Abstract

We describe a hybrid approach to the treatment of aortic obstruction after stage 1 palliation (S1P) of hypoplastic left heart syndrome. Recurrent aortic obstruction is a common problem after S1P of hypoplastic left heart syndrome. Even mild aortic obstruction is poorly tolerated so early and definitive therapy is desirable. Although stent implantation is an effective treatment for aortic obstruction in older children and adults, technical issues due to small vessels and concerns regarding future potential for expansion have generally precluded the use of stents in this setting. Six patients underwent hybrid aortic reconstruction (HAR) in the operating room or catheterization laboratory, with the interventional cardiologist and cardiac surgeon working in cooperation. Patients had a mean weight of 5.8 kg (2.9-7.7) and a mean age of 5.6 months (0.5-12.9) at the time of HAR. Five patients had undergone prior balloon angioplasty at a mean age of 2.8 months (2.1-3.5), and five had moderately depressed single ventricular function prior to HAR. The balloons used had a diameter of 7-10 mm and introducer sheath size ranged from 6 to 10 F. There were no immediate or late procedure related complications. Stent redilation was performed in 5 patients for relief of recurrent obstruction or to keep pace with somatic growth. At a median follow up of 4.8 years (0.2-7.9), there were 3 patients progressing well after Fontan palliation and 3 deaths. HAR allows for placement of stents that can ultimately reach adult size in small infants who have recurrent aortic obstruction after balloon angioplasty following S1P. Advantages include freedom from delivery sheath constraints when determining stent type/size, facilitation of precise stent position, and avoidance of vascular damage or hemodynamic compromise during the procedure. Longer follow-up and larger experience are required to determine if this therapy will provide a long-term solution to this difficult problem.

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