Innovators at heart
Meet the Manitoba health-care providers who helped make this province a leader in the detection of fetal heart defects
|Dr. Reeni Soni, Karen Letourneau, Randy Fransoo, Dr. Fern Karlicki and Keith McDonald.
BY SUSIE STRACHAN
Winnipeg Health Region
Wave, November / December 2016
Watching young Ben Sparling as he skates down the ice chasing a hockey puck, you’d never know he has a serious heart defect.
But he does. In fact, the nine-year-old Winnipeg boy was diagnosed four months before he was born.
His mother, Laura McKay, remembers the day in 2007 when she went in for a routine fetal sonogram four-and-a-half months into her pregnancy. The test took longer than expected. She was booked for a second, more in-depth look at her fetus.
Then the news came: the sonogram revealed that her unborn child had a variation of a condition called hypoplastic left heart syndrome. Essentially, it meant Ben’s left ventricle, one of the two main pumping chambers of the heart, could not do its job. “We were devastated,” remembers McKay, about the day she and her husband, Brad Sparling, learned of the diagnosis.
The news, however, wasn’t all bad.
Left untreated, hypoplastic left heart syndrome is uniformly fatal within hours of birth. But thanks to the sonogram – a digital image taken inside the body using an ultrasound machine – McKay’s health-care team was able to prepare for what they knew would be a high-risk birth.
As soon as he was born, Ben was transferred to the neonatal intensive care unit (NICU), where he was later attached to a ventilator. At just five days of age, Ben underwent surgery to address the problem, with a second surgery at five months, and a third at three-and-a-half years of age.
Ben Sparling (left) spends some time on the playground with his brother, Jasper.
“They’ve monitored his heart right from the very start,” says McKay. “Today, Ben is healthy and active. We’re very grateful that it was caught ahead of time,” she says.
How that early diagnosis came about is an interesting tale all on its own.
While stories like Ben’s are relatively common in Manitoba today, that wasn’t always the case. Fifteen years ago, about 30 babies a year were being born with severe heart defects, with only about 25 per cent detected before birth.
Since then, things have changed, and Manitoba has quietly emerged as a leader in using sonograms to detect fetal heart defects. Statistics show that about 45 babies are now born annually with severe heart defects, with about 90 per cent detected before birth.
The dramatic improvement can be attributed to the efforts of a small group of health-care providers and researchers who have spent more than a decade developing and perfecting a new protocol for performing sonograms – a protocol that has helped save the lives of dozens of babies over the years.
The group includes:
- Karen Letourneau, who recently retired as a sonographer at Victoria Hospital;
- Dr. Randy Fransoo, a research scientist at the Manitoba Centre for Health Policy and an assistant professor in community health sciences at the University of Manitoba;
- Dr. Reeni Soni, a pediatric cardiologist at the Variety Children’s Heart Centre at Health Sciences Centre Winnipeg;
- Keith McDonald, Operations Manager of Diagnostic Imaging at St. Boniface Hospital;
- Dr. Fern Karlicki, a sonologist/radiologist at St. Boniface Hospital;
- Dr. David Horne, who is now a pediatric cardiac surgeon in Halifax.
The protocol developed by this team was outlined in a paper written in 2014 by Fransoo, Horne and Letourneau. That paper won the Kenneth R. Gottesfeld Award for excellence in 2015, which is given annually by the Society of Diagnostic Medical Sonography to the authors of the top three papers published in its Journal of Diagnostic Medical Sonography.
Now, the group is on the verge of publishing a second paper that will outline the positive results that have been achieved using the protocol. Once the paper is published, it is expected the protocol will be adopted in other parts of Canada and around the world, a move that will likely lead to many more young lives being saved through early diagnosis and treatment of severe fetal heart defects.
The origins of the effort to develop the new sonogram protocol can be traced back to a conversation that took place in 2000 while Letourneau was attending a conference in Montreal, along with three other sonographers from Winnipeg.
At the time, sonograms were considered to be an indispensable diagnostic tool. In the case of a pregnant woman, a sonogram could show if there were any problems with the development of a fetus’s brain, spine, stomach and digestive system, umbilical cord, limbs, fingers, toes and face.
But Letourneau felt that a sonogram was not as effective when it came to identifying potential problems with the fetal heart, a point she broached with her colleagues while they were at that conference.
“When I asked them how confident they felt about scanning fetal hearts, they all admitted similar concerns,” says Letourneau.
“In medicine, it is not easy to admit that you can’t do an aspect of your job perfectly, every time,” says Letourneau. “The fact that more experienced sonographers felt the same way gave me the motivation to do something about it, so that we could all provide the best possible service.”
When she returned to Winnipeg, Letourneau decided to start researching the subject.
She called on Fransoo, who is her brother, to help her design a study that could evaluate the need for continuing education in fetal heart screening, and a method to measure the impact additional training could have on the prenatal detection rate in Manitoba.
Together, they developed and sent a survey to all sonographers in the province to assess their confidence level, and find out how they were conducting the scans.
The survey results confirmed Letourneau’s suspicion: routine ultrasounds done of fetuses around the 20-week mark were only catching fetal heart defects 25 per cent of the time.
“That convinced me that we were on to something,” she says, adding that 100 per cent of the surveys were returned, showing the dedication of the province’s sonographers.
Letourneau also contacted two instructors from her student days – McDonald and Karlicki – to see if they knew of a better method for detecting fetal heart defects.
McDonald and Karlicki also believed sonograms were not as effective as they could be. And, as it turned out, McDonald also had some ideas about how the ultrasound machine could be used to generate more comprehensive images of the fetal heart.
At the time, guidelines set out by the American Institute of Ultrasound in Medicine (AIUM) recommended that sonographers produce images depicting a four-chamber view of the fetal heart, showing the right and left atriums and right and left ventricles.
The problem was that the four-chamber view did not include two outflow vessels from the heart – the aorta and pulmonary artery. In other words, the sonogram scans weren’t catching certain heart defects that could only be diagnosed by evaluating these outflow vessels.
But the AIUM guidelines also noted there was a way to address that problem. By changing the angle of the scan, sonographers could pick up the two outflow vessels, allowing them to diagnose heart defects.
“Keith proposed that we were looking at the wrong target, that we were looking at the heart from the wrong angle,” says Karlicki.
As she explains, changing the angle meant sonographers could generate a view of a fetus’s chest, with the elongated ribs to each side, and a pumping heart on the left side of the chest – a heart, which is about the size of a strawberry at 20 weeks of age.
But sonograms done this way can also show too much information about the fetal heart.
“We have the potential to look at 25 features of the heart, with maybe 30 views and 100 actions,” says McDonald, explaining that there are hundreds of cardiac problems that could be captured by the sonogram. “This was too complicated. We wanted a test that could be done by every sonographer on any piece of equipment, anywhere in the province.”
At this point, the group contacted Dr. Reeni Soni, who was the only pediatric cardiologist in the province at that time. The question they posed to Soni was: how can we make a simple enough test that will still catch the critical heart abnormalities?
Soni, who now heads a team of four pediatric cardiologists at Variety Children’s Heart Centre at HSC Winnipeg, understood the need to simplify the detection of defects. She outlined the case for only looking for what she calls the “top three” defects: hypoplastic left heart syndrome (which is what Ben has); transposition of the great arteries; and severe cardiomyopathies (a group of diseases that affect the heart muscle).
“When I started here, the mortality rate was 75 per cent for hypoplastic left heart syndrome. Thanks to earlier detection, and better surgical techniques, that rate has dropped to five per cent,” says Soni. “We knew that early detection would put the health-care team ahead of the eight ball when it came to the right treatment, be it medication, surgery or a heart transplant.”
The group agreed it was better to ask sonographers to scan for a limited number of specific problems and refer any fetus with a heart anomaly to the Fetal Assessment Unit at either Women’s Hospital or St. Boniface Hospital for a second stage of testing. The final diagnosis would then be made through a fetal echocardiogram performed by Soni.
“It’s like they say on Sesame Street. We wanted the sonographers to be able to think, ‘One of these things is not like the others,’” says McDonald.
In 2004, the group hosted a weekend seminar attended by all but two of the sonographers in the province, along with a number of radiologists and maternal fetal medicine specialists. The seminar began with a panel of families telling their stories of how a prenatal diagnosis made the very stressful situation of having a child with a heart defect easier to bear.
Then, Letourneau and her group made their case, showing how proper positioning of the fetus during the scan would result in a more accurate assessment of the fetal heart. This emphasis on technique and strict adherence to the protocol was not previously published in the medical literature.
Under the proposed protocol, the first thing everyone had to learn to do in a prenatal scan was to get the fetus into the right position. The scan has to show a rounded chest with ribs on either side. This often means getting the pregnant mother to go for a walk in the hopes of having the fetus roll over.
“Sonographers were shown how to look at the four chambers – the left and right ventricle and the left and right atrium – and how the big vessels like the aorta, superior vena cava and the pulmonary artery join into it,” says Karlicki.
Then, they were shown how to look for anomalies, such as whether the chambers are pumping in parallel or if they’re crisscrossed, or if a chamber isn’t present,” she says.
After that weekend, practicing sonographers went back to their clinics and began using the new protocol right away, says McDonald, adding that sonography students were also being taught the protocol.
Right from the start, the new method began catching previously undetected fetal heart defects. “At first, we were worried about sending more pregnant moms to Dr. Soni, as she was the only one who could do the fetal echocardiograms,” says Letourneau. “But as she explained to us, early detection was making a world of difference.”
It takes about one hour for Soni to do an echocardiogram on a fetus, compared to eight hours of intense work in NICU after a child is born with a previously undetected heart problem. That made seeing more pregnant mothers the best solution.
Catching unborn babies’ heart defects meant that neonatal teams could prepare for treating a newborn. It also meant parents could prepare for what would happen after birth.
“The more lead time, the better,” says Soni. “This allows us to provide the counselling and to walk the parents through the various options. We also have peer-to-peer support, where families can support each other, in our Circle of Hearts program.”
Soni is the only Manitoba pediatric cardiologist who does fetal heart echocardiograms. She sees around 120 pregnant women a year. Once a defect has been diagnosed, preparations are then made to deliver the babies at HSC Winnipeg, and stabilize them in the NICU. Then, if need be, they are sent to Edmonton or Vancouver for surgery. The most difficult cases are sent to Edmonton before birth, so they can be taken into surgery soon after the baby is delivered.
That expertise in early detection, followed up by care from Soni and her team, has made all the difference to children like Ben Sparling.
Today, the young boy is up-front about his heart. He has talked to his class about it, and he understands a lot more about self-regulation than the average nine-year-old, says his mother.
“He came home and told us he wanted to be in the marathon running club. It took a little while, but because he understands how to self-limit, we had to trust him. We talked to Dr. Soni, and she gave it the go-ahead. The same goes for him playing hockey,” says McKay, adding that Ben will stop and take breaks when he feels he needs to.
“You’d never know to look at him that he has a history of heart problems,” says McKay.
Susie Strachan is a communications specialist with the Winnipeg Regional Health Authority.
Wave is published six times a year by the Winnipeg Health Region in cooperation with the Winnipeg Free Press. It is available at newsstands, hospitals and clinics throughout Winnipeg, as well as McNally Robinson Books.
Read the November / December 2016 issue of Wave