Title
Management of Neonates and Infants with Ebstein Anomaly
Authoring Organization
Publication Month/Year
April 18, 2024
Last Updated Month/Year
April 30, 2024
Document Type
Consensus
Country of Publication
US
Document Objectives
Symptomatic neonates and infants with Ebstein Anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management. Risk stratification is essential in neonates and infants with Ebstein anomaly. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Unstable neonates are most commonly palliated with the Starnes procedure. Stable neonates should undergo ductal closure. Stable neonates with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures following Starnes palliation include either single ventricle palliation or biventricular repair strategies.
Target Patient Population
Neonates and Infants with Ebstein Anomaly
Target Provider Population
Pediatric Surgeons, Pediatricians, and Other Clinicians Caring for Neonates and Infants with Ebstein Anomaly
Inclusion Criteria
Male, Female, Infant
Health Care Settings
Ambulatory, Hospital, Outpatient, Operating and recovery room
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Management
Diseases/Conditions (MeSH)
D004437 - Ebstein Anomaly
Keywords
Ebstein Anomaly
Source Citation
Konstantinov IE, Chai P, Bacha E, Caldarone CA, Da Silva JP, Da Fonseca Da Silva L, Dearani J, Hornberger L, Knott-Craig C, Del Nido P, Qureshi M, Sarris G, Starnes V, Tsang V. The American Association for Thoracic Surgery (AATS) 2024 Expert Consensus Document: Management of neonates and infants with Ebstein anomaly. J Thorac Cardiovasc Surg. 2024 Apr 18:S0022-5223(24)00360-X. doi: 10.1016/j.jtcvs.2024.04.018. Epub ahead of print. PMID: 38685467.