Guidelines Summary
2018 AHA/ACC guidelines for adults with Ebstein anomaly
The American Heart Association/American College of Cardiology (AHA/ACC) released updates to their 2008 guideline for the management of adults with congenital heart disease (CHD) in August 2018. [55, 56] Their recommendations for adults with Ebstein anomaly are outlined below.
The AHA/ACC classifies the CHD anatomy of Ebstein anomaly as being of moderate complexity (II). [55] This disease spectrum includes mild, moderate, and severe variations. Electrocardiographic findings in these patients may include a diagnosis of Wolff-Parkinson-White syndrome. [55]
Diagnostic recommendations: Ebstein anomaly
Class IIa (moderate strength, moderate quality from nonrandomized studies)
In adults with Ebstein anomaly, cardiac magnetic resonance imaging (CMRI) can be useful in the evaluation of cardiac anatomy, right ventricular (RV) dimensions, and systolic function. Transesophageal echocardiography (TEE) can be useful for surgical planning if the transthoracic echocardiographic (TTE) images are inadequate for assessing the tricuspid valve morphology and function.
Electrophysiologic study (EPS) with or without catheter ablation may provide benefit in the diagnostic evaluation of adults with Ebstein anomaly and ventricular preexcitation but without supraventricular tachycardia (SVT). EPS (and catheter ablation, if needed) is reasonable before surgery on the tricuspid valve in adults with Ebstein anomaly, even in the absence of preexcitation or SVT.
Treatment recommendations: Ebstein anomaly
Class I (strong recommendation, moderate quality from nonrandomized studies)
Surgical repair or reoperation for adults with Ebstein anomaly and significant tricuspid regurgitation (TR) is recommended when one or more of the following are present:
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Heart failure symptoms
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Objective evidence of worsening exercise capacity
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Progressive RV systolic dysfunction by echocardiography or CMRI
Class I (strong recommendation, limited data)
Catheter ablation is recommended for adults with Ebstein anomaly and high-risk pathway conduction or multiple accessory pathways.
Class IIa (moderate recommendation, moderate quality from nonrandomized studies)
Surgical repair or reoperation for adults with Ebstein anomaly and significant TR may provide benefit in the presence of progressive RV enlargement, systemic desaturation from right-to-left atrial shunt, paradoxical embolism, and/or atrial tachyarrhythmias.
Class IIb (weak recommendation, moderate quality from nonrandomized studies)
Consider bidirectional superior cavopulmonary (Glenn) anastomosis at the time of Ebstein anomaly for adults in the presence of severe RV dilation or severe RV systolic dysfunction, preserved left ventricular (LV) function, and nonelevated left atrial pressure and LV end diastolic pressure.
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Anatomic features of Ebstein anomaly. Note the atrialized portion of the right ventricle and displacement of the tricuspid valve. AV = atrioventricular.
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Characteristic chest radiograph of a neonate with Ebstein anomaly. The heart shadow demonstrates cardiomegaly, with evidence of severe right atrial enlargement.
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Surgical repair of Ebstein anomaly as described by Danielson. (A) The right atrium is incised and reduced, and the atrial septal defect is closed with a patch. The arrow identifies the large anterior leaflet. (B) Mattress sutures with felt pledgets are used to pull the tricuspid annulus and valve together in a horizontal plane, obliterating the atrialized right ventricle. (C) Sutures are tied after all have been inserted. The arrow identifies the septal leaflet. (D) A posterior annuloplasty is used to narrow the orifice of the tricuspid annulus. (E) Completed repair, resulting in a competent tricuspid valve.
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Surgical repair of Ebstein anomaly as described by Carpentier. The anterior and posterior leaflets are detached from the tricuspid annulus. In type D lesions, fenestrations are used to create interchordal spaces for the passage of blood into the right ventricle outflow tract (insert). Mattress sutures with pledgets are placed in a vertical plane to plicate the atrialized portion of the right ventricle (top right). The anterior leaflet is reattached at the level of the true annulus with a continuous running suture (bottom left). An annuloplasty ring is inserted to reinforce the repair (bottom right).
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Surgical repair of Ebstein anomaly in the neonate as described by Starnes. The atrial septal defect is enlarged by excising the remaining septum. The tricuspid valve orifice is closed with a Gore-Tex patch, effectively creating tricuspid atresia. A Gore-Tex shunt (not shown) is then placed to connect the innominate artery to the right pulmonary artery. PTFE = polytetrafluoroethylene.
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Surgical replacement of the tricuspid valve in Ebstein anomaly. (A) The atrialized right ventricle is plicated in a horizontal plane. (B) Sutures are placed on the atrial side of the coronary sinus and atrioventricular node to avoid injury to the conduction system. (C) Sutures are tied with the heart beating and perfused to ensure the conduction system is intact.
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Surgical replacement of the tricuspid valve using a pericardial patch to avoid injury to the conduction system. The valve insertion is begun anterior to the coronary sinus using a continuous running suture. A glutaraldehyde-treated pericardial patch is sutured to the septal portion of the prosthetic valve sewing annulus. The free margin of the patch is then sutured to the atrial tissue beyond the area of the conduction tissue. AV = atrioventricular.
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Operative steps for Ebstein anomaly repair. (A) Opened right atrium showing displacement of the tricuspid valve. ASD = atrial septal defect, CS = coronary sinus, TTA = true tricuspid annulus. (B) Detached part of the anterior and posterior leaflet as a single piece. (C) Clockwise rotation of the posterior leaflet edge to be sutured to the anterior leaflet septal edge and plication of the true tricuspid annulus to bring the valve to a uniform level. (D) Completion of valve attachment to the true tricuspid annulus and closure of the atrial septal defect. Used with permission from Elsevier (Fig 1 from Da Silva JP, Baumgratz JF, da Fonseca L, et al. The cone reconstruction of the tricuspid valve in Ebstein's anomaly. The operation: early and midterm results. J Thorac Cadiovasc Surg. 2007 Jan;133(1):215-23).
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The cardiac silhouette is usually enlarged in Ebstein anomaly. A chest radiograph will usually show an enormous shadow of the right atrium.
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Pathology of Ebstein anomaly: Dilated right atrium (RA) with inferior displacement of an abnormal tricuspid valve (TV) septal leaflet, which results in a small right ventricle (RV) that is atrialized. ASD = atrial septal defect, LA = left atrium, LV = left ventricle, PA = pulmonary artery, R-L = right to left.
