Critical Congenital Heart Disease Screening

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.

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.

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.

Screening results and documentation

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 arteriosus
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: