Ebstein Anomaly Treatment & Management

Ebstein anomaly presents with a spectrum of congenital abnormalities of the tricuspid valve and the right ventricle. The age of presentation with symptoms is variable, and a wide range of treatment options is available. Treatment of Ebstein anomaly is complex and dictated mainly by the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Transfer to a cardiothoracic surgery unit if necessary.

Treatment options include medical therapy, radiofrequency ablation, and surgical therapy.

Administer antibiotic prophylaxis for infective endocarditis

Pharmacotherapy for heart failure includes the use of angiotensin-converting enzyme (ACE) inhibitors, diuretics, and digoxin.

Arrhythmia treatment includes antiarrhythmic drugs or radiofrequency ablation of the accessory pathways. Curative therapy of supraventricular tachycardia (SVT) with radiofrequency ablation is currently the treatment of choice; however, the success rate is lower than that in patients without significant structural heart disease. Factors associated with lower likelihood of success include the following:

• Accessory pathways located along the atrialized right ventricle

• Multiple accessory pathways

• Complex geometry of the pathways

• Abnormal morphology of the endocardial action potentials in this region

Consultations

Consult an electrophysiologist and cardiac surgeon for the management of patients with Ebstein anomaly.

Diet and activity

A low-sodium diet is recommended for symptomatic relief from fluid overload. Activity may be allowed as tolerated.

Surgical care includes correction of the underlying tricuspid valve and right ventricular abnormalities, correction of any associated intracardiac defects, palliative procedures in early days of life as a bridge to more definitive surgical treatment later, and surgical treatment of associated arrhythmias. Complete repair of Ebstein anomaly in symptomatic neonates has been shown to be feasible, with good early and late survival and excellent functional status. ^[10] In patients aged 50 years or older with Ebstein anomaly, surgery is associated with good long-term survival and improved functional status, although long-term survival might be improved by performing surgery earlier. ^[11]

The trend is to perform surgery earlier rather later in the course of heart failure. Indications for surgery are generally as follows:

• New York Heart Association (NYHA) class I-II heart failure with worsening symptoms or with a cardiothoracic ratio of 0.65 or greater ^[12]

• NYHA class III-IV heart failure

• History of paradoxical embolism

• Significant cyanosis with arterial O₂ saturation of 80% or less and/or polycythemia with hemoglobin of 16 g/dL or more

• Arrhythmias refractory to medical and radiofrequency ablation

Various approaches are available to treat structural abnormalities. Tricuspid valve repair is preferred over valve replacement, and bioprosthetic valves are preferred over mechanical prosthetic valves. The atrialized portion of the right ventricle can be resected surgically, and the markedly dilated, thin-walled right atrium can be resected. Associated septal defects may be closed.

In a study of 27 consecutive cone reconstructions to treat severe tricuspid valve (TV) regurgitation associated with Ebstein anomaly, Ibrahim and colleagues retrospectively compared the clinical characteristics, echocardiography, magnetic resonance imaging, and exercise data between preoperative baseline and follow-up. Patients showed improvements in left ventricle filling and objective exercise capacity. The investigators concluded that in patients with severe regurgitation associated with Ebstein anomaly, cone reconstruction of TV offers effective repair. ^[13]

Da Silva's cone repair is a technique for the surgical reconstruction of the tricuspid valve and the right ventricle in Ebstein anomaly. In one study, investigators evaluated echocardiographic studies and magnetic resonance imaging (MRI) before and after Da Silva's cone repair for Ebstein anomaly in 20 patients. They found that Da Silva's cone repair created excellent valve function; the size of the right ventricle decreased and the antegrade net stroke volume increased 6 months post-surgery. ^[14]

Palliative procedures include creation of atrial septal defect, closure of tricuspid valve with plication of the right atrium, and maintenance of pulmonary blood flow through aortopulmonary shunt. Palliative procedures usually are reserved for severely ill infants with otherwise dire prognosis.

Left ventricular dysfunction should not be considered a contraindication to tricuspid valve surgery. In these patients, although early mortality is greater with tricuspid valve surgery, the late results are favorable and left ventricular function seems to improve postoperatively. ^{[3, 15]}

Functional status improves after surgery.

Surgical treatments of arrhythmias include the following:

• Ablation of the accessory pathways

• Modified right atrial maze procedure for atrial arrhythmias.

• In the presence of atrial fibrillation, the addition of left atrial Cox Maze III procedure can reduce the risk of recurrent atrial fibrillation

Cardiac transplantation is appropriate in selected patients.

Outpatient follow-up is directed toward the following:

• Assessment of general symptoms (eg, fatigue, lassitude)

• Assessment for development of bacterial endocarditis with careful history and examination for signs of bacterial endocarditis, such as fever, weight loss, anorexia, night sweats, splinter hemorrhages, clubbing, Roth spots, Janeway lesions, Osler nodes, splenomegaly, and hematuria

• Assessment of signs of worsening heart failure, such as weight gain, ankle edema, and rales

• Assessment for arrhythmias by history of symptoms, such as faintness or syncope

• Periodic chest radiographs to assess for worsening cardiomegaly

• Evaluation and adjustment of medications