Response to Letter Regarding Article, “Mortality in First 5 Years in Infants With Functional Single Ventricle Born in Texas, 1996 to 2003”
We concur with several of the issues addressed in the letter by Dr Hill and his colleagues. The low 5-year survival of 38% for hypoplastic left heart syndrome in our population-based study, in contrast to recent surgical survival reports for the Norwood procedure, may be explained by several factors. First, from 1996 to 2003, surgical outcomes were improving but had not reached current levels. Second, our report includes deaths occurring before surgery and after hospital discharge. Third, surgical reports of Norwood procedure outcomes frequently include other cases with hypoplastic systemic ventricles and small aortic roots (eg, unbalanced atrioventricular septal defects), which have a more favourable outcome than classic hypoplastic left heart syndrome.
As pointed out by Dr Hill and colleagues, the highest risk of death occurs early, before the second-stage palliation Glenn procedure. In our study, 80% of the deaths in hypoplastic left heart syndrome occurred in the first 3 months of life. During this period, access to care for these fragile infants is important, as is the need for effective communication between the specialty team and the home caregivers. The higher mortality risk seen in infants born in Texas counties bordering Mexico and in Hispanic infants may reflect the fact that their families live far from a specialty center and many are primarily Spanish speaking.
David E. Fixler, MD, MSc
Department of Pediatrics
Division of Pediatric Cardiology
University of Texas Health Science Center
Wendy N. Nembhard, PhD
Jason L. Salemi, MPH
Department of Epidemiology and Biostatistics
College of Public Health
University of South Florida
Mary K. Ethen, MPH
Mark A. Canfield, PhD
Texas Birth Defects Epidemiology and Surveillance Branch
Texas Department of State Health Services
- © 2010 American Heart Association, Inc.