Staged surgical management of hypoplastic left heart syndrome: a single-institution 12-year experience

Simon P McGuirk ^1*, Massimo Griselli ¹, Oliver Stumper ¹, Elizabeth M Rumball ¹, Paul Miller ¹, Rami Dhillon ¹, Joseph V de Giovanni ¹, John G Wright ¹, David J Barron ¹ and William J Brawn ¹

¹ Birmingham Children's Hospital NHS Trust, United Kingdom

^* To whom correspondence should be addressed. E-mail: dr_mcguirk{at}dsl.pipex.com.

Accepted 27 May 2005

Abstract

Objective: This study sought to describe the 12-year experience with staged surgical management for hypoplastic left heart syndrome (HLHS) and identify the factors that influenced outcome.

Methods: Between December 1992 and June 2004, 333 patients with HLHS underwent a Norwood procedure (median age 4 days, range 0 to 217 days). A bidirectional Glenn (Stage II) was subsequently performed in 203 patients and 81 patients underwent a modified Fontan procedure (Stage III). Follow-up was complete (median interval 3.7 years, range 32 days to 11.3 years).

Results: Early mortality following the Norwood procedure was 29% (n=95); this decreased from 46% (first year) to 16% (last year; p<0.05). There were 49 inter-stage deaths, 27 prior to Stage II and 22 between Stages II and III. There was one early and 3 late deaths following Stage III. Actuarial survival (SEM) was 58% (3%) at 1 year and 50% (3%) at 5 and 10 years. On multivariable analysis, five factors influenced early mortality following the Norwood procedure (p<0.05). Pulmonary blood flow supplied by a right ventricle to pulmonary artery (RV-PA) conduit; arch reconstruction with pulmonary homograft patch and increased operative weight improved early mortality. Increased periods of cardiopulmonary bypass and deep hypothermic circulatory arrest increased early mortality. Similar factors also influenced actuarial survival following the Norwood procedure.

Conclusion: This study identified an improvement in outcome following staged surgical management of HLHS, which was primarily attributable to changes in surgical technique. The RV-PA conduit, in particular, was associated with a marked and independent improvement in early and actuarial survival.

Keywords: congenital heart defects, hypoplastic left heart syndrome, Norwood procedure, paediatrics, risk factors

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