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Ebstein's Anomaly

What is Ebstein's disease? Ebstein's anomaly is a congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced towards the apex of the right ventricle of the heart. What is the cause of Ebstein's anomaly? Ebstein's anomaly occurs due to improperdevelopment of the tricuspid valve in the first eight weeks of fetal growth. It can be caused by a number of factors, though, most of the time, this heart defect occurs sporadically (by chance), with no apparent reason for its development. Is Ebstein's anomaly a cyanotic heart disease? Patients with the most severe form of this disease may present with severe congestive heart failure and cyanosis at birth while mild cases may not be discovered until adulthood. Ebstein's anomaly may also lead to enlargement of the heart or heart failure. It's usually associated with an atrial septal defect. How is Ebstein's anomaly diagnosed? If your doctor suspects an underlying problem, such as congenital heart disease, or if you have other signs and symptoms that may suggest Ebstein's anomaly, your doctor may recommend several tests, including: Echocardiogram. This test is often used to diagnose Ebstein's anomaly and other congenital heart defects Treatment Medication Ebstein's cardiophysiology typically presents as an (antidromic) AV reentrant tachycardia with associated pre-excitation. In this setting, the preferred medication treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs such as beta blockers, calcium channel blockers, and digoxin are contraindicated. If atrial fibrillation with pre-excitation occurs, treatment options include procainamide, flecainide, propafenone, dofetilide, and ibutilide, since these medications slow conduction in the accessory pathway causing the tachycardia and should be administered before considering electrical cardioversion. Intravenous amiodarone may also convert atrial fibrillation and/or slow the ventricular response. Surgery The Canadian Cardiovascular Society (CCS) recommends surgical intervention for these indications • Limited exercise capacity (NYHA III-IV) • Increasing heart size (cardiothoracic ratio greater than 65%) • Important cyanosis (resting oxygen saturation less than 90% - level B) • Severe tricuspid regurgitation with symptoms • Transient ischemic attack or stroke The CCS further recommends patients who require operation for Ebstein's anomaly should be operated on by congenital heart surgeons who have substantial specific experience and success with this operation. Every effort should be made to preserve the native tricuspid valve.