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Ann Thorac Surg 1991;52:1329-1330
© 1991 The Society of Thoracic Surgeons

Articles

Aplasia of the right aortic cusp in a neonate: A life-threatening but curable anomaly

Axel Ch. Henze, MD, PhD*, Torsten K. Malm, MD, Jan T. Sunnegårdh, MD, PhD, Mårten B. Hallberg, MD

Departments of Cardiothoracic Surgery and Pediatric Cardiology, University Hospital, Uppsala, Sweden

Accepted for publication May 31, 1991.

* Address reprint requests to Dr Henze, Department of Cardiothoracic Surgery, University Hospital, S-751 85 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.






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