The Annals of Thoracic Surgery, Vol 52, 1329-1330, Copyright © 1991 by The Society of Thoracic Surgeons
Aplasia of the right aortic cusp in a neonate: a life-threatening but curable anomaly
AC Henze, TK Malm, JT Sunnegardh and MB Hallberg
Department of Cardiothoracic Surgery, University Hospital, Uppsala, Sweden.
A case of absent right aortic cusp causing serious aortic incompetence in a
neonate is reported. A "bicuspidalization" repair broke down within 24
hours, but reoperation with annular enlargement and insertion of a 19-mm
prosthetic valve resulted in complete recovery. This rare but
life-threatening anomaly of the aortic valve can be recognized with Doppler
echocardiography. Valve replacement in conjunction with aortic annular
enlargement should be performed as primary repair.