Objective: Supravalvar aortic stenosis is an uncommon form of left ventricular outflow tract obstruction, with a typical localized form of the disease, and a less frequent diffuse form involving the ascending aorta and beyond.

Methods: A retrospective review of 41 patients undergoing surgery between 1980 and 2007 at the Royal Children’s Hospital, Melbourne.

Results: There were 41 patients (27 males, 14 females) with a median age at operation of 50 months. William’s syndrome was diagnosed in 63% of patients. There were 33 (80%) with localised stenosis. Surgery provided effective relief, reducing the gradient from 84 mm Hg to 15mm Hg post-operation (p<0.001). The long term peak gradient remained low, and at 5, 10, and 20 years, the mean peak gradient was 20mm Hg, 28mm Hg and 23 mm Hg respectively. Three different types of repair were used. There were 6 deaths (2 early and 4 late), 4 reoperations, and the survival at 5, 10 and 20 years was 86% (95% CI: 70-94%), 86% (95% CI: 70-94%), and 79% (95% CI: 55-91%). Diffuse stenosis, residual post-operative gradient and younger age at presentation were univariate risk factors for mortality. The residual gradient was a risk factor for re-operation. There was no difference between the surgical techniques for mortality, reoperation or gradient.

Conclusion: Surgical management results in immediate and effective relief of the stenosis and provides a stable long term result. However, the diffuse type of stenosis is associated with a poorer outcome. Residual gradient confers a significant risk of death and reoperation.