Pediatric Ebstein Anomaly Workup
- Author: Raymond T Fedderly, MD; Chief Editor: Stuart Berger, MD more...
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.
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.
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.
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.
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 (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.
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