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common], dyspnoea, fatigue, poor exercise tolerance, chest pain, and peripheral and/or central cyanosis.

4.9.3 Diagnostic work-up See section 3.3 for general principles. Clinical findings may include cyanosis and hepatomegaly. Auscultation findings include a widely split first sound and second sound, serial clicks, third and fourth sound, and a systolic murmur from TR. ECG may show RA hypertrophy, a prolonged PR interval, right bundle branch block (often with a splintered QRS complex), deep Q in II, III, aVF, and V1V4, pre-excitation, low voltage, multiple pathways (AVRT), and supraventricular and ventricular arrhythmias. • Chest X-ray is helpful to follow changes in the heart’s size. • Echocardiography provides information on: anatomy and function of the TV; apical distal displacement of the septal or posterior leaflet (in adults >_0.8 cm/m2 BSA); size of the anterior leaflet; tethering of the septal or posterior TV leaflet on the septum or ventricular wall; size and function of the different cardiac sections (RA, atrialized ventricle, remaining functional RV, and LV); and RVOTO and associated lesions. • CMR has value with regards to prognostication225, and for evaluation before and after surgery, as it offers unrestricted views for assessment and quantification of the dilated right heart, RV function, and TV function.

4.9.4 Surgical/catheter interventional treatment Clinical symptoms determine the treatment. Conservative therapy can alleviate symptoms temporarily and create a beneficial basis for the following operation.226 Oral anticoagulation is recommended for patients with a history of paradoxical embolism or AF. In the presence of an increased thromboembolic risk or a RL shunt, oral anticoagulation may be considered. Symptomatic rhythm disorders can be treated conservatively or, preferably, with EP intervention.227 Transcatheter access to right-sided accessory pathways and the slow pathway in AV node reentry may be hindered by TV surgery, such that it may be reasonable to assess for arrhythmia substrates and proceed with catheter ablation, if identified, before surgery. Occasionally, there may be an indication to close the atrial communication in isolation. However, this needs to be discussed carefully, as it may lead to a further increase in right heart pressures and a decrease in systemic cardiac output. Surgical repair remains challenging and should only be performed by surgeons with specific experience in this lesion. TV repair, if feasible, is preferred over TV replacement (with closure of an associated interatrial communication). If the RV is too small for repair or RV dysfunction has developed, an additional bidirectional cavopulmonary (Glenn) anastomosis may be considered in adults with preserved LV function when LA pressure and LV end diastolic pressure are not elevated.228 In patients with failed repair, or in severe biventricular dysfunction, heart transplantation may be the only option. The previously high operative mortality (>25%) has fallen to <6% in specialized centres. Over 90% of patients operated on by an experienced surgeon survive >10 years, many in functional class I or II. Late fatalities are probably due to arrhythmias. In a large series, survival free of late reoperation was 86%, 74%, 62%, and 46% at 5, 10, 15, and 20 years, respectively.229 4.9.5 Follow-up recommendations Regular follow-up (at least yearly) is required in all patients in specialized ACHD centres. Typical post-operative residual anomalies to look for are persisting or new TR, the usual complications after valve replacement, failure of RV or LV, residual atrial shunts, arrhythmias, and higher-grade AV blocks.

Reintervention may become necessary for recurrent TR and failure of prosthetic valves.

4.9.6 Additional considerations

• Exercise/sports: patients without residual anomalies can usually lead normally active lives without restriction, except for extensive static

Recommendations for intervention in Ebstein anomaly

Recommendations Classa Levelb

Indications for surgery Surgical repair is recommended in patients with severe TR and symptoms or objective deterioration of exercise capacity. I C It is recommended that surgical repair is performed by a congenital surgeon with specific experience in Ebstein surgery. I C If there is an indication for TV surgery, ASD/ PFO closure is recommended at the time of valve repair if it is expected to be haemodynamically tolerated. I C Surgical repair should be considered regardless of symptoms in patients with progressive right heart dilation or reduction of RV systolic function. IIa C Indications for catheter intervention In patients with symptomatic arrhythmias, or pre-excitation on the ECG, electrophysiologic testing followed by ablation therapy, if feasible, or surgical treatment of the arrhythmias in the case of planned heart surgery is recommended. I C In the case of documented systemic embolism, probably caused by paradoxical embolism, isolated device closure of ASD/PFO should be considered but requires careful evaluation before intervention to exclude induction of RA pressure increase or fall in cardiac output. IIa C If cyanosis (oxygen saturation at rest <90%) is the leading problem, isolated device closure of ASD/PFO may be considered but requires careful evaluation before intervention to exclude induction of RA pressure increase or fall in cardiac output. IIb C

ASD = atrial septal defect; ECG = electrocardiogram; PFO = patent foramen ovale; RA = right atrium/atrial; RV = right ventricle/ventricular; TR = tricuspid regurgitation; TV = tricuspid valve. aClass of recommendation. bLevel of evidence. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026