Critical Congenital Heart Disease

Beginning January 1, 2014, all babies born in Missouri will be screened for critical congenital heart disease. Critical congenital heart disease (CCHD) is the name given to specific congenital heart defects. These defects in the heart occur before birth, cause blood to flow in an abnormal pattern, and may lead to blockage of blood flow throughout the body. If left untreated, these defects can lead to death or can cause serious developmental delay.

CCHD screening is a simple bedside test to determine the amount of oxygen in the baby’s blood. Low oxygen levels can be a sign of CCHD. The test is done using a machine called a pulse oximeter. The pulse oximeter is an infrared light sensor that is gently wrapped around the baby’s hand or foot. Light passes through the skin and tissue and is read by the sensor to estimate the blood oxygen level. The test is painless and takes just a few minutes.

If the results are “negative,” the baby’s test results did not show signs of a CCHD. This type of screening test does not detect all CCHDs, so it is possible to still have a CCHD or other congenital heart defect with a negative screening result. If the results are “positive” (“fail” or out-of-range result), it means that the baby’s test results showed low levels of oxygen in the blood, which can be a sign of a CCHD. This does not always mean that the baby has a CCHD. It just means that more testing is needed.

Pulse oximetry screening is most likely to detect seven specific CCHDs. These include:

Laws & Regulations

Chloe's Law -- critical congenital heart disease screening, requirements--rulemaking authority.
191.334.

  1. This section shall be known and may be cited as "Chloe's Law".
  2. Effective January 1, 2014, every newborn infant born in this state shall be screened for critical congenital heart disease in accordance with the provisions of this section.
  3. Every newborn delivered on or after January 1, 2014, in an ambulatory surgical center, birthing center, hospital, or home shall be screened for critical congenital heart disease with pulse oximetry or in another manner as directed by the department of health and senior services in accordance with the American Academy of Pediatrics and American Heart Association guidelines. Screening shall occur prior to discharge if delivery occurs in a facility. If delivery occurs in a home, the individual performing the delivery shall perform the screening within forty-eight hours of birth. Screening results shall be reported to the parents or guardians of the newborn and the department of health and senior services in a manner prescribed by the department for surveillance purposes. The facility or individual shall develop and implement plans to ensure that newborns with positive screens receive appropriate confirmatory procedures and referral for treatment as indicated.
  4. The provisions of this section shall not apply if a parent or guardian of the newborn objects to the screening on the grounds that it conflicts with his or her religious tenets and practices. The parent or guardian of any newborn who refuses to have the critical congenital heart disease screening administered after notice of the requirement for screening shall document the refusal in writing. Any refusal of screening shall be reported to the department of health and senior services in a manner prescribed by the department.
  5. The department of health and senior services shall provide consultation and administrative technical support to facilities and persons implementing the requirements of this section including, but not limited to, assistance in:
    1. Developing and implementing critical congenital heart disease newborn screening protocols based on the American Academy of Pediatrics and American Heart Association guidelines;
    2. Developing and training facilities and persons on implementation of protocols;
    3. Developing and distributing educational materials for families; and
    4. Implementing reporting requirements.
  6. Any rule or portion of a rule, as that term is defined in section 536.010, that is created under the authority delegated in this section shall become effective only if it complies with and is subject to all of the provisions of chapter 536 and, if applicable, section 536.028. This section and chapter 536 are nonseverable and if any of the powers vested with the general assembly pursuant to chapter 536 to review, to delay the effective date, or to disapprove and annul a rule are subsequently held unconstitutional, then the grant of rulemaking authority and any rule proposed or adopted after August 28, 2013, shall be invalid and void.
    (L. 2013 S.B. 230)

CCHD Rules 19 CSR 40-12.010

Publications & Manuals

Additional Resources

General Information

Resources for Parents

Resources for Health Care Providers