Forty-one years of surgical experience with congenital supravalvular aortic stenosis

Objective: Several techniques for symmetric reconstruction of the aortic ro ot in congenital supravalvular aortic stenosis have been developed, but it remains unclear whether these grove superior to patch enlargement of the no ncoronary sinus alone, We reviewed our experience with surgical treatment o f supravalvular aortic stenosis and investigated the impact of the surgical technique on long-term results, Methods and results: Seventy-five patients underwent operations to treat congenital supravalvular aortic stenosis at our institution between 1957 and 1998, Surgical procedures included patch e nlargement of the noncoronary sinus only (n = 34), inverted bifurcated patc h plasty (n = 35), and 3-sinus reconstruction of the aortic root (n = 6), T here were 7 early deaths. Among those who survived the operation, 100% were alive at 5 years, 96% were alive at 10 years, and 77% were alive at 20 yea rs, According to time-related analysis diffuse stenosis of the ascending ao rta proved a risk factor for both survival and reoperation (P < .01 for eac h), Patients,vith multiple-sinus reconstructions of the aortic root account ed for only 2 of the 14 reoperations and none of the late deaths (both P < .001). Residual gradients were lower after multiple-sinus reconstruction of the aortic root (median 10 mm Hg vs 20 mm Hg for patch enlargement of the noncoronary sinus only P = .008), as was the prevalence of moderate aortic regurgitation at follow-up (3% vs 22%, P = .05), Conclusions: Results of op erations for supravalvular aortic stenosis improved greatly after the intro duction of more symmetric reconstructions of the aortic root. Multiple-sinu s reconstructions (inverted bifurcated patch plasty and 3-sinus reconstruct ion) resulted in superior hemodynamics and were associated with reductions in both mortality rate and need for reoperation.