Ebstein’s and me

by Margaret Smith

I was born in 1952 and diagnosed with Ebstein’s Anomaly at birth. The prognosis was that I would not survive with this condition. I spent my first few years in and out of Sully Hospital in South Wales. During this time I underwent two lots of cardiac catheterisation. The doctors advised my parents not to wrap me in cotton wool and to let me have as normal a life as possible. I learnt to live with my condition and managed to play netball and took up horse riding, which all helped to strengthen my heart.

In 1975 I moved to Jersey where I met my husband. We were married in 1976 and then I was given several tests to see if my heart was strong enough to go ahead with a pregnancy. Unfortunately, my condition was too severe and I was advised that sterilisation was the best option. I am sure that with the advance of medical science it would be different in this day and age.

My condition was stable until 1985 when I was taken into hospital in Jersey suffering from fast atrial fibrillation with a constant rate of 150 beats per minute. My cardiologist prescribed Flecainide, which kept my symptoms under control until around 2004 when I was diagnosed with a suspected blood clot. Warfarin was prescribed with regular blood tests.

In 2006 I was once again taken into hospital with atrial flutter, severe tricuspid regurgitation and cyanosis. After spending four days in intensive care, Dr Peter Strauss managed to get a transfer for me to The Heart Hospital in London. I was flown out of Jersey by air ambulance to Biggin Hill and then taken by ambulance to London. On admission I was examined by Dr Shay Cullen. He spent time with me explaining my condition and the operation that was going to be performed, which was an ablation. An amazing team at the hospital carried this out. After a few days, I was discharged back to Jersey with the contact number of my nursing specialist who I could call at any time.

Twelve months later my heart started to deteriorate again and my blood saturation was anything between 60 and 85 bpm. I received a cardioversion but this was not successful. Dr Mitchell, my cardiologist in Jersey, prescribed Amiodorone. This kept me stable for around another year then I began to get very breathless and my blood saturation was falling again.

The Heart Hospital had been monitoring my progress with sixmonthly checks in London and always had a keen interest in my condition. On a visit to them for one of my checks I was advised that I might now be able to have an operation to repair the Tricuspid Valve. This operation was called the Cone Procedure. Mr Victor Tsang had been to America where the operation had been carried out. It was stressed that this was pioneering surgery and that Mr Tsang had only performed the operation on one 14-year-old boy. When I returned to Jersey, Mr Tsang telephoned me and talked me through the operation. I then discussed it with Dr Mitchell in Jersey who told me that he was really pleased they were offering me an operation as it’s likely that without it I would have no quality of life because my condition was getting worse.

I was admitted to The Heart Hospital in June 2009 and Mr Tsang visited me and gave me a lot of confidence whilst explaining the operation once again. Being so far from home was quite daunting but all the doctors, nurses and staff were second to none in their care, kindness and consideration, especially to my husband while I was recovering.

The Heart Hospital is a small hospital but part of the larger UCLH (University College London Hospital) Foundation Trust. It is deemed by The Somerville Foundation to be a specialist centre for people with congenital heart conditions, which, having a very rare condition myself, I found extremely comforting. I believe that this operation has now been carried out on at least nine patients.

It has been three years this month since my operation and in this time I have also been able to stop taking any heart tablets and warfarin for my condition. My life has certainly changed significantly for the better and I am so grateful to the Heart Hospital.