Critical Congenital Heart Disease (CCHD)

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Information for Providers

What counts as Critical Congenital Heart Disease or CCHD?

It is estimated that more than 100 babies are born in Utah with one of these CCHD diagnoses each year. Although there is no perfect definition, there is a broad agreement that CCHD is one of the following diagnoses. HRSA and the Centers for Disease Control have identified:

  • Coarctation of the Aorta
  • Double Outlet Right Ventricle
  • Ebstein’s Anomaly
  • Interrupted Aortic Arch
  • Hypoplastic Left Heart Syndrome
  • Pulmonary Atresia
  • Single Ventricle
  • Tetralogy of Fallot
  • Total Anomalous Pulmonary Venous Return
  • Transposition of the Great Arteries
  • Tricuspid Atresia
  • Truncus Arteriousus

How do you do the screening?

We recommend using the screening protocol endorsed by the US Secretary of Health and Human Services and the American Academy of Pediatrics. This approach uses a “Two Sites, Three Strikes” approach. Pulse oximetry measurements are made in the right hand and one foot. A baby can have as many as three chances to pass the screening unless one of the oxygen saturations is <90%.

Learn more about the screening process and how to respond to a failed screening.

What information is being reported?

Birth facilities and midwives will record the results of screening to the Utah Department of Health on the birth certificate. Pulse oximetry has its lowest false positive rate if performed 24 hours after birth or later. The vital records clerks at each birthing facility will be trained to locate the CCHD screening results and record one of three options (PASS, FAIL, NOT SCREENED) on the birth certificate.

Which babies should be screened?

Every baby born in Utah should have pulse oximetry screening. Most babies will be screened in the first 24 -48 hours following birth. Some babies will need to be screened at a different time.

  • Babies who are on supplemental oxygen should have pulse oximetry screening deferred until they no longer need supplemental oxygen. Babies being discharged home from the hospital on supplemental oxygen should have pulse oximetry screening close to the time of discharge from the hospital. Results of the CCHD screening should be recorded in the chart. This process allows screening for CCHD at a time when the results can be accurately interpreted.

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