My plan was to clear the obstruction and close the hole in the heart, an ambitious attempt to restore normal physiology. This was straightforward in principle yet taxing in a back-to-front heart in the wrong side of the chest, and I didn’t want any surprises. So I did what I always do in difficult circumstances – set about to draw the anatomy in detail.
Was the surgery possible? I didn’t know, but we had to try. Even if we failed to remove the tumour completely it would still help him, although should it prove to be a rare malignancy his outlook would be bleak. But between us we were convinced that it was a rhabdomyoma.
It was time to meet the boy and his mother. Mayo man took me to the paediatric high-dependency unit where he was still being fed via a tube through his nose, which he disliked intensely. His mother was sitting cross-legged on a mat on the floor beside the cot and she never left his side, day or night.
As we approached she rose up. She was not at all what I’d expected – stunningly beautiful, with a striking resemblance to the model Iman, the widow of David Bowie. Her jet black hair was straight and long, her skinny arms folded across her chest. The Red Cross had established that she was Somali, and as she was a Christian her head was not covered.
Her long delicate fingers were clutching the bundle in which she held her son, precious rags that had shielded him from the hot sun then kept him warm in the cold desert nights. An umbilical cord of drip tubing emerged from these swaddling clothes and stretched to the drip pole and a bottle of feed, which was a milky-white solution replete with glucose, amino acids, vitamins and minerals to put meat back on his little bones.
Her eyes turned towards the stranger, the English heart surgeon whom she had heard about. Head gently tilted backwards in an attempt to remain detached, a bead of sweat appeared in the root of her neck and slithered down over the sternal notch. She was becoming anxious and her adrenaline was flowing.
I tried to engage with her in Arabic. ‘Sabah al-khair, aysh ismuk?’ (Good morning, what’s your name?). She said nothing and looked at the floor. Showing off, I continued, ‘Terref arabi?’ (Do you know Arabic?), then, ‘Inta min weyn?’ (Where are you from?). Still no response. Finally in desperation I asked, ‘Titakellem ingleezi?’ (Do you speak English?). ‘Ana min ingliterra’ (I’m from England).
Then she looked up, wide eyed, and I knew that she understood. Her lips parted but still no words. She was mute. Mayo man was speechless too, stunned by my linguistic skills, which unbeknownst to him had almost been exhausted. She appeared to appreciate my efforts and her shoulders dropped. She relaxed. I wanted to show her kindness, to take her hand, but I couldn’t in this environment.
I indicated that I’d like to examine the boy, which was fine as long as she could continue to hold him. But I was shocked as she pulled back his linen covering. The lad was emaciated, with all his skinny ribs protruding. There was virtually no fat on him and I could see his bizarre heart pounding against his chest wall. He was breathing rapidly to overcome the stiffness in his lungs, his protuberant belly full of fluid and his enlarged liver clearly visible on the opposite side to normal. From the different skin tone to his mother I assumed that his father was an Arab. A curious rash covered his dark olive skin and I thought I saw fear in his eyes.
His mother pulled the linens back over his face, protectively. He was all she had in the world, this boy and a few rags and rings, and I couldn’t help the upwelling of pity I felt for both of them. Surgery was my business but I was sucked into this whirlpool of despair, my objectivity gone.
In those days I had a red stethoscope and I placed it on the infant’s chest, trying to look professional. There was a harsh murmur as blood squeezed past the tumour and out through the aortic valve, then the crackling sounds of wet lungs, even the gurgling and bubbling of empty guts. The cacophony of the human body.
Next I said, ‘Mumken asaduq?’ (‘Will you let me help you’?). For a second I thought she responded. Her lips moved and those eyes fixed on mine. I sensed that she’d murmured, ‘Naam’ (Yes). I tried to explain that I needed to operate on the boy’s heart to make him well so they could both have a better life. When tears appeared in her eyes I knew that she understood.
But how could I persuade her to sign a consent form? We sent for a Somali interpreter who repeated my words, yet still we had nothing in return. She remained impassive as I struggled to convey the complexities of the surgery. Name of the operation: ‘Relief of left ventricular outflow tract obstruction in dextrocardia’. Then a short sentence for my own benefit: ‘High risk case!’ Absolving me of any blame, on paper at least. I was confident that this was the boy’s only chance of survival, so just an ‘X’ from the mother would be enough. But she was signing over her whole life, her only reason for living. Eventually she took the pen from my hand and scribbled on the form, then I asked Mayo man to countersign and I signed it myself, looking directly into her eyes not at the document, searching for approval, I guess. By now her skin was glistening with perspiration; she was pouring out adrenaline and trembling with anxiety.
It was time for us to leave her alone. I indicated that I’d do the operation on Sunday when the best paediatric anaesthetist was available, then I said goodbye in both English and Arabic to show that I was still making an effort.
This was Thursday afternoon, the day before the Saudi weekend, and my colleagues were planning to take me out to the desert to camp on the dunes beneath the night sky as a way of escaping the oppression of the city. The convoy left in the early evening, just as the searing heat was starting to abate. When we ran out of road, the jeeps ploughed through the sand, miles and miles of it. They had a rule – never travel in just one vehicle. If it broke down that could be the end, even within twenty miles of the hospital.
The desert night was clear and cold. We sat around the campfire drinking homemade hooch and watching shooting stars. A Bedouin camel train passed by silently barely two hundred yards away, swords and Kalashnikovs glinting in the moonlight. They didn’t even acknowledge us.
I felt uneasy and wondered how the mother had coped. Walking at night, hoping to find shelter during the day, and carrying water and child together, she must have been fuelled by hope but little else. However difficult it would prove to be, I was driven to save the boy and watch them both grow stronger.
The operation would be far from straightforward as I was still unsure of how to get at this tumour. The obstruction could only be accessible by opening the apex of the left ventricle wide, and that would impair its pumping ability. I kept working through the steps of the operation in my mind, always returning to the same question – ‘What if?’ With conventional surgery the technical challenges posed by this dextrocardia heart were virtually insurmountable. So would the boy be better off if he were operated on by a more experienced surgeon in the States? I couldn’t see why, because his combination of pathologies was probably unique. No one else would have greater experience, even if they did have a better team. I had a good enough team and great equipment, the best that money could buy. So I was the man for the job, wasn’t I?
It was then that I had my eureka moment, while staring up at the Milky Way. I suddenly knew the obvious way to get at the tumour. It might have been an outrageous idea, but I had a plan.
On Saturday I brought the anaesthetic and surgical teams together to discuss the case, and showed them the novel pictures of the unusual anatomy. Then, unusually – as much of what happens in an operating theatre remains impersonal, which is perhaps best when operating on those who may not survive – I told them the heart-rending story of the mother and boy. Everyone agreed that the boy was doomed if we made no effort but voiced justifiable concerns that the tumour was inoperable in dextrocardia. I said that we’d only know that through trying, although I kept the operating plan to myself.
I spent a hot, restless night in the apartment, my mind racing, disturbed by irrational thoughts. Would I have risked this back in England? And was I doing it for the patient or for the mother �
� or even for myself, so I could publish a paper about it? If I succeeded, who would care for this slave girl and her illegitimate child? The boy was an inconvenience. In Yemen he would be left out under a bush for the wolves to eat. It was the mother they wanted.
The early-morning call to prayer put an end to my discomfort. It was already 28°C as I walked from the apartment to the hospital. Mother and boy came down to the operating theatre complex and anaesthetic room at 7 am. She’d stayed awake until morning with her child in her arms, and all through the night the nurses had been concerned that she might capitulate and run away. She didn’t, but they were still worried whether she would hand the boy over.
Despite premedication he was screaming and thrashing around when they tried to put him asleep. Dreadful for the mother – and difficult for the anaesthetic staff – this was pretty much routine in paediatric surgery. Gas through the face mask eventually subdued him sufficiently to insert a cannula into a vein and stun him into unconsciousness. His mother still wanted to follow him into the operating theatre, so the ward nurses eventually dragged her away. Finally raw emotion had broken through the mask – whatever she had suffered physically, this was worse for her. Yet there were still no words.
I sat, dispassionate, in the coffee room until the mayhem abated, enjoying thick Turkish coffee and dates for breakfast. The caffeine hit was good for my ADHD but racked up my sense of responsibility. What if the boy dies? Then she has nothing. Nobody in the world.
One of the Australian scrub nurses came through to ask that I check the equipment, the extra bits I’d requested for the radical plan conceived under the dark desert sky. I’d yet to share it with my team.
Uncovered on the shiny black vinyl of an operating table, this emaciated little body was a pathetic sight, with none of the puppy fat that every infant deserves. Instead his skinny legs were swollen with fluid. The heart failure paradox – the muscle is replaced by water but the weight stays the same. His prominent, skinny ribs rose and fell with the ventilator, as he was no longer struggling for breath on his own. Now everyone understood why the mother was so fiercely protective. We could see the heart beating away in the wrong side of the chest and the outline of his swollen liver in the contrary side of the bulging abdomen. Everything was the wrong way round, all a source of fascination for the onlookers and presenting a daunting challenge for me. I’d seen one operation on dextrocardia in the US and another at Great Ormond Street. This would be the first I’d attempted myself.
There were still streaks of dried salt down his cheeks from the traumatic separation from his mother. What was it I used to say when asked if I was ever anxious about undertaking an operation? ‘No. It’s not me on the table!’ But although I don’t do anxiety, I was now in tiger country with an untested procedure in an unfamiliar environment and could feel sweat trickling down my back. It all felt a very long way from Oxford.
Everyone was happier when that fragile little body was covered in blue drapes, leaving just a rectangular window of dark skin exposed over the breastbone. He was now no longer a child, just a surgical challenge. That is until we heard his tormented mother banging on the operating theatre doors. She’d given her minders the slip and rushed back, and after a brief struggle they allowed her to sit in the corridor just outside. Her day had been traumatic enough without being dragged away for a second time.
Back inside the operating theatre the scalpel blade slid left to right along the length of the boy’s sternum until a trickle of bright red blood skidded over the plastic drape. The electrocautery soon put a stop to that as it sizzled down onto white bone, reminding me of that line from Apocalypse Now – ‘I love the smell of napalm in the morning.’ The whiff of white smoke told me that the diathermy had too much power and I reminded the orderly that we were operating on a child, not electing a pope, so would he please turn down the voltage.
Heart failure fluid was pushing up the diaphragm. I made a small hole in the boy’s abdominal cavity and straw-coloured fluid poured out like piss into the wound. The noisy sucker removed almost a pint into the drainage bottle and his belly flattened out. A very quick way to lose weight. The saw zipped up the sternum, spraying beads of bone marrow onto the plastic. It breached the right chest cavity, releasing a knuckle of stiff, pink, waterlogged lung. Yet more fluid spilled out, so the sucker bottle had to be changed. It left no one in any doubt that this kid was seriously unwell.
Impatient to view the congenitally distorted heart, I dissected away the redundant thymus gland and sliced open the pericardium – the fibrous sac that encases the heart – with the same excitement and anticipation as unwrapping a surprise parcel at Christmas.
Everyone wanted to get a good look at the dextrocardia heart before I started, so I took a step back and relaxed for a minute. The plan was to open up the narrowed channel below the aortic valve by coring out as much solid tumour as possible, then close the hole in the atrial septum. I gave the order to go onto the heart–lung machine and proceeded to stop the empty heart with cardioplegia fluid. It lay cold, still and flaccid in the bottom of the pericardial sac. I gently pressed the muscle and could feel the rubbery tumour through the heart wall. By now I was sure that I couldn’t reach it all with a conventional approach and that there was little point cutting into the ventricle that his circulation depended upon purely on an exploratory basis. So I told myself, ‘Just do it.’ Plan B. The eureka option, one that had probably never been done before. The perfusionist began to cool the whole body down from 37°C to 28°C. The boy was likely to be on the bypass machine for at least two hours.
At that point I had no option but to share Plan B with the rest of the team. I would chop out the boy’s heart from his chest and, with it lying on a kidney dish full of ice to keep it cool, operate on it on the bench. Then I could twist and turn the thing as much as I needed to do a good job. I considered it to be a brilliant idea, but I had to work fast.
The process was equivalent to removing a donor heart for transplant then sewing it back into the same patient. Back in my research days I’d transplanted tiny rat hearts. This boy’s heart should be no problem, even if the anatomy was unusual, so I transected the aorta just beyond the origin of the coronary arteries, then the main pulmonary artery. By pulling these vessels towards me, the roof of the left atrium was exposed at the back of his heart. I cut through the atria, leaving all the large veins from the body and lungs in place, then, lifting the ventricles out, I left most of the atria in situ. It was then, as you’d do with a donor heart, that I placed the cold, floppy muscle onto the ice.
Now I could see the tumour within the outflow part of the left ventricle. I started to dissect it out, cutting a channel through it so that it would no longer obstruct the heart. The tumour’s rubbery texture was consistent with it being benign, making me optimistic that we had done the right thing. Both my assistants were shocked and mesmerised by the empty chest and were not assisting well. And the longer this heart remained without a blood supply, the more likely that it would fail when I re-implanted it. Frankly, the Australian scrub nurse was much sharper than these trainees, so I asked her to help. She knew instinctively what was required and injected the necessary pace into the procedure.
I was torn between just doing enough or making a radical job of it. But I wanted to tell the boy’s mother that I’d succeeded in removing all of the tumour so I pursued it into the ventricular septum, close to the heart’s electrical wiring system. I knew where this was situated in a normal heart, but its location was less certain in this case. After thirty minutes I infused another dose of cardioplegia solution directly into both coronary arteries to keep the heart really cold and flaccid, and fifteen minutes later the job was done.
I took the boy’s heart back to his body, aligned the ventricles with the atrial cuffs and started to sew it in. I was really quite impressed with myself, the journal article already half-written in my head. The re-implantation process also closed the hole in the heart, so – with luck – he was cured.
<
br /> This part of the operation had to be fail-safe as these stitch lines would be completely inaccessible in a beating heart. With both atria joined up again it was time to re-join the aorta and let blood back into the coronary arteries. The heart would start beating again and we could warm the boy’s body up. All that was left to do was to reconnect the main pulmonary artery. By then the surgical assistants had also warmed up a bit, on familiar territory once more with the heart back where it belonged.
Usually a child’s heart starts to beat spontaneously and quickly when its blood flow is restored, but this one was too slow. What’s more, I could see that the atria and the ventricles were contracting at different rates. This told me that the conduction system between the two was not working, which is not good as a coordinated heart rhythm is much more efficient. The anaesthetist had already noticed this on the electrocardiogram but said nothing. After cooling, the conduction system often goes to sleep for a while then recovers spontaneously.
Ten minutes later and nothing had changed. I must have cut through the electrical bundle while dissecting out the tumour. Shit and derision. He’d need a pacemaker. This made me more anxious about another issue. A transplanted heart also loses its connection with nerves from the brain, nerves that automatically speed up or slow down the heart during exercise or changes in blood volume. This denervation, together with the disruption of the electrical conduction system, could be a real problem.
My earlier euphoria, optimism and self-congratulation quickly abated, and the young mother drifted back into my thoughts. This wasn’t a good time to lose focus. There was still air within the heart chambers and it had to be let out, so I inserted a hollow needle into the aorta and pulmonary artery. Air fizzed out of both. When air entered the uppermost right coronary artery the right ventricle distended and stopped pumping.
Fragile Lives Page 6