Active clinical trials for "Bicuspid Aortic Valve Disease"

The purpose of this study is to determine whether long-term treatment with a beta-blocker (BB) such as atenolol and/or an angiotensin receptor blocker (ARB) such as telmisartan, given to adult patients with bicuspid aortic valve (BAV) disease (aortopathy) reduces the widening (dilatation) of the aorta from its baseline size.

Bicuspid Aortic Valve (BAV) is the most common congenital heart disease affecting 1-2% of the population. The aortic dilation and aortic valve degeneration are common complications in patients with BAV. Statins have shown a reduction in the expression of metalloproteinases of the extracellular matrix observed in aortic aneurisms. Several retrospective studies have suggested the benefit of the statins to reduce aortic dilation in patients with BAV. Moreover, although statins did not show to be useful in the progression of aortic stenosis, different studies have suggested a higher profit when the valve affection is not severe. The objective of this study is to determine whether atorvastatin is effective at reducing the progression of aortic dilation in patients with BAV.

The aorta is the principal arterial vessel arising from the left heart that transfers blood to the body. Certain genetic and familial disease processes are known to weaken the aortic wall resulting in dilation and potential rupture. These aortic complications carry high mortality (>25%) and current management is orientated towards early detection and preventive treatment. Aortic dilation can also result in aortic valve dysfunction leading to heart failure. The estimated UK incidence of aortic disease per year is around 10 per 100,000 individuals, with 2000 people per year dying from aortic complications. The 2017-2020 National Adult Cardiac Surgery Audit report identified the number of people receiving surgery for aortic dissection in Scotland is per population proportionately lower compared to England (4.6 per million per year in Scotland vs. 6.6 per million per year in England). The reasons for this are unclear but may relate to the prevalence of aortic disease or a large geographic distribution with compromised access to specialized centres. Currently surgery is recommended when the aortic diameter exceeds a certain threshold. There are several types of effective surgical procedures, but there is still limited information on their long-term outcomes and the advantage of one procedure over another. The aims of the project are firstly to determine the clinical outcomes of the surgical procedures that are currently employed in Scotland to treat proximal aortic disease and secondly to describe the prevalence and distribution of proximal aortic disease within the Scottish population. The project will be hosted by the Golden Jubilee Research Institute. Contemporary and retrospective data will be collected from all the Scottish Cardiothoracic Surgery units which are based in Glasgow, Edinburgh and Aberdeen. This will be the first study to analyse surgical outcomes for ascending aortic disease in Scotland, and the first to describe the epidemiology of aortic disease within the population. It is anticipated that the results will guide current surgical practise, and provide data to inform national service provision for the management of proximal aortic disease.

BAVgenetics is a partnership between Investigators at Boston University, Brigham and Women's Hospital, and Massachusetts General Hospital dedicated to discovering the genetic causes of bicuspid aortic valve disease and associated aortic disease.

Early development of calcified aortic valve disease (CAVD) is a commonly occurring complication in patients with a bicuspid aortic valve (BAV, an aortic valve consisting of two leaflets instead of three). In general, CAVD is characterized by progressive narrowing of the aortic valve, with involvement of altered calcium metabolism. CAVD progression in fact may lead to necessity of valve replacement, since to date, no other therapies have been shown effective in the treatment of CAVD. The primary objective of our study is to test the hypothesis that supplementation of vitamin K2 will slow down the calcium metabolism in CAVD. Vitamin K2 is essential in the activation of matrix Gla Protein (MGP), an important inhibitory factor in the regulation of calcification. In this randomized controlled trial, 44 patients will be allocated to either the vitamin K2 or placebo group. To assess the calcification process in a detailed manner in these patients, a Positron Emission Tomography (PET) scanner using a tracer (18F-fluoride [NaF]) that has been shown to bind to regions of newly developing microcalcification in aortic valve tissue is used. We expect that vitamin K2 supplementation will reduce the calcium metabolism in the aortic valve on 18NaF-PET (primary endpoint) and slow progression of CAVD as measured by the calcium score on CT and echocardiography after 18 months (secondary endpoints), when compared to placebo.

Aortic valve sparing operations are applied in patients with a non-calcified tricuspid or bicuspid aortic valve associated with a root aneurysm, in order to preserve the valve, and avoid new aortic dilatation by stabilizing the aortic valve anulus with a graft prosthesis. This results in a normal functioning aortic valve, with a low forward gradient across the left ventricular outflow tract. Little is known about how the repaired aortic valve behaves in conditions of controlled exercise, and how the gradients across the valve change during exercise.

To observe the clinical outcomes of the different valve sizing strategies treating type-0 Bicuspid Aortic Stenosis (BAS) with self-expandable transcatheter aortic valve implantation (TAVI) valve, compared with those of a standard sizing strategy of normal Tricuspid Aortic Stenosis(TAS).

In order to determine the effectiveness of medical therapy options to prevent enlargement of the aorta in patients with a bicuspid aortic valve, a randomized study will be planned. This feasibility study will gather accurate data on number needed to screen, changes in medication use over time, and variance of the intended study endpoint.

Bicuspid Aortic Valve (BAV) disease is a common cardiac anomaly that is associated with valvular abnormalities, both stenosis and regurgitation, and aortopathy. It is also shown to play role in abnormal aortic distensibility and stiffness with impairment of aortic elasticity and Left ventricular dysfunction. Mechanism of aortopathy is complex and is not understood completely. In a recent study podocan is found in extracellular matrix (ECM) of human aorta and is found to be accumulated in human abdominal aortic aneurysms. There is no current effective therapy that can alter the progression of aortic dilatation in bicuspid valve disease. Aortic surgery and aortoplasty is the only treatment in severely dilated aorta and aortic dissection. In this study the aim is to investigate the association between podocan and Wnt pathway in development and pathogenesis of aortopathy. This could provide more effective and physiological understanding of disease process and potential target in prevention and treatment for aortopathy.

The purpose of this study is to evaluate the clinical outcomes of transcatheter aortic valve replacement (TAVR) in patients with bicuspid aortic valve stenosis.