Modified Norwood procedure for hypoplastic left heart syndrome

Since June 1994, we have used a modification of the Norwood technique in 13 patients presenting with the hypoplastic left heart syndrome or similar variants. This technique involves coarctation repair, arch reconstruction, and creation of a neo-ascending aorta using autologous great vessel tissue only. Pulmonary blood flow is provided by a central shunt of 3.0- to 4.0-mm Gore-Tex. The mean age and weight at operation were 15 days (range, 1 to 77 days) and 3.2 kg (range, 1.7 to 4.6 kg), respectively. The mean circulatory arrest time was 32 minutes (range, 25 to 50 minutes). There was one operative death, and there have been no late deaths. Seven patients have gone on to conversion to a bidirectional cavopulmonary shunt at a mean age of 6 months. There have been no cases of recurrent coarctation, arch obstruction, or left pulmonary artery stenosis. Significant coronary insufficiency requiring revision of the ascending aortic reconstruction has developed in 2 patients. We believe this approach offers the advantage of using the patient's own native tissue for all great vessel reconstruction. This technique may also allow 10 to 15 minutes less of circulatory arrest time. The theoretic benefits include improved growth of repaired structures and avoidance of homograft or prosthetic material. The long-term results remain to be elucidated.