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J Pediatr. 2008 Sep;153(3):354-8. doi: 10.1016/j.jpeds.2008.03.009. Epub 2008 Apr 28.

Decreasing number of deaths of infants with hypoplastic left heart syndrome.

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Division of Pediatric Cardiology, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.



To assess mortality rates and demographic characteristics for infants with hypoplastic left heart syndrome (HLHS) in California.


We used California death registry files from 1990 to 2004 to compare overall mortality and demographic characteristics between infants with HLHS (n = 856) who received surgical intervention and those who received comfort care. The California discharge database was used to calculate the annual incidence of disease and survival rates for infants with HLHS undergoing surgery between 1995 and 1999.


The annual number of deaths for infant with HLHS decreased by nearly 50% over the study period, even though the incidence of the disease remained constant during this period. For all deaths, the proportion of infants receiving comfort care decreased significantly over time compared with those infants who underwent surgery. Although the total number of deaths in infants with HLHS who underwent surgical intervention increased, the mortality rate for this cohort decreased. Interstage unexpected mortality and the median age at death both increased in the infants who underwent surgery.


Over the study period of 1990 to 2004 in California, fewer families chose comfort care for infants diagnosed with HLHS, and the number of deaths for those infants who underwent surgical intervention increased. These changes likely reflect improved treatment outcomes and an increased number of families desiring surgical intervention in higher-risk infants.

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