Antenatal Risk of Coarctation of the Aorta (ARCH)

Pathway Background and Objectives

Coarctation of the aorta can develop as the ductus arteriosus closes after delivery. Because the ductus arteriosus is open in utero, diagnosis prenatally can be challenging. Coarctation of the aorta can never be completely ruled out in the presence of a patent ductus arteriosus, but secondary signs can be present on fetal evaluation to increase suspicion. A hypoplastic aortic arch or reversal of flow in the transverse arch can be reliable in predicting ductal dependency. Other prenatal signs of potential coarctation, such as ventricular size discrepancy, have a 60-70% sensitivity of coarctation and ductal dependency. 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 pathway are to:

• Ensure maximal safety for babies with potentially dependent coarctation of the aorta
• Optimize the use of echocardiogram with the available resources
• Decrease variability in the assessment process of newborns with suspicion of coarctation in the NICU or well- baby nursery
• Decrease multiple visits for newborns for cardiac evaluation
• Minimize delay in discharge of newborn babies and their mothers

Algorithm

Download the ARCH Pathway Algorithm

Quality Metrics

• Percentage of eligible patients treated per pathway
• Percentage of eligible patients with order set usage
• Number of patients requiring surgery for coarctation prior to discharge
  • Stratified by ARCH risk score and presence of coarctation
• Number of patients who had umbilical lines placed who did not require treatment for coarctation of the aorta
  • Stratified by ARCH risk score and presence of coarctation
• Number of patients who received prostaglandins started
  • Stratified by ARCH risk score and presence of coarctation

Educational Module

Download the ARCH Educational Module

Key References

• Franklin O, Burch M, Manning N, Sleeman K, Gould S, Archer N. Prenatal diagnosis of coarctation of the aorta improves survival and reduces morbidity. Heart. 2002 Jan;87(1):67–9.
• Head CE, Jowett VC, Sharland GK, Simpson JM. Timing of presentation and postnatal outcome of infants suspected of having coarctation of the aorta during fetal liHeart. 2005 Aug;91(8):1070-4.
• Donofrio MT, Moon-Grady AJ, Hornberger LK, et al. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation. 2014 May;129(21):2183-242.
• Familiari A, Morlando M, Khalil AA, et al. Risk factors for coarctation of the aorta on prenatal ultrasound: a systematic review and meta-analysis. Circulation. 2017 Feb;135(8):772–785.

Pathway Contacts

• Alicia Wang, MD

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.

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