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Advanced Procedures - Bentall Procedure

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Buttons inside graft.jpg
dissecting right cusp side.jpg

This page is under construction

Objectives - Topics to be Covered

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  • Anatomy - refer to aortic valve and aortic replacements pages

  • Diseases associated with a combination of:

    • Aortic Root Dilation​

    • Aortic Valve Insufficiency

    • Aortic Aneurysm

  • Procedure

    • Cannulation strategy​

    • When to use deep hypothermic circulatory arrest (DHCA) 

    • Preparing the aortic root

    • ​Removal of the Aorta and aortic valve

    • Creating and preparing the coronary buttons

    • Sizing and placing the aortic valve/graft composite

  • Distal anastomosis

  • Modifications 

    • reenforcing the root

Manifestations of aortic valve disease in the context of connective tissue disorders include valvular stenosis, regurgitation, and/or thoracic aortic aneurysms (Tables 1 and 2). Both inherited and inflammatory connective tissue disorders contribute to aortic valve damage with increased susceptibility associated with specific gene variants. There is insufficient knowledge regarding the underlying mediators, and thus appropriate immunotherapy to target specific cytokines in order to dampen the inflammatory response. For example, in Marfan’s syndrome, TGF-β has been documented to play a crucial role and thus presents a therapeutic target. Ultimately, the goal of such therapies would be the induction of remission without the need for valve replacement, which is associated with inherent risk. Despite demonstrating promising results in the treatment of Ehlers–Danlos syndrome (EDS), surgical intervention of the aortic valve has been associated with many complications, including the need for recurring operations in Behcet disease and ankylosing spondylitis and a high mortality and morbidity rate in systemic lupus erythematosus (SLE).

 

​Exerts from Disorders of the Aorta and Aortic Valve in Connective Tissue Diseases Bogna Grygiel-Górniak1 Published online: 19 June 2020 #The Author(s) 2020 &Mary-Tiffany Oduah1 & Abdulbaril Olagunju1 & Michal Klokner1; Current Cardiology Reports (2020) 22: 70

Table 1: Connective tissue diseases and their manifestations in the aorta and aortic valve

Aortic disorders and aortic valve manifestations

Aortic valve regurgitation

Aortic Valve Stenosis

Aortic valve stenosis and regurgitation

Aortic valve thickening

Dilation of aortic root and/or ascending aorta; dissecting thoracic aortic aneurysms; aortic valve regurgitation

Connective tissue diseases

• Ankylosing spondylitis

• Churg–Strauss syndrome

• Behcet disease

• Ehlers–Danlos syndrome

• Takayasu vasculitis

• Primary antiphospholipid syndrome

• Systemic sclerosis

• Primary antiphospholipid syndrome-related bioprosthetic valve stenosis

• SLE

• Sjogren syndrome

• Rheumatoid arthritis

• Granulomatosis with polyangiitis

• Marfan’s syndrome

Table 2: Aortic valve changes in certain vasculitis

Name of vasculitis        Takayasu arteritis         Granulomatosis          Churg–Strauss syndrome   Behcet disease

                                                                               with polyangiitis

Type of vasculitis

Large vessel arteritis

ANCA-associated small vessel vasculitis

ANCA-associated small vessel vasculitis

Various vessel vasculitis

Aortic involvement

Aortic regurgitation

Aortic regurgitation (rarely aortic stenosis)

Mainly aortic regurgitation

Aortic regurgitation

Aneurysms

Pseudoaneurysms

Aortic rupture

Stenotic lesions (brachiocephalic artery)

Characteristic of aortic valve changes

• Secondary to dilation of the aortic root

• Normal valvular cusps in echocardiography shows

• Aneurysm can be present

• Thickened cusps of the aortic valve

• May be the only sign of relapse in GPA

• The onset: at the time of diagnosis or few years after diagnosis and correlates with elevated PR3-ANCA

• Chronic or acute manifestations (e.g., the perforation of the cusps of the aortic valve)

The valve is dense and thick due to fibrosis and the inflammatory reaction

Aortic regurgitation

• The most common valvular pathology

• Aneurysm

• Elongation and prolapse of aortic cusps

• Vegetation like lesions

Aortic valve regurgitation

• Can be secondary to aneurysm of the ascending aorta due to vasculitis

• Normal or fibrosed cusps of the valves

• Annulus dilatation

• Cusp fenestration and fusion

Histopathologic findings

• Disrupted elastic fibers in the media

• Granuloma

• Marked collagen deposition in the adventitial

• Granulomatous inflammation

• Polymorphonuclear microabscesses (differentiate with infective endocarditis)

• Foci of necrosis

• All findings located in the central layer of the valve

• Fibrosis and inflammation => dense and thick valve

• Valve leaflets infiltrated by eosinophils, lymphocytes and plasma cells

• Necrotizing granulomas

Lymphoplasmacytic infiltrates, neutrophils, histiocytes, eosinophils and occasional giant cells, myxoid degeneration, focal necrosis, and fibrotic thickening

Other causes include

Association with bicuspid aortic valve

Hypertension and Atherosclerosis

 

Other Contributing Factors

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