Also, there are cohort series of Ebstein patients to inform decisions.S4.3.4-6,S4.3.4-7,S4.3.4-10 Surgical repair generally consists of tricuspid valve repair (preferred when feasible) or replacement, selective plication of atrialized right ventricle, re- duction atrioplasty, arrhythmia surgery, and/or closure of atrial level shunt. Surgery may result in improvement of symptoms and functional ability, and prevent or delay worsening symptoms. 6. Adults with Ebstein anomaly and ventricular pre- excitation often have multiple accessory path- ways, which are associated with a higher risk of SCD. Surgical interruption of accessory pathways is largely reserved for patients who have failed attempts at catheter ablation. High-risk path- ways are those with an increased risk of SCD, largely related to VF resulting from rapidly con- ducting AF. Definition and discussion of high-risk Table 26. Ebstein Anomaly: Routine and Follow-Up and Testing Intervals Frequency of Routine Follow-Up and Testing Physiological Stage A* (mo) Physiological Stage B* (mo) Physiological Stage C* (mo) Physiological Stage D* (mo) CXR As needed As needed 12 24 12 24 Pulse oximetry 24 12 Each visit Each visit Holter monitor As needed As needed 24 12 24 *See Tables 3 and 4 for details on the ACHD AP classification system. Routine TTE may be unnecessary in a year when CMR imaging is performed unless clinical indications warrant otherwise. CMR may be indicated for assessment of right ventricular size and function. Baseline study is recommended with periodic follow-up CMR, with frequency of repeat imaging determined by anatomic and physiological findings. CCT may be used if CMR is not feasible; the frequency should be weighed against radiation exposure. 6-minute walk test or CPET, depending on clinical indication. ACHD indicates adult congenital heart disease; CCT, cardiac computed tomography; CMR, cardiovascular magnetic resonance imaging; CPET, cardiopulmonary exercise test; CXR, chest x-ray; ECG, electrocardiogram; and TTE, transthoracic echocardiogram. 