title: Supravalvar aortic stenosis: clinical and hemodynamic profile, and surgical outcome.
abstract: BACKGROUND
  Supravalvar aortic stenosis is the rarest of left ventricular outflow obstructions. Data on this rare entity from India are scarce.
  
  
  RESULTS
  We retrospectively analyzed the data of 15 patients (13 males, mean age 15.5+/-10.18 years) with a diagnosis of supravalvar aortic stenosis confirmed by cardiac catheterization. Five patients had morphological features of Williams' syndrome. One patient had diffuse while the rest had discrete type of supravalvar aortic stenosis. Five patients did not have any associated lesions. A 9-year-old male had an ascending aortic aneurysm, and 3 patients had associated peripheral pulmonary artery stenosis. One child had a subaortic ventricular septal defect, and another had severe mitral regurgitation. Twelve patients had electrocardiographic evidence of left ventricular hypertrophy. Three patients had mild aortic valvar stenosis while 2 had aortic regurgitation. Six patients had dilated coronary arteries. Two patients with supravalvar aortic gradients of 20 and 40 mmHg were kept on close follow-up. One patient was not willing to undergo surgery while the other is awaiting surgery. Eleven patients underwent surgical correction. Dacron or pericardial patch aortoplasty was done in all the patients. In addition, one patient each underwent pulmonary artery plasty, ventricular septal defect closure, repair of ascending aortic aneurysm, and mitral valve replacement. The patient with diffuse type of supravalvar aortic stenosis underwent augmentation aortoplasty. Two patients died perioperatively. One was lost to follow-up. Two had moderate residual gradients. The rest of the patients were in New York Heart Association functional class I on follow-up of 6.3+/-4.7 years.
  
  
  CONCLUSIONS
  Repair of supravalvar aortic stenosis by single sinus aortoplasty is safe and produces good results.
authors.affiliation: Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram. drharikrishnan@hotmail.com
authors.forename: S
authors.initials: S
authors.lastname: Harikrishnan
authors.forename: S R Krishna
authors.initials: SR
authors.lastname: Manohar
authors.forename: Krishna Kumar
authors.initials: KK
authors.lastname: Nair
authors.forename: Jaganmohan
authors.initials: J
authors.lastname: Tharakan
authors.forename: Thomas
authors.initials: T
authors.lastname: Titus
authors.forename: V K Ajith
authors.initials: VK
authors.lastname: Kumar
authors.forename: Anil
authors.initials: A
authors.lastname: Bhat
authors.forename: S
authors.initials: S
authors.lastname: Sivasankaran
authors.forename: Francis
authors.initials: F
authors.lastname: Bimal
authors.forename: K M
authors.initials: KM
authors.lastname: Krishna Moorthy
authors.forename: R Padma
authors.initials: RP
authors.lastname: Kumar
mesh_terms: D021921:Aortic Stenosis, Supravalvular / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D006328:Cardiac Catheterization; D017023:Coronary Angiography; D006439:Hemodynamics; D006801:Humans; D012189:Retrospective Studies; D016896:Treatment Outcome