Abstract
Supravalve aortic stenosis (SAS) and arch hypoplasia are features of Williams syndrome. The effect of aortoplasty on growth of the aorta is not established. We hypothesize that growth of the aorta remains deficient whether or not aortoplasty has been performed. Review of the Children’s Hospital of Pittsburgh database revealed 18 patients with Williams syndrome and SAS. Fourteen had sufficient data for inclusion. Patients were divided into two groups based on whether or not they had undergone aortoplasty (groups 1 and 2, respectively). Echocardiographic measurements and Doppler velocity estimates of the aorta were made at two time points in all patients and one additional time point postoperatively for group 1. Measurements were converted to z scores and compared. Peak pulsed echo Doppler velocity (m/sec) in the ascending aorta was higher in patients who underwent aortoplasty. This decreased significantly after surgery. Preoperatively, there was no difference between the groups’ annulus, ascending aorta, transverse aorta, and isthmus measurements. At a mean of 43 months postoperatively, there was no significant change in size of the ascending aorta, transverse aorta, and isthmus. Children with Williams syndrome have hypoplasia of the aortic arch that remains constant. Aortoplasty decreases the aortic gradient but has no effect on the size of the ascending aorta, transverse aorta, and aortic isthmus over the short term.
Similar content being viewed by others
References
LM Bird GF Billman RV Lacro et al. (1996) ArticleTitleSudden death in Williams syndrome: report of ten cases. J Pediatr 129 926–931 Occurrence Handle1:STN:280:ByiC38vlslQ%3D Occurrence Handle8969740
JW Brown M Ruzmetov P Vijay MW Turrentine (2002) ArticleTitleSurgical repair of congenital supravalvular aortic stenosis in children. Eur J Cardiothorac Surg 21 50–56 Occurrence Handle10.1016/S1010-7940(01)01087-9 Occurrence Handle11788256
T Chowdhury W Reardon (1999) ArticleTitleElastin mutation and cardiac disease. Pediatr Cardiol 20 103–107 Occurrence Handle10.1007/s002469900415 Occurrence Handle1:STN:280:DyaK1M7ktleisQ%3D%3D Occurrence Handle9986885
L Debelle AM Tamburro (1999) ArticleTitleElastin: molecular description and function. Int J Biochem Cell Biol 31 261–272 Occurrence Handle10.1016/S1357-2725(98)00098-3 Occurrence Handle1:CAS:528:DyaK1MXhvFehsbY%3D Occurrence Handle10216959
JJ Denie AP Verheugt (1958) ArticleTitleSupravalvular aortic stenosis. Circulation 18 902 Occurrence Handle1:STN:280:CyaD3MvpsVQ%3D Occurrence Handle13585567
NS Giddins JP Finley MA Nanton DL Roy (1989) ArticleTitleThe natural course of supravalvar aortic stenosis and peripheral pulmonary artery stenosis in Williams’s syndrome. Br Heart J 62 315–319 Occurrence Handle1:STN:280:By%2BD38jptFw%3D Occurrence Handle2803879
YM Kim SJ Yoo JY Choi et al. (1999) ArticleTitleNatural course of supravalvar aortic stenosis and peripheral pulmonary arterial stenosis in Williams syndrome. Cardiol Young 9 37–41 Occurrence Handle1:STN:280:DyaK1M3lsFWitw%3D%3D Occurrence Handle10323536
Y Kumada H Yasuda E Sasaki et al. (1998) ArticleTitleReoperation for diffuse supravalvular aortic with Williams syndrome—extended patch aortoplasty and extra-anatomic bypass from the ascending aorta to the descending aorta in a median sternotomy. Jpn J Thorac Cardiovasc Surg 46 1061–1064 Occurrence Handle1:STN:280:DyaK1M%2FmsVSmtA%3D%3D Occurrence Handle9847590
L Mencarelli (1930) ArticleTitleStenosi-soprvalvolare aortica adanells. Arch Ital Anat Path 1 829
CA Morris CO Leonard C Dilts SA Demsey (1990) ArticleTitleAdults with Williams syndrome. Am J Med Genet 6 102–107 Occurrence Handle1:STN:280:By%2BA2szgvFQ%3D Occurrence Handle2118770
CA Morris J Loker G Ensing AD Stock (1993) ArticleTitleSupravalvular aortic stenosis cosegregates with a familial 6; 7 translocation. Am J Med Genet 46 737–744 Occurrence Handle1:STN:280:ByyA2s%2FkvFA%3D Occurrence Handle8362925
TM Olson VV Michels NM Lindor et al. (1993) ArticleTitleAutosomal dominant supravalvular aortic stenosis: localization to chromosome 7. Hum Mol Genet 2 869–873 Occurrence Handle1:CAS:528:DyaK3sXlvFOjsbg%3D Occurrence Handle8364568
TA Peterson DB Todd JE Edwards (1965) ArticleTitleSupravalvular aortic stenosis. J Thorac Cardiovasc Surg 50 734–741 Occurrence Handle1:STN:280:CCmD2cvkvFI%3D Occurrence Handle5845458
BR Pober RV Lacro C Rice V Mandell RL Teele (1993) ArticleTitleRenal findings in 40 individuals with Williams syndrome. Am J Med Genet 46 271–274 Occurrence Handle1:STN:280:ByyB2MnhtVM%3D Occurrence Handle8488870
DJ Radford PG Pohlner (2000) ArticleTitleThe middle aortic syndrome: an important feature of Williams syndrome. Cardiol Young 10 597–602 Occurrence Handle1:STN:280:DC%2BD3M%2Fmtlyhsw%3D%3D Occurrence Handle11117392
GC Rastelli DC McGoon PA Ongley HT Mankin JW Kirklin (1966) ArticleTitleSurgical treatment of supravalvular aortic stenosis. J Thorac Cardiovasc Surg 51 873–882 Occurrence Handle1:STN:280:CCmC28ngsFI%3D Occurrence Handle5944111
A Wessel R Pankau D Kececioglu W Ruschewski JH Bursch (1994) ArticleTitleThree decades of follow-up of aortic and pulmonary vascular lesions in the Williams–Beuren syndrome. Am J Med Genet 52 297–301 Occurrence Handle1:STN:280:ByqC3cbls1I%3D Occurrence Handle7810560
JCP Williams MB Barratt-Boyes JB Lowe (1961) ArticleTitleSupravalvular aortic stenosis. Circulaton 24 1311–1318 Occurrence Handle1:STN:280:CC2C1MbgvFQ%3D
C Wren P Oslizlok C Bull (1990) ArticleTitleNatural history of supravalvular aortic stenosis and pulmonary artery stenosis. J Am Coll Cardiol 15 1625–1630 Occurrence Handle1:STN:280:By%2BB28vmtV0%3D Occurrence Handle2345244
WJ Wu AS Weiss (1999) ArticleTitleDeficient coacervation of two forms of human tropoelastin associated with supravalvular aortic stenosis. Eur J Biochem 266 308–314 Occurrence Handle10.1046/j.1432-1327.1999.00891.x Occurrence Handle1:CAS:528:DyaK1MXnsVantbo%3D Occurrence Handle10542079
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
English, R., Colan, S., Kanani, P. et al. Growth of the Aorta in Children with Williams Syndrome Does Surgery Make a Difference? . Pediatr Cardiol 24, 566–568 (2003). https://doi.org/10.1007/s00246-003-0302-0
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-003-0302-0