Promoting and protecting the health and safety of all Idahoans

Critical Congenital Heart Disease (CCHD) Screening

During the 2018 Idaho legislative session, rules were added to existing Idaho Administrative Code 16.02.12 - Procedures and Testing to be Performed on Newborn Infants, to include screening for critical congenital heart disease (CCHD) using pulse oximetry. Effective July 1, 2018 screening for CCHD is mandated for all Idaho newborns.

About CCHD Screening

What is critical congenital heart disease (CCHD)?

Critical congenital heart disease (CCHD) is one of the conditions found through newborn screening. CCHD refers to a group of heart defects that can be serious and life-threatening. These defects can include structural problems in the heart or problems with blood flow in the heart. These problems range from mild to severe. CCHD is often treatable, if caught early.

Congenital heart defects are the most common birth defect and impact approximately 8 out of every 1,000 babies born. Of these 8 babies, approximately 25% (about 2 per 1,000 babies) are born with heart defects that are considered critical and require immediate intervention. In Idaho, it's estimated that about 55 babies are born each year with CCHD.

Why is CCHD screening done?

Babies born with CCHD may appear healthy at first and may be sent home before their heart defect is detected. These babies are at risk of experiencing serious complications within the first few days or weeks of life and often need emergency care. Screening identifies some of these babies so they can receive immediate care and treatment. Prompt care may prevent disability or death early in life.

Who is screened for CCHD?

As of July 1, 2018, all newborn babies in Idaho shall be screened for CCHD. 

When is the screening done?

Screening must be done when the baby is at least 24 hours of age, or as late as possible if the baby will be discharged from the hospital before 24 hours of age.

How is the CCHD screening done?

Healthcare professionals check for CCHD by using pulse oximetry (or "pulse ox"). Pulse oximetry is a painless, noninvasive test that measures the amount of oxygen in your baby's blood.

What if my baby's pulse ox reading is low?

This means that the oxygen levels in your baby's blood are low, which can have many causes. An out-of-range screening result does not necessarily mean that your baby has CCHD. However, since some babies do have the condition, it is very important to talk about next steps with the health professionals at the facility or birth center where your baby was born.

Because the harmful effects of CCHD can develop shortly after birth, follow-up testing should be completed as soon as possible to determine whether or not your baby has a heart condition. 

Please watch the following short video for more information about CCHD screening

Critical congenital heart disease (CCHD) screening has been recommended nationally by the Secretary for Health and Human Services and pulse oximetry screening for CCHD is endorsed by the American Heart Association, American Academy of Pediatrics, and American College of Cardiology. Congenital heart disease is the most common birth defect and may be detected during the prenatal or postnatal period. Failing to detect CCHD may lead to serious events such as cardiogenic shock or death. Survivors who present late are at greater risk for neurologic injury and subsequent developmental delay. Early detection of CCHD can potentially improve the prognosis and decrease the mortality and morbidity rate of affected infants. Pulse oximetry has been investigated and proven to be successful in detecting some forms of CCHD in newborns.

CCHD Screening Methods

As of July 1, 2018, all Idaho newborns must be screened for CCHD, including newborns born outside of a hospital or birthing facility. Timing is important in regard to CCHD screening. It is recommended that pulse oximetry screening be done in conjunction with other standard-of-care newborn screening that requires the infant be at least 24 hours of age (such as metabolic or hearing screening). A pulse oximeter is used to measure the percentage of hemoglobin in the blood that is saturated with oxygen.

In contrast to blood-based newborn screens such as those for metabolic disorders in which testing is performed at the state's laboratory and providers are notified of the results, the CCHD screen is a point-of-care test. Point-of-care testing refers to those tests administered outside of a laboratory but close to the site of direct delivery of medical care for a patient. Intervention will take place at the hospitals and birthing facilities if the infant fails the pulse oximetry test. All hospitals, birthing centers, and midwives should have a written plan in place as part of their protocol to guide them if a newborn fails the CCHD screen.

The following algorithm was developed to show the steps in CCHD screening.

Printable Version

Pulse oximetry screening should not replace obtaining a complete family health history and pregnancy history, nor should it replace completing a physical examination, which can sometimes detect a CCHD before the newborn develops low levels of oxygen (hypoxemia) in the blood. 

Types of CCHD Pulse Oximetry Can Detect

  • Coarctation of the aorta

  • Double outlet right ventricle

  • Ebstein anomaly

  • Hypoplastic left heart syndrome

  • Interrupted aortic arch

  • Pulmonary atresia

  • Single ventricle

  • Tetralogy of Fallot

  • Total anomalous pulmonary venous return

  • d-Transposition of the great arteries

  • Tricuspid atresia

  • Truncus ateriosus

  • Other CCHDs requiring treatment in the first year of life

While not the primary focus of screening, many conditions other than CCHD may be detected via pulse oximetry testing as they present with hypoxemia.

Failed Screens

A CCHD screening is considered failed if

  • Any oxygen saturation measure is <90% in the initial screen or in repeat screens,

  • Oxygen saturation is <95% in the right hand and foot on three measures, each separated by one hour, or

  • A >3% absolute difference exists in oxygen saturation between the right hand and foot on three measures, each separated by one hour. 

Newborns who fail the screen should have an evaluation for the causes of hypoxemia. Typically, this will include an echocardiogram, but if a reversible cause of hypoxemia is identified and appropriately treated, the echocardiogram may not be necessary. The newborn's pediatrician should be notified immediately and the newborn might need to be seen by a cardiologist.

Passed Screens

Any screening with an oxygen saturation measure that is ≥95% in the right hand or foot with ≤3% absolute difference between the right hand or foot is considered a passed screen and the screening would end. Pulse oximetry screening does not detect all CCHDs, so it is possible for a newborn with a passing screening result to still have a CCHD or other CHD. 

Help Reduce False Positive Screens

  • Screen the newborn while they are alert

  • Screen the newborn when they are at least 24 hours old

Documentation of the CCHD Screening

Hospitals, birthing facilities, and midwives are required to document the CCHD screening results on the birth certificate as part of the Idaho Vital Records System. Often, the screening results are initially captured in an electronic health record (EHR) or some other charting mechanism as part of the newborn's medical record. These results must be entered into the electronic birth certificate (EBC) system or on a hard-copy of the birth certificate. 

The following fields should be completed:

  • Final CCHD screening result
  • Pulse oximetry results for right hand, foot, and age in hours at time of screen
  • Pulse oximetry results for repeated screens, up to 3 times per the algorithm
  • Reason for not screened
  • Action taken if screening was failed 

Images of the EBC system:




 

Please watch the following instructional videos for more information about CCHD screening

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Contact Us

Business Hours: 

Monday - Friday 8:00 a.m. - 5:00 p.m. (MST)

 

Mailing Address:

Idaho Newborn Screening Program

PO BOX 83720

Boise, ID 83720-0036

 

Shipping Address:

Idaho Newborn Screening Program

450 W. State St., 4th Floor

Boise, ID 83720-0036

 

Program Main Phone: (208) 334-5962

Fax: (208) 334-4946