Guidelines for the Management of Adults with Congenital Heart Disease (ESC, 2020)
The European Society of Cardiology (ESC) updated their 2010 guidelines on the management of adult congenital heart disease (ACHD) in 2020. [16, 17] Recommendations on the management of Ebstein anomaly are outlined below.
Surgical repair is recommended in patients with severe tricuspid regurgitation and symptoms or objective deterioration of exercise capacity. Surgical repair should be performed by a congenital cardiac surgeon with expertise in Ebstein surgery.
If tricuspid valve surgery is indicated, atrial septal defect/patent foramen ovale closure is recommended at the time of valve repair if it is expected to be hemodynamically tolerated.
In patients with symptomatic arrhythmias or electrocardiographic (ECG) preexcitation, electrophysiology testing followed by ablation therapy, if feasible, or surgical treatment of the arrhythmias in the case of planned heart surgery is recommended.
Guidelines for the Management of Adults with Congenital Heart Disease (ACC/AHA, 2018)
The American College of Cardiology/American Heart Association (ACC/AHA) 2018 Guidelines for the Management of Adults with Congenital Heart Disease provides information regarding the diagnosis, workup, and treatment of patients with Ebstein anomaly. [18, 19] Recommendations for the evaluation and management of patients with Ebstein anomaly are summarized below.
Workup
Chest radiography, echocardiography and electrocardiography (ECG) are recommended in the evaluation of Ebstein anomaly in adult patients.
Pulse oximetry, Doppler transesophageal echocardiography (TEE), electrophysiological studies, Holter monitoring, and coronary angiography can be useful in the diagnostic evaluation of Ebstein anomaly
Data obtained from cardiovascular magnetic resonance (CMR) can inform clinical care and surgical planning or decision-making, because CMR data correlate well with intraoperative findings. Two-dimensional and 3D TEE can better define the anatomy and function of the tricuspid valve before surgery.
Management
Anticoagulation, catheter ablation, and surgical intervention have a role in the management of adult patients with Ebstein anomaly.
A patent foramen ovale, otherwise usually considered normal, may have significant impact in Ebstein anomaly. Accessory pathways and arrhythmias are relatively common. Patient surveillance and management varies depending on age, severity of the lesion, and associated abnormalities. Treatments include medical and surgical therapy for patients with manifest symptoms as well as catheter-based structural and electrophysiological interventions when indicated.
Adults with Ebstein anomaly and ventricular preexcitation can have multiple accessory pathways and a high risk of sudden cardiac death; concealed pathways are also common. High prevalence of atrial tachyarrhythmia which, in the presence of ventricular preexcitation increase the risk of lethal ventricular arrhythmias. It may be reasonable to assess for arrhythmia substrates and proceed with catheter ablation if identified, before tricuspid valve surgery.
Early surgery, before heart failure or right ventricular (RV) systolic dysfunction ensues, results in improvement of symptoms and functional ability, and prevents or delays worsening symptoms. Surgical interruption of accessory pathways is largely reserved for patients who have failed attempts at catheter ablation.
Bidirectional cavopulmonary shunt in the adult patient is usually reserved for patients with severe RV dysfunction. Preoperative invasive hemodynamic assessment and feasibility of applying the bidirectional cavopulmonary shunt becomes more important in older patients, especially in those with long standing systemic hypertension with diastolic dysfunction and elevated pulmonary artery pressures.
