Critical Congenital Heart Disease Screening
Critical Congenital Heart Disease (CCHD), involve types of heart defects that lead to low levels of oxygen in a newborn. This screening is important because some babies born with CCHD appear healthy at first but will need surgery or other procedures in the first year of life.
In the U.S. about 7,200 babies are born every year with a critical congenital heart defects. Undiagnosed babies are at risk of having serious complications within the first few days or weeks of life and often require emergency care.
Newborn Screening for Critical Congenital Heart Diseases
Newborn screening for CCHD involves a simple, bedside test called pulse oximetry. The test estimates the amount of oxygen in the baby's blood. Low levels of oxygen can be a sign of CCHD. The test is done using a machine called a pulse oximeter. The nurse places sensors on the baby's right hand and either foot. It is painless and takes only a few minutes.
Time of Screening
Screening is done when a baby is at least 24 hours of age, or as late as possible if the baby is to be discharged from the hospital earlier. In Maine, it is required by law that every baby be screened for CCHD before being discharged.
Pulse Oximetry Screening Results
Pulse oximetry screening is likely to detect seven of the CCHDs. The seven defects are hypoplastic left heart syndrome, pulmonary atresia, tetralogy of Fallot, total anomalous pulmonary venous return, transposition of the great arteries, tricuspid atresia, and truncus arteriosus. Other heart defects can be just as severe, but pulse oximetry screening may not detect them as consistently.
Parental Refusals of Newborn Screening
A parental refusal of CCHD screening must be stated in writing and then becomes a part of the infant's medical record. The hospital or birthing center designee, midwife, or principal birthing attendant must ensure the Maine CDC Maternal and Child Health Program is notified in writing of the parental refusal within five days of the infant's birth by using the CCHD parental refusal form (PDF).
Fax refusal forms to the Maine CDC Newborn Health Coordinator at 207-287-4743.
Recording and Reporting CCHD Screening Results
The person who performs the CCHD screening is required by Maine rule to:
- Fully and clearly complete the CCHD section of the Newborn Bloodspot Filter Paper card
- Document in the infant's medical record that the CCHD screen was done and include the date and time when conducted and the results.
If the CCHD screening did not occur prior to shipment of the bloodspot filter paper card, providers must complete the online CCHD Screening form (coming soon) or download the CCHD Reporting Form (PDF), complete and fax to Maine CDC Newborn Health Program.
Presumptive positive results
Suspected or confirmed heart defects, and the diagnostic method used, shall also be reported to the Maine CDC Birth Defects Surveillance Program as required by 22 M.R.S.A. § 1687, and Chapter 280, Rules by submitting the online Reportable Birth Defects Form.
To learn more, contact the Newborn Health Coordinator:
- Tel: (207) 287-5357
- TTY: Maine Relay 711
Families can learn more from the Baby's First Test website.