Fragile Lives Page 24

  In the end she donated her liver and both kidneys, so three patients benefited. The fact that these organs still functioned normally was a testament to ECMO.

  Within days we needed the equipment again, this time for a young woman who’d just given birth and had suffered an embolism of uterine fluid to her lungs. All I could advise was to send her directly to an ECMO centre, knowing full well that the delay would prove fatal. Unfortunately, I was right.

  Then I could have used it for a forty-year-old patient who’d suffered an accidental air embolism and cardiac arrest in our own intensive care unit. She died. And so the saga goes on.

  The young woman’s death caused real distress amongst her university friends and teachers at Brookes, so much so that I wrote to the vice-chancellor, expressing my own regret that we couldn’t have saved her after her friends had made such a valiant effort when she collapsed. Months later I received an invitation to the university graduation ceremony. They intended to award her a posthumous degree, and would I please come with the parents?

  I sat with her mum, dad and boyfriend in the front row, and we watched the bright young men and women come up to the stage to collect their awards. Then the chancellor Shami Chakrabarti gave an explanation about the special award and thanked the surgeon for his valiant attempts to save her. Someone had to go up and receive the certificate. Mum was the one. Dad was frozen with grief and her boyfriend sat desolate. I was choked. I couldn’t speak, but I helped the poor lady stumble up the stairs. This was not how it was meant to be, not the anticipated end to her university career. All her friends and tutors rallied round. The family were happy to see them and bravely they went to the reception.

  But I was bitter and twisted. I went away crushed, the weight of the world on my shoulders. It was the saddest day of my whole career.

  In memory of Alice Hunter, so others might be saved.

  15

  double jeopardy

  When I was young and full of life,

  I loved the local doctor’s wife,

  And ate an apple every day

  To keep the doctor far away.

  Thomas W. Lamont, My Boyhood in a Parsonage

  Julia was forty. Pretty, blonde and feisty, she had a busy career in London. At the weekend she was an accomplished event rider, not far from the top tier, often rubbing shoulders – or bridle – with the best. So she’d left it late to have her first baby. But it would be fine as she was fit, both physically and mentally. Indeed as a psychology student at Durham she’d played hockey for the university, then for her county Leicestershire. And football. And cricket.

  There was one funny thing, however. She could never complete a bleep test, something always holding her back. And she regularly fell asleep in meetings, so much so that she was admitted to a private hospital for sleep studies. They suspected narcolepsy, but nothing was found and it cost a fair bit.

  She was deliriously excited when the pregnancy test turned blue in April 2015 in only the second month of trying. Bingo! But then she started to get really tired, then a bit breathless, then more breathless – just getting onto the horse – although she was reassured that it was normal in pregnancy, and was all down to hormones and retained fluid.

  Desperate not to let her exhaustion beat her she started running again, determined to get fitter. The first time she pushed herself for five kilometres, but the following week she was breathless by the end of the street, with a burning throat and a tight chest. Her breasts were tender and swollen, and she thought sore breasts could be part of the problem. She just had to slow down a bit, but at least she could still ride.

  At thirteen weeks she saw the midwife at the doctor’s surgery on a Monday. She was advised to take aspirin as prophylaxis against pre-eclampsia – the dangerously high blood pressure some women suffer during the later stages of pregnancy. She mentioned how unfit she felt and how quickly the situation had deteriorated. Instead of dismissing Julia as neurotic, the midwife’s response was that she should get her heart and lungs checked out, promising to have a word with her doctor. Good for the midwife – it was a critically important decision.

  The doctor listened as doctors should. He was kind and reassuring. ‘Blood volume increases by a third during pregnancy,’ he told her. ‘It can make you breathless. Let me just listen to your chest.’ Then his tone changed and he suddenly looked serious. ‘Just a slight murmur. But we should get someone to take a look at you quickly.’

  Soon he was on the phone to the Windsor Clinic in Maidenhead. A cardiologist would see her on Wednesday, the day after tomorrow. Julia was anxious but went back to work. United Biscuits needed her and it would keep her mind off the ‘murmur’ word.

  The Windsor Clinic had a nice waiting room, an efficient receptionist and a comfortable sofa, although none of this mattered a bugger to Julia. There were two important tests planned before she saw the cardiologist. So off with her smart black dress and on with the ubiquitous white gown; it showed her bum as it had ties down the back that you just couldn’t reach.

  First the electrocardiogram. She got up on the couch and a lady asked her to pop the top of her gown off. Sensors detected the electrical activity on her wrists, ankles and across her chest wall, then the ECG machine rapidly spewed out a long strip of pink paper with a black squiggly line. This was very important to doctors, completely meaningless to anyone else, but the technician said it was fine. How reassuring was that! Except it wasn’t fine.

  To the trained eye Julia’s ECG showed what we call left ventricular hypertrophy – heart muscle under strain. Next she had an echocardiogram, the non-invasive window on the heart, which took ultrasound pictures with a probe and projected them on a screen. This time Julia coloured up a bit as it was done by a man, although he was nice and chatty as he smeared viscous jelly on her chest. All part of the job.

  It took a while to get good pictures. He worked around her swollen left breast, trying not to hurt her. He started with her heart chambers – the left and right ventricles – seen best through the ‘four chamber’ view. The left ventricle was thicker than expected. The right ventricle, left atrium and right atrium all normal. But the ultrasound hadn’t yet got the money shot. He shifted the probe to the top of the breastbone and angled it downwards.

  Then his demeanour and expression changed abruptly. He went quiet and fiddled with the probe, and Julia sensed impending bad news. Her heart sank and she experienced that sudden cold, empty feeling, like your guts just fell out.

  ‘What is it?’ she couldn’t help asking.

  ‘Tight aortic stenosis.’ It sounded like an automated reply. ‘I’m so sorry. I’ll go and tell the doctor.’

  Then another lady came with a different echo machine to look for the baby, this time smearing slimy jelly on her belly. It was Julia’s first introduction to her foetus, and there was concern whether it was still alive. Then, from the ensuing dialogue, she sensed that it might be better if it wasn’t. While Julia’s day was unravelling, the foetal heart was still beating away normally at around 150 beats per minute.

  It was now time to see the doctor, a smart young cardiologist who also worked in the NHS. He had already reviewed the investigations, and while he knew the diagnosis there was nothing he could do to help. But at least Julia had her clothes back on now, so she felt less exposed, less physically vulnerable, although she was verging on psychological meltdown. From her undergraduate studies she knew a lot about psychology, yet it didn’t make it easier to control her own.

  She spoke first, with no exchange of pleasantries. ‘I’m in trouble, aren’t I?’

  ‘Yes, I’m sorry.’

  That bloody word again. All doctors used it but none of them meant it.

  ‘You’ve got very severe aortic stenosis. Congenital aortic stenosis, in fact. Didn’t anyone hear the murmur before you decided to have the child?’

  Julia thought carefully. Other doctors had listened to her chest, yes; but no, no one had mentioned a heart murmur.

  When the
valve becomes very tight it can be difficult to hear one. Now it was very tight and her symptoms had been unveiled by the expanded blood volume – the extra work that a heart has to do to support the placenta.

  To explain the physiology of what had happened, it’s important to appreciate that from the twelfth to the thirty-sixth week of gestation the volume of blood pumped by the heart rises to a peak of 50 per cent above non-pregnant levels. Julia had hit the buffers by thirteen weeks because she had a severe narrowing of the valve at the outlet of the left ventricle. The crushing chest pain when she exercised stemmed from poor flow in the coronary arteries. When the pressure in her arm was 100 mm Hg, it was 250 mm Hg in the left ventricle – dangerously high. In addition, blood trying to enter her heart was held back in the lungs, causing them to be stiff. Further strain of any kind could cause oedema fluid to flood the lungs, risking sudden death. And Julia thought she was fit!

  Now the coup de grâce. The life expectancy for severe aortic stenosis without the baby would be between a further six and twenty-four months at best. In her current situation it was only weeks. It was far too dangerous to continue with the pregnancy, so the cardiologist felt that he should arrange an abortion to take place before the weekend. Then it would be possible for her to have an operation to replace the aortic valve. She needed it soon.

  This was not at all what Julia wanted. She’d left it late to have kids, but after three months of excitement and expectation she was attached to her foetus. And not just by the placenta. What if she never had another chance? She felt fine as long as she was doing nothing. Surely she could just do nothing until the baby was born? Simple logic and a price worth paying. But wrong. The cardiologist was in no doubt whatsoever that if nothing was done both Julia and the baby would die long before it could be delivered, even if it was delivered prematurely in twenty weeks’ time.

  Her options were limited. No surgeon would operate on her aortic valve while she was pregnant. If she wanted he would discuss her case the following day at a multi-disciplinary team meeting with a group of cardiologists, surgeons, intensive care doctors – and in Julia’s case, obstetricians – who would review the information in detail, consider the alternatives and recommend the right thing.

  But Julia was no wilting violet. ‘What about my opinion?’ she insisted. ‘I want to keep my baby. Not have people ganging up on me. What’s my best chance of keeping the baby?’

  This wasn’t an easy question to answer. There was no straightforward solution. He thought for a minute, then said, ‘I’ll get you to a cardiologist in Oxford who specialises in heart problems during pregnancy.’

  The ethical principles pertaining to pregnancy are straightforward. The doctor’s first responsibility is to the mother, and while a baby may be sacrificed in utero to sustain the mother’s health, it’s not acceptable to put a mother at risk for the sake of an unborn child. A baby will normally survive if delivered after thirty weeks, even twenty-eight weeks. But only rarely have dying mothers been kept alive with the sole objective of sustaining a foetus.

  The cardiologists at the district general hospital saw the echo images. They judged the valve to be far too tight for Julia to reach thirty weeks’ gestation for a Caesarean section. The hormonal changes and the increase in blood volume were already life-threatening and she wouldn’t survive another sixteen weeks. Everyone’s opinion was the same – Julia should be advised to have a termination of pregnancy within days, then have the aortic valve replaced soon afterwards. An abortion would turn a complex problem into a simple one, assuming you judge cardiac surgery to be simple.

  The cardiologist phoned her at work that Thursday afternoon and summarised the depressing consensus from his colleagues. She winced at that ‘sorry’ word again, but he’d arranged an appointment for her to see Dr Oliver Ormerod in Oxford the following afternoon, on the NHS. He emphasised that there was no time to spare and that in the meantime she absolutely must not ride or do any exercise of any sort.

  Getting to the appointment itself was a nightmare – traffic queuing on the main roads to reach the hospital, traffic queuing to get into the car parks, no parking spaces, no help. She was going to be late for the most important appointment of their two lives and on top of it all she had that crushing chest pain again, followed by crippling anxiety. Last Friday she was an excited mother to be. Now she was filled with impending doom.

  Oliver changed all that, as he was altogether different. Not wearing a suit or tie, and not seeming to take things at all seriously, he reminded Julia of one of her childhood favourites, Popeye the Sailor Man. He made her feel that she was the special one in that consulting room.

  ‘You want to keep your baby? Let’s see how we can help you with that.’

  The tightness in her chest disappeared, a wave of relaxation flowed through her body and her hand involuntarily dropped down to the little bump as if to say, ‘Don’t worry! This doctor will look after us both.’

  So what were the possibilities for keeping Julia safe and the baby alive? Oliver agreed that the valve couldn’t wait until the baby was viable at twenty-eight weeks’ gestation. Therefore the valve would have to be dealt with while trying to preserve the pregnancy. There were two potential ways forward. The first was a balloon dilatation of the critically narrowed valve orifice as a temporising manoeuvre, to buy them some time. The second was to get on with open heart surgery on the heart–lung machine. All previous medical opinion had been against the latter.

  Balloon dilatation was done in the catheterisation laboratory under X-ray guidance but the uterus could be shielded from the radiation. The balloon would be inflated within the narrowed valve orifice to split and open the fused parts. If this would carry Julia through to thirty weeks the valve could be replaced after the baby was delivered. She’d then face safer heart surgery as a new mother.

  My colleague Professor Banning was an expert in balloon valve interventions, and Oliver needed more detailed echo pictures of the valve to show him. If he agreed, the procedure would be carried out early the following week. But what were the risks? The valve might split and leak badly, causing acute heart failure, so a surgical team would need to stand by in the operating theatres. Alternatively, the valve might not open sufficiently to make a difference. Either way there was a significant risk to the mother and baby. It was not straightforward.

  Oliver decided to admit her to the cardiology ward after the weekend. In the meantime he would talk to the only surgeon he knew who’d operated on patients in similar circumstances.

  Oliver called me at home on the Friday evening and we talked about our previous experience. The last pregnant patient we’d managed between us had an unusual murmur discovered at twenty-eight weeks. She was found to have a massive but benign tumour in the left atrium, a left atrial myxoma like Anna’s. We watched her carefully for four weeks in hospital, then delivered the baby by Caesarean section in a cardiac operating theatre at thirty-two weeks. Three days later I removed the tumour. Both did well.

  Before that we’d treated a young woman with an infected artificial heart valve that was disintegrating and leaking badly. We took her to the operating theatre at thirty-three weeks and performed the Caesarean section, then I re-replaced the aortic valve at the same sitting. Mother and baby did well, although we had problems with bleeding from the uterus.

  Then I reminded Oliver that in another hospital I’d replaced an aortic valve in a thirty-five-year-old with a twenty-week foetus. The valve replacement was fine and the baby had a detectable heartbeat afterwards. But in the middle of the night she aborted and haemorrhaged profusely. We came close to losing the mother as well as the baby.

  Heart surgery in pregnancy is one of those rare procedures in which you can actually lose two patients – mother and baby. I’d read and analysed every published report about heart surgery during pregnancy, then produced a detailed review. At the time there were only 133 cases worldwide, only nineteen being aortic valve replacements. No mothers had died but seven of the
babies were lost. Was this reassuring? No.

  The big problem is that surgeons prefer to report successes, so there could have been hundreds of unreported cases where the babies – or even the mothers – had died. But best keep schtum about these ones, eh? It’s human nature. Nevertheless, we had some statistics to share with Julia and her family.

  Oliver then asked me what I thought about the balloon option. I said it was a good idea but there were practical issues. Most congenitally deformed aortic valves didn’t have defined parts that would separate under balloon pressure, not like rheumatic mitral valves for which the technique was well established. It was essentially a blind procedure – the valve might be destroyed, and the aorta might even split and bleed torrentially. We needed to ask Banning what he thought about the chances of success. If they decided to take the valvuloplasty route, I’d do my best to provide back-up. We left it at that.

  After the weekend Julia was brought back into hospital for more tests. News of her pregnancy dilemma had spread fast, so there was an impressive turnout at the congenital heart team meeting at the crack of dawn on Thursday morning. We were joined by our paediatric cardiology colleagues from Southampton, and Oliver presented the case with superb new pictures of her heart.

  Julia’s aortic valve orifice was a narrow slit, and instead of having three cusps there appeared to be just one, what we call a monocusp valve. This looked like a rocky volcano, and was almost 1 cm in depth and rigid. The muscle below was ominously thick and it was curious that she’d reached the age of forty in this state. Would the balloon make a difference? Unlikely. Was it safe? Unlikely.