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. 2014 Jan 21;129(3):285-92.
doi: 10.1161/CIRCULATIONAHA.113.003711. Epub 2013 Oct 17.

Prenatal diagnosis, birth location, surgical center, and neonatal mortality in infants with hypoplastic left heart syndrome

Shaine A Morris  1 , Mary K Ethen, Daniel J Penny, Mark A Canfield, Charles G Minard, David E Fixler, Wendy N Nembhard
Affiliations

Prenatal diagnosis, birth location, surgical center, and neonatal mortality in infants with hypoplastic left heart syndrome

Shaine A Morris et al. Circulation. .

Abstract

Background: Most studies have not demonstrated improved survival after prenatal diagnosis of critical congenital heart disease, including hypoplastic left heart syndrome (HLHS). However, the effect of delivery near a cardiac surgical center (CSC), the recommended action after prenatal diagnosis, on HLHS mortality has been poorly investigated.

Methods and results: Using Texas Birth Defects Registry data, 1999 through 2007, which monitored >3.4 million births, we investigated the association between distance (calculated driving time) from birth center to CSC and neonatal mortality in 463 infants with HLHS. Infants with extracardiac birth defects or genetic disorders were excluded. The associations between prenatal diagnosis, CSC HLHS volume, and mortality were also examined. Neonatal mortality in infants born <10 minutes from a CSC was 21.0%, 10 to 90 minutes 25.2%, and >90 minutes 39.6% (P for trend <0.001). Prenatal diagnosis alone was not associated with improved survival (P=0.14). In multivariable analysis, birth >90 minutes from a CSC remained associated with increased mortality (odds ratio, 2.03; 95% confidence interval, 1.19-3.45), compared with <10 minutes. In subanalysis, birth >90 minutes from a CSC was associated with higher pretransport mortality (odds ratio, 6.69; 95% confidence interval, 2.52-17.74) and birth 10 to 90 minutes with higher presurgical mortality (odds ratio, 4.45; 95% confidence interval, 1.17-17.00). Higher surgical mortality was associated with lower CSC HLHS volume (odds ratio per 10 patients, 0.88; 95% confidence interval, 0.84-0.91).

Conclusions: Infants with HLHS born far from a CSC have increased neonatal mortality, and most of this mortality is presurgical. Efforts to improve prenatal diagnosis of HLHS and subsequent delivery near a large volume CSC may significantly improve neonatal HLHS survival.

Keywords: heart defects, congenital; hypoplastic left heart syndrome; mortality; population; prenatal diagnosis.

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Conflict of interest statement

Conflict of Interest Disclosures: None.

Figures

Figure 1.
Figure 1.
Neonatal mortality of infants with hypoplastic left heart syndrome by driving time from birth location to cardiac surgical center, among birth eras (A) and with prenatal versus postnatal diagnosis (B). Comparison of mortality among birth eras, accounting for time to CSC, p=0.002. Comparison of mortality by prenatal diagnosis, accounting for time to CSC, p=0.90. Error bars represent 95% confidence intervals.
Figure 2.
Figure 2.
Pre-transport, Pre-Stage 1, and Stage 1 neonatal mortality in infants with hypoplastic left heart syndrome by driving time from birth location to cardiac surgical center. Error bars represent 95% confidence intervals.
Figure 3.
Figure 3.
A. Percent pre-Stage 1 mortality in infants with hypoplastic left heart syndrome (HLHS) after arrival at a cardiac surgical center (CSC), by CSC, with HLHS volume (cases) on the x-axis, and mortality on the y-axis. B. Percent Stage 1 neonatal mortality by CSC, with HLHS volume on the x-axis, and mortality on the y-axis.
See this image and copyright information in PMC

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