I am 67 years old and was diagnosed with Ebsteins Anomaly (EA) in March 2014.

In late 2013 I was having problems with fuzzy vision and MRI indicated abnormal blood vessels in my eyes. I was referred for cardiovascular investigation and in early 2014 atrial fibrillation was found and EA diagnosed by echocardiogram. The EA diagnosis was a complete surprise. The doctor said there was something unusual in my echocardiogram and went to consult a colleague. He came back and said I had EA and was only the second adult case he had seen in his long career. I was told to avoid vigorous exercise – his words were: don’t run for a bus! I’m a retired biomedical scientist and looked on the internet for information about EA and was quite alarmed at the seriousness of the condition and the apparent low incidence of survival to my age.

In subsequent weeks I had instances of palpitations and shortness of breath in the night resulting in emergency visits to A&E and became very aware of right jugular pulse due to tricuspid valve regurgitation. Simply going upstairs to bed causes shortness of breath with a strong jugular pulse making it difficult to go to sleep because of hearing blood flow in my head.
Over my whole life I have avoided vigorous sporting activities (did not have the energy!) and had several instances of transient blackouts in recent years. However, as a scientist I have travelled widely round the world without health issues. The recent emergence of EA symptoms may be stress related. My father died in 2013 and I have no siblings to help me support my 90 year old mother who has memory loss and lives at the other end of England.

I have been told that although tricuspid valve repair is possible, for me the risk outweighs the benefit at this time. Taking daily beta blocker, histamine H2 antagonist and warfarin tablets I am now feeling much better but have to carefully manage my daily amount of exercise. Since starting warfarin in January 2015 the jugular pulse seems less intrusive.

I would be very interested to hear about the experiences of anyone who has been diagnosed with Ebsteins Anomaly late in life!

Thanks,
Trevor

Posted by Trevor Stephens.

3 Comments »

  1. Hi,
    I was diagnosed with Ebsteins Anomaly in my mid 40’s. I had my first operation at the Brompton Hospital aged 48 to repair my tricuspid valve and maze treatment. Unfortunately this was unsuccessful and 2 years later I was operated on again. This time they repaired the valve by fitting a Carpentier ring and also fitted Glenn shunts (Google with explain).
    I am now 63 and have regarded myself as being very fortunate to have had such wonderful treatment from the Brompton. I have had to have several bouts of ablation over the years and a pacemaker fitted 4 years ago. Next week I go in again for more tests as need further treatment but the hospital have always been brilliant with everything.

    Comment by Lynds — August 15, 2015 @ 6:56 am

  2. Hi Trevor,
    I am 64 and was diagnosed with EA about 20 years ago. When I was young I had some trouble with tachycardia and they found a murmur so they knew I had some heart problem. The EA diagnosis, as I said came much later.
    I have known about my tricuspid regurgitation but it was not that bad and I have led a reasonably active life but, as with you, not very sporty as I ran out of breath too quickly.
    The tachycardia, or as they now call it atrial fibrillation, gradually got worse and when combined with pneumonia in 1997 led to me having an ablation in 1998. This pretty well sorted out the atrial tachycardia.
    My shortness of breath has got worse over the last year or so and my doctors are now rather keen on me having a valve repair or replacement. Yes, there are risks involved but, for me, the degeneration of the tricuspid means I will have to have this done in the next six months, probably January or February.
    Best wishes,
    Steve

    Comment by SteveH — September 12, 2015 @ 3:28 pm

  3. Hi Trevor,
    I am 64 and had a funny heart since childhood. They found a murmur and the odd feelings in my chest were called paroxysmal tachycardia. From then on I was checked about once a year. The tachycardia (AF) gradually got worse into my teens and I had further investigations. They said there was a possible shunt (hole in the heart) and not to worry unless I had serious pain in my chest. Much later I got the diagnosis of Ebstein’s Anomaly and told me a bit about it. I was used to not being very fit and whatever I did, not getting any fitter. The atrial fibrillation was irregular and only rarely a real bother to me. Then I got pneumonia which gradually got worse, then at the same time my heart does its fast beat (AF) and finally I was admitted to hospital for a few weeks. OK after discharge, just the fast heart beat (AF) continued. This worried the docs and they did an ablation which worked, stopped the fast heart (AF), mostly. But a bit later my regular checks found occasional heart block and I ended up having a pacemaker. This last stuff about 12 years ago. In recent months my docs have been talking about valve repair surgery. As you know EA is a problem tricuspid valve and odd left ventricle. The docs were noticing more tricuspid leaking which puts further strain on the right ventricle and this was getting worse. They were concerned that things would soon get to the point were surgery would not be possible. So I am booked in for tricuspid valve repair or replacement in a few weeks. Hopefully I will be fit for Xmas!
    Hope this helps,
    Steve

    Comment by Steve Holloway — November 2, 2015 @ 4:59 pm

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