54
Specific Conditions
4.3.1. Ebstein Anomaly
COR LOE
Recommendations
2b B-NR
9. In adults with Ebstein anomaly and severe RV dysfunction
in whom there is concern that the RV will not tolerate
supporting the entire stroke volume, a bidirectional superior
cavopulmonary (Glenn) anastomosis at the time of tricuspid
valve surgery may be considered in carefully selected
individuals to improve cardiac output through ventricular
unloading.
2b C-LD
10. In adults with Ebstein anomaly who have an ASD/patent
foramen ovale, and cyanosis or paradoxical embolism,
isolated closure may be considered after hemodynamic
assessment to improve clinical outcomes.
(cont'd)
Table 26. Ebstein Anomaly: Routine Follow-Up and Testing
Intervals
Type of Follow-Up
or Testing
Physiological
Stage A* (mo)
Physiological
Stage B* (mo)
Physiological
Stage C* (mo)
Physiological
Stage D* (mo)
Outpatient ACHD
cardiologist
12–24
12 6–12
3–6
Electrocardiogram 12–24
12 12 12
Transthoracic
echocardiogram
†
12–24
12–24
12 12
For recommendations about timing of CMR imaging, see Section 4.3.1 supportive text for
recommendation #2.
Modified with permission from Stout et al. Copyright © 2018 American Heart Association, Inc.
and American College of Cardiolog y Foundation.
* See Section 2.2 for details on the ACHD anatomic and physiological classification system.
†
Routine transthoracic echocardiogram may be unnecessary in a year when CMR imaging is
performed, unless clinical indications warrant otherwise.
ACHD indicates adult congenital heart disease; and CMR, cardiovascular magnetic resonance.
