3. Causes of Aortic regurgitation : Congenitally abnormal valves

Kathryn Statham , Genevieve Carbonatto

Congenitally abnormal valves

  • Bicuspid
  • Unicuspid
  • Quadricuspid


Bicuspid valves (BAV) have been classified in many different ways. An easy classification  looks at  the number of cusps, the presence of raphes and the position and symmetry of cusps. A raphe refers to a conjoined area of two underdeveloped leaflets turning into a malformed commissure between both leaflets . When no raphe is present, the valve is called strictly bicuspid.

BAV are

  •  3 X more common in men
  • May  be associated with non valvular lesions such as abnormal coronary artery anatomy. Most patients have left dominant coronary circulation. The left main dominant may arise from the pulmonary artery.
  • Often  associated with an aortopathy involving dilatation of the thoracic aorta
  • Associated with coarctation of the aorta in at least 20% of cases

Below are clips of a bicuspid valve seen from the PLAX, the PSAX and the PSAX view with colour Doppler

Notice the bowing of the aortic valve

Notice the soccer ball closing of the aortic valve and compare this with a normal trileaflet AV

Bicuspid valve above

Normal tricuspid valve above

PSAX view of bicuspid valve with  colour Doppler showing regurgitation.

Quadricuspid valves are rare congenital abnormalities with an estimated frequency of < 0.05%. The most common abnormality associated with quadricuspid aortic valves is aortic root dilatation. AR is found in 30% of patients with quadricuspid valves


Unicuspid aortic valves is a very rare congenital abnormality presenting in the 3rd or 5th decade of life (unless identified in infancy) with severe aortic stenosis and regurgitation



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