The Knife's Edge

by Stephen Westaby

  Cara came back at the age of ten. She couldn’t run or play with the other kids at school – simply walking across the playground made her breathless, giving her panic attacks as she felt she was being strangled. Then there was the crushing pain in the centre of her chest when she became excited about anything. Life was increasingly miserable for her, while her parents were consumed by the anxiety that inevitably comes with third-time heart surgery. Complex reoperations were always fraught with uncertainty and the prospect that we might terminate a young life, although in reality that rarely happened. Yet as I grew older and less able to assemble a consistent operating team, I became more conscious of the risks.

  We discussed every case at a multidisciplinary team meeting before executive decisions were made. By then, Neil Wilson had become the country’s leading light on balloon dilatation of narrowed valves in children. He would insert a balloon-tipped catheter into a leg artery and retrogradely feed it up the aorta under X-ray guidance. The latex balloon was then inflated under high pressure to split open the obstructive valve leaflets. Hopefully this would take place along the lines of fused valve cusps, but it didn’t always work out that way. Sometimes the valve tore in the wrong direction, then leaked badly. Yet Wilson was so talented and bold with his catheters that he began to dilate valves like Cara’s when the baby was still in the womb. Really scary stuff.

  I was expecting the meeting, dominated as it was by cardiologists, to recommend an attempted balloon valvotomy for Cara – but it didn’t. The technology of magnetic resonance imaging had recently been introduced, and the pictures they gave of her thickened, gnarled and rigid valve were detailed and depressing. There was no point submitting her to another general anaesthetic on a ‘have a go’ basis. I had already published an account of the first infant Ross procedure in the journal Heart and that’s what the meeting wanted for Cara, not more temporising efforts.

  Was there any alternative? Although Cara was small for her age, it might be possible to remove the valve, further enlarge the channel out of the ventricle and implant the smallest mechanical heart valve, possibly a much more straightforward operation – if third-time operations can ever be described as straightforward. But she’d have to take the anticoagulant drug warfarin for the rest of her life and would require a larger valve in a few years’ time. Moreover pregnancy, although possible, would be a nightmare for her in these circumstances.

  After the group discussion about which operation to do, Wilson came right out with it: ‘A mechanical valve will leave her with the lifelong risk of stroke and anticoagulant-related bleeding. That’s the coward’s way out. You described the Ross operation in children. Just get on and do it.’

  So that is what we did – and fortunately it went well. Our homograft bank found an adult-sized donor pulmonary valve that I hoped would last Cara indefinitely. When we followed the Ross operation in children over the years, we found that their own pulmonary valve did indeed grow normally in its new position. As a result we had some miraculous long-term successes. What’s more, the donor valves lasted much longer than we expected in the right side of the heart because the pressure and stresses are much lower.

  So finally, as I have no way of tracing her, here’s my reply to the letter from Cara that I discovered in my papers at the Texas Heart Institute.

  Dear Cara,

  I was desperately sorry to have missed you when you came back to the hospital in Oxford. You must feel that you never met me during the rollercoaster of those three operations but please understand that I knew you very well. I wanted to let you know that I cared about you and your parents during those difficult times. You helped to prove how successful that Ross procedure could be. Donald is no longer with us but he would have loved to have heard your story. I wish you many successful pregnancies and a happy life. I hope someone sees this letter and tells you about it.

  Love to you and the family,

  Prof.

  9

  hope

  Grim Reaper tirelessly stalked the hospital corridors with his scythe, hoping for me to screw up. Sometimes I did, mostly I didn’t, but I never let anyone go without a fight. My motto was Winston Churchill’s message to the country in the dark days of the Second World War: ‘We shall never surrender.’ Winston’s grave was the halfway point of my Blenheim Estate jogging – perhaps ‘staggering’ – circuit, which I’m too old for now. I would sit and talk with him on the bench donated by the Polish resistance. And there were always flowers there all year round, often with the message ‘Hope springs eternal’. I had hope, my patients had it too, as did their loved ones. In the hospital, love, hope and triumph are bedfellows, while disappointment and grief lurk menacingly in the wings – or under my operating table. The difference between these extremes was skill, resilience and unstinting effort. Does that glorious trio still exist these days?

  On a cold, miserable February morning I was about to take a patient off the bypass machine after an aortic valve replacement when a nervous blonde head poked through the operating theatre door. This time it was the paediatric cardiology registrar. Would I come straight away to the children’s intensive care unit? There was a dire crisis. My patient’s heart was beating vigorously, so my assistant came around to take over. With a sense of déjà-vu I backed away and peeled off my blood-caked gloves.

  ‘It had better be an emergency’ was the only thing I could say.

  My registrar was a safe pair of hands, but through bitter experience I had learned that it was not a sensible time for me to leave. The intensive care unit was just a hundred yards down a straight corridor, past the accident department. The messenger moved quickly at a trot, betraying her considerable stress. By the time I arrived she was already holding the heavy door open, while the nursing sister held open a second swing door between the relatives’ waiting room and the inner sanctum. It was a clear statement. We want you in here quick. And what kept you?

  Green drapes were drawn around the bed, but through a parting I saw the frenetic activity and the fact that the stark tableau within spelled death. Someone announced, ‘He’s here,’ but no one looked up. They were in the process of trying to resuscitate Sophie, a thin, deathly pale fifteen-year-old. Anaesthetists, cardiologists and paediatricians – all were huddled around the one body. My eye was drawn to the long wide-bore needle poking into her chest directly over the heart. A large syringe was withdrawing heavily blood-stained fluid, then pushing it out into a plastic bag through a three-way tap. So far there was half a litre of the stuff, which had been compressing the ventricles. The anaesthetist was rhythmically squeezing a black rubber gas bag, blowing in oxygen through corrugated plastic piping down into the girl’s stiff lungs through the tube sticking out of her throat. I glanced instinctively at the monitor screen. Her heart rate was 130 beats per minute, much too fast; her blood pressure was half what it should have been, but that was OK – better low than nothing. Mercifully the girl was already deeply unconscious, completely unaware of being transfixed through the chest by that needle. Grim Reaper was trying to take her, but the resuscitation team wouldn’t let go.

  It was the start of the real battle, although she’d already been through multiple skirmishes. Her brown case-notes folder lay open on the table. The opening entry was from the district general hospital to which she had been first taken. It read:

  Sunday 16 February. 11 pm. Fever, neck stiffness, headache and muscle pains. Started having knee and elbow pain on Saturday afternoon. Went to sleep it off but still present. Went to Dad’s on Saturday evening when the temperature reached 104°F. Worsening headache, vomiting and generalised body ache. Stayed in bed yesterday. Today worsening headache despite 200 mg Ibuprofen x 4. Vomited three times. Now has neck stiffness and generalised limb pains. Heart rate 104. Blood pressure 95/50. Diagnosis viral illness but exclude meningitis. Pupils OK.

  As if all this were not miserable enough, she then had to endure three unsuccessful attempts at a lumbar punctu
re by junior doctors, the aim being to tap a specimen of cerebrospinal fluid from the spinal canal, the same fluid that surrounds the brain. In meningitis, this crystal-clear fluid becomes cloudy with white blood cells and in the worst cases it looks milky, full of bacteria. I did lots of lumbar punctures as a houseman at Charing Cross, shoving the long needle in one direction then another, trying to find a way through into a narrow space past arthritic vertebral bones. The patients hated it and I hated it, but I always found the fluid in the end. Often lives depended on it. But Sophie’s doctors had failed and given up, which was inexcusable. If it had been meningitis she would be dead already. Instead, they put in a drip in her arm to give her fluids and took blood for culture to see if there were any bacteria in her blood stream.

  Over the next twelve hours Sophie became desperately ill. Antibiotics were given into the drip, but without isolating a bug it was all guesswork and her condition worsened. The next evening her blood pressure started to fall and her heart rate climbed to 120. Next, they transferred her to the coronary care unit of the district hospital, then by ambulance to our children’s intensive care unit in Oxford. Powerful vasopressor drugs were needed to keep her blood pressure above 60 mm Hg as she deteriorated into septic shock. The next morning, the bacteriology laboratory reported a heavy growth of Staphylococcus aureus bacteria in Sophie’s blood, a common organism that thrives on skin but is extremely dangerous when it enters the blood stream.

  It was then that a painful hot red swelling appeared on the back of her right hand, diagnosed as septic arthritis by the intensive care doctors. An urgent cardiac echo was performed that evening by one of the unit registrars but was deemed unremarkable. Worryingly, this particular staphylococcus organism was found to be resistant to treatment with penicillin, so the antibiotics regime was changed. Sophie was becoming short of breath and began hallucinating. An urgent chest X-ray showed fluffy shadowing and fluid accumulating in both chest cavities around the lungs.

  The following morning the plastic surgeons decided to drain the pus from the infected hand, believing it to be the source of the blood-stream infection. Or was the blood-stream infection the cause of the septic arthritis? Impossible to say, given that single snapshot in time and an ‘amateur night’ echo that had missed the point. Because she was desperately sick, the surgery was carried out with only local anaesthetic, which made it miserable – to say the least – for poor Sophie. But sure enough, the fluid evacuated from the joint did grow the same staphylococcus bug. Then another cardiac echo performed by Dr Archer showed fluid accumulating around the heart. She was becoming anaemic and needed more intravenous fluids to keep her blood pressure up, added to which her temperature chart was still swinging up and down like Wall Street on a nervy day.

  One week after Sophie first came to Oxford, Dr Archer detected a new heart murmur. The echocardiogram showed the mitral valve to be leaking and yet more fluid around the heart. With increasing echo density, this fluid was beginning to look like pus, and she was now so anaemic that she needed a blood transfusion. Her anxious little family gathered around the bedside. Despite heavy-duty antibiotics, Sophie remained critically ill. Her mum Fiona and sister Lucy already believed that they would lose her, so they had moved into a room in the hospital. There followed more antibiotics, more fluids and more blood pressure drugs – but still no improvement. With the relentless swinging temperature, she suffered worsening delirium and terrifying nocturnal hallucinations. How sick can one kid get without an obvious diagnosis?

  Next morning came the catastrophic deterioration that caused me to be summoned to her bedside. Sophie finally suffered terminal cardiovascular collapse and respiratory arrest, prompting a call for the paediatric resuscitation team and finally the heart surgeon. Without aggressive resuscitation measures she was dead at this point. First they had to intubate her windpipe and take control of her breathing, then relieve the pressure on her heart by drawing out the infected fluid that was compressing it, hence the big hollow needle and syringe. As her blood pressure started to improve, Archer took the echo probe to show me the cause of the crash. Sophie’s mitral valve had been infected all along and had suddenly disintegrated under pressure. Huge lumps of infected proteinaceous material were dangling from it, ready to break free and fly off into her brain. Had she needed cardiac compressions, the likelihood was that these would have already detached and caused a massive stroke.

  But now it was essential for her to go directly to the operating theatre to replace that buggered valve. No ifs, no buts, no debate. The honeymoon period with improved blood pressure wouldn’t last. The infection was winning. Every time the left ventricle contracted – a frenetic 130 times per minute – more blood was refluxing backwards into the left atrium than passing forwards to the aorta. Sophie’s heart was barely pumping any blood onwards and her lungs were filling with fluid. Either the previous echoes had missed this morass of teaming bacteria eating their way through the tissues or the bug was so damned aggressive that we were unlikely to succeed whatever we did.

  The second planned patient of the day had been given premedication and was already on her way round to the anaesthetic room. The poor woman had to be turned back at the operating theatre doors, a dire experience for any anxious patient and their waiting family, all now psychologically prepared for heart surgery. I went back to the theatres myself and told them to get ready for an urgent mitral valve replacement in a desperately sick teenager. What was the name of the patient? I didn’t even know that. I hadn’t asked. Nor had I spoken with the frantic parents and sister. There simply hadn’t been time.

  Having announced the change in plan, I returned to the intensive care unit. Archer was in the relatives’ room with them all, a highly intelligent family who had been told that Sophie was dying. He had already done the difficult bit and explained the bad news, and profound worry was now etched on three desolate faces. How do you make the impending death of a child any easier? Give them some hope, maybe. That magic word. Hope was my job, and with my ingrained optimism and lack of self-doubt I explained that although an infection had completely destroyed the mitral valve, we could replace it. We were fortunate to have the chance of getting into an operating theatre quickly and needed to do so as soon as possible. There was one last thing to warn them about – if Sophie was given an artificial valve she would have to be anticoagulated with warfarin for the rest of her life. Warfarin is needed for prosthetic valves to reduce the production of clotting proteins by the liver. I sidestepped further questions by saying I was going to push Sophie around to the theatres myself as we couldn’t wait for porters. It had to be now.

  We bypassed the anaesthetic room and deposited Sophie’s unconscious body directly on the operating table. Most of the necessary cannulas and electrodes were already in place to hook up to the operating theatre monitors, my instruments were all laid out on blue linen – a paediatric set and an adult set, because she was in between – and the nurses were ready. The white fluffy swabs were carefully counted and stacked in a pile. Sophie was skinny and pale, white like her gown that was soon tossed on the floor. The scrub nurse and registrar painted her yellow with iodine solution, then put on the blue drapes. I tried to adjust the operating theatre lights, but they were brand new, cheap and with a life of their own. Everything we had was either new and cheap, or old – the instruments, the saws, the ventilators, the heart–lung machine, all repaired time after time. That’s what we had to work with, but there was no point moaning about it.

  I opened her quickly. Already the blood biochemistry was poor as her disintegrating heart gave up the ghost. Sophie was pretty close to being a ghost herself now and needed to be safely on cardiopulmonary bypass, when we could correct everything by filtering her blood. When I bisected her breast-bone I could see that there was still a substantial amount of yellow fluid and debris within the pericardium around the heart, strands of infected protein, teeming with staphylococcus, that had precipitated out of the inflammatory s
oup. We sucked and scraped it all out, then I put in the bypass pipes and the perfusionist started his machine. For now, Sophie was safe. It was time to assess the damage.

  Her juvenile left atrium was very small, so I approached it through the right atrium then across the septum between the two. Now I had sight of the mitral valve, which looked as if it were covered in seaweed. There was an abscess in the junction between the anterior and posterior leaflets to my left, the tissue deeply eroded by the bacteria and torn, causing the valve to separate away from the wall of the heart. My first impression was that it all needed to come out, but instinctively I first started to clean away the infected gunge to see what was left, gradually cutting back to the healthy tissue that would hold stitches. The first assistant was hauling uncomfortably on the atrial retractor, bewildered as to why I wasn’t making my usual rapid progress. But I’d decided to try to repair the damage and save the valve, sparing Sophie the risk of lifelong anticoagulation that would most likely rob her of a safe pregnancy.

  I wondered whether I could rebuild the damaged edges of both leaflets with pericardium. The fibrous membrane from around the heart was the most appropriate material to use, but because Sophie’s own pericardium was teeming with bacteria I used cow pericardium instead, sheets of sterilised tissue that are prepared by the cardiovascular industry for the specific purpose of repairing the heart or blood vessels. I cut out an oval patch to sew into the mushy heart wall, onto which I reattached the flail valve leaflets. This resulted in the valve orifice becoming smaller, but not sufficiently to obstruct the flow. At one corner I used an extra strip of human aorta to reinforce the repair. Picasso would have been proud of me. Or perhaps Henry Moore, the sculptor. Sophie’s heart now contained bits of both dead person and dead cow. I hoped this piece of applied fine art would stand up to the pressure when the heart started to pump again. We would soon find out.