Jonathan's Heart

 Jonathan in Mater Hospital, Auckland New Zealand, 2 days after open heart surgery, July 1970

Earlier in these memoirs I described how I discovered that there was something seriously wrong with Jonathan’s heart, when he was a few weeks old.  It was an indictment of our family doctor’s competence that he failed to detect this. I discovered it late one Sunday evening when Jonathan couldn’t sleep because of his cough and breathlessness. I broke the rule about treating my own family when I listened to his chest myself. He had a very loud heart ‘murmur’ a rasping, rough sound so loud I could hear it without a stethoscope if I put my ear a few inches away from his chest.

He was slow to sit, crawl and walk, spent much of his time sleeping, had a poor appetite, was always short of breath and had a perpetual juicy cough. He was never able to walk more than a short distance and when we went anywhere, Wendy or I had to carry him. By the time he was 4 years old, it was usually I who carried him piggy-back, his matchstick legs hooked under my elbows. When he started kindergarten, Wendy took over the role of carrying him to and fro. He was taking a maintenance dose of digitalis (medication to treat heart failure) and oral penicillin to prevent lethal infection of his heart valves. Clinically, his symptoms and signs suggested that he had a large hole between the chambers of his left and right ventricles.  Blood rushing to and fro through this hole produced the very loud heart murmur. But there were some puzzling features that hinted at a more complex problem than just a ‘hole’ between left and right sides of his heart.  We knew from his infancy onward that without surgical correction of this, his chances of surviving to adult life were very poor.  Open heart surgery in those days was just developing, and a third or more of all small children did not survive their operation.

In 1967 in Edinburgh, Jonathan had a thorough investigation, including cardiac catheter studies with contrast media which enabled blood flow inside his heart to be seen on a computer screen. I watched all this because the cardiologist wanted a parent to hold his hand, rather than from a sense of clinical curiosity, although of course I was intensely curious. It was fascinating to observe this new technology.  To everyone’s surprise, Jonathan was shown to have the Tetralogy of Fallot, transposition of the great blood vessels entering and leaving the heart, rather than the large ventricular septal defect that we had previously believed was the cause of his severe disability. Infants with Fallot’s Tetralogy have a complex cluster of malformations that arise during development of the heart in early embryonic life. They are ‘blue babies’ because oxygen-poor blood is shunted through the peripheral circulation instead of being replenished with oxygen in the lungs. Jonathan had the uncommon variation of the condition in which oxygenated blood was shunted back through the lungs, so his body and limbs simply were not getting enough blood whereas his lungs were permanently engorged by too much blood.  Rather than being a 'blue baby' Jonathan was a 'white baby' because his malformed heart wasn't able to pump enough blood through his body - too much of his blood was being diverted back into his lungs, engorging these and making him breathless. Without surgery he would have had a maximum life expectancy of about 10-12 years. 

After the diagnosis was confirmed I began exploring where we could get the surgical repair that could restore his health. I discussed this at length with his cardiologist, Bobbie Marquis and studied the outcome of series of cases operated on in various places. One place to avoid was Scotland where the postoperative complication and fatality rates were very high. Two surgeons in the world at that time had the best record. Both trained at the Mayo Clinic and had gone back to their home cities, James Kirklin to Birmingham, Alabama, and Brian Barrett-Boyes to Auckland, New Zealand. I met Brian Barrett-Boyes at an international medical congress in Sydney in 1968, and discussed Jonathan’s condition with him in person then, and later, in several exchanges of letters after he had received the full details from Bobbie Marquis. But it seemed at that time an impossible dream to take Jonathan to New Zealand for the necessary operative repair. One motive for us to leave Edinburgh was to relocate to a place where it would be financially and logistically feasible to get the world-leading advanced pediatric cardiovascular surgery that might enable Jonathan to live a normal life span.

The dream of timely surgical intervention came true shortly after we arrived in Ottawa. I was invited to New Zealand to present papers at an international conference, and my air fare was paid with enough over to cover Wendy’s fare as well as mine. We scraped together the funds for Jonathan’s child-size fare. To cap it all, the generous provisions of the Ontario Health Insurance Plan, OHIP, covered virtually all of the costs of having the operation done privately on the other side of the world: it was cheaper than it would have been to have the operation done in Canada because hospital costs in Canada were much higher than in Auckland, New Zealand.

In the early 1950s in England I had seen the dramatic and instantaneous benefit from surgical treatment of rheumatic valvular heart disease, but even so I was amazed at how rapidly Jonathan recovered and was able to do things that previously were unimaginable. A few days after the operation he was running up hills on the steep slopes of John Wendelken’s home in Wellington; and soon after he came back to Ottawa, I looked out the window one day to see him running after one of his little friends who was riding a bike, and catching up with him. It was a miracle.

Everything went spectacularly well for Jonathan until he was about 14, when he began having alarming attacks of transient unconsciousness that turned out to be caused by bursts of rapid irregular heart-beat. These occur because he doesn’t have the neuromuscular bundle that controls heart rhythm. He has a Teflon graft where that neuromuscular bundle in the septum between right and left sides ought to be. That and other problems impair his health somewhat, so he gets an invalid pension. However he is not so severely incapacitated as to need constant help: he lives independently and augments his pension with a small income from computer graphics and computer trouble-shooting. Jonathan is a beneficiary of spectacular technical advances in pediatric cardiovascular surgery that are among the many breathtaking achievements of the second half of the 20^th century. Without these advances, first the discovery of penicillin which prevented fatal infection of his defective heart valves, then the development of surgical techniques, intensive care, cryosurgery – cooling the body to prevent brain damage when blood circulation to the brain is interrupted during the operation – and skilled postoperative nursing care, Jonathan’s maximum life expectancy would have been no greater than 12 years, and for almost all of that time he would have been confined to bed or a wheel chair. Jonathan is alive and reasonably well at almost 50 years of age because of the advances in preventive medicine, diagnostic imaging, surgical techniques, intensive care, medical and hospital insurance, and globalization of air travel that combined to make it all possible.