eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Aortic Stenosis, Supravalvar: Treatment & Medication

Author: Gautam K Singh, MD, DCh, MRCP, FACC, Associate Professor of Pediatrics, Division of Cardiology, Director of Noninvasive Imaging Research, Co-director of Echocardiography Laboratory, Washington University School of Medicine; Attending Faculty, Department of Pediatrics, Division of Cardiology, St Louis Children's Hospital
Contributor Information and Disclosures

Updated: Oct 27, 2009

Treatment

Medical Care

  • Surgery is the primary treatment for supravalvar aortic stenosis (SVAS).

Surgical Care

  • Children and adolescents with catheter peak-to-peak (or Doppler mean) gradient of 50 mm Hg or more should have surgical intervention similar to what is indicated for valvular aortic stenosis.13 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).
  • Surgical resection of the supravalvular 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. In patients with associated coronary artery involvement, patch aortoplasty encompassing the left main ostium for circumferential narrowing of 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 supravalvular ridge; and bypass grafting for diffuse narrowing of the left main coronary artery should be performed at the same time as aortoplasty. In patients who have supravalvular aortic stenosis with diffuse narrowing, the ascending aorta and the arch of the aorta can be reconstructed using an aortic allograft or a pulmonary autograft.
  • Overall early and late mortality is 1–2%.9 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.14
  • Standard postoperative care and precautions for pediatric cardiac patients are also required for patients with supravalvular aortic stenosis.

Consultations

  • Cardiologist
  • Cardiothoracic surgeon
  • Geneticist

Diet

  • No special diet is required.

Activity

Recommended physical activities are similar to those for aortic valve stenosis because they are progressive diseases of similar nature.

Patients with coronary artery stenosis or abnormal anatomy should be restricted to participate in physical activities only at a recreational level. Exercise recommendations for children with supravalvular aortic stenosis are as follows:

  • Mild degree of stenosis (<20 mm Hg), normal ECG findings, no symptoms - Full sports participation
  • Moderate degree of stenosis (21-49 mm Hg), mild left ventricular hypertrophy (LVH), no symptoms - Low static or moderately dynamic sports participation
  • Severe degree of stenosis (>50 mm Hg) or moderate degree of stenosis with symptoms - No competitive sports participation (only recreational)

Medication

More on Aortic Stenosis, Supravalvar

Overview: Aortic Stenosis, Supravalvar
Differential Diagnoses & Workup: Aortic Stenosis, Supravalvar
Treatment & Medication: Aortic Stenosis, Supravalvar
Follow-up: Aortic Stenosis, Supravalvar
Multimedia: Aortic Stenosis, Supravalvar
References
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References

  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. Oct 21 2009;[Medline].

  2. Peterson TA, Todd DB, Edwards JE. Supravalvular aortic stenosis. J Thorac Cardiovasc Surg. Nov 1965;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. Dec 2000;120(6):1040-6. [Medline].

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

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

  7. 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. Sep 8 2004;292(10):1179-87. [Medline].

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

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

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

  11. 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. Apr 2000;13(4):255-63. [Medline].

  12. 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. Jul 1 2000;86(1):114-6. [Medline].

  13. [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. Aug 1 2006;114(5):e84-231. [Medline].

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

  15. 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. Jun 2007;138(6):739-45, 747-60. [Medline]. [Full Text].

  16. 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. May-Jun 2005;42(3):190-201. [Medline].

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

  18. Keane JF, Fellows KE, LaFarge CG, et al. The surgical management of discrete and diffuse supravalvar aortic stenosis. Circulation. Jul 1976;54(1):112-7. [Medline].

  19. Kirklin JW, Barratt-Boyes BG. Congenital aortic stenosis. In: Cardiac Surgery. Vol 2. 2nd ed. New York, NY: Churchill Livingstone Inc; 1993:1195-1238.

  20. Latson LA. Aortic stenosis: Valvular, supravalvular, fibromuscular subvalvular. In: Garson A, Bricker JT, McNamara DG, eds. The Science and Practice of Pediatric Cardiology. Philadelphia, Pa: Lea & Febiger; 1990:1334-52.

  21. Rodriguez-Revenga L, Badenas C, Carrio A, Mila M. Elastin mutation screening in a group of patients affected by vascular abnormalities. Pediatr Cardiol. Nov-Dec 2005;26(6):827-31. [Medline].

  22. Sugiyama H, Veldtman GR, Norgard G, et al. Bladed balloon angioplasty for peripheral pulmonary artery stenosis. Catheter Cardiovasc Interv. May 2004;62(1):71-7. [Medline].

  23. 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. Jul 1 2000;86(1):114-6. [Medline].

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Further Reading

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Keywords

supravalvular aortic stenosis, SVAS, left ventricular outflow tract obstruction, LVOT obstruction, Williams syndrome, Williams-Beuren syndrome, atherosclerosis, myocardial ischemia, Coanda effect, diffuse peripheral pulmonary artery stenosis

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Contributor Information and Disclosures

Author

Gautam K Singh, MD, DCh, MRCP, FACC, Associate Professor of Pediatrics, Division of Cardiology, Director of Noninvasive Imaging Research, Co-director of Echocardiography Laboratory, Washington University School of Medicine; Attending Faculty, Department of Pediatrics, Division of Cardiology, St Louis Children's Hospital
Gautam K Singh, MD, DCh, MRCP, FACC is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Echocardiography, and Royal College of Physicians
Disclosure: Nothing to disclose.

Medical Editor

Charles I Berul, MD, Professor of Pediatrics, 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, American College of Cardiology, American Heart Association, Cardiac Electrophysiology Society, Heart Rhythm Society, Pediatric and Congential Electrophysiology Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

John W Moore, MD, MPH, Professor of Clinical Pediatrics, Section of Pediatric 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, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

 
 
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