Critical Congenital Heart Disease Screening Program

Critical CHDs, or CCHDs, are types of heart defects that lead to low levels of oxygen in a newborn. Critical CHD’s may be identified by using pulse oximetry screening after birth. This screening is important because some babies born with a Critical CHD appear healthy at first and may be sent home with their families before their heart defect is detected. Babies with a Critical CHD need surgery or other procedures in the first year of life.

In the United States, about 7,200 babies are born every year with 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 CHD

Newborn Screening for Critical CHD involves a simple, bedside test called pulse oximetry. The test estimates the amount of oxygen in the baby’s blood. Low levels of oxygen in the blood can be a sign of Critical CHD.

The test is done using a machine called a pulse oximeter. The nurse places sensors on the baby’s right hand and either foot. The test is painless and takes only a few minutes.

Pulse Oximetry does not replace a complete history or physical examination. It should be used alongside an assessment by your baby’s physician.

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 before they are 24 hours of age. In Maine, it is required by law that every baby be screened for Critical CHD before being discharged.

Pulse Oximetry Screening Results

Pulse oximetry screening is most likely to detect seven of the Critical CHDs. These 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 as these seven and require treatment soon after birth. However, pulse oximetry screening may not detect these heart defects as consistently as the seven listed above.

Parental Refusals of Newborn Screening

In the instance of parental refusal of critical CHD screening, the parental refusal must be stated in writing which then becomes a part of the infant’s medical record. The hospital or birthing center designee, midwife, or principal birthing attendant must ensure that 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 refusal form below.

Refusal forms must be faxed to the Maine CDC – Maternal and Child Health Program, Attn Newborn Health Coordinator at 207-287-4743.

Recording and Reporting CCHD Screening Results

The person who performs the CCHD screening as required by Maine rule must fully and clearly complete the CCHD section of the Newborn Bloodspot Filter Paper card, and document in the infant’s medical record, the fact that the Critical CHD screen was done, including 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 Critical CHD Screening form located below or download the paper form, 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 (below).

For Healthcare Providers

For Families

For more information, please contact the Newborn Health Coordinator:

  • Tel: (207) 287-5357
  • TTY: Maine Relay 711
  • Fax: (207) 287-4743

Maine State Law – relating to Critical Congenital Heart Defects