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4.3.1. Ebstein Anomaly
COR LOE
Recommendations
Diagnostic
1 B-NR
1. In adults with Ebstein anomaly, an electrophysiological study
is recommended for patients with symptomatic arrhythmia or
asymptomatic ventricular preexcitation to assess for substrates
for ablation, including multiple or high-risk accessory
pathways.
2a B-NR
2. In adults with Ebstein anomaly, CMR imaging can be useful
to determine anatomy, RV dimensions, and systolic function
to guide surgical planning and management.
2a B-NR
3. In adults with Ebstein anomaly, electrophysiological study
(and catheter ablation, if needed) can be useful before
tricuspid valve surgery to improve clinical outcomes.
2a C-LD
4. In adults with Ebstein anomaly, if transthoracic
echocardiography is inadequate to guide surgical planning,
transesophageal echocardiography can be beneficial to
characterize tricuspid valve morpholog y and function.
2b B-NR
5. In adults with Ebstein anomaly and severe RV dysfunction in
whom there is concern that the RV will not support the entire
stroke volume, preoperative diagnostic cardiac catheterization
may be considered to assess candidacy for a bidirectional
superior cavopulmonary (Glenn) anastomosis.
erapeutic
1 B-NR
6. In adults with Ebstein anomaly and at least moderate
tricuspid regurgitation in the presence of heart failure,
worsening exercise capacity, or progressive RV systolic
dysfunction, surgical intervention is recommended to restore
valve function and augment cardiac output.
1 B-NR
7. In adults with Ebstein anomaly and high-risk accessory
pathway conduction or multiple accessory pathways, catheter
ablation is recommended to reduce the risk for SCD.
2a B-NR
8. In asymptomatic adults with Ebstein anomaly and at
least moderate tricuspid regurgitation in the presence of
progressive RV enlargement, systemic desaturation from
a right-to-left atrial shunt, recurrent arrhythmias, and/or
paradoxical embolism, surgical intervention can be beneficial
to improve clinical outcomes.
4.3. Right-Sided Lesions
