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Original Articles: Pediatric Cardiac

Risk Factors for Mortality After the Norwood Procedure Using Right Ventricle to Pulmonary Artery Shunt

^a Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan
^b Department of Cardiovascular Surgery, National Taiwan University Hospital, Taipei, Taiwan

Accepted for publication August 11, 2008.

^_* Address correspondence to Dr Sano, Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Okayama, 700-8558, Japan (Email: s_sano{at}cc.okayama-u.ac.jp).

Presented at the Forty-fourth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 28–30, 2008.

Background: The purpose of this study was to describe the experience with staged surgical reconstruction of the hypoplastic left heart syndrome (HLHS) with a right ventricle to pulmonary artery conduit and to identify the risk factors that influence late outcome.

Methods: Between February 1998 and June 2007, 62 patients with HLHS underwent a Norwood procedure by using right ventricle to pulmonary artery conduit (median age, 9 days [range, 1 to 57]; median body weight 2.7 kg [range, 1.6 to 3.9 kg]). The subsequent 47 patients underwent a bidirectional Glenn procedure (stage 2). Thirty-two patients underwent a modified Fontan procedure (stage 3). Follow-up was complete (median, 32 months; range, 1 to 101).

Results: Hospital mortality after the Norwood procedure was 8% (5 of 62 patients). Between stages, 9 patients died, 3 before stage 2 and 6 before stage 3. There was 1 late death after stage 3. Overall survival was 76% (47 of 62). The estimated 1-year and and 5-year survival rates were 80% and 73%, respectively. Using the any-mortality as the endpoint, prematurity (gestational age <37 weeks), body weight less than 2.5 kg at stage 1 operation, and tricuspid regurgitation 2+ or more were associated with mortality. Using Cox regression analysis, body weight less than 2.5 kg and tricuspid regurgitation 2+ or more were two independent factors associated with midterm survival.

Conclusions: From 9 years of experience, despite good early survival after Norwood stage 1 palliation, low body weight and tricuspid valve regurgitation were still associated with worse outcome. More efforts should be made to improve the late results for patients with hypoplastic left heart syndrome.