Complete Indicator Profile of Birth Defects: Congenital Heart Defects
Definition
Number of cases of major heart defects per 1,000 births.Numerator
Number of cases of major heart defects among live births and fetal deaths occurring 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.Denominator
Number of live births and stillbirths among women residing in Utah.Why Is This Important?
Congenital heart defects are among the most common birth defects. Many 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, 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 health care needs.
Tracking congenital heart defects in Utah is crucial in assessing the impact of these common conditions, evaluating its causes, and helping to plan appropriately for the care of affected children and adults.
Burden of disease and medical costs associated with congenital heart defects are substantial. $1.2 billion is the estimated lifetime cost for U.S. children born in a single year with one of four major heart defects (tetralogy of Fallot, transposition of the great arteries, single ventricle, truncus arteriosus) (Waitzman and collaborators, 1994).
How Are We Doing?
The Utah Birth Defect Network began tracking selected congenital heart defects (conotruncal and left obstructive heart defects) in 1997 and later expanded to include all major heart defects. This report is based on 1,418 affected births reported to the Network from 1997 through 2003.The Network reports that the overall rate of major congenital heart defects in 2003 in Utah was 0.6% (6.3 per 1,000), or 1 in 159 births. On average, approximately 300 affected babies or more are born every year in Utah.
Overall rates of major heart defects for birth year 2003 are similar in different racial and ethnic groups. Some rates are based on small numbers of affected babies and thus their confidence intervals are wide. For some groups such as Native Americans and African Americans the number of affected babies in 2003 were small (<10) and because the corresponding rates are statistically very unstable and not meaningful they are not reported.
How Do We Compare With U.S.?
Comparing rates among different areas requires caution as many aspects of the tracking programs in these areas can impact the reported rates. This is true generally for birth defects and particularly for heart defects, that require special examinations for diagnosis (e.g., echocardiography) and that often are suspected or diagnosed well after the newborn period. Thus programs with varying access to pediatric cardiology data and with different follow up periods can vary in their ability to detect and register affected babies and can therefore generate apparently different rates of heart defects.Moreover, the reported overall rate of heart defect also depends on inclusion criteria. Because many minor or mild conditions (e.g., muscular ventricular septal defects, patent ductus arteriosus, patent foramen ovale) are quite frequent and are variably included in different tracking programs, their inclusion or exclusion can lead to considerable variations in reported rates.
It is therefore sensible to compare overall and specific rates among programs with similar methods of ascertainment and reporting. Comparisons are likely to be more meaningful for the more specific and severe heart defects, whose ascertainment and reporting are less likely to vary by much.
The overall rate of major heart defects in Utah (6.3 per 1,000) is comparable to that observed in the well-known Baltimore Washington Infant Study, where the rate varied between 4 and 8 per 1,000 depending on time and inclusion criteria. The rate in Utah is lower than that reported in the Atlanta registry, managed by the Centers for Disease Control and Prevention, whose methods of ascertainment are similar to those in Utah Birth Defect Network. The lower rate in Utah is likely due to the use of more restrictive reporting criteria, such as the exclusion from rate computations of common mild heart conditions (see definition of numerator). For specific, severe heart defects such as tetralogy of Fallot, d-transposition of the great arteries, and hypoplastic left heart, observed rates in Utah are comparable to those observed in Metropolitan Atlanta and in the Baltimore Washington Infant Study.
What Is Being Done?
The Utah Birth Defect Network (UBDN) currently is tracking rates of congenital heart defects, evaluates their reported origin and time/space clustering, and, more recently, is involved in studies of causation with the goal of finding strategies for primary prevention.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: http://www.medhomeportal.org
Available Services
Pediatric Cardiology100 North Medical 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 N. Medical Drive
Salt Lake City, Utah 84132
Phone (801) 581-8943
Children with Special Health Care Needs
44 North Medical 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 Medical 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, and speech and language therapy.
More Information
Utah Birth Defect Networkhttp://www.health.utah.gov/birthdefect/
Parent to Parent/Utah Parent Center
2290 East 4500 South, Suite 110
Salt Lake City, Utah 84117-4428
Phone (801) 272-1051
Toll Free (800) 468-1160 voice or TDD
Espanol (801) 272-1067
Family to Family/ARC
455 East 200 South, Suite 202
Salt Lake City, Utah 84111
Phone (801) 364-5060
Related Indicators
Related IBIS-PH Indicator Profile Links for Relevant Population Characteristics:
- Birth Defects: Overall
- Birth Defects: Down Syndrome
- Birth Defects: Infant Mortality
- Birth Defects: Neural Tube Defects
- Birth Defects: Orofacial Clefts
Graphical Data Views
Heart Defects: Rate Overall by Race/Ethnicity, Utah, 2003
| Race/Ethnicity | Rate per 10,000 Births | Lower Limit | Upper Limit | Numer | Denom |
|---|---|---|---|---|---|
| All Races | 62.9 | 56.0 | 69.8 | ||
| American Indian/Native Alaskan | 66.8 | 1.3 | 132.2 | ||
| Asian | 71.6 | 29.3 | 113.9 | ||
| Black | 0.0 | 0.0 | 114.7 | 53 | 7,120 |
| Hispanic | 74.4 | 54.4 | 94.5 | ||
| White | 61.5 | 53.8 | 69.1 |
Record Count: 6
Data Sources
Utah Birth Defect Network; Utah Department of Health;Heart Defects: Rates of Selected Defects, Utah, 1997-2003
| Heart Defects | Rate per 10,000 Births |
|---|---|
| Atrial Septal Defects | 19.0 |
| Ventricular Septal Defects, perimembranous | 9.4 |
| d-Transposition of Great Arteries | 3.4 |
| Tetralogy of Fallot | 3.3 |
| Hypoplastic Left Heart | 3.5 |
| Coarctation of Aorta | 7.8 |
Record Count: 6
Data Sources
Utah Birth Defect Network;Heart Defects: Rates Over Time of Selected Heart Defects, Utah, 1997-2003
| BrthDefHrt | Year | Rate per 10,000 Births |
|---|---|---|
| Coarctation of Aorta | 1997 | 7.2 |
| Coarctation of Aorta | 1998 | 10.1 |
| Coarctation of Aorta | 1999 | 8.6 |
| Coarctation of Aorta | 2000 | 5.7 |
| Coarctation of Aorta | 2001 | 8.1 |
| Coarctation of Aorta | 2002 | 7.3 |
| Coarctation of Aorta | 2003 | 7.6 |
| Hypoplastic Left Heart | 1997 | 3.5 |
| Hypoplastic Left Heart | 1998 | 3.3 |
| Hypoplastic Left Heart | 1999 | 3.9 |
| Hypoplastic Left Heart | 2000 | 3.6 |
| Hypoplastic Left Heart | 2001 | 3.7 |
| Hypoplastic Left Heart | 2002 | 4.0 |
| Hypoplastic Left Heart | 2003 | 2.8 |
| Tetralogy of Fallot | 1997 | 4.9 |
| Tetralogy of Fallot | 1998 | 2.2 |
| Tetralogy of Fallot | 1999 | 4.5 |
| Tetralogy of Fallot | 2000 | 3.2 |
| Tetralogy of Fallot | 2001 | 3.3 |
| Tetralogy of Fallot | 2002 | 2.8 |
| Tetralogy of Fallot | 2003 | 2.2 |
| d-Transposition | 1997 | 1.6 |
| d-Transposition | 1998 | 3.8 |
| d-Transposition | 1999 | 5.4 |
| d-Transposition | 2000 | 4.0 |
| d-Transposition | 2001 | 3.1 |
| d-Transposition | 2002 | 3.8 |
| d-Transposition | 2003 | 2.0 |
Record Count: 28
Data Sources
Utah Birth Defect Network;
Page Content Updated On 08/18/05,
Published on 10/28/08
