HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): John W. Brown Mark Ruzmetov Mark W. Turrentine Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Brown, J. W. Articles by Turrentine, M. W. Search for Related Content PubMed PubMed Citation Articles by Brown, J. W. Articles by Turrentine, M. W. Related Collections Congenital - acyanotic

Eur J Cardiothorac Surg 2002;21:50-56
© 2002 Elsevier Science NL

Surgical repair of congenital supravalvular aortic stenosis in children

Section of Cardiothoracic Surgery, James W. Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN 46202-5123, USA

Received 3 July 2001; received in revised form 20 August 2001; accepted 31 October 2001.

* Corresponding author. Tel.: +1-317-274-7150; fax: +1-317-274-2940
e-mail: jobrown{at}iupui.edu

Objective: Supravalvular aortic stenosis (SVAS) is an uncommon congenital cardiac anomaly characterized by varying degrees of left ventricular outflow tract obstruction beginning distal to the aortic valve. Methods: Between March 1962 and December 2000, 101 consecutive patients underwent surgical correction for congenital SVAS at Riley Children's Hospital. There were 61 male (60%) and 40 female (40%) ranging in age from 3 month to 17 years (medium age, 6.1 years). Fourteen patients (14%) had Williams syndrome. Preoperatively, 11 patients were in New York Heart Association (NYHA) functional class I, 55 in class II, 28 in class III, and seven in class IV. Of the 101 patients, 73 (72%) had localized type SVAS and 28 (28%) diffuse type SVAS. Results: Those with localized SVAS were successfully treated with patch aortoplasty, whereas those with diffuse SVAS required either an apical aortic conduit or extensive endarterectomy with patch aortoplasty. The overall mean pressure gradient was reduced to 21 mmHg (P<0.001) in the early postoperative period. There were one early death (<30 days postoperatively) (1%), two (2%) late deaths, and 14 patients (14%) underwent one or two additional operation (n=17) in a follow-up period ranging from 6 months to 30 years (medium 9.4 years). Postoperatively, there were 72 patients (73%) in NYHA functional class I and 26 (27%) in class II. Overall survival including operative mortality was 98% at 10 years, 97% at 20 and at 30 years. Conclusion: Good surgical outcome of congenital SVAS can be achieved with the appropriate method of treatment in patients with both localized and diffuse SVAS.

Key Words: Congenital valve disease • Aortic stenosis • Children • Left ventricular outflow tract

This article has been cited by other articles:

				O. Metton, W. Ben Ali, D. Calvaruso, D. Bonnet, D. Sidi, O. Raisky, and P. R. Vouhe Surgical management of supravalvular aortic stenosis: does Brom three-patch technique provide superior results? Ann. Thorac. Surg., August 1, 2009; 88(2): 588 - 593. [Abstract] [Full Text] [PDF]

				D. J. Scott, D. N. Campbell, D. R. Clarke, S. P. Goldberg, D. R. Karlin, and M. B. Mitchell Twenty-year surgical experience with congenital supravalvar aortic stenosis. Ann. Thorac. Surg., May 1, 2009; 87(5): 1501 - 1508. [Abstract] [Full Text] [PDF]

				R. Formigari, G. Michielon, M. C. Digilio, G. Piacentini, A. Carotti, A. Giardini, R. M. Di Donato, and B. Marino Genetic syndromes and congenital heart defects: how is surgical management affected? Eur. J. Cardiothorac. Surg., April 1, 2009; 35(4): 606 - 614. [Abstract] [Full Text] [PDF]

				E. J. Hickey, G. Jung, W. G. Williams, C. Manlhiot, G. S. Van Arsdell, C. A. Caldarone, J. Coles, and B. W. McCrindle Congenital Supravalvular Aortic Stenosis: Defining Surgical and Nonsurgical Outcomes Ann. Thorac. Surg., December 1, 2008; 86(6): 1919 - 1927. [Abstract] [Full Text] [PDF]

				E. Arnaiz, D. Koolbergen, A. Adsuar, and M. G. Hazekamp Surgery for supravalvular aortic stenosis - the three-patch technique MMCTS, September 15, 2008; 2008(0915): 2329. [Abstract] [Full Text] [PDF]

				J. W. Brown, M. Ruzmetov, P. Vijay, M. D. Rodefeld, and M. W. Turrentine Surgery for aortic stenosis in children: a 40-year experience Ann. Thorac. Surg., November 1, 2003; 76(5): 1398 - 1411. [Abstract] [Full Text] [PDF]

				M. Yamagishi, K. Shuntoh, T. Matsushita, K. Fujiwara, T. Shinkawa, T. Miyazaki, and N. Kitamura Complete augmentation of diffuse narrowing of the aorta with Williams syndrome by using an overturn approach J. Thorac. Cardiovasc. Surg., June 1, 2003; 125(6): 1556 - 1558. [Full Text] [PDF]