• Normally aortic valve is having 3 leaflets in human beings but occasionally this valve is having 2 leaflets instead of 3 leaflets, this is known as bicuspid aortic valve.
  • Aortic valve is a valve that is present in one major blood vessel of heart that is known as Aorta. This valve regulates pure blood from the left sided chamber of the heart to the body.
  • The aortic valve allows oxygen-rich blood to flow from the heart to the aorta. It prevents the blood from flowing back from the aorta into the heart when the pumping chamber relaxes.
  • This is most common congenital heart disease seen in children. Reason is unclear for its cause. Sometimes, it is genetic as well.
  • BAV is more common in boys as compared to girls.
  • BAV most commonly associated with coarctation of the aorta (COA, narrowing of major blood vessel of heart).
  • This valve can leads to symptoms as well as child may be asymptomatic

Symptoms

  • Symptoms due bicuspid aortic valve depending whether this valve is narrowed or leaking (and what is its severity and age of presentation), these are known as aortic valve stenosis (AS) or aortic valve regurgitation (AR).
  • In critical stenosis (seen in newborn babies), babies present in shock like state due to poor cardiac output to the body. Baby will have poor feeding, difficulty in breathing, poor urine output etc.
  • If there is severe stenosis(Aortic stenosis) then child may have chest pain, fainting attacks or shortness of breath etc.
  • If severe leaking (Aortic regurgitation) is there, then child mainly have difficulty in breathing as an initial symptom.

Diagnosis

  • “Pediatric Echocardiogram” is the definitive investigative modality for making the diagnosis.
  • Other investigations that can be done if severe stenosis or regurgitation are: ECG, Chest x ray.
  • Sometimes, CT or MRI needs to be done when dilatation of aorta is also present along with stenosis or regurgitation.

Treatment

  • Child with bicuspid aortic valve should undergo regular follow up with pediatric cardiologist as valve may get narrowed or can leak or aorta can be enlarged later on if no problems due to valve on diagnosis.