• There are 2 great vessels in heart. One is named as aorta that supplies pure blood to the body. This artery is having a valve.
  • When there is an obstruction either at the level of valve, or below or above the valve, it is known as valvular, subvalvular or supravalvular aortic stenosis.
  • The most common etiology of aortic valve obstruction in neonate or children is bicuspid aortic valve.
  • Normally aortic valve has 3 leaflets; when it is having 2 leaflets then it is known as bicuspid aortic valve; when having one leaflet then known as unicuspid aortic valve.
  • Subvalvular aortic stenosis can be due to fibrous ridge, membrane below aortic valve etc. Besides obstruction below the aortic valve, the most common issue that occurs with this variety of aortic stenosis is that it damages the aortic valve leaflets also.
  • Supravalvar aortic stenosis generally occurs with a syndrome known as ‘Williams’s syndrome’. It can be in the form of hourglass shape or funnel shaped type of obstruction.

Clinical Features

  • Presentation of patient with any variety of aortic stenosis depends on severity of obstruction.
  • In mild form, it is generally asymptomatic and detected on routine examination by a local doctor in the form of murmur on examination.
  • As severity of obstruction increases, patient presents with easy fatigue, chest pain, loss of consciousness (syncope) and in later stages with shortness of breath (dyspnea due to heart failure).

Diagnosis of Aortic Stenosis

  • It can be suspected on examining the child but best modality for detection of aortic stenosis is by Pediatric Echocardiography.

Management of Aortic Stenosis

  • Treatment of aortic stenosis (AS) depends on severity of obstruction.
  • In mild to moderate obstruction, patient needs medical follow-up at periodic intervals most of the time.
  • In severe obstruction, patient needs immediate intervention for resolving the obstruction.
  • In Valvular aortic stenosis, treatment of choice is by doing balloon dilatation of Aortic valve’’.

In subvalvular or supravalvular aortic stenosis, treatment modality for correction the obstruction is by open heart surgery. (Kindly refer to pediatric cardiac surgical section on www.childrenheartcare.com”.
For detailed procedure, kindly refer to intervention section of balloon dilatation of aortic valve on www.childrenheartcare.com”.

Supravalvar aortic stenosis

Valvular Aortic Stenosis

Subaortic membrane with stenosis