Three-stage palliation of hypoplastic left heart syndrome in the University HealthSystem Consortium

The objective of this study was to describe current management and short-term outcomes for patients with hypoplastic left heart syndrome representative of nationwide experience. Additionally, this study identifies risk factors associated with mortality in patients undergoing staged surgical palliation.

The University HealthSystem Consortium database was queried to identify all hospital admissions between 1998 and 2007 with a diagnosis of hypoplastic left heart syndrome. Procedure codes were used to determine surgical management, and outcomes were ascertained by discharge status (discharged, transferred, or expired).

Discharge data were present from 118 hospitals in the United States. First-stage surgical palliation was performed in 1949 neonates with 30% mortality. Mortality decreased from 43% in 1998 to 18% in 2007. Large institutional case volume and later era of surgery were associated with improved operative mortality in first-stage palliation. Primary cardiac transplantation was performed in 28 neonates, and rescue transplantation in 11, with 36% mortality. Second-stage palliation was performed in 1244 patients with 5.2% mortality, and third-stage palliation was performed in 1084 patients with 4.1% mortality. An additional 62 patients over 1 month of age at time of admission received cardiac transplantation with 15% mortality.

First-stage palliative mortality for hypoplastic left heart syndrome fell dramatically over the past decade, while that for second- and third-stage procedures remained stable. The cumulative operative mortality for three-staged repair of hypoplastic left heart syndrome was 39% over the decade, but fell to 24% for procedures in 2007.