Quadricuspid Aortic Valve

The normal aortic valve is trileaflet and comprised of the non, left, and right coronary cusps. Quadricuspid aortic valve (QAV) is an extremely rare malformation occurring in only 0.008% of autopsies and 0.043% of cases examined through echocardiography. Hurwitz and Roberts proposed seven classes of QAV based on the arrangement and relative sizes of the cusps; Nakamura et al. created a simplified classification system based on the position of the extra cusp.

Substantial valvular insufficiency (and less often stenosis) occurs, typically leading to surgical intervention in the fifth or sixth decade of life. Symptoms include those found in chronic aortic valve insufficiency – fatigue, shortness of breath, palpitations, chest pain, swelling, and even heart failure.

Credit: TEE submitted by my colleague, Dr. Paden Karnes, MD.

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