Ebstein Malformation Surgery Workup
- Author: Sergey G Toshinskiy; Chief Editor: John Kupferschmid, MD more...
Routine laboratory study results in patients with Ebstein malformation may be in the reference range. In symptomatic neonates, acidosis may lead to metabolic and electrolyte derangements. ABG levels determine the severity of cyanosis and indicate the amount of right-to-left shunting at the atrial septal defect.
The heart shadow may be normal or marked by massive cardiomegaly. Typically, the shadow of the great vessels is narrow because of a small aorta and main pulmonary artery. Right atrial and right ventricular enlargement produces a heart shadow with a globular shape. The apical region of the left ventricle may be elevated from the diaphragm, as seen in right ventricular enlargement (see the image below).
Pulmonary vascularity findings may range from normal to significantly decreased depending on the severity of the defect and the amount of pulmonary blood flow.
A cardiothoracic ratio greater than 0.65 is a predictor of sudden death; some physicians consider this finding an indication for surgery.
Echocardiography has evolved as the primary diagnostic tool to aid in diagnosing Ebstein malformation in patients. A well-documented correlation is noted between echocardiography findings and operation or autopsy findings.
Echocardiography can define the morphology of the tricuspid valve as well as specific abnormalities of the leaflets. In addition, the function, thickness, and size of the right and left ventricles can be assessed. Coexisting cardiac lesions can also be identified.
Color flow Doppler allows for better assessment of tricuspid valve incompetence and degree of shunting at the atrial level.
Two systems that classify disease severity have been developed based on echocardiographic findings. The classification of Carpentier is based on tricuspid valve morphology, whereas the classification of Celermajer is based on cardiac chamber size.[8, 31] More recently, real-time 3-dimensional transthoracic echocardiography has provided a more comprehensive assessment of the anatomic and morphologic features in patients with Ebstein anomaly.
If echocardiography is inadequate or indeterminate, cine CT and MRI are also useful for diagnostic purposes.
Prior to recent advances in echocardiography, cardiac catheterization was the definitive diagnostic study for patients with Ebstein malformation. Early studies demonstrated that cardiac catheterization in patients with Ebstein malformation was associated with an increased risk of arrhythmia and significant mortality. Currently, cardiac catheterization is reserved for patients with associated cardiac defects, previous shunt placements, or possible pulmonary artery stenosis.
During cardiac catheterization, hemodynamic findings usually demonstrate elevated right atrial pressures with a pulse wave contour showing a dominant v wave and a steep y descent. For patients with severe right atrial enlargement, the right atrial pulse wave may be normal despite the presence of severe tricuspid regurgitation.
Right ventricular pressure may be normal or mildly elevated. Significantly elevated right ventricular pressure may be found in patients with right ventricular outflow tract obstruction or elevated pulmonary artery pressures. The demonstration of right atrial pressure in the proximal right ventricle strongly indicates the presence of Ebstein malformation.
Contrast angiography demonstrates right atrial enlargement and displacement of the septal and posterior tricuspid valve leaflets below the true tricuspid annulus. In most studies, the origin of the displaced septal and posterior leaflets can be seen on the right ventricular wall.
If the tricuspid valve is incompetent, contrast often moves back and forth between the right atrium and the right ventricle. Right-to-left shunting of contrast is seen across any atrial septal defect.
In most patients, blood flow through the right side of the heart and pulmonary arteries is slower than normal.
ECG findings are almost always abnormal. Right axis deviation and right bundle-branch block are common. P wave morphology usually indicates right atrial enlargement, and the PR interval is often prolonged. Since both atrial arrhythmias and ventricular arrhythmias are common, the results of a 24-hour Holter monitor should be obtained from any patient with a history of arrhythmia or palpitation.
Electrophysiologic studies should be performed on those patients with a history of ventricular preexcitation, wide-complex arrhythmia, or syncope.
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