Pediatric Supravalvar Aortic Stenosis Treatment & Management

  • Author: Anita Krishnan, MD; more...
 
Updated: Mar 29, 2011
 

Approach Considerations

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

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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.[17]

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 supravalvar 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.

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
  • 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.[18]

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).

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Activity

Exercise recommendations for children with SVAS 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 (only recreational)
  • Coronary artery stenosis or abnormal anatomy - No competitive sports participation (only recreational)
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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.

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Consultations

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

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Long-term Monitoring

Follow-up care is recommended for all patients with SVAS, whether or not their condition has been surgically corrected. Infants and children with SVAS require follow-up at 3-month to 6-month intervals; older children require follow-up at 6-month to 12-month intervals. Rapid progression of SVAS may occur preoperatively.

Detailed cardiac examination and ECG should be performed at the follow-up visit. An echocardiogram may be indicated based on physical examination or ECG findings. Changes in intensity of the murmur may indicate progressive stenosis; the development of ST-segment or T-wave changes may signal coronary involvement.

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Contributor Information and Disclosures
Author

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.

Coauthor(s)

Gautam K Singh, MD, DCh, MRCP  Associate 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, DCh, MRCP 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.

Specialty Editor Board

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, American College of Cardiology, American Heart Association, Cardiac Electrophysiology Society, Heart Rhythm Society, Pediatric and Congenital Electrophysiology Society, and Society for Pediatric Research

Disclosure: Johnson & Johnson Consulting fee Consulting

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 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.

References
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  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. 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].

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  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. Jun 2007;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. Sep 1993;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. Jan 2002;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. Jun 1990;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. Sep 8 2004;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. Dec 1996;129(6):926-31. [Medline].

  13. 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].

  14. 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].

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

  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. [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].

  18. 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].

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Two-dimensional suprasternal echocardiographic image of supravalvar aortic stenosis.
Aortogram of a patient with supravalvar aortic stenosis and dilated sinus of Valsalva.
 
 
 
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