Aortic valve stenosis

Congenital aortic stenosis is a malformation of the heart with a narrowing (stenosis ) of the large main artery ( aorta), which arises from the left ventricle and supplies the body with oxygen-rich blood. Congenital aortic stenosis accounts for about 5-6 % of all congenital heart defects. They can be divided three different forms depending on the level of the constriction. The valvular stenosis with 75 to 80 % of the total is the most common form. Furthermore, there are still the subvalvular aortic stenosis and supravalvular aortic stenosis rare.

All three forms of a flow obstacle causing stenosis of the left ventricle into the systemic circulation. From the degree of stenosis, it depends on how high the pressure load of the left ventricle, which then become enlarged and thickening (hypertrophy ) leads to the heart muscle. The complete closure or agenesis of the aortic valve is called Aortenklappenatresie.

Hypertrophic obstructive cardiomyopathy ( HOCM ), in which the increase in muscle mass in the left ventricle and the septum moved the discharge channel is expected to be acquired aortic stenosis.

• 4.1 Ross operation

Forms of congenital aortic stenosis

Three congenital constrictions can account for the aortic stenosis:

Valvular stenosis

The valvular stenosis is the typical aortic stenosis. It can be caused by a too-tight valve ring or by a malformation, deformity or thickening of the valve leaflets. Often, instead of the three valve leaflets normally applied only two ( = bicuspid aortic valve zweisegelige ) that allow only a small slot-shaped opening. This bicuspid aortic valve may also constitutes the most common primary cause of aortic stenosis in adults who are conspicuous only later by secondary inflammation or calcification. In addition, this deformity leads to an increased risk of other diseases of the aorta such as aortic aneurysm which.

Subvalvular stenosis

The subvalvular stenosis below the aortic valve in the outflow tract of the left ventricle is also called subaortic stenosis. Cause is usually a connective tissue membrane, but they can also occur as a tunnel-like narrowing of the outflow tract of the left ventricle.

Supravalvular stenosis

The supravalvular stenosis is located above the aortic valve in the initial part of the aorta. In this rarely occurring form to find mostly hourglass constrictions in the initial part of the aorta above the outgoing circuit of the coronary arteries. The ascending portion of the aorta can be narrowed but also a long stretch.

The supravalvular aortic stenosis is an essential feature of the Williams -Beuren syndrome.

Symptoms

Mild aortic stenosis cause harmful interference, in physical development, efficiency and general well-being of children. Medium and high-grade stenoses cause shortness of breath and rapid fatigue on exertion. Older children with severe aortic stenosis, severe arrhythmia and syncope associated with loss of consciousness can occur with sudden efforts.

Diagnostics

The cardiac ultrasound ( echocardiography ) is a non- invasive method nowadays the method of choice dar. For high-resolution images of the heart and functional magnetic resonance imaging ( MRI) is employed. For the diagnosis of concomitant cardiac arrhythmia, an electrocardiogram (ECG) is performed. The heart shape and size are shown in a radiograph. For specific questions regarding the decision on the therapeutic approach may also be required cardiac catheterization.

Therapy

• A balloon valvuloplasty (the " blasting " the narrowed aortic valve using a balloon catheter heart ) is the current state (2012 ) only for unstable patients as a bridge to definitive surgery or for inoperable patients as a palliative measure. A newer method, called percutaneous aortic valve replacement, in which a balloon catheter, a stent with artificial aortic valve is used ( for example, from porcine ), is currently the treatment of choice.
• If this is not possible, the " bonded pocket flaps " in an operation can be separated by the surgeon cuts the commissures. Both procedures are performed carefully to no valvular regurgitation ( leakage through lack of closure) to provoke.
• If an aortic valve replacement necessary today include both the use of a homograft (human donor valve) as well as the use of an artificial heart valve in question.

While a homograft with time can calcify (especially in children) and then must be replaced, holds an artificial heart valve decades. The "disadvantage " is that the patients on the lifelong use of anticoagulant medication (eg coumarin derivative) are dependent with regular monitoring of blood counts. Also the risk of internal bleeding after falling exists. Coumarins are teratogenic. Therefore, a woman under coumarin dose should not be pregnant.

Ross operation

The Ross procedure is gaining in relation to aortic valve replacement in importance. While the aortic valve is removed and replaced by the own pulmonary valve of the patient. Instead of a pulmonary valve homograft is implanted. Especially for children and young adults, this operation is an attractive solution. The aortic valve grows with normal, there is no taking anticoagulant medications necessary to the practice of sport is possible.

A replacement of the homograft to Pulmonalisposition is less serious because the pulmonary valve is less loaded than the aortic valve. Of concern may be that of a "one- valve patients " a " two-valve patient" is. Therefore, the decision for or against the Ross operation in intensive exchange between doctor and patient about the patient's wishes to his quality of life and expected to be done. In the hands of experienced cardiac surgeons who have this surgery often performed in patients at any age, can be regarded as a very good alternative to the " simple" heart valve replacement today.

In people with Marfan syndrome not Ross operation is performed, as the genetic disposition would lead to aortic insufficiency ( inability circuit ) or an aneurysm again.

Long-term expectations

Lifetime heart check-ups are necessary. Also on endocarditis must be respected.