Háskóli ÍslandsUniversity of IcelandIngason, ArnarSigfússon, GunnlaugurTorfason, Bjarni2018-12-072018-12-072018-06-07Ingason, A. B., Sigfusson, G., & Torfason, B. (2018). Congenital aortic stenosis due to unicuspid unicommissural aortic valve: a case report. Journal of Cardiothoracic Surgery, 13(1), 61. doi:10.1186/s13019-018-0755-01749-8090https://hdl.handle.net/20.500.11815/942Publisher's version (útgefin grein)Background Unicuspid unicommissural aortic valve is an extremely rare congenital anomaly that usually presents in adulthood but can rarely 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 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.eninfo:eu-repo/semantics/openAccessUnicuspid aortic valveCongenital aortic stenosisAortic valve replacementCase reportBlóðrásarsjúkdómarinfo:eu-repo/semantics/articleJournal of Cardiothoracic Surgery10.1186/s13019-018-0755-0