OBJECTIVE: 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. DESIGN: 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). RESULTS: 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. CONCLUSIONS: 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.
OBJECTIVE: 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. DESIGN: 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). RESULTS: 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. CONCLUSIONS: 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.
Authors: Howard P Gutgesell; Diane G Hillman; Kimberly E McHugh; Peter Dean; G Paul Matherne Journal: World J Pediatr Congenit Heart Surg Date: 2011-10-01
Authors: Joseph Deptula; James Hammel; Kari George; John Detwiler; Kimberly Glogowski; Melinda Valleley; Kim Duncan Journal: J Extra Corpor Technol Date: 2011-12
Authors: Peter N Dean; Kimberly E McHugh; Mark R Conaway; Diane G Hillman; Howard P Gutgesell Journal: Pediatr Cardiol Date: 2013-05-31 Impact factor: 1.655