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

Aortic Stenosis, Supravalvar: Follow-up

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

Follow-up

Further Inpatient Care

  • Follow-up care is recommended for all patients, whether or not they have had surgical correction of supravalvar aortic stenosis (SVAS). Infants and children with supravalvular aortic stenosis 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 supravalvular aortic stenosis may occur preoperatively.

Further Outpatient Care

  • Further outpatient care is required for preoperative evaluation and postoperative follow-up care.

Transfer

  • Transfer may be required for further surgical intervention.

Deterrence/Prevention

  • Advise patients to avoid strenuous activities and competitive sports (see Activity).

Complications

  • Preoperative
    • Progressive coronary osteal stenosis and atherosclerosis
    • Infective endocarditis
    • Sudden death
  • Postoperative
    • Aortic insufficiency in 25% of patients
    • Higher mortality rate in patients with diffuse supravalvular stenosis than in those with localized supravalvular aortic stenosis

Prognosis

  • In one series, the actuarial survival rate following operative repair of supravalvular aortic stenosis was approximately 85% at 15 years. After surgery, most patients remain in class I of the New York Heart Association's (NYHA) functional classification; most do not require reoperation.
  • In other large series follow-up (ranging from 6 mo to 30 y, medium 9.4 y), 73% patients were in NYHA functional class I, and 27% were in NYHA functional class II.9 Overall survival including operative mortality was 98% at 10 years and 97% at 20 years and at 30 years.
  • The survival after operative repair of supravalvular stenosis is as much as 98% at 10 years and 97% at both 20 and 30 years, with most in New York Heart Association functional class I.9 Prognosis is influenced by the presence of genetic disorders, coronary artery lesions, and associated obstructive lesions of pulmonary arteries.

Patient Education

  • Restrictions
    • Activity: Preoperative recommendations for physical activities should be followed as mentioned in Activity. Physical activity restrictions are not required postoperatively if no residual lesion is present and the pressure gradient is less than 20 mm Hg across the left ventricular (LV) outflow tract (LVOT), which is similar to the preoperative recommendation.
    • Lifestyle implications: In general, persons with supravalvular aortic stenosis should have risk stratification for coronary artery disease early in adult life because supravalvular aortic stenosis may predispose the coronary artery to premature atherosclerotic changes.
  • Counseling: Council patients and their families regarding the need for long-term follow-up care.

Miscellaneous

Medicolegal Pitfalls

  • Failure to inform patients and their families about exercise restrictions
  • Failure to inform patients and their families about antibiotic prophylaxis for bacterial endocarditis
  • Failure to recognize development of cardiac ischemia, arrhythmias, potential for sudden death particularly during administration of anesthesia, or need for intervention

Special Concerns

  • Many children with supravalvar aortic stenosis (SVAS) have Williams syndrome, which 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 because of both cardiac and noncardiac causes.
  • Most children are asymptomatic from a cardiovascular standpoint. A timely diagnosis of supravalvular aortic stenosis may not be made, and patients and their families may comply poorly with follow-up care.
  • No published reports have documented the outcome of pregnancy in postoperative patients with supravalvular aortic stenosis. 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.
 


More on Aortic Stenosis, Supravalvar

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Treatment & Medication: Aortic Stenosis, Supravalvar
Follow-up: Aortic Stenosis, Supravalvar
Multimedia: Aortic Stenosis, Supravalvar
References
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References

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  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].

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  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].

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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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