Shelley: Heart
Shelley was born both unlucky and lucky. She was unlucky because she was born with a serious congenital heart defect that, in 1971 or really any other time, didn’t have a very good untreated survival rate beyond a day or two.
She was lucky because, just a couple of hospitals over, there was a surgeon available who had just recently become very, very good at the operation that fixed it.
As a refresher course in how a healthy heart works: the heart has two sides (left and right), each with two chambers (atrium and ventricle, going from top to bottom) separated by a valve each. Blood is received into the atria from the veins, and during the first half of each heartbeat (the ‘ba’ of the ‘ba-boom’) the atria contract, pushing the collected blood through valves to the ventricles below. The valves stop the blood from flowing back into the atria.
During the ‘boom’ of the ‘ba-boom’, the ventricles contract, sending the blood off to a new destination: either the lungs or the body, depending on which side of the heart it’s leaving from. There’s another valve at the entrance of the arteries the blood leaves through, again to stop any messy backwash. Three of the four valves in your heart have three flaps or ‘cusps’ that open and close depending on the amount of blood and therefore pressure there is on either side of them. The other one has two flaps. Weirdo.
The left side of the heart receives lovely bright red oxygenated blood from the lungs into the left atrium, squishes it through a valve to the left ventricle, and squirts it out through the aorta via the aortic valve to all your organs and extremities, giving them the oxygen they need to keep going. The right side of the heart receives duller, bluer blood that’s on its way back from your organs and extremities after they’ve wrung all the oxygen out of it. The right atrium squishes it through a valve to the right ventricle, which sends it on to the lungs via the pulmonary valve to cram it full of oxygen again. And so on. And so on. Ba-boom. Ba-boom.
The problem
Shelley’s heart had a couple of problems, and these problems are often found together. The proper science-y names for the problems don’t provide many clues as to what’s actually going on, so I’ll leave those until later. But Shelley was born an unusual blue/gray colour and noticeably not very well, which was the first clue that something was up, most likely with her heart. She was diagnosed with, in layman’s terms, a valve that wasn’t working and a hole in her heart. Surgery was strongly and urgently advised, which Dad strongly and urgently agreed to, reportedly with the phrase “where do I sign?”. Mum was pretty out of it on 1970s-strength blood pressure medication, so Dad was subjected to a wall of information and decision-making, which he received and delivered in turn like an atrium and ventricle that’s working properly.
The three flaps of Shelley’s pulmonary valve — the one at the entrance to the pulmonary artery, which sends blood from the heart to the lungs — were fused together when she was born. The valve just wasn’t finished. As a result, blood didn’t take its planned route from her heart to her lungs to get oxygenated. The fancy doctor name for this is pulmonary valve atresis, and babies born with it usually look a bit blue at birth, because their blood is far less oxygenated than that of a healthy baby.
The second part of Shelley’s problem was the ‘hole’ in her heart. Every baby has a bunch of holes in their heart while they’re developing in the uterus, including some in the wall (septum) between the left and right atria, but the holes are supposed to close up shortly after birth. As a baby’s lungs aren’t operational until after they’re born, there’s no point in sending blood from the heart to the lungs in utero. A fetus’s blood is oxygenated via the placenta rather than the lungs, and the whole circulatory system is a lot more complicated in the womb than it is after they’re born. When a baby is developing, there’s a connection between the aorta (which sends blood to the body) and the pulmonary artery (which will eventually send blood to the lungs) called the ductus arteriosis. When it stays open after birth, deoxygenated blood can flow in the wrong direction, which isn’t ideal. In Shelley’s case, it’s likely that this connection staying open may have helped to keep her alive in the two days between her birth and surgery. Her fused valve meant that blood wasn’t being sent to the lungs via normal means, so some blood had to make it into the pulmonary artery somehow, and the ductus arteriosis was probably it.
On top of that mess, Shelley had been diagnosed pre-operatively with an atrial septal defect, which is often part of the package, and is the actual ‘hole in the heart’ business. At least one of the holes between the atria that are supposed to close didn’t, meaning oxygenated blood flowing through and mixing with deoxygenated blood. The resulting extra blood flow to the lungs makes a noticeable swishing noise, or ‘murmur’. Not as cute as it sounds.
Mum didn’t even know she was having twins when we were born, partly due to the limited availability of ultrasound scans in 1971, partly due to Shelley and me combined being the size and weight of one big baby, and partly due to the fact that doctors with stethoscopes could hear one strong heartbeat (mine) without paying too much attention to the second, much weaker one.
The surgery
Around 6–8 out of every thousand babies in 1971 were born with congenital heart defects, and at the time there was a general move toward treating tiny little babies with open heart surgery rather than with less invasive, less aggressive, and substantially less successful treatment. Early surgical intervention with new techniques was proving to increase the survival rate of babies with congenital heart defects, and Shelley was lucky enough to be referred to Dr Timothy Cartmill, who had taken over as chief paediatric surgeon at Camperdown Children’s Hospital two years previously, after training and specialising in neonatal heart surgery in the USA. Simply put, he was the undisputed right person for the job, and a leading expert in what Shelley needed, a pulmonary valvectomy. Dad remembers that Cartmill had only performed the procedure in Australia once before, on a baby boy, so Shelley was the second very small thing to undergo the surgery in this country, and likely the smallest. While I stayed and probably slept through everything in St Leonards, Shelley was transported promptly to Camperdown.
Shelley and I were born slightly prematurely, a month before expected, and the heart of a full-term newborn baby is roughly the size of a walnut or a big strawberry. The size of the pulmonary valve is obviously smaller still, so imagining the size of the instruments Dr Cartmill used and the strength of the magnification necessary is just utterly mind-boggling.
On the 2nd of June 1971, Dr Cartmill opened Shelley’s chest and sternum and clamped her veins and arteries. He waited for her heart to empty and using a (tiny) blade and my father’s description, “cut along the dotted lines”, slicing her pulmonary valve into the three cusps it should have organised itself into originally. He released the vein and artery clamps, let the heart fill up again, checked that the muscles of her heart became pink, closed her sternum with metal staples and stitched up her chest.
The whole thing still freaks me out.
Afterwards
Shelley had a scar on her chest that our family affectionately referred to as her ‘zipper’. It started near the hollow where her neck meets her chest, and ended near the bottom of her breastbone. Her ribs were always at a slightly different angle to mine, and as she got older, sometimes the staples in her ribcage would ache. You could see the staples in chest x-rays she had later to check on them — bizarre bright white tangles against muted grey ribs. Occasionally if Shelley exerted herself her lips would go a little blue, but she managed to get a black belt in karate and give birth twice, so it’s fair to say the whole experience never really slowed her down.
Shelley’s ongoing cardiologist was another legend of the industry, Dr John Celermajer, and when she was five she had a coronary angiography to check everything was as it should be — basically a camera on a long tube that was inserted into a vein in her groin and fed up to her heart to have a look around. Thinking about it makes me feel icky, but I’m pretty glad it happened.
As an adult, Shelley went to hospital for a probably-unrelated heart problem which is nicknamed ‘broken heart syndrome’ because its actual name — Takotsubo cardiomyopathy — is just impolitely complicated. It’s named after Japanese octopus traps that apparently mimic the heart’s shape in sufferers, where stress or intense emotions weaken and enlarge the muscles in the left ventricle. The symptoms are extremely similar to those of a heart attack, which is very much a no thank you situation, but with a significantly lower risk. After checking that it wasn’t a heart attack, Shelley stayed in hospital for a couple of days for observation. While Mum and I visited her the day before a federal election, an Electoral Commission official brought a cart of voting slips around asking if patients wanted to vote absentee, and offered all three of us the opportunity. We sat on Shelley’s hospital bed, ticking boxes and chatting.
Treatment for Takotsubo cardiomyopathy is temporary medication and learning how to relax more. Shelley did, and I can report confidently that a yoga session on the excellent lawn in her backyard followed by a cup of tea and a biscuit is an extremely good way to spend time.
