Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period  Tara Karamlou, MD, Brian S. Diggs, PhD, Ross M.

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Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period  Tara Karamlou, MD, Brian S. Diggs, PhD, Ross M. Ungerleider, MD, MPH, Karl F. Welke, MD, MS  The Journal of Thoracic and Cardiovascular Surgery  Volume 139, Issue 1, Pages 119-127 (January 2010) DOI: 10.1016/j.jtcvs.2009.04.061 Copyright © 2010 Terms and Conditions

Figure 1 Distribution of case-mix in year sextiles. An increase in the Norwood operation is seen over time concomitant with a small decrease in the number of OHTx. Despite increasing application of the Norwood operation, comfort care remains the most common treatment choice. Raw numbers (estimates) of the total number of neonates with HLHS within each sextile are as follows: sextile 1: 406 total (national estimate, 2393); sextile 2: 458 total (national estimate, 2469); sextile 3: 437 total (national estimate, 2202); sextile 4: 583 total (national estimate, 2921); sextile 5: 606 total (national estimate, 2935); sextile 6: 796 total (national estimate, 3862). The Journal of Thoracic and Cardiovascular Surgery 2010 139, 119-127DOI: (10.1016/j.jtcvs.2009.04.061) Copyright © 2010 Terms and Conditions

Figure 2 Mortality for the Norwood operation and OHTx is displayed over time, divided into year sextiles. Norwood mortality decreased in a nearly linear manner over time. Note the widening confidence intervals for OHTx mortality within all but 1 of the year sextiles, indicating that increased mortality may be confounded the small number of OHTx cases in these periods. The Journal of Thoracic and Cardiovascular Surgery 2010 139, 119-127DOI: (10.1016/j.jtcvs.2009.04.061) Copyright © 2010 Terms and Conditions

Figure 3 Graphic display of the admission sources for neonates with HLHS over time. The years are shown on the horizontal axis and the proportion of neonates with each admission code is on the vertical axis. The legend at the figure right shows the different codes within the NIS. Neonates who were transferred in bear an admission code “admitted from another hospital,” shown in dark blue. The number of transferred neonates has decreased only slightly during the 18-year period. LTC, Long-term care. The Journal of Thoracic and Cardiovascular Surgery 2010 139, 119-127DOI: (10.1016/j.jtcvs.2009.04.061) Copyright © 2010 Terms and Conditions

Figure 4 Number of neonates having each treatment type over time segregated by whether they were transferred from another hospital (transferred in) or whether they were admitted directly (not transferred in). The legend at top describes the color-coded 4 treatment types, the years are shown at the horizontal axis, and the proportion of neonates having each treatment type is on the vertical axis. Regarding the subset of patients who were transferred, there has been an increase in the number of neonates who underwent subsequent surgical intervention at the receiving hospital over time, from 4% (11/248) in 1988 to 47% (348/738) in 2005. There was a corresponding decrease in the proportion of patients having comfort care at the receiving hospital, although this group still comprised nearly 50% of the total in 2005. The Journal of Thoracic and Cardiovascular Surgery 2010 139, 119-127DOI: (10.1016/j.jtcvs.2009.04.061) Copyright © 2010 Terms and Conditions

Figure 5 Graphic description of the proportion of neonates managed with a particular treatment strategy within each hospital (vertical axis), as a function of increasing percent intervention, excluding those hospitals whose annual HLHS volume was less than 3 cases. The different colored shaded areas are confluent histograms from each institution and represent the 4 possible treatment options. There are clear treatment biases, with the majority of hospitals choosing to transfer neonates with HLHS or provide comfort care (blue and red shaded areas at left). A minority of hospitals uniformly choose Norwood operation (green areas), and a small number provide only OHTx (pink areas). Importantly, hospitals providing both the Norwood operation and OHTx have the highest percentage of intervention (and are therefore located at the far right on the plot) in neonates with HLHS and used transfer with decreasing frequency. The Journal of Thoracic and Cardiovascular Surgery 2010 139, 119-127DOI: (10.1016/j.jtcvs.2009.04.061) Copyright © 2010 Terms and Conditions

Figure 6 Percent of neonates with HLHS admitted to either an interventional or noninterventional hospital (vertical axis) segregated by year sextiles. An interventional hospital was defined as a hospital that provided either Norwood operation or OHTx at least once during an annual period. A significant increase in the proportion of neonates admitted to interventional hospitals is noted up to 1999, after which point there is no significant increase. The Journal of Thoracic and Cardiovascular Surgery 2010 139, 119-127DOI: (10.1016/j.jtcvs.2009.04.061) Copyright © 2010 Terms and Conditions