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

Grewal, Nimrat; Groot, Adriana C. Gittenberger‐de; Lindeman, J.; Klautz, Arthur; Driessen, Antoine H.G.; Klautz, Robert J.M.; Poelmann, Robert E.

doi:10.21037/acs-2021-bav-14

Cited by 21 publications

(17 citation statements)

References 57 publications

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“…In bicuspidy, it has earlier been postulated that thoracic aortopathy is caused by an early embryonic defect in the progenitor cells which are responsible for both the aortic valve and vascular smooth muscle cells development ( 41 ). In MFS, the link between a FBN1 mutation and early embryonic defects is less well described.…”

Section: Discussionmentioning

confidence: 99%

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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Section: Discussionmentioning

confidence: 99%

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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“…Cases requiring surgery for bicuspid aortic valve and ascending aorta aneurysm are not particularly rare [ 1 ]. We occasionally encounter patients in whom mediastinitis develops after open heart surgery and placement of a thoracic aortic prosthesis, which can be treated by omentoplasty to the pleural cavity [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

A case of multiple median sternotomy for infection and expanding hematoma in 10 years

Nakajima,

Iba,

Shibata

et al. 2023

Egypt Heart J

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Background After a median sternotomy, mediastinitis may develop, necessitating reopening of the chest. Rarely, reoperation due to hematoma after cardiovascular surgery is experienced. In the present case, we experienced a patient who initially had mediastinitis, but later developed a chronic hematoma and underwent multiple surgeries. Case presentation The patient was a 40-year-old man who underwent aortic valve replacement for a bicuspid aortic valve and a graft for a dilated ascending aorta. Postoperatively, he developed hematoma in the anterior mediastinum on multiple occasions with repeated episodes of infection that required multiple median sternotomies. Conclusions We reported our experience with a rare case of multiple median sternotomies. In the early stage, mediastinitis due to infection was observed, and in the late stage, mediastinal dilatation due to hemorrhage was observed.

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“…Bicuspid aortic valve and Marfan syndrome (MFS) are both associated with an extremely high risk to develop a thoracic aortic dissection ( 5 , 6 ). During the last decade, a number of studies dealing with the embryonic development of aortopathy in BAV and MFS have significantly improved our knowledge on the etiology ( 7 , 8 ). In both conditions an altered neural crest cell and second heart field contribution, separately or in combination, can account for a maturation defect of the vascular smooth muscle cells resulting in a structurally different aortic root and ascending aortic wall ( 8 , 9 ).…”

Section: Introductionmentioning

confidence: 99%

Are acute type A aortic dissections atherosclerotic?

Grewal

Dolmaci²,

Jansen³

et al. 2023

Front. Cardiovasc. Med.

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BackgroundType A aortic dissections (TAAD) are devastating aortic complications. Patients with Marfan syndrome, a bicuspid aortic valve or a thoracic aortic aneurysm have an increased risk to develop a TAAD. These predisposing conditions are characterized by a histologically thin intimal layer and hardly any atherosclerosis. Little is known about the susceptibility for atherosclerosis in patients with a type A aortic dissection.ObjectiveWe aim to systematically describe atherosclerotic lesions in TAAD patients.Materials and methodsA total of 51 patients with a TAAD (mean age 62.5 ± 10.8 years, 49% females) and 17 control patients (mean age 63 ± 5.5 years, 53% females) were included in this study. Cardiovascular risk factors were assessed clinically. All sections were stained with Movat pentachrome and hematoxylin eosin. Plaque morphology was classified according to the modified AHA classification scheme proposed by Virmani et al.ResultsIn the TAAD group thirty-seven percent were overweight (BMI > 25). Diabetes and peripheral arterial disease were not present in any of the patients. Fifty-nine percent of the patients had a history of hypertension. The intima in TAAD patients was significantly thinner as compared to the control group (mean thickness 143 ± 126.5 μm versus 193 ± 132 μm, p < 0.023). Seven TAAD patients had a normal intima without any form of adaptive or pathological thickening. Twenty-three TAAD patients demonstrated adaptive intimal thickening. Fourteen had an intimal xanthoma, also known as fatty streaks. A minority of 7 TAAD patients had progressive atherosclerotic lesions, 4 of which demonstrated pathological intimal thickening, 3 patients showed early fibroatheroma, late fibroatheroma and thin cap fibroatheroma. In the control group the majority of the patients exhibited progressive atherosclerotic lesions: three pathologic intimal thickening, two early fibroatheroma, six late fibroatheroma, one healed rupture and two fibrotic calcified plaque.DiscussionThis study shows that TAAD patients hardly exhibit any form of progressive atherosclerosis. The majority of TAAD patients showcase non-progressive intimal lesions, whereas the control group mostly demonstrated progressive intimal atherosclerotic lesions. Findings are independent of age, sex, or the presence of (a history of) hypertension.

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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

Cited by 21 publications

References 57 publications

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

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

A case of multiple median sternotomy for infection and expanding hematoma in 10 years

Are acute type A aortic dissections atherosclerotic?

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