Medscape is available in 5 Language Editions – Choose your Edition here.


Pediatric Supravalvar Aortic Stenosis Treatment & Management

  • Author: Anita Krishnan, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
Updated: Aug 26, 2015

Approach Considerations

Surgery is the primary treatment for SVAS. Activity restrictions may be indicated, depending on the severity of the disorder.


Surgical Intervention

Children and adolescents with catheter peak-to-peak (or Doppler mean) gradient of 50 mm Hg or more should have surgical intervention. The choice of procedures in these patients is similar to that indicated for valvar aortic stenosis.[19]

Children and adolescents with catheter peak-to-peak (or Doppler mean) gradient of 30-50 mm Hg may be considered for surgical intervention if they are symptomatic, with angina, syncope, or dyspnea on exertion (class I). Asymptomatic patients who have developed ST/T-wave changes over the left precordium on ECG at rest or with exercise should also be considered for surgical intervention (class I). Aortic valve involvement and lower supravalve gradients may also warrant surgical intervention.  

Surgical resection of the supravalve obstruction and patch aortoplasty and multiple-sinus reconstructions (inverted bifurcated patch plasty and 3-sinus reconstruction) are the procedures of choice for the fibrous diaphragm and hourglass deformities.[20]

Associated coronary artery involvement is addressed with the following measures, which are performed at the same time as aortoplasty:

  • Patch aortoplasty encompassing the left main ostium for circumferential narrowing of the left main ostium
  • Excision of the fused leaflet from the aortic wall for ostial obstruction caused by a fusion of the aortic cusp to the supravalvar ridge [21]
  • Bypass grafting for diffuse narrowing of the left main coronary artery

In patients who have SVAS with diffuse narrowing, the ascending aorta and the arch of the aorta can be reconstructed using an aortic allograft or a pulmonary autograft.

Surgical treatment of associated abnormalities of aortic valve and aortic arch vessels should be undertaken at the same time to optimize the overall surgical outcome.[22]

Standard postoperative care and precautions for pediatric cardiac patients are also required for patients with SVAS. Postoperative complications include aortic insufficiency (in 25% of patients).



Exercise recommendations for children with SVAS and no coronary artery involvement are as follows:

  • Mild stenosis (< 20 mm Hg), normal ECG findings, no symptoms - Full sports participation
  • Moderate stenosis (21-49 mm Hg), mild left ventricular hypertrophy (LVH), no symptoms - Low static or moderately dynamic sports participation
  • Severe stenosis (>50 mm Hg) or moderate degree of stenosis with symptoms - No competitive sports participation (at most recreational)
  • Coronary artery stenosis or abnormal anatomy - No competitive sports participation (at most recreational and dependent on the level of obstruction)

Additional Management

Williams syndrome, which is found in many children with SVAS, may be associated with infantile hypercalcemia with some risk of nephrocalcinosis, osteosclerosis with progressive joint limitation and abnormal gait, and neurodevelopmental delay. These children require multidisciplinary support. Use a coordinated management approach. They are also at risk of higher mortality than the normal population is, because of cardiac and noncardiac causes.

No published reports have documented the outcome of pregnancy in postoperative patients with SVAS. Address pregnancy on an individual basis, taking into account the type of lesion and surgical procedure performed, the presence of residual lesion, and the associated cardiac and noncardiac conditions and syndromes.



Consult a pediatric cardiologist and a pediatric cardiac surgeon, as needed.


Long-term Monitoring

Follow-up care is recommended for all patients with SVAS, whether or not their condition has been surgically corrected, and the frequency is based on the severity of the obstruction and whether there is coronary artery involvement. Rapid progression of SVAS may occur preoperatively.

Detailed cardiac examination and echocardiography should be performed at the follow-up visit. Changes in intensity of the murmur may indicate progressive stenosis; the development of ST-segment or T-wave changes may signal coronary involvement.

Contributor Information and Disclosures

Anita Krishnan, MD Assistant Professor of Pediatrics, George Washington University School of Medicine; Attending Physician, Division of Cardiology, Children’s National Medical Center

Disclosure: Nothing to disclose.


Gautam K Singh, MD, , MRCP Professor of Pediatrics, Division of Cardiology, Director of Noninvasive Imaging Research, Co-Director of Echocardiography Laboratory, Washington University in St Louis School of Medicine; Attending Faculty, Department of Pediatrics, Division of Cardiology, St Louis Children's Hospital

Gautam K Singh, MD, , MRCP is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Echocardiography, Royal College of Physicians

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatic Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Charles I Berul, MD Professor of Pediatrics and Integrative Systems Biology, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center

Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, Heart Rhythm Society, Cardiac Electrophysiology Society, Pediatric and Congenital Electrophysiology Society, American College of Cardiology, American Heart Association, Society for Pediatric Research

Disclosure: Received grant/research funds from Medtronic for consulting.

  1. Micale L, Turturo MG, Fusco C, et al. Identification and characterization of seven novel mutations of elastin gene in a cohort of patients affected by supravalvular aortic stenosis. Eur J Hum Genet. 2009 Oct 21. [Medline].

  2. Peterson TA, Todd DB, Edwards JE. Supravalvular aortic stenosis. J Thorac Cardiovasc Surg. 1965 Nov. 50(5):734-41. [Medline].

  3. Edwards JE. Pathology of left ventricular outflow tract obstruction. Circulation. 1965. 31:586-99.

  4. Thistlethwaite PA, Madani MM, Kriett JM, Milhoan K, Jamieson SW. Surgical management of congenital obstruction of the left main coronary artery with supravalvular aortic stenosis. J Thorac Cardiovasc Surg. 2000 Dec. 120(6):1040-6. [Medline].

  5. French JW, Guntheroth WG. An explanation of asymmetric upper extremity blood pressures in supravalvular aortic stenosis: the Coanda effect. Circulation. 1970 Jul. 42(1):31-6. [Medline].

  6. Gersony WM, Hayes CJ, Driscoll DJ, et al. Bacterial endocarditis in patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect. Circulation. 1993 Feb. 87(2 Suppl):I121-6. [Medline].

  7. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. J Am Dent Assoc. 2007 Jun. 138(6):739-45, 747-60. [Medline]. [Full Text].

  8. Ewart AK, Morris CA, Atkinson D, et al. Hemizygosity at the elastin locus in a developmental disorder, Williams syndrome. Nat Genet. 1993 Sep. 5(1):11-6. [Medline].

  9. Brown JW, Ruzmetov M, Vijay P, et al. Surgical repair of congenital supravalvular aortic stenosis in children. Eur J Cardiothorac Surg. 2002 Jan. 21(1):50-6. [Medline].

  10. Wren C, Oslizlok P, Bull C. Natural history of supravalvular aortic stenosis and pulmonary artery stenosis. J Am Coll Cardiol. 1990 Jun. 15(7):1625-30. [Medline].

  11. Wessel TR, Arant CB, Olson MB, et al. Relationship of physical fitness vs body mass index with coronary artery disease and cardiovascular events in women. JAMA. 2004 Sep 8. 292(10):1179-87. [Medline].

  12. Bird LM, Billman GF, Lacro RV, et al. Sudden death in Williams syndrome: report of ten cases. J Pediatr. 1996 Dec. 129(6):926-31. [Medline].

  13. Pieles GE, Ofoe V, Morgan GJ. Severe Left Main Coronary Artery Stenosis with Abnormal Branching Pattern in a Patient with Mild Supravalvar Aortic Stenosis and Williams-Beuren Syndrome. Congenit Heart Dis. 2013 May 22. [Medline].

  14. Martin R, Lairez O, Boudou N, Méjean S, Lhermusier T, Dumonteil N, et al. Relation between left ventricular outflow tract obstruction and left ventricular shape in patients with hypertrophic cardiomyopathy: A cardiac magnetic resonance imaging study. Arch Cardiovasc Dis. 2013 Aug-Sep. 106(8-9):440-7. [Medline].

  15. Jureidini SB, Marino CJ, Singh GK, et al. Main coronary artery and coronary ostial stenosis in children: detection by transthoracic color flow and pulsed Doppler echocardiography. J Am Soc Echocardiogr. 2000 Apr. 13(4):255-63. [Medline].

  16. Tani LY, Minich LL, Pagotto LT, Shaddy RE. Usefulness of doppler echocardiography to determine the timing of surgery for supravalvar aortic stenosis. Am J Cardiol. 2000 Jul 1. 86(1):114-6. [Medline].

  17. Sugiyama H, Veldtman GR, Norgard G, Lee KJ, Chaturvedi R, Benson LN. Bladed balloon angioplasty for peripheral pulmonary artery stenosis. Catheter Cardiovasc Interv. 2004 May. 62(1):71-7. [Medline].

  18. Dridi SM, Foucault Bertaud A, Igondjo Tchen S, et al. Vascular wall remodeling in patients with supravalvular aortic stenosis and Williams Beuren syndrome. J Vasc Res. 2005 May-Jun. 42(3):190-201. [Medline].

  19. [Guideline] Bonow RO, Carabello BA, Kanu C, et al. ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (writing committee to revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease): developed in collaboration with the Society of Cardiovascular Anesthesiologists: endorsed by the Society for Cardiovascular Angiography and Interventions and the Society of Thoracic Surgeons. Circulation. 2006 Aug 1. 114(5):e84-231. [Medline].

  20. Kavarana MN, Riley M, Sood V, Ohye RG, Devaney EJ, Bove EL, et al. Extended single-patch repair of supravalvar aortic stenosis: a simple and effective technique. Ann Thorac Surg. 2012 Apr. 93(4):1274-8; discussion 1278-9. [Medline].

  21. Ayoub C, Ranasinghe I, Yiannikas J. Successful negative inotropic treatment of acute left ventricular outflow tract obstruction by elongated mitral valve leaflet. J Clin Ultrasound. 2013 Sep 20. [Medline].

  22. McElhinney DB, Petrossian E, Tworetzky W, Silverman NH, Hanley FL. Issues and outcomes in the management of supravalvar aortic stenosis. Ann Thorac Surg. 2000 Feb. 69(2):562-7. [Medline].

Two-dimensional suprasternal echocardiographic image of supravalvar aortic stenosis.
Aortogram of a patient with supravalvar aortic stenosis and dilated sinus of Valsalva.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.