Prenatal Diagnosis, Birth Location, Surgical Center, and Neonatal Mortality in Infants with Hypoplastic Left Heart Syndrome
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, 1999-2007, 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%, 10-90 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, OR 2.03 (95%CI 1.19-3.45), compared to <10 minutes. In subanalysis, birth >90 minutes from a CSC was associated with higher pre-transport mortality (OR 6.69, 95%CI 2.52-17.74) and birth 10-90 minutes with higher pre-surgical mortality (OR 4.45, 95%CI 1.17-17.00). Higher surgical mortality was associated with lower CSC HLHS volume (OR per 10 patients 0.88, 95%CI 0.84-0.91).
Conclusions—Infants with HLHS born far from a CSC have increased neonatal mortality, and most of this mortality is pre-surgical. Efforts to improve prenatal diagnosis of HLHS and subsequent delivery near a large volume CSC may significantly improve neonatal HLHS survival.
- fetal echocardiography
- hospital performance
- access to care
- Stage I palliation
- hypoplastic left heart syndrome
- prenatal diagnosis
- heart defects, congenital
- population studies
- Received May 9, 2013.
- Revision received September 10, 2013.
- Accepted October 1, 2013.