Aortic root replacement in a patient with left ventricular noncompaction.

We describe the case of a 57-year-old woman with noncompaction of the left ventricle and regurgitant bicuspid aortic valve who presented with progressive congestive heart failure and was successfully treated with aortic root replacement. The long-term outcome for these patients is poor because of progressive left ventricular impairment, increased rates of life-threatening arrhythmias, and intraventricular thrombi. To our knowledge, only 3 patient with noncompaction of the left ventricle has been reported to have undergone aortic valve replacement for severely regurgitant bicuspid aortic valve. Herein, we describe a patient with noncompaction of the left ventricle who underwent successful mechanical aortic root replacement.