Unravelling aetiology and risk factors in patients with a Bicuspid Aortic Valves * study of clinical outcome and genetics

Unravelling aetiology and risk factors in patients with a Bicuspid Aortic Valves * study of clinical outcome and genetics - BAV

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

NL-OMON

Registry ID

NL-OMON44982

Enrollment

220

Registered

2014-06-30

Start date

2014-11-20

Completion date

Unknown

Last updated

2024-07-22

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

bicuspid aortic valve turner syndrome

Interventions

None listed

Eligibility

Age

18 Years to 99 Years

Inclusion criteria

Inclusion criteria: The study population consist of 100 adults with *high-risk* BAV and 100 patients with Turner syndrome, who regularly seen at the outpatient clinic of the three academic hospitals (Erasmus MC, RadboudUMC and LUMC). ;Inclusion criteria In order to be eligible to participate in this study, a subject must meet all of the following criteria: * Men and women aged > 18 years; * Capable of understanding and signing informed consent. ;Inclusion criteria for *high-risk* BAV patients are: No prior aortic valve surgery, but with 1 or more of the following risk factors: * Aortic stenosis: gradient >3 m/sec * Aortic regurgitation at least moderate * Aortic aneurysm: >40 mm ascending aorta and/or aortic index > 2.1;Inclusion criteria for patients with Turner syndrome: * Know with chromosoom XO ;In addition, 20 healthy participants will be included who will receive echocardiography once at baseline.

Exclusion criteria

Exclusion criteria: A potential subject who meets any of the following criteria will be excluded from participation in this study: Subjects who have any of the conditions below at the time of the enrollment or scan session are excluded from this study. Further, should it be determined by study staff that an enrolled subject meets any of the conditions below during the course of the study, the subject will be removed from the study. * Contra-indication to radiation exposure (CT/MRI): pregnancy * Contra-indication to iodine contrast media: renal failure, iodine Allergy * Any contraindication to an MRI scan or gadolinium contrast media (renal failure, allergy)

Design outcomes

Primary

Measure	Time frame
The strong endpoints in real life setting are death, surgery, heart failure and/or arrhythmia but as we expect to see only low incidence of these hard endpoints within a 4-year timeframe, we will use surrogate endpoints including progression of aortic valve stenosis/regurgitation (echocardiography and MRI), progression of left ventricular hypertrophy (MRI), progression of aortic dilatation (MRI and ECG synchronized CT), aortic distensibility (CTA and MRI), decrease in exercise tolerance (VO2max exercise test), and subclinical worsening of left ventricular function (Speckle echocardiography). This study will form the basis of a longer prospective study of 10 years in which the hard endpoints can be assessed.	—

Measure

Time frame

The strong endpoints in real life setting are death, surgery, heart failure and/or arrhythmia but as we expect to see only low incidence of these hard endpoints within a 4-year timeframe, we will use surrogate endpoints including progression of aortic valve stenosis/regurgitation (echocardiography and MRI), progression of left ventricular hypertrophy (MRI), progression of aortic dilatation (MRI and ECG synchronized CT), aortic distensibility (CTA and MRI), decrease in exercise tolerance (VO2max exercise test), and subclinical worsening of left ventricular function (Speckle echocardiography). This study will form the basis of a longer prospective study of 10 years in which the hard endpoints can be assessed.

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Countries

Netherlands

Outcome results