Title

Management of Neonates and Infants with Ebstein Anomaly

Authoring Organization

American Association for Thoracic Surgery

Publication Month/Year

April 18, 2024

Last Updated Month/Year

August 13, 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.