Living with Ebstein’s Anomaly.

What is Ebstein’s Anomaly

Ebstein’s Anomaly is a rare condition that is present at birth (congenital). With Ebstein’s Anomaly, your tricuspid valve — the valve between the two right heart chambers — is not positioned inside the heart as it should be.

As a result, blood may leak back through the valve. This can make your heart work less efficiently. Ebstein’s anomaly can also lead to enlargement of the heart and even heart failure.

Ebstein’s anomaly is not just a valve disease because it can also involve the heart muscle.

How common is Ebstein’s Anomaly?

Ebstein’s Anomaly occurs in 0.5% of people born with congenital heart disease and is frequently associated with other congenital heart problems, such as atrial septal defect (ASD), ventricular septal defect (VSD), pulmonary stenosis and pulmonary atresia.

What are the common symptoms associated with Ebstein’s Anomaly?

Ebstein’s can be mild, and people may have no symptoms for many decades until they are diagnosed.

On the other hand, it can be very apparent in newborn babies, or later on in life, and can be so severe that surgery is necessary.

Where signs and symptoms are present they can include:

  • Shortness of breath
  • Poor exercise tolerance
  • Chest pain
  • Fatigue
  • Heart palpitations or abnormal heart rhythms (e.g. atrial flutter, fibrillation and arrhythmias)
  • A bluish discolouration of the lips and skin caused by low oxygen (cyanosis)
  • A bluish discolouration of the finger and toenails.

How is Ebstein’s Anomaly treated?

In mild cases of Ebstein’s Anomaly, careful monitoring of your heart may be all that’s necessary. However, if the symptoms are bothering you, and/or if the heart is enlarging or becoming weaker, treatment may be necessary. Treatment options include medications and surgery.

If you have Ebstein’s, it is vital that you are seen and assessed in a specialist CHD centre where CHD cardiologists agree on the necessary investigations and treatment that are right for you.

The usual indications for surgery are the appearance of heart failure, cyanosis, arrhythmia and deteriorating exercise capacity.
Surgery aims to repair the valve. If this is not possible, a valve replacement will be performed. Surgery to treat the arrhythmia may also be required at the same time.

With the right, ongoing medical care people with Ebstein’s’ Anomaly can have a good quality of life.

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With your help, we’ve helped fund new and improved treatments for congenital heart disease. we opened the UK’s first dedicated Congenital Heart Research Centre. we’ve organised receptions at the House of Lords and House of Commons, a film première, concerts, parachute jumps, marathon runs, annual sponsored walks and masquerade balls. we ensure that adult CHD patients enjoy access to every opportunity, are free from discrimination, and are fully supported throughout their lives. we’ve staged annual conferences, regional patient information days and workshops throughout the UK. we’ve published a range of leaflets for CHD patients on a wide-range of topics. we’ve launched a Freephone patient helpline to provide practical advice, a listening ear, and emotional reassurance at times of stress. we’ve created online networks where congenital heart patients can connect with each other. we successfully applied for a Comic Relief grant to help develop mental health support services. we’ve contributed to adult congenital heart disease nurse training days and taken part in various study days and conferences for medical and health professionals. we’ve established a benevolent fund for members who are in financial hardship. we’ve organised residential weekends and outward bound holidays for teenagers and adults, providing congenital heart patients with the opportunity to meet and gain support from each other.

Just think what else we could do if you donate, fundraise or volunteer for us.