The modified Norwood procedure for hypoplastic left heart syndrome: Early to intermediate results of 120 patients with particular reference to aorticarch repair

Background: Classic first-stage Norwood repair of hypoplastic left heart sy ndrome uses a homograft patch enlargement to obtain an unobstructed aorta a nd coronary arteries, Because of possible disadvantages of the homograft, s uch as lack of growth, degeneration and calcification, and availability, we have tried to repair the aorta without patch supplementation. Methods: Bet ween February 1993 and September 1997, 120 patients, aged birth to 47 days (median 4 days) and weighing 1.7 to 4.4 kg (median 3.1 kg), underwent first -stage palliation for hypoplastic left heart syndrome. The diameter of the ascending aorta ranged from 1.5 to 8.0 mm (median 3.0 mm), Eight patients h ad an aberrant right subclavian artery arising from the descending thoracic aorta. In 95 patients (group I), all duct tissue was excised and the desce nding aorta was anastomosed to the aortic arch, which had been opened back into the ascending aorta. Then to this confluence was anastomosed the proxi mal main pulmonary artery. In the remaining 25 patients (group II), continu ity of the aortic arch was maintained and the repair was performed with a D amus-Kaye-Stansel anastomosis. The size of the systemic-to-pulmonary shunt was 3 mm in 48 patients, 3.5 mm in 70, and 4.0 mm in 2, Results: Circulator y arrest time ranged from 19 to 105 minutes (median 54 minutes). A homograf t patch was necessary for the arch reconstruction in 18 patients (15%); 9 g roup I patients (10%) and 9 group II (36%) (P =.001). There were 82 hospita l survivors (68%): 69 group I patients (73%) and 13 group IT (52%) (P =.04) , 71 patients without a patch (70%) and 11 with a patch (61%) (P >.2). By m ultiple logistic regression, the aberrant right subclavian artery was a sig nificant risk factor for hospital death (P =.008), There were 6 late deaths . Sixteen of 71 patients (23%) who underwent second-stage palliation had a neoaortic arch obstruction develop, with a peak gradient greater than 10 mm Hg; 14 group I patients (23%) and 2 group II(22%) (P >.2), 15 without a pa tch (23%) and 1 with a patch (17%) (P >.2). Overall survivals were 57% at 1 year and 55% at 2 years. Conclusion: The modified Norwood procedure for fi rst-stage palliation of hypoplastic left heart syndrome is possible in the majority of patients without the use of exogenous materials and does not re sult in an increased incidence of neoaortic arch obstruction. Repair of the aorta without patch supplementation may improve the potential for long-ter m growth of the new aorta.