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Pediatric Ebstein Anomaly Workup

  • Author: Raymond T Fedderly, MD; Chief Editor: Stuart Berger, MD  more...
Updated: Jan 04, 2016

Imaging Studies

Echocardiography is the definitive test for diagnosis of Ebstein anomaly. Typical findings include the segmental anatomy, inferior displacement of the septal and/or posterior leaflet of the tricuspid valve, large tricuspid valve annulus, dilated right atrium and right ventricle, tricuspid valve regurgitation, right ventricular outflow tract obstruction, and an atrial septal defect (ASD) or patent foramen ovale (PFO).

Chest radiography may be helpful in determining the size of the cardiac silhouette, which is related to the degree of tricuspid valve regurgitation. In severe cases, the cardiothoracic ratio may approach 1. A dilated right atrium is most often the cause of cardiomegaly. In patients who are cyanotic, the pulmonary vascular markings appear decreased.


Other Tests


Typical findings on the ECG are large P waves, a prolonged PR interval, right axis deviation, and a right bundle branch block. Atrial or ventricular arrhythmias, as well as a delta wave indicative of Wolff-Parkinson-White syndrome (WPW) syndrome, can also be seen.

Holter monitoring

Ambulatory ECG recording may be performed to evaluate the possibility of occult arrhythmias or to verify symptomatology, such as palpitations over 24 or 48 hours.

Stress testing

Exercise testing may be done both preoperatively and postoperatively to objectively evaluate exercise tolerance, oxygen consumption, systemic arterial oxygenation, and possible arrhythmia vulnerability during exercise.[4]



Cardiac catheterization

Cardiac catheterization may be performed to evaluate both the hemodynamic and angiographic characteristics of the patient before surgical intervention. Depending on the particular patient's anatomic and physiologic findings, echocardiography findings are often sufficient, and may reduce the necessity for cardiac catheterization.

Postcatheterization precautions include hemorrhage, vascular disruption after balloon dilation, pain, nausea and vomiting, and arterial or venous obstruction from thrombosis or spasm.

Complications may include rupture of blood vessel, tachyarrhythmias, bradyarrhythmias, and vascular occlusion.

Electrophysiological studies

Electrophysiological (EP) studies are performed in many patients suspected of having an arrhythmia secondary to paroxysmal supraventricular tachycardia (SVT) and WPW syndrome, both of which have a high incidence of occurrence. During invasive EP testing, an accessory pathway is most often identified along the abnormal tricuspid annulus. Manifest accessory pathways (WPW) and concealed pathways (unidirectional retrogradely conducting accessory pathways) account for most SVT mechanistically in patients with Ebstein anomaly. The frequency of multiple accessory pathways is significantly higher in Ebstein anomaly than in the general population of patients with SVT and normal tricuspid valve anatomy.

Contributor Information and Disclosures

Raymond T Fedderly, MD Associate Professor, Department of Pediatric Cardiology, Children's Hospital of Wisconsin, Medical College of Wisconsin

Raymond T Fedderly, MD is a member of the following medical societies: American College of Cardiology, American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatic Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Charles I Berul, MD Professor of Pediatrics and Integrative Systems Biology, George Washington University School of Medicine; Chief, Division of Cardiology, Children's National Medical Center

Charles I Berul, MD is a member of the following medical societies: American Academy of Pediatrics, Heart Rhythm Society, Cardiac Electrophysiology Society, Pediatric and Congenital Electrophysiology Society, American College of Cardiology, American Heart Association, Society for Pediatric Research

Disclosure: Received grant/research funds from Medtronic for consulting.

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Frontal chest radiograph in an infant with severe Ebstein anomaly shows a large heart that leaves little space for the lung. Although the appearance is relatively nonspecific, the large heart should suggest Ebstein anomaly in the differential diagnosis.
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