No. DSHS requires reporting of confirmed cases of Critical Congenital Heart Disease (CCHD) conditions only. These are usually diseases that present with cyanosis at birth. The CCHD screening core conditions are: 

1. Coarctation of the aorta
2. Double-outlet right ventricle
3. Ebstein's anomaly
4. Hypoplastic left heart syndrome
5. Interrupted aortic arch
6. Pulmonary atresia
7. Single ventricle (not otherwise specified)
8. Tetralogy of Fallot
9. Total anomalous pulmonary venous return
10. D-transposition of the great arteries
11. Tricuspid atresia
12. Truncus arteriosus
13. Other critical cyanotic lesions not otherwise specified

Congenital Heart Disease (CHD) involves problems with the heart's structure that are present at birth. There are many types of congenital heart disease that range from simple defects with no symptoms to complex defects. One example of a common but often not usually critical congenital heart disease is ventricular septal defect, or VSD. 

About 25% of CHDs will be Critical Congenital Heart Disease (CCHD) that causes severe, life-threatening symptoms and requires medical intervention within the first few hours, days, or months of life. The CCHD screening core conditions are: 

1. Coarctation of the aorta
2. Double-outlet right ventricle
3. Ebstein's anomaly
4. Hypoplastic left heart syndrome
5. Interrupted aortic arch
6. Pulmonary atresia
7. Single ventricle (not otherwise specified)
8. Tetralogy anomalous pulmonary venous return
9. D-transposition of the great arteries
10. Tricuspid atresia
11. Truncus arteriosus
12. Other critical cyanotic lesions not otherwise specified

Confirmed cases of these disorders must be reported to the DSHS. Other CHDs do not need to be reported to DSHS. 

Submit a CCHD Reporting Form when a diagnosis of CCHD is confirmed. CCHD conditions are confirmed via diagnostic testing, whether prenatally or postnatally, per Texas Health and Safety Code (HSC), Section 33.015. It is not necessary or a requirement to report the results of the CCHD screen. 

Yes. Once the diagnosis is confirmed, the CCHD reporting form should be completed and submitted to DSHS. On page two of the form, there is a “Diagnosis Timeframe” section that will identify when the CCHD was diagnosed – prenatally or postnatally. 

Yes. Anytime a CCHD diagnosis is confirmed, the CCHD reporting form should be completed and faxed to DSHS. If the infant is diagnosed prior to performing pulse oximetry screening, then the pulse oximetry screening algorithm is not required. On page two of the form, the “Diagnosis Timeframe” section provides an option for “Post-natal diagnosis prior to pulse oximeter screening.” 

DSHS does not have a specific timeframe for reporting confirmed cases of a CCHD condition. Since the care of a newborn with low oxygen saturation should be immediate and provided at the point of care, DSHS did not identify a time critical reporting requirement. However, the case should be reported as near the time of diagnosis (or birth for those diagnosed prenatally) as reasonably possible. Cases not reported quickly have a greater risk that the reporting will not occur.

The CCHD reporting form is the only form that should be submitted to the Newborn Screening Program. The law requires reporting of confirmed CCHD cases only. 

Pulse oximeters used to conduct CCHD newborn screening must meet the standards and accuracy as determined by the Food and Drug Administration for hospital use in newborns per Texas Administrative Code, Section 37.78(c).

There is no separate reimbursement coding for CCHD screening. 

It is now recommended that the newborn be screened for CCHD utilizing pulse oximetry at 24-48 hours of life once they have weaned off of supplemental oxygen. If the newborn is expected to be on supplemental oxygen for an extended period of time, the CCHD pulse oximetry screen should be repeated when the newborn no longer requires supplemental oxygen or at the time of discharge, regardless of oxygen status, unless an echocardiogram has already been performed on the infant during their hospitalization. 

A physician attending a newborn child may delegate the physician's responsibilities related to Newborn Screening to any qualified and properly trained person acting under the physician's supervision (HSC, Section 33.0111(h)). 

The provider who confirms the diagnosed CCHD condition is responsible for submitting the CCHD reporting form to DSHS. A physician attending a newborn child may delegate the physician's responsibilities related to Newborn Screening to any qualified and properly trained person acting under the physician's supervision (HSC, Section 33.0111(h)). 

Scenario 1 – A baby is born under the care of Provider A, who performs the CCHD pulse oximetry screening. The baby has a failed screen and diagnostic testing is ordered by Provider A. Provider A confirms a CCHD condition based on test results. Provider A completes and submits the CCHD reporting form to DSHS.

Scenario 2 – A baby is born under the care of Provider A, who performs the CCHD pulse oximetry screening. The baby has a failed CCHD screen and the baby is transferred to the care of Provider B for additional diagnostic testing. Provider B confirms a CCHD condition. In this case, Provider B completes and submits the CCHD reporting form to DSHS.

Scenario 3 – A baby is born under the care of Provider A, who performs the CCHD pulse oximetry screening. The baby has a failed CCHD screen and the baby is transferred to the care of Provider B for additional diagnostic testing. Provider B confirms a CCHD condition. Provider B completes and submits the CCHD reporting form to DSHS. The baby is then transferred to the care of Provider C for additional treatment or procedures where it is determined that the baby has a different CCHD condition than was originally diagnosed. Provider C then completes and submits an updated CCHD reporting form to DSHS with the new CCHD condition marking the box “Update to a previously reported case.” 

If a subsequent test/procedure identifies a different CCHD condition than was originally submitted, a second CCHD reporting form should be submitted with the box checked to indicate that it is an “Update to a previously reported case.” 

The American Academy of Pediatrics (AAP) policy recommends that screening be done no earlier than 24 hours after birth and prior to discharge from the birth facility. Because of the transition from fetal to neonatal circulation and stabilization of systemic oxygen saturation levels, screening earlier than 24 hours is not as reliable and can produce increased false-positive results (Kemper et al., 2011). If the newborn is to be discharged before 24 hours of age, screening should be done as immediately before discharge as possible.

Babies not screened in the newborn nursery or prior to discharge from a birthing center should be screened as soon as possible, preferably at the first pediatric checkup within a few days of birth. However, CCHD screening does not need to be conducted before discharge or post-discharge if echocardiography has been performed as part of the newborn’s care in the birth facility.

The test used for CCHD screening is a pulse oximetry screen using the CCHD Algorithm. If a screen is positive, an echocardiogram can help confirm the diagnosis of a CCHD condition.