Health Indicator Report of Birth Defects: Congenital Heart Defects
Congenital heart defects are the most common birth defects. They are also the most expensive birth defects in the United States. As a group, they are the leading cause of infant death due to congenital anomalies. These three features - common, costly, critical - highlight the importance of tracking, care, and prevention. Congenital heart defects are the leading cause of infant death due to congenital anomalies - accounting for approximately 1 in 3 such infant deaths. The contribution to neonatal deaths is also significant - in the United States and in several European countries congenital heart defects account for an estimated 1 in 4 neonatal deaths to birth defects. In developed countries, congenital heart defects are estimated to account for approximately 1 in 10 infant deaths from any cause. Affected children require medical and surgical treatment. Even after treatment, some children may be at increased risk of illness and death because of cardiovascular complications, rhythm abnormalities, or other long-term adverse events. As treatment and support continually improves, an increasing number of affected people live longer and healthier lives. Adolescents and adults that have been successfully treated now represent a growing group in the population and have specific healthcare needs. The burden of disease and medical costs associated with congenital heart defects are substantial. In the United States, congenital heart defects are the most expensive of all birth defects (Robbins et al., 2007; Keren et al., 2012). In fact, six of the ten birth defects with highest hospital charges were congenital heart defects and two - hypoplastic left heart and truncus arteriosus-topped the list (Robbins et al., 2007). Tracking congenital heart defects in Utah is crucial in assessing the impact of these common conditions, evaluating their causes, and helping to plan appropriately for the care of affected children and adults.
Heart Defects: Prevalence of Heart Defects, Overall and by Race/Ethnic Groups, Utah, 2003-2011
- Utah Birth Defect Network
- Utah Department of Health
DefinitionNumber of cases of major heart defects per 1,000 births.
NumeratorNumber of cases of major congenital heart defects among live births and stillbirths among women residing in Utah. This number excludes mild or minor conditions such as isolated patent foramen ovale, patent ductus arteriosus, and muscular ventricular septal defect. Diagnoses are confirmed by echocardiography, catheterization, surgery, or autopsy.
DenominatorNumber of live births and stillbirths among women residing in Utah.
How Are We Doing?The Utah Birth Defect Network began tracking selected congenital heart defects in 1997, including conotruncal conditions (e.g., tetralogy of Fallot) and left obstructive heart defects (e.g., hypoplastic left heart syndrome ). In 1999, tracking expanded to include all major heart defects, except ventricular septal defects, and in 2003 it further expanded to include also ventricular septal defects. To be inclusive and consistent across years, this report is based mainly on the cohort born since 2003. For birth years from 2003 through 2011, the Network identified 4,230 babies with a major congenital heart defect born to resident mothers. The overall prevalence was 0.9% (88.9 per 10,000), or 1 in 112 births. Thus, 470 babies with a major congenital heart defect are born on average every year in Utah. Figure 1 shows how the prevalence varies by race/ethnicity. These variations are small and not statistically significant - congenital heart defects affect every racial and ethnic group. Note that the prevalence of some race/ethnicities are based on few affected babies: thus, the confidence intervals are wide (Figure 1) and the corresponding prevalence is statistically unstable. Figure 2 shows the prevalence of selected major heart defects, including some that are associated with the greatest health impact (e.g., hypoplastic left heart syndrome). Figure 3 shows the time trends for those major heart defects that have been tracked the longest by the Utah Birth Defect Network.
How Do We Compare With the U.S.?Comparing birth prevalence among different areas requires caution as many aspects of the tracking programs in these areas can impact the reported prevalence. This is true generally for birth defects, and particularly for heart defects, which require special examinations for diagnosis (e.g., echocardiography) and may become clinically evident only after discharge from the newborn nursery. For this reason, prevalence between tracking programs may easily vary just because of their different access to data sources (e.g., pediatric cardiology services) and the programs' different follow up periods (e.g., the length of time after birth during which they are able to collect data). Reported prevalence also depends on inclusion criteria. Because many minor or mild conditions (e.g., ventricular septal defects, patent ductus arteriosus, patent foramen ovale) are frequent and are variably included in different tracking programs, their inclusion or exclusion can lead to considerable variations in the reported prevalence. For these reasons, when comparing prevalence across programs, it is crucial to examine their methods of ascertainment and reporting. In general, comparisons are likely to be meaningful for the more severe heart defects, which present earlier and pose fewer diagnostic ambiguities, leading to lesser spurious variations in prevalence. The overall prevalence of major heart defects in Utah (88.9 per 10,000, or 8.9 per 1000) is comparable to that observed in the well-known Baltimore Washington Infant Study, where the prevalence varied between 4 and 8 per 1,000 depending on time and inclusion criteria. The prevalence in Utah is similar or slightly higher than in other birth defect programs. For example, recent reports from the Atlanta registry, managed by the Centers for Disease Control and Prevention, noted an overall prevalence of major heart defects of 8.1 per 1,000 (Reller et al, 2008) and 6.8 per 1,000 (Bjornard et al, 2013) depending on time period and inclusion criteria. Because of the variations due to inclusion criteria, it is recommended to focus on individual types of heart defects (rather than all types combined) when comparing the local prevalence with other programs or with national estimates.
What Is Being Done?The Utah Birth Defect Network (UBDN) continues to track the prevalence of congenital heart defects in Utah. It also collaborates in studies to assess their health impact (e.g., early mortality), the associated costs and use of resources, and potential risk factors (with the goal of finding strategies for primary prevention). Since 2013, the UBDN has been focusing in particular on Critical Congenital Heart Defects (CCHD), a subgroup of severe, early onset conditions that account for most of the morbidity and mortality associated with heart defects. CCHDs are amenable to newborn screening by pulse oximetry. Several of the specific heart defects selected as IBIS indicators are CCHDs. Specific services for families of affected children from birth to three years of age are provided by the Utah Early Intervention Program, located within the Bureau of Children with Special Health Care Needs, Utah Department of Health. Services include child health assessment; service coordination among providers, programs and agencies; occupational and physical therapy; and speech and language therapy. Information on these services is available at http://www.utahbabywatch.org. A further resource is the Utah Collaborative Medical Home, which is a project designed to provide information, tools and resources for Primary Care Physicians to enhance their ability to care for children with special health care needs. Information on the Utah Collaborative Medical Home is available at their web site: www.medicalhomeportal.org.
Available ServicesPediatric Cardiology 100 North Mario Capecchi Drive, Suite 1500 Primary Children?s Medical Center Salt Lake City, Utah 84113 Division of Medical Genetics Department of Pediatrics University of Utah Health Sciences Center 2C412 School of Medicine 50 North Mario Capecchi Drive Salt Lake City, Utah 84132 Phone (801) 581-8943 Children with Special Health Care Needs 44 North Mario Capecchi Drive PO Box 144610 Salt Lake City,UT 84114-4610 Phone (801) 584-8284 Toll Free (800) 829-8200 http://health.utah.gov/cshcn CSHCN provides services for children who have or are at risk for a chronic physical, developmental, behavioral, or emotional condition. State Early Intervention Services 44 North Mario Capecchi Drive PO Box 144720 Salt Lake City, Utah 84114-4720 Phone (801) 584-8226 Toll Free (800) 961-4226 http://www.utahbabywatch.org Statewide EI services are available for families of affected children from birth to three years of age that include child health assessment, service coordination among providers, occupational and physical therapy, speech and language therapy.
Page Content Updated On 11/18/2014, Published on 07/23/2015