Supravalvar aortic stenosis: clinical and hemodynamic profile, and surgical outcome.
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.
Harikrishnan
S
S
Department of Cardiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram. drharikrishnan@hotmail.com
Manohar
S R Krishna
SR
Nair
Krishna Kumar
KK
Tharakan
Jaganmohan
J
Titus
Thomas
T
Kumar
V K Ajith
VK
Bhat
Anil
A
Sivasankaran
S
S
Bimal
Francis
F
Krishna Moorthy
K M
KM
Kumar
R Padma
RP
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