title: Surgical options in subaortic stenosis associated with endocardial cushion defects.
abstract: Over a 15-year period, 12 patients with endocardial cushion defects undergoing correction had subaortic stenosis requiring operative intervention. Ages ranged from 4 months to 17 years (mean, 7 +/- 6 years) and subaortic gradients from 15 to 100 mm Hg (mean, 60 +/- 25 mm Hg). Subaortic stenosis was due to discrete fibromuscular tissues in 7 patients, mitral valve malattachment in 3, and tunnel outflow in 2. In 2, the subaortic stenosis was clinically significant at the time of endocardial cushion defects repair, whereas in 10 it was noted 2 to 14 years postoperatively (mean, 6.3 +/- 5 years). Surgical relief of subaortic stenosis was accomplished by resection of muscle tissues in 7, apicoaortic conduit insertion in 2, modified Konno procedure (aortic valve preserved) in 2, and lifting of malattached mitral valve from the outflow in 1. There was no early death and one late death (infected conduit). Severe mitral insufficiency developed in the patient who had the mitral valve lifted and necessitated valve replacement. Postoperative echocardiographic gradient in 9 patients ranged from 0 to 36 mm Hg (mean, 10.5 +/- 14 mm Hg). Clinically significant subaortic stenosis has not developed in any patient in 15 years of follow-up (mean, 5 +/- 4 years). We conclude that in subaortic stenosis associated with endocardial cushion defects, resection is effective for discrete obstruction, whereas a modified Konno procedure is preferable for obstruction due to tunnel outflow or mitral valve malattachment.
authors.affiliation: Heart Institute for Children, Christ Hospital and Medical Center, Oak Lawn, Illinois.
authors.forename: S Y
authors.initials: SY
authors.lastname: DeLeon
authors.forename: M N
authors.initials: MN
authors.lastname: Ilbawi
authors.forename: W R
authors.initials: WR
authors.lastname: Wilson
authors.forename: R A
authors.initials: RA
authors.lastname: Arcilla
authors.forename: O G
authors.initials: OG
authors.lastname: Thilenius
authors.forename: S
authors.initials: S
authors.lastname: Bharati
authors.forename: M
authors.initials: M
authors.lastname: Lev
authors.forename: F S
authors.initials: FS
authors.lastname: Idriss
mesh_terms: D001024:Aortic Valve Stenosis / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D001807:Blood Vessel Prosthesis; D002648:Child; D004452:Echocardiography; D004694:Endocardial Cushion Defects / Q000150:complications / Q000601:surgery*; D005500:Follow-Up Studies; D006801:Humans