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Hypoplastic Left Heart Syndrome Aortic Stenosis Single Ventricle

Aortic Stenosis

  • Aortic valvular stenosis refers to any obstruction in outflow from the left ventricle. Prevalence of this anomaly increases with age and almost all patients are male (80%).
  • The most common aortic valve anomaly in children is a bicuspid valve. Obstruction is common. In 33% of cases, thickening of the leaflets and adhesion of the remaining commissure results in progression of obstruction.
  • The degree of obstruction is determined by the amount of ventricular pressure required to adequately perfuse tissues. For example, conditions that require increased cardiac output such as exercise or anemia require higher pressure in the left ventricle which in turn, worsens obstruction.
  • Patients with aortic valvular stenosis are usually asymptomtatic with normal growth and development. These cases are often diagnosed during routine childhood physical exams when an abnormal heart murmur is heard. Exercise intolerance and fainting are less common, although not unheard of.
  • Management of valvular aortic stenosis requires life-long monitoring, as it is progressive and associated with sudden death, often requires repeat intervention, and is a site of endocarditis. Calcification, aortic regurgitation, and left ventricular dysfunction also become more common with age.
    • It is generally recommended that peak-peak catheterization gradients and te results of electrocardiograms and stress tests be used as a guide to management.
      • Intervention always includes endocarditis prophylaxis and depending on these tests, may also include restrictions on physical activities, balloon dilation, and replacement of the valve.