending aortic wall: a comprehensive overview of the embryology and pathology of the bicuspid aortic valve

Grewal¹,

Groot²,

Lindeman³

et al. 2022

Ann Cardiothorac Surg

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“…Furthermore, the intimal layer is embryologically altered resulting in a significantly thinner intima, with minimal features of atherosclerosis as compared to patients with a TAV ( 11 – 13 ). In line with the histopathological findings of less atherosclerosis, clinically the BAV patients have also demonstrated a significantly lower risk for coronary artery sclerosis ( 14 – 16 ).…”

Section: Introductionsupporting

confidence: 64%

Risk for acquired coronary artery disease in genetic vs. congenital thoracic aortopathy

Dolmaci

Ayyildiz

Poelmann

et al. 2023

Front. Cardiovasc. Med.

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ObjectivePatients 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.MethodsMarfan 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.ResultsA 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.ConclusionMarfan 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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“…The possibility to adapt the intimal thickness to shear stress is an important aspect which the MFS and BAV lack, although the thinner intima might be protective for atherosclerosis during their life. Lack of atherosclerosis in bicuspid aortopathy has earlier been described clinically (43,44). It is interesting to note that a specific group of animals, being reptiles, normally present with bicuspid semilunar valves.…”

Section: Discussionmentioning

confidence: 85%

Thoracic aortopathy in Marfan syndrome overlaps with mechanisms seen in bicuspid aortic valve disease

Grewal

Dolmaci²,

Jansen³

et al. 2023

Front. Cardiovasc. Med.

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BackgroundThoracic aortopathy is a serious complication which is more often seen in patients with Marfan syndrome (MFS) and patients with a bicuspid aortic valve (BAV) than in individuals with a tricuspid aortic valve (TAV). The identification of common pathological mechanisms leading to aortic complications in non-syndromic and syndromic diseases would significantly improve the field of personalized medicine.ObjectiveThis study sought to compare thoracic aortopathy between MFS, BAV, and TAV individuals.Materials and methodsBicuspid aortic valve (BAV; n = 36), TAV (n = 23), and MFS (n = 8) patients were included. Ascending aortic wall specimen were studied for general histologic features, apoptosis, markers of cardiovascular ageing, expression of synthetic and contractile vascular smooth muscle cells (VSMC), and fibrillin-1 expression.ResultsThe MFS group showed many similarities with the dilated BAV. Both patient groups showed a thinner intima (p < 0.0005), a lower expression of contractile VSMCs (p < 0.05), more elastic fiber thinning (p < 0.001), lack of inflammation (p < 0.001), and a decreased progerin expression (p < 0.05) as compared to the TAV. Other features of cardiovascular ageing differed between the BAV and MFS. Dilated BAV patients demonstrated less medial degeneration (p < 0.0001), VSMC nuclei loss (p < 0.0001), apoptosis of the vessel wall (p < 0.03), and elastic fiber fragmentation and disorganization (p < 0.001), as compared to the MFS and dilated TAV.ConclusionThis study showed important similarities in the pathogenesis of thoracic aortic aneurysms in BAV and MFS. These common mechanisms can be further investigated to personalize treatment strategies in non-syndromic and syndromic conditions.

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Comparative evaluation of coronary disease burden: bicuspid valve disease is not atheroprotective

Cited by 12 publications

References 8 publications

Normal and abnormal development of the aortic valve and ascending aortic wall: a comprehensive overview of the embryology and pathology of the bicuspid aortic valve

Normal and abnormal development of the aortic valve and ascending aortic wall: a comprehensive overview of the embryology and pathology of the bicuspid aortic valve

Risk for acquired coronary artery disease in genetic vs. congenital thoracic aortopathy

Thoracic aortopathy in Marfan syndrome overlaps with mechanisms seen in bicuspid aortic valve disease

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