Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period

J Thorac Cardiovasc Surg. 2010 Jan;139(1):119-26; discussion 126-7. doi: 10.1016/j.jtcvs.2009.04.061. Epub 2009 Nov 11.


Objectives: We aimed to describe management strategies for neonates with hypoplastic left heart syndrome over the past 18 years in the United States and to identify determinants of institutional management decisions.

Methods: Neonates with hypoplastic left heart syndrome were retrospectively identified by use of the Nationwide Inpatient Sample 1988-2005. Treatment was categorized as (1) transplantation, (2) Norwood operation (as defined by Risk Adjustment in Congenital Heart Surgery), (3) transfer to another facility, or (4) no surgical intervention (comfort care).

Results: A total of 3286 neonates were identified, yielding a national estimate of 16,781 + or - 586 cases. Of these, 2% (348 + or - 47) underwent transplantation, 16% (2767 + or - 286) had Norwood operations, 25% (4143 + or - 156) were transferred to another facility, and 57% (9523 + or - 436) had comfort care. Changes in practice patterns occurred over time, with an increasing number of neonates undergoing Norwood, concomitant with decreasing numbers undergoing transplantation (P < .001). Bias toward the Norwood operation over time paralleled a significant, nearly linear decrease in the in-hospital mortality rate for the Norwood operation, from 86% in the earliest sextile to 24% in the most recent sextile (P < .001). Prevalence of transfer to definitive care hospitals remained constant over time, as did the number of infants (approximately half) who received no surgery (comfort care).

Conclusions: Despite improved surgical outcomes, the majority of infants continue to receive no surgical care. There has been an increase in the number of infants offered the Norwood operation for hypoplastic left heart syndrome over the past 2 decades, which seems to have come mostly owing to a decrease of transplants. The advent of prenatal diagnosis has not decreased the proportion of neonates born at institutions unequipped to provide definitive care.

MeSH terms

  • Cardiac Surgical Procedures
  • Decision Making
  • Female
  • Heart Transplantation
  • Humans
  • Hypoplastic Left Heart Syndrome / mortality
  • Hypoplastic Left Heart Syndrome / surgery
  • Hypoplastic Left Heart Syndrome / therapy*
  • Infant, Newborn
  • Male
  • Patient Transfer
  • Treatment Outcome