Appendix B
A True Haunting
The referral slip was like all the rest: too little information and, what there was of it, haphazardly arranged. I knew that it would take a while to decipher. It lay among the 20 or so other referrals that also clamoured for attention on my desk, and there it was: the anticipated scrap of paper with written details of 17-year-old Jim, about whom the referring cardiologist had telephoned me a few days before. We’d talked in general terms about a young man, a huge aneurysm of the ascending aorta, poor heart function, and when was the right time to operate to replace this naturally weak and already hugely enlarged aorta with an artificial tube, so that imminent and fatal rupture could be averted.
I had been told that Jim was a young and otherwise healthy lad who had complained of recent, severe central chest pains that troubled him at unpredictable times. He was said to display all the characteristics of a condition called ‘Marfan’s syndrome’: he was tall, slim, and ‘double-jointed’, in that he was much more flexible than most people. In a yoga class, that would be a good thing, but, in Marfan’s syndrome, it means that the connective tissue is abnormal. This inherited weakness in connective tissue affects many parts of the body, but none more so than the root of the aorta, the origin of the largest artery in the body, and the one that every organ bar none depends on for blood supply. When that weakness is present, the aortic root begins to balloon out, so that its normal size of three centimetres or so increases. Jim’s aortic root was a whopping 11 centimetres, way past the point at which rupture and death are a very real and distinct probability.
The aortic root is basically a tube, but one that contains some truly vital components. At its origin is the aortic valve, a delicate, gossamer-light three-leaflet structure that lets blood freely out of the heart into the aorta when the heart beats, but stops it from leaking back when the heart relaxes. Jim’s aortic valve had been stretched so widely by the enlarged root that its leaflets had no chance of meeting in the middle to close and stop the backflow, and it was leaking freely back into the heart. This is called aortic valve regurgitation, and the heart does not like it. As far as the poor heart is concerned, it works hard to pump the blood into the aorta only to see most of it leak back and need to be pumped again. The heart can cope with this for a while, but was never designed to pump so much blood at every beat for a sustained period of time, and, when that happens, the heart enlarges with the volume overload and fails. This had already happened to Jim’s heart: it was a bloated, weak, tired, and failing pump. The second important bit of ‘clockwork’ in the tube that is the aortic root is that the all-important left and right coronary arteries arise from that bit of aorta. These are, of course, the arteries that feed the heart muscle itself, and any operation to replace the aortic root needs to ensure that these arteries remain functional if the heart is to survive.
I telephoned the cardiologist, and we agreed that Jim should come over to my hospital as soon as possible so that he, his family, and I could review the picture together and agree to a surgical plan. We knew we had to deal with his aortic root as a matter of urgency, as it was about to ‘pop’. At the back of our minds, we also knew that other bits of his aorta might also need attention in future, and a series of operations over time to replace the whole of his aorta ‘piecemeal’ was the likely scenario, but, for the time being, his aortic root was the problem that demanded immediate attention. The rest would wait.
He arrived on the ward, exceptionally tall as expected, and accompanied by his moderately tall parents. They settled into the ward quickly, and, between us, we decided that Jim would be well served by having his aortic root replaced with a woven artificial-tube graft, and a mechanical prosthetic valve to replace his own stretched and leaky one. We would also have to detach his coronary arteries from the diseased aortic root and re-implant them into the new artificial tube so as to maintain their ability to keep the heart alive. This was a big operation, with a lot to do: taking out the aortic root, leaving the coronary arteries, taking out the valve, implanting a new valve, implanting a new aortic root, re-attaching the coronary arteries to the new aortic root, and joining that to the (for the time being) relatively healthy remaining aorta that feeds the body. It was not an operation that could be conducted quickly, and as the heart would be starved of oxygen while all this was being done, and was already weak and failing, protecting the heart muscle from damage during that time would be crucial. We would use a cold solution of potassium to do this, and we would need a lot of the stuff, and we would also need to complete the operation in as short a time as possible to minimise the damage of oxygen starvation. Aortic root and aortic valve replacement, though unusual, was a familiar operation to me with my interest and focus on aortic surgery.
All agreed to go ahead, and, encouraged by Jim and his family, we even promised to take photographs during this somewhat exceptional operation. The day the operation was scheduled for finally came, and we had a detailed and carefully worked out operation plan.
We began by attaching Jim to the heart–lung machine, by placing a tube to collect the blue, de-oxygenated blood from the right atrium of the heart so that the blood could be oxygenated and pumped into one of Jim’s arteries, so that the body remained alive while the heart is isolated. As we were expecting to cut away as much of the diseased aorta as we could, we would make the final join quite close to the part of the aorta that gives off the brain arteries. That would need stopping the circulation for a brief period, and, for the brain to survive that, it had to be cold, so, as soon as the heart–lung machine started, we began to cool Jim by cooling his blood from 37°C to 18°C. While the cooling was being performed, we clamped the aorta downstream from the heart, and stopped and cooled the heart with the potassium solution, planning to repeat the potassium cooling every 20 minutes to reduce any heart damage by oxygen starvation. We then opened the aorta, cut out Jim’s aortic valve, and took out the aortic root, leaving the openings of the coronary arteries. We then replaced the valve with a large mechanical valve, and attached that in turn to the synthetic graft that would be the new ‘aorta’. So far, all was going well and according to plan. The next step was the delicate procedure of re-implanting the coronary artery openings into the synthetic graft. The graft was about three centimetres in diameter, Jim’s enormous aorta had been 11 centimetres, and so the coronary openings were too far to reach the new aorta. We needed ‘extensions’ to reach. We therefore quickly cut into Jim’s leg, and took two pieces of saphenous vein, which we knew he could do without, and attached these to the openings of the two coronary arteries. With these extensions, we could reach the synthetic graft easily, and we plumbed them into the graft. Temperature had now reached 18°C, and it was safe to stop the circulation altogether to perform the final procedure of joining the new artificial ‘aorta’ to the stump of the old one, level with the branches that feed the brain.
This was completed without a problem. We flushed out any air from the heart and restarted the circulation by restarting the heart–lung machine. We also began to rewarm the blood to bring Jim’s temperature from 18°C back to normality of 36–37°C. As we were doing this, the heart was again connected to the circulation, and the blood flowing down the coronary arteries washed out the potassium, so that the heart began to beat again. The surgically sewn joins looked free of leaks. We checked all surgical areas, and noted good, well-filled vein extensions between native coronary arteries and the new aorta. As we approached normal body temperature, we began to wean Jim from the heart–lung machine, and that is when the problems started.
Jim’s heart was incapable of supporting his circulation. This was a very worrying development. Could this have been a troubled coronary artery leading to poor heart-muscle blood supply? We could not be sure, but, to be on the safe side, I quickly took more pieces of vein from the leg, and used them to bypass the two coronary arteries. To our utter dismay, it did not work: there was no improvement in heart function. We had little in the way of alternatives despite his youth. My hospital is a centre that offers heart transplantation for failing hearts, but it is impossible to obtain a heart from a donor on demand: such hearts are not there to be taken off the shelf — a brain-dead donor needs to be found, and that could take days or weeks. I asked for help from colleagues. We considered long-term support using a mechanical ‘assist device’ or artificial heart: there were technical reasons why this could not be done. All we had left was the hope that, by leaving Jim on the heart–lung machine for some more time, his heart might recover sufficiently to be able to take on the circulation so that he would survive. We did this, and to cut a long story short, there was absolutely no recovery. We had to face the fact that Jim was to die on the operating table … and indeed this is how Jim died.
I was overwhelmed. We had, I felt, undertaken all the manoeuvres to ensure the best preservation possible of the poorly functioning heart muscle, so why did it not work like it should at the end? ‘Cheated’ really summarised my feelings, and, clouded with this thought, I broke the dreadful and unexpected news to his worried parents. Perhaps the excessively prolonged operation had already seeded bad news in their minds. They appeared to accept this outcome in better fashion than I did.
Reflecting, as we all do in a keen attempt to accommodate and so come to terms with such events, I recognised the substantial risk of such an outcome using EuroSCORE. This is a tool we use to estimate the chance of death using pre-operative characteristics. Death was estimated to be likely to be the result in one in five of such operations, but the inescapable fact that led to Jim’s death was that his damaged heart function had suffered further during my surgery.
Death is a most powerful and eloquent natural criticism, and yet more so when your patient dies before leaving the operating room, and so much more so when the patient is so very young. Even to this day, years later, I find myself reviewing in my mind all the steps of the operation, and especially those to do with the precautions we had taken to protect against hurting Jim’s troubled heart further, only to find again and again that by any standards we’d done what appeared to be ‘a good job’, though the eventual outcome screamed that we had not. And so Jim’s days in Papworth return to me often, haunting if you will, so that I certainly know these events will stay with me forever with little chance of my acceptance, accommodation, and reconciliation of them; a true haunting.