دریچه آئورت دولتی و نکات برگزیده

دريچه ي آئورت دولتي (bicuspid Aortic valve)و نكات برگزيده:

نماي يك دريچه نرمال آئورت

*مقوله اي كه درگذشته هنگام ارائه ي بيماريهاي مادرزادي قلب به كنارگذاشته مي شد،اكنون يكي از شايعترين و پراهميت ترين مسائل قلبي مادرزادي قلمداد ميگردد

نمايي از يك دريچه ي آئورت دولتي

*علت مهم شدن آن همراهي بالايش با مسائل روت آئورت مثل آنوريسم مي باشد

*شايعترين زمينه براي وقوع آنوريسم –ديسكشن آئورت درجوانان ديگر مارفان نمي باشد.دريچه ي آئورت دولتي است!!

*خود دريچه ي دولتي ميتواند منشاءASو AIنيز گردد

*درصورت نبود تنگي يا نارسايي دريچه، تشخيص با دقت زياد براي سمع كليك دركانون آئورت يا آپكس مقدور است

پس هر نوع صداي غير طبيعي را درسمع قلب جدي بگيريم و تنها بدنبال سمع سوفل نباشيم

*به علت وقوع بالاي مسائل روت دراين بيماران سايزينگ سالانه و مرتب روت الزامي است تا بر اساس آن محدوديتهاي ورزشي لازم اعمال شود

*درصورتي كه سايز ريشه ي آئورت از 4.5سانتي متر بيشتر بوده يا سرعت افزايش سايز بالاباشد بيمار كانديد تعويض روت بايا بدون دريچه در سن كودكي و نوجواني ميگردد.

*تنگي دريچه با PTAC يا بالون دريچه ي آئورت قابل مدارا ست.هرچند احتمال عود ASو يا پيشرفتAIوجود دارد.

دريچه ي نرمال سه لتي آئورت               دريچه ي غيرطبيعي دولتي

دريچه ي آئورت دولتي از نگاه MRI

The congenitally bicuspid aortic valve is the most common congenital malformation, occurring in approximately 2% of the general population. The most common presenting sign or symptom is the presence of a systolic ejection murmur and usually an ejection click. However, depending on the severity of valvular disease, patients with aortic stenosis or aortic insufficiency may present with exercise intolerance, dyspnea on exertion, or chest pain. Those without significant aortic stenosis (usually defined as a gradient <25 mm Hg) and less than mild aortic insufficiency are not restricted. However, over time, the lesion can progress.

The development of aortic stenosis is variable and may be related to valvular characteristics.

pressure gradients increased approximately 18 mm Hg each decade, concomitant with valve sclerosis. Patients with anteroposterior (as opposed to right-left) and eccentric (versus symmetric) valve leaflets had a faster rate of progression, with an aortic valve pressure gradient increase averaging 27 mm Hg per decade.

in those with a gradient >50 mm Hg, arrhythmias, sudden death, endocarditis, syncope, and angina occurred at a rate of 1.2% per year.

Aortic root dilation may develop in some patients with bicuspid aortic valve. Interestingly, some studies provide support for the theory that the bicuspid aortic valve is part of a single developmental anomaly that also affects the aortic root with an aortopathy that eventually leads to aortic root dilation.

For this reason, if aortic valve replacement is necessary, replacement of the aortic root should be considered, particularly if the aortic root exceeds 45 mm.

Therefore, those with bicuspid aortic valve but without significant aortic valve stenosis or regurgitation require routine assessment for aortic root enlargement. Echocardiography may be used to screen and follow the aortic root but may not provide adequate imaging beyond the first few centimeters above the sinuses of Valsalva. Therefore, echo potentially will miss significant enlargement of the distal ascending aorta. A CMR or computerized tomography (CT) study as screening tools are necessary at some point to evaluate the entire aortic root from the valve annulus to the takeoff of the great vessels