Birth weight and complexity are significant factors for the management of hypoplastic left heart syndrome.

BACKGROUND: Classic options for treatment of hypoplastic left heart syndrome include the Norwood procedure (NW) and heart transplantation (HT). Recently off-pump palliative procedures were introduced in the management of these patients. Risk factors influencing the decision between the NW with staged reconstruction or off-pump palliation and HT were assessed.
METHODS: Between January 2002 and January 2006, 69 patients with hypoplastic left heart syndrome were referred for either a NW (n = 33) or HT (n = 36). Patients referred for HT underwent off-pump palliation (catheter-based, n = 20, or surgical hybrid procedures, n = 7) until a donor organ became available: including patent ductus arteriosus stenting (80.6%), atrial septostomy (41.7%), and branch pulmonary artery banding (55.6%).
RESULTS: Heart transplantation patients were more complex, based on a higher Aristotle Comprehensive Complexity score calculated at birth (HT 18.8 +/- 2.4 versus NW 17.7 +/- 1.7; p < 0.05). Presurgical death was significantly greater for HT referrals (HT 27.8% versus NW 3.0%; p < 0.01); however, there was no difference between the two groups in operative (HT 11.5% versus NW 21.9%; not significant) or overall mortality (HT 36.1% versus NW 24.2%; not significant). No independent risk factors for death were identified with HT; however, a lower birth weight (<2.5 kg) and a higher Aristotle score (>20) correlated with surgical death with NW (p < 0.01). Noticeably, surgical survival was 85.7% for infants with birth weight of 2.5 kg or greater undergoing NW.
CONCLUSIONS: Overall survival is similar for hypoplastic left heart syndrome patients referred for the NW or HT. Lower birth weight and higher complexity are risk factors for patients undergoing NW. Off-pump palliation followed by HT or further staged palliation should be considered for these high-risk hypoplastic left heart syndrome patients.