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Evolution of National Treatment Options and Outcomes for Hypoplastic Left Heart Syndrome Over an 18-Year Period
T. Karamlou, B. S. Diggs, R. M. Ungerleider*, K. F. Welke*. Oregon Health & Science University, Portland, OR,
BACKGROUND: The purpose of this investigation is to describe the patterns of management strategies for HLHS over the past 18 years in the US. METHODS: Neonates with HLHS were retrospectively identified using the Nationwide Inpatient Sample 1988 - 2005. Treatment was categorized as either: 1) transplantation (OHTx), 2) Norwood operation (as defined by RACHS-1), 3) transfer to another facility, or 4) no surgical intervention (comfort care).
RESULTS: Overall, 16,781 neonates were identified. Of these, 348 (2%) underwent OHTx, 2767 (17%) underwent Norwood, 4143 (24%) were transferred to another facility, and 9523 (57%) had no intervention. Important changes in practice patterns occurred over time, with increasing number of neonates undergoing Norwood, concomitant with decreasing numbers undergoing OHTx (P<0.001; Figure 1). Bias toward Norwood over time paralleled a significant, nearly linear decrease in the in-hospital mortality rate for Norwood, from 86% in the earliest sextile to 24% in the most recent sextile (P<0.001; Figure 2). 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, national practice patterns have not changed substantially in favor of surgery. There has been an increase in the number of infants offered Norwood for HLHS over the past two decades which seems to have come mostly due to decrease of OHTx. The majority of infants continue to receive no surgical care. The advent of prenatal diagnosis has not decreased the proportion of neonates born at institutions unequipped to provide definitive care.
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