Supravalvar aortic stenosis: current surgical approaches and outcomes

Abstract

Supravalvar aortic stenosis (SVAS) is a congenital anomaly characterized by a discrete or diffuse narrowing of the ascending aorta. It may also be associated with right-ventricular outflow tract obstruction, aortic valve pathology and coronary ostial stenosis. While present in both familial and sporadic forms, it demonstrates a strong association with William–Beuren syndrome, both being anomalies associated with defects in the elastin gene. In this article, the authors have discussed the etiology, morphology, clinical presentation and genetic basis of SVAS. Various surgical approaches, both conventional and recent, have been discussed and demonstrated with the aid of diagrams. Single-, two- and three-sinus methods have been presented, along with a comparative analysis of early results, associated procedures, late mortality and reoperation. In conclusion, the authors have described their institutional experience of more than 40 years in the surgical management of SVAS.

Keywords::

• congenital cardiac surgery
• congenital heart disease
• supravalvar aortic stenosis

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.