Congenital Supravalvular Aortic Stenosis: Defining Surgical and Nonsurgical Outcomes

Edward J. Hickey, Gordon Jung, William G. Williams, Cedric Manlhiot, Glen S. Van Arsdell, Christopher A. Caldarone, John Coles, Brian W. McCrindle

Research output: Contribution to journalArticle

Abstract

Background: Supravalvular aortic stenosis is a rare stenotic lesion of the left ventricular outflow tract (LVOT). We characterized the natural history of the disease and the effect of surgical intervention. Methods: Ninety-five children diagnosed with supravalvular aortic stenosis between 1976 and 2006 were studied. Procedural and repeated echocardiography reports were analyzed. Results: Stenosis morphology (localized, 82%; diffuse, 18%) was independent of Williams syndrome (n = 59, 62%). The risk of open operation (n = 47) was 46% ± 6% at 10 years. Increased risk of operation was associated with higher baseline LVOT peak gradients (p < 0.001), smaller minimum LVOT z scores (p < 0.01; thresholds > 50 mm Hg and < -3, respectively), and the absence of Williams syndrome (p = 0.01). Patients who did not undergo operations had gradually reducing LVOT gradients and enlarging ascending aorta z scores over time. Persistently small minimum LVOT z scores and higher gradients were associated with children who required an operation. Operation resulted in persistent relief of LVOT obstruction and accelerated increases in ascending aorta dimensions. Overall survival was 94% ± 3% and 85% ± 7% at 10 and 15 years and was similar for surgical and nonsurgical groups. No independent risk factors for death were identified on univariate or multivariable analysis. Conclusions: Many children-particularly those with Williams syndrome-show regression of stenosis without intervention. Children who undergo operation have high LVOT gradients and smaller LVOT z scores that do not improve over time. Surgical intervention alters the natural history: LVOT obstruction is relieved and does not recur, and ascending aortic dimensions progressively enlarge towards normal values.

Original languageEnglish (US)
Pages (from-to)1919-1927
Number of pages9
JournalAnnals of Thoracic Surgery
Volume86
Issue number6
DOIs
StatePublished - Dec 1 2008
Externally publishedYes

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Supravalvular Aortic Stenosis
Williams Syndrome
Ventricular Outflow Obstruction
Aorta
Pathologic Constriction
Natural History
Echocardiography
Reference Values
Survival

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Pulmonary and Respiratory Medicine

Cite this

Hickey, E. J., Jung, G., Williams, W. G., Manlhiot, C., Van Arsdell, G. S., Caldarone, C. A., ... McCrindle, B. W. (2008). Congenital Supravalvular Aortic Stenosis: Defining Surgical and Nonsurgical Outcomes. Annals of Thoracic Surgery, 86(6), 1919-1927. https://doi.org/10.1016/j.athoracsur.2008.08.031

Congenital Supravalvular Aortic Stenosis : Defining Surgical and Nonsurgical Outcomes. / Hickey, Edward J.; Jung, Gordon; Williams, William G.; Manlhiot, Cedric; Van Arsdell, Glen S.; Caldarone, Christopher A.; Coles, John; McCrindle, Brian W.

In: Annals of Thoracic Surgery, Vol. 86, No. 6, 01.12.2008, p. 1919-1927.

Research output: Contribution to journalArticle

Hickey, EJ, Jung, G, Williams, WG, Manlhiot, C, Van Arsdell, GS, Caldarone, CA, Coles, J & McCrindle, BW 2008, 'Congenital Supravalvular Aortic Stenosis: Defining Surgical and Nonsurgical Outcomes', Annals of Thoracic Surgery, vol. 86, no. 6, pp. 1919-1927. https://doi.org/10.1016/j.athoracsur.2008.08.031
Hickey, Edward J. ; Jung, Gordon ; Williams, William G. ; Manlhiot, Cedric ; Van Arsdell, Glen S. ; Caldarone, Christopher A. ; Coles, John ; McCrindle, Brian W. / Congenital Supravalvular Aortic Stenosis : Defining Surgical and Nonsurgical Outcomes. In: Annals of Thoracic Surgery. 2008 ; Vol. 86, No. 6. pp. 1919-1927.
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abstract = "Background: Supravalvular aortic stenosis is a rare stenotic lesion of the left ventricular outflow tract (LVOT). We characterized the natural history of the disease and the effect of surgical intervention. Methods: Ninety-five children diagnosed with supravalvular aortic stenosis between 1976 and 2006 were studied. Procedural and repeated echocardiography reports were analyzed. Results: Stenosis morphology (localized, 82{\%}; diffuse, 18{\%}) was independent of Williams syndrome (n = 59, 62{\%}). The risk of open operation (n = 47) was 46{\%} ± 6{\%} at 10 years. Increased risk of operation was associated with higher baseline LVOT peak gradients (p < 0.001), smaller minimum LVOT z scores (p < 0.01; thresholds > 50 mm Hg and < -3, respectively), and the absence of Williams syndrome (p = 0.01). Patients who did not undergo operations had gradually reducing LVOT gradients and enlarging ascending aorta z scores over time. Persistently small minimum LVOT z scores and higher gradients were associated with children who required an operation. Operation resulted in persistent relief of LVOT obstruction and accelerated increases in ascending aorta dimensions. Overall survival was 94{\%} ± 3{\%} and 85{\%} ± 7{\%} at 10 and 15 years and was similar for surgical and nonsurgical groups. No independent risk factors for death were identified on univariate or multivariable analysis. Conclusions: Many children-particularly those with Williams syndrome-show regression of stenosis without intervention. Children who undergo operation have high LVOT gradients and smaller LVOT z scores that do not improve over time. Surgical intervention alters the natural history: LVOT obstruction is relieved and does not recur, and ascending aortic dimensions progressively enlarge towards normal values.",
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AU - Van Arsdell, Glen S.

AU - Caldarone, Christopher A.

AU - Coles, John

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