Results of staged palliation for hypoplastic left heart syndrome: A complete population‐based series

Aim: To study the outcome of staged palliation for classic hypoplastic left heart syndrome. Methods: Retrospective chart review. Risk factors for mortality were analysed using Cox's proportional hazard regression modelling. Results: From 1993 to 2004, 55 infants underwent Norwood stage I procedure at a median age of 8 d (range 1–19 d). Hospital survival was 39/55 (71%), and there were six late deaths (before stage II). Birthweight, circulatory arrest time and cardiopulmonary bypass time were independent risk factors for stage I hospital mortality ( p =0.029, p =0.001 and p =0.003, respectively). Poor right ventricular function prior to stage I was a significant predictor for interstage mortality ( p =0.02). Thirty‐two patients underwent bidirectional cavopulmonary anastomosis, at a median age of 6.5 mo (range 2.0–9.5 mo), with seven late deaths. Two patients had a heart transplant after stage II. Total cavopulmonary connection (TCPC) was performed in 13 patients, at a median age of 33 mo (range 21–45 mo), without mortality. Kaplan‐Meier survival was 58%, 52% and 45% at 6, 12 and 48 mo, respectively. Conclusion: Low birthweight, long time on circulatory arrest and cardiopulmonary bypass were risk factors for stage I mortality. Poor right ventricular function was detrimental to intermediate outcome.