Living with an anomalyBMJ 2006; 332 doi: https://doi.org/10.1136/bmj.332.7550.1141-a (Published 11 May 2006) Cite this as: BMJ 2006;332:1141
The first indication I had that something was wrong was when I went to my prep school and found myself quite unable to keep up on cross country runs. Paradoxically, I excelled at soccer and won the school hurdles. This inconsistency did not concern anyone until, in my early teens, I had a day-long attack of paroxysmal tachycardia. There was no treatment for this in 1952, so I made an unlikely bargain with God that if he stopped it I would never bet or gamble except on horses. He kept his side of the bargain, so I had to keep mine, although I did come to an arrangement about poker later on.
This episode led to my having an atrial septal defect diagnosed, and while I was a medical student at Cambridge it was decided that this should be closed. Cardiac catheterisation established that I had a huge right atrium, and Ebstein's anomaly was diagnosed, for which I was told there was no treatment. I was not encouraged to read that sudden death is a feature of the condition.
I continued my medical studies but was constantly troubled by extrasystoles and runs of rapid beats and started taking quinidine. Life for a junior doctor in the 1960s could be very demanding, and, as a senior house officer, I was in sole charge of the busy Norwich casualty department from Friday to Monday on alternate weekends. However, the acute sleep deprivation that this involved did not seem to do me any harm.
Ebstein's struck again one morning in 1972, when I had my first attack of atrial fibrillation. After 36 hours, I was cardioverted, but I was advised that I must expect to go into this rhythm permanently, and, without atrial transport, this would reduce my activities and shorten my life. Although I was, by then, in a strong position to compete for consultant jobs, I decided not to and took a personal associate specialist job.
Soon afterwards, β blockers arrived and eliminated the sinus tachycardias to which I was so prone at the end of a long day, improved my exercise tolerance, and reduced the rate of atrial fibrillation and probably the number of attacks. The result is that, with the additional help of flecainide and a supportive cardiologist, I am still fighting off atrial fibrillation more than 30 years later—although with plenty of cardioversions and burnt chests on the way.
Operations and ablation therapy are now available for Ebstein's anomaly. In the United States the Mayo clinic has now operated on more than 600 cases, but I believe nowhere in Britain has done more than a few. There is an urgent need for centres specialising in this condition.
The moral is never be too pessimistic about life. It can turn out very differently from the way you expect. Fortunately, I found a wife who was prepared to take a gamble on my life, and we now have four healthy grandchildren.