Extended septoplasty for subaortic stenosis developed 19 years after double-outlet right ventricle repair

A 27-year-old woman developed subaortic stenosis 19 years after double-outlet right ventricle repair. Subaortic stenosis was caused by a narrow ring of fibromuscular ridge associated with a bulge of the underlying septal muscle. The aortic valve was bicuspid and stenotic. We conducted extended septoplasty, replacing the aortic valve. Postoperative cineangiogram showed an adequate left ventricular outflow pathway. Double-outlet right ventricle repair may thus be followed by subaortic stenosis as long as 19 years after initial surgery. This lesion was assumed due to acquired disease secondary to flow disturbances in the left ventricular outflow, so reconstructing an adequate outflow pathway is effective and appears to help avoid recurring stenosis.