Objectives We sought to identify risk factors for mortality and morbidity during the Norwood hospitalization in newborn infants with hypoplastic left heart syndrome and other single right ventricle anomalies enrolled in the Single Ventricle Reconstruction trial. Methods Potential predictors for outcome included patient- and procedure-related variables and center volume and surgeon volume. Outcome variables occurring during the Norwood procedure and before hospital discharge or stage II procedure included mortality, end-organ complications, length of ventilation, and hospital length of stay. Univariate and multivariable Cox regression analyses were performed with bootstrapping to estimate reliability for mortality. Results Analysis included 549 subjects prospectively enrolled from 15 centers; 30-day and hospital mortality were 11.5% (63/549) and 16.0% (88/549), respectively. Independent risk factors for both 30-day and hospital mortality included lower birth weight, genetic abnormality, extracorporeal membrane oxygenation (ECMO) and open sternum on the day of the Norwood procedure. In addition, longer duration of deep hypothermic circulatory arrest was a risk factor for 30-day mortality. Shunt type at the end of the Norwood procedure was not a significant risk factor for 30-day or hospital mortality. Independent risk factors for postoperative renal failure (n = 46), sepsis (n = 93), increased length of ventilation, and hospital length of stay among survivors included genetic abnormality, lower center/surgeon volume, open sternum, and post-Norwood operations. Conclusions Innate patient factors, ECMO, open sternum, and lower center/surgeon volume are important risk factors for postoperative mortality and/or morbidity during the Norwood hospitalization.

Feeding difficulties are not uncommon following surgery for the correction of congenital heart defects, especially in the neonate. The most important risk factors appear to be an increased RACHS score and prolonged postoperative intubation. Hopefully, by defining the risk factors, proactive management strategies can be developed to minimize these problems following neonatal congenital heart surgery.

Outcomes were adversely affected primarily by prolonged cardiopulmonary bypass time, elevated central venous pressure and transpulmonary gradient, and right ventricular morphology. Specifically, outcomes were unaffected by the presence of a left superior vena cava, cannulation strategy, or antegrade pulmonary blood flow. There were few differences between the unilateral and bilateral groups, none of which were postoperative outcomes.

Operative survival after the Norwood procedure has significantly improved during the past 10 years. However, there remains attrition among Norwood survivors before reaching planned second-stage palliation. The purpose of this study was to evaluate potential risk factors for interstage mortality among Norwood survivors. All patients undergoing the Norwood procedure at the Medical University of South Carolina from January 1996 through January 2001 were retrospectively reviewed. Patient and procedural variables were examined as potential risk factors for interstage mortality. Among 50 Norwood survivors, 8 (16%) died prior to second-stage palliation. The mean age at death was 102 +/- 72 days (median, 61; range, 35-208). By multivariate analysis, the presence of an arrhythmia in the postoperative period (p = 0.02) and decreased ventricular function at hospital discharge (p = 0.05) were identified as risk factors for interstage mortality. There remains a significant risk for interstage mortality among Norwood survivors. Patients with postoperative arrhythmias and/or decreased ventricular function at discharge are at increased risk for interstage death after Norwood procedure. More frequent follow-up and aggressive medical management of arrhythmia or decreased function may be warranted for these high-risk patients.