Ebstein Anomaly

Diagnosis

The physician conducts a physical examination, which includes listening to the patient’s heart and lungs. In cases of an Ebstein anomaly, a doctor may detect a murmur, which is an abnormal heart sound. Additionally, children with severe Ebstein anomaly may exhibit bluish or grayish skin coloration due to reduced blood oxygen levels.

Tests

Tests used to assist in the diagnosis of the Ebstein anomaly include:

• Pulse oximetry. A sensor that is affixed to a finger or toe measures the blood’s oxygen content in this test.
• Chest X-ray. An X-ray of the chest can show blood vessels, the heart, and the lungs. If the heart is enlarged, it can be seen.
• Electrocardiogram (ECG or EKG). This easy test monitors heartbeat. Attached to the chest, and occasionally the arms and legs, are sticky patches. The patches are wired to a computer so that it may print or show the results.
• Holter monitor. You can wear this portable ECG gadget for up to a day to monitor the heart’s activity while you go about your everyday business.
• Echocardiogram. Pictures of the beating heart are produced by the use of sound waves. The flow of blood via the heart and heart valves can be seen on an echocardiography.
• CardiacMagnetic Resonance Imaging (MRI). A cardiac MRI produces detailed pictures of the heart using radio waves and magnetic fields. This examination can provide a thorough look at the tricuspid valve. It also displays the dimensions and functionality of the heart chambers.
• Exercise stress tests. Frequently, these examinations entail using a stationary bike or treadmill while the heart is monitored. The heart’s response to activity can be seen via an exercise stress test.
• Electrophysiology study (EP). In order to do this test, the doctor inserts a thin, flexible catheter into a blood vessel and directs it toward the heart. One may utilize many catheters. The catheter’s tip has sensors that transmit electrical impulses and monitor the heart’s electrical activity. This test aids in identifying the area of the heart that is producing an irregular or rapid heartbeat. This test may be used to treat an irregular heartbeat.
• Cardiac catheterization. Your doctor will be able to take measurements of oxygen and pressure in various cardiac chambers during the exam. A catheter is a long, thin, flexible tube that is placed into a blood vessel, typically in the wrist or groin, and is directed toward the heart. The catheter allows dye to enter the heart’s arteries. The dye enhances the visibility of the arteries in film and X-ray images. This test can also be used to provide certain heart disease treatments.

Treatment

The treatment approach for the Ebstein anomaly is determined by the severity of the heart condition and the presence of symptoms. Treatment options may encompass regular check-ups, medication, surgical interventions, or specific procedures. The primary goals of treatment are to alleviate symptoms and prevent potential complications, such as heart failure and irregular heartbeats.

Routine medical examination

A doctor might advise routine examinations if the Ebstein anomaly isn’t producing any symptoms, such as irregular heartbeats.

Typically, follow-up visits are made at least annually. A physical examination and cardiac imaging studies are typically included of the checkup.

Medications

You might be prescribed medication if you have an Ebstein anomaly.

• Manage irregular heartbeats or other heart rhythm abnormalities.
• Prevent the body from accumulating fluid. Heart failure may be indicated by an excess of fluid in the body.
• Prevent blood clots, which could happen in the event of an Ebsteinanomaly with a hole in the heart.

Additionally, some babies receive inhaled nitric oxide to help enhance lung blood flow.

Surgery or other procedures

If the Ebstein anomaly results in severe tricuspid regurgitation, heart failure, or increased difficulties exercising, surgery is usually advised.

If additional symptoms, including some irregular heartbeats, are severe or negatively impact quality of life, surgery might also be advised.

If surgery is required, it is crucial to select a surgeon experienced in treating Ebstein anomaly. The surgeon should be qualified and experienced in carrying out the necessary procedures to fix the issue.

In order to treat the Ebstein anomaly and associated cardiac issues, surgery may involve:

• Closure of the atrial septal defect. The purpose of this surgery is to seal a hole that exists between the heart’s upper chambers. During this surgical procedure, it is possible to address and rectify other cardiac issues as well.
• Tricuspid valve repair. A faulty tricuspid valve is repaired during this open cardiac surgery. The surgeon may remove excess tissue surrounding the valve opening or repair holes or tears in the valve flaps. There may be more repairs made.
  One such method of valve repair is the cone procedure. The tissue that should have formed the tricuspid valve is separated from the heart muscle by the cardiac surgeon. Next, a functional tricuspid valve is created using the tissue.
  The valve may occasionally require additional repairs or replacement in the future.
• Tricuspid valve replacement. Surgery to replace the valve can be required if it cannot be fixed. Both minimally invasive and open heart surgery can be used to replace a tricuspid valve. A valve consisting of cow, pig, or human heart tissue is used in place of the damaged or diseased valve that the physician removes during surgery. We refer to this as a biologic valve. It is rare to replace tricuspid valves with mechanical valves.
• Maze procedure. This technique may be carried out during valve replacement or repair surgery if the Ebstein anomaly is the source of irregular heartbeats. To build a pattern or maze of scar tissue, the surgeon creates tiny incisions in the heart’s upper chambers. The scars can also be produced by using heat or cold energy. Scar tissue is non-conductive for electrical signals. Thus, the maze prevents irregular heart rhythms.
• Radiofrequency catheter ablation. Fast or irregular heartbeats are addressed with this medical intervention. The physician inserts one or more catheters, slender and flexible tubes, into a blood vessel, usually in the groin area. These catheters are then navigated by the doctor to reach the heart. Sensors located at the catheter tips employ radiofrequency energy, in the form of heat, to cause little tissue damage to the heart tissue. Scarring results from this, and the heart signals that produce irregular heartbeats are blocked.
• Heart transplant. A heart transplant may be necessary if a significant Ebstein anomaly is the reason for the heart failure.