Supravalvular aortic stenosis: Long-term results of surgical treatment

J. A M Van Son, G. K. Danielson, F. J. Puga, Hartzell V Schaff, A. Rastogi, W. D. Edwards, R. H. Feldt, J. J. Amato, J. F. Stark, L. C B De Souza

Research output: Contribution to journalArticle

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Abstract

To determine long-term outcome after operation for supravalvular aortic stenosis, we reviewed the case histories of 80 patients who had repair of the localized form (group A) (n = 67) or diffuse form (group B) (n = 13) from 1956 to 1992, including 31 patients with the Williams-Beuren syndrome. Ages ranged from 7 months to 54 years (mean = 12.6 years). Forty-six patients had one or more associated cardiovascular anomalies; the most common was aortic valve stenosis (33.8%). Eighteen patients had 22 previous cardiovascular operations, and 28 patients had one or more additional anomalies repaired during their initial procedure at our institution. In group A, the aortic root was enlarged with a teardrop-shaped patch (n = 61) or a pantaloon- shaped patch (n = 6). In group B, patch enlargement of the aorta was confined to the root (n = 4) or extended into the ascending aorta or aortic arch (n = 7); one patient had a graft placed between the ascending and descending thoracic aorta and one patient had a left ventricular-aortic conduit. There were no deaths in group A; two patients in group B in whom patch enlargement was confined to the aortic root died during the operation (2.5%). Follow-up extended to 33.4 years (mean = 14.2 years); there were five late deaths in group A and one in group B. Survival excluding operative mortality was 94% at 10 years and 91% at 20 years. All patients were in functional class I or II. There was no significant difference between patients with a teardrop-shaped or pantaloon-shaped patch in terms of late gradient, survival, or aortic insufficiency. By Cox multivariate model, the only independent predictor of late death for all patients was associated aortic valve disease (p = 0.02), which was also a risk factor for late reoperation (p = 0.02). In group B, overall survival was better in patients who received an extended patch versus aortic root patch only (p = 0.02). We reached the following conclusions: (1) Associated aortic valve disease was strongly correlated with late death and need for reoperation. (2) Both the teardrop-shaped and pantaloon-shaped patch techniques provide excellent long-term relief of localized supravalvular gradients and preservation of aortic valve competence. (3) In diffuse supravalvular aortic stenosis, aortic enlargement should be extended into the ascending aorta or beyond as required to relieve the gradient; some patients may require a graft or conduit. (4) Early surgical intervention before development of coronary artery disease or ventricular dysfunction may improve long-term survival.

Original languageEnglish (US)
Pages (from-to)103-115
Number of pages13
JournalJournal of Thoracic and Cardiovascular Surgery
Volume107
Issue number1
StatePublished - 1994

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Supravalvular Aortic Stenosis
Therapeutics
Thoracic Aorta
Aorta
Survival
Aortic Valve
Reoperation
Williams Syndrome
Transplants
Ventricular Dysfunction
Aortic Diseases
Aortic Valve Stenosis

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery

Cite this

Van Son, J. A. M., Danielson, G. K., Puga, F. J., Schaff, H. V., Rastogi, A., Edwards, W. D., ... De Souza, L. C. B. (1994). Supravalvular aortic stenosis: Long-term results of surgical treatment. Journal of Thoracic and Cardiovascular Surgery, 107(1), 103-115.

Supravalvular aortic stenosis : Long-term results of surgical treatment. / Van Son, J. A M; Danielson, G. K.; Puga, F. J.; Schaff, Hartzell V; Rastogi, A.; Edwards, W. D.; Feldt, R. H.; Amato, J. J.; Stark, J. F.; De Souza, L. C B.

In: Journal of Thoracic and Cardiovascular Surgery, Vol. 107, No. 1, 1994, p. 103-115.

Research output: Contribution to journalArticle

Van Son, JAM, Danielson, GK, Puga, FJ, Schaff, HV, Rastogi, A, Edwards, WD, Feldt, RH, Amato, JJ, Stark, JF & De Souza, LCB 1994, 'Supravalvular aortic stenosis: Long-term results of surgical treatment', Journal of Thoracic and Cardiovascular Surgery, vol. 107, no. 1, pp. 103-115.
Van Son JAM, Danielson GK, Puga FJ, Schaff HV, Rastogi A, Edwards WD et al. Supravalvular aortic stenosis: Long-term results of surgical treatment. Journal of Thoracic and Cardiovascular Surgery. 1994;107(1):103-115.
Van Son, J. A M ; Danielson, G. K. ; Puga, F. J. ; Schaff, Hartzell V ; Rastogi, A. ; Edwards, W. D. ; Feldt, R. H. ; Amato, J. J. ; Stark, J. F. ; De Souza, L. C B. / Supravalvular aortic stenosis : Long-term results of surgical treatment. In: Journal of Thoracic and Cardiovascular Surgery. 1994 ; Vol. 107, No. 1. pp. 103-115.
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N2 - To determine long-term outcome after operation for supravalvular aortic stenosis, we reviewed the case histories of 80 patients who had repair of the localized form (group A) (n = 67) or diffuse form (group B) (n = 13) from 1956 to 1992, including 31 patients with the Williams-Beuren syndrome. Ages ranged from 7 months to 54 years (mean = 12.6 years). Forty-six patients had one or more associated cardiovascular anomalies; the most common was aortic valve stenosis (33.8%). Eighteen patients had 22 previous cardiovascular operations, and 28 patients had one or more additional anomalies repaired during their initial procedure at our institution. In group A, the aortic root was enlarged with a teardrop-shaped patch (n = 61) or a pantaloon- shaped patch (n = 6). In group B, patch enlargement of the aorta was confined to the root (n = 4) or extended into the ascending aorta or aortic arch (n = 7); one patient had a graft placed between the ascending and descending thoracic aorta and one patient had a left ventricular-aortic conduit. There were no deaths in group A; two patients in group B in whom patch enlargement was confined to the aortic root died during the operation (2.5%). Follow-up extended to 33.4 years (mean = 14.2 years); there were five late deaths in group A and one in group B. Survival excluding operative mortality was 94% at 10 years and 91% at 20 years. All patients were in functional class I or II. There was no significant difference between patients with a teardrop-shaped or pantaloon-shaped patch in terms of late gradient, survival, or aortic insufficiency. By Cox multivariate model, the only independent predictor of late death for all patients was associated aortic valve disease (p = 0.02), which was also a risk factor for late reoperation (p = 0.02). In group B, overall survival was better in patients who received an extended patch versus aortic root patch only (p = 0.02). We reached the following conclusions: (1) Associated aortic valve disease was strongly correlated with late death and need for reoperation. (2) Both the teardrop-shaped and pantaloon-shaped patch techniques provide excellent long-term relief of localized supravalvular gradients and preservation of aortic valve competence. (3) In diffuse supravalvular aortic stenosis, aortic enlargement should be extended into the ascending aorta or beyond as required to relieve the gradient; some patients may require a graft or conduit. (4) Early surgical intervention before development of coronary artery disease or ventricular dysfunction may improve long-term survival.

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