Newborn Management of Prenatally Diagnosed Tetralogy of Fallot and Risk of Ductal Dependency Clinical Pathway

Pathway Background and Objectives

The diagnosis of Tetralogy of Fallot carries a wide range of treatment possibilities based on the detailed cardiac anatomy. Neonates that are ductal dependent for adequate pulmonary blood flow can be at high risk for severe hypoxemia after delivery if not immediately recognized. Babies that are not ductal dependent may be discharged home if they have normal oxygen saturations, and elective surgery is planned at several months of age. Because the ductus arteriosus is open in utero and oxygen saturations cannot be predicted, determination of ductal dependency prenatally can be challenging. Secondary signs on a fetal echocardiogram can be helpful in predicting ductal dependency, such as flow across the pulmonary valve, size of the pulmonary arteries, and direction of flow across the ductus arteriosus. It is not, however, until after a baby is born that ductal dependency can be accurately determined. Based on the prenatal signs, we can estimate the degree of risk in order to guide our postnatal management and assessment.

The objectives of this clinical pathway are to:

Ensure maximal safety for babies with potentially ductal dependent Tetralogy of Fallot
Optimize the use of echocardiogram and available resources
Decrease ambiguity and variability in evaluation and treatment of newborns with prenatally diagnosed Tetralogy of Fallot in the Neonatal Intensive Care Unit or Well Baby Nursery
Minimize unnecessary separation of newborn babies from their mothers

Algorithm Educational Module

Quality Metrics

Number patients managed with the pathway
Percentage of patients on the pathway for which the pathway was followed appropriately
Number of patients requiring surgery for Tetralogy of Fallot prior to discharge
- Stratified by TET risk score
Number of patients who had umbilical lines placed or who were made NPO who did not require surgery prior to discharge
- Stratified by TET risk score
Number of patients who had prostaglandins started who did not require surgery prior to discharge
- Stratified by TET risk score
Length of stay (days)
Number of echocardiograms performed either prior to discharge or prior to surgery

Key References

Quartermain MD, Glatz AC, Goldberg DJ, Cohen MS, Elias MD, Tian Z, Rychik J. Pulmonary outflow tract obstruction in fetuses with complex congenital heart disease: predicting the need for neonatal intervention. Ultrasound Obstet Gynecol. 2013 Jan;41(1): 47-53.
Tuo G, Volpe P, Buffi D, De Robertis V, Marasini M. Assessment of the ductus arteriosus in fetuses with tetraology of Fallot and the implication for postnatal management. Congenit Heart Dis. 2014 Sept,9(5):382-390.
Donofrio MT, Moon-Grady AJ, Hornberger LK, et al. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. 2014 May;129(21):2183-242.

Disclaimer

The clinical pathways in the above links have been developed specifically for use at Connecticut Children’s and are made available publicly for informational and/or educational purposes only. The clinical pathways are not intended to be, nor are they, a substitute for individualized professional medical judgment, advice, diagnosis, or treatment. Although Connecticut Children’s makes all efforts to ensure the accuracy of the posted content, Connecticut Children’s makes no warranty of any kind as to the accuracy or completeness of the information or its fitness for use at any particular facility or in any individual case.