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Ebstein’s Anomaly Treatment in Delhi, India
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Ebstein anomaly is an uncommon heart defect that has been present since birth. In this condition, the tricuspid valve is in the wrong position, and the valve's flaps, also called leaflets, are deformed, as a result of which the valve does not work properly. Normally, these leaflets open up so that the direction of blood flow is from the right atrium into the right ventricle, and the valve closes while the heart pumps so that blood cannot flow backwards.
Symptoms
Mild forms of Ebstein anomaly might not depict any symptoms initially until found later in adulthood. Signs and symptoms include:
- Dyspnoea, especially with a lot of exertion.
- Fatigue
- Heart palpitations or arrhythmias.
- Cyanosis of the lips and skin caused by low oxygen.
Causes
Ebstein anomaly is a heart defect whose cause is mainly unknown.
Diagnosis
If the child doesn't have any signs or symptoms of a heart-related issue, the doctor can determine a problem only after hearing abnormal heart sounds during a routine physical exam. The doctor might also recommend several tests, including:
- Echocardiogram: It shows the basic structure of the tricuspid valve and the blood flow through the heart. Sometimes, a transesophageal echocardiogram is done. Since the oesophagus lies close to the heart, this test can provide a detailed image of the heart.
- Electrocardiogram (ECG): An ECG can help to highlight issues with the heart's rhythm as well as structure.
- Holter Monitor: This is a portable ECG device worn by the patients while they are away from the doctor's clinic. It records the heart's electrical activity as the patient performs their normal activities for a day or two.
- Chest X-ray: This is performed to detect enlargement of the heart.
- Cardiac MRI: A cardiac MRI makes use of magnetic fields and radio waves to form detailed images of the heart. This test yields a detailed view of the tricuspid valve. It allows the doctor to see the size of the heart chambers and how well they work.
- Pulse Oximetry: In this test, a sensor is attached to the finger or toe to measure the amount of oxygen in the blood.
- Electrophysiology Study (EP): This test is performed to map the heart's electrical impulses.
In addition, the doctor can use the electrodes to stimulate the heart to beat at rates that may trigger or halt an arrhythmia. Eventually, they can treat them by ablating it.
- Cardiac Catheterisation: A catheter, which is a small, thin tube, is placed into a blood vessel near the groin, arm, or neck region and accompanied to the heart with the help of an X-ray. Afterwards, a dye injected through the catheter gives the doctor a clearer view of blood flow through the heart, blood vessels, and surrounding valves. During the test, the doctor can measure pressure and oxygen levels in the heart and check for problems present inside the heart and lungs.
Treatment
Treatment of the Ebstein anomaly depends on the severity of the defect and the signs and symptoms. The treatment is aimed to reduce future complications, such as heart failure and arrhythmias.
Regular Monitoring
If there are no signs or symptoms or abnormal heart rhythms, the doctor might recommend monitoring the heart condition with regular follow-up appointments.
Medications
If the patient is experiencing heart rhythm disturbances, medications can be of some help for sure as they help to control the heart rate and also maintain a normal rhythm of the heart. The doctor might also prescribe specific medications to relieve signs and symptoms of heart failure, such as drugs to avoid water retention.
If the patient has any heart rhythm problems or a hole located between the upper heart chambers, then the doctor may recommend certain medications to prevent the formation of blood clots.
Some babies are given a certain kind of substance for inhalation purposes called nitric oxide to help improve blood flow to the lungs.
Surgical Procedures
Several types of procedures performed by doctors to surgically treat Ebstein anomaly and associated defects are as follows:
- Tricuspid valve repair
- Tricuspid valve replacement
- Closure of the atrial septal defect
- Maze procedure
- Heart transplant
Risk Factors
Doctors aren't too sure about the risk factors associated with this defect. Genetic as well as environmental factors are expected to play a vital role. Generally, a family history of heart defects or a mother's consumption of certain medications, like lithium, during the time of pregnancy is supposed to increase the risk of an Ebstein anomaly in the baby.
Complications
Mild Ebstein anomaly generally doesn't lead to any complication as such; however, various possible complications of Ebstein anomaly consist of:
- Heart failure
- Cardiac arrest
- Arrhythmias
- Stroke
Following a few precautions before indulging in any sports or becoming pregnant may certainly help to avoid complications.
If the heart size is almost normal and there are no disturbances in the heart rhythm, the patient can probably take part in most of the physical activities. The doctor might recommend skipping a few competitive sports, like football or basketball.
Many women with mild Ebstein's anomaly can safely have kids. But at times, pregnancy and delivery put an additional strain on the heart. Rarely, very severe complications can develop, resulting in the death of either mother or baby.
Sometimes, other modes of treatment for the condition or symptoms may be recommended before pregnancy.
Outlook
Overall, the outlook for children with Ebstein's anomaly is very bright. Children with mild Ebstein's may never experience any sort of symptoms or problems. Latest research and advancements in surgical and medical therapies have improved outcomes even for those children with the severest form of the defect.
Review
Reviewed by Dr. Arun Kumar Gupta, Associate Director - Cardiac Sciences, Cardiology, Cardiac Electrophysiology-Pacemaker, Interventional Cardiology.
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