Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report

Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report

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Abstract Background Unicuspid unicommissural aortic valve is an extremely rare congenital anomaly that usually presents in adulthood but can rarely Ball - Softballs present in infancy.We report a 17-year-old patient with congenital aortic stenosis secondary to unicuspid unicommissural aortic valve that was successfully treated with aortic valve replacement.Case presentation The patient was diagnosed with aortic stenosis after a murmur was heard in the newborn nursery and subsequently underwent aortic balloon valvuloplasty 6 weeks after birth.

He had been regularly followed up since and underwent numerous cardiac catheterizations, including another aortic balloon valvuloplasty at age 13.During follow-up at age 17, the patient presented with symptomatic severe aortic stenosis and mild left ventricular Horse Tail Cords hypertrophy.Aortic valve replacement was planned since the patient was nearly adult-sized and to reduce the risk of cardiac decompensation.

During the operation an unicuspid unicommissural aortic valve was revealed.The patient recovered well post-operatively.He was discharged 5 days after the surgery in good condition and was completely symptom-free at follow-up 6 weeks later.

Conclusions Unicuspid aortic valve is a rare congenital anomaly that can cause congenital aortic stenosis.It is seldom diagnosed pre-operatively but should be suspected in infants presenting with aortic stenosis.