“I Am Astounded That I Survived”

In the months leading up to the birth of her first child, Sudi Barre, a 36-year-old former social worker who lives in Edmonton, was told by her doctors that she was a “textbook ideal pregnancy”—healthy and fit, she was still working close to her due date and even hiking up until a week before. “I was feeling uncomfortable with how comfortable I was,” she recalls now with a small, knowing smile.

But on a warm morning in August 2017, while recovering in hospital from an emergency Caesarean, Barre began to experience excruciating pain down her neck, between her shoulders and down her arm  to her elbow. She couldn’t breathe, and she was worried she wouldn’t survive to hold her child. At first, she was told by a nurse to “walk it off.” But eventually, the attending physician ordered an electrocardiogram (ECG), which revealed Barre had experienced a heart attack. Doctors placed a stent in her heart, but things only grew worse as she then experienced what she estimates was at least another seven heart attacks over several weeks. The medication—along with the pain and fear—was copious.

After later being transferred to the Mazankowski Alberta Heart Institute, Barre was diagnosed with spontaneous coronary artery dissection (SCAD), the result of a tear in an artery wall, which leads to blood building up between the layers of the wall and blocked or reduced blood flow to the heart. That, of course, can lead to a heart attack or cardiac arrest. Dr. Sharonne Hayes, a cardiologist, professor of cardiovascular medicine and lead researcher for the Mayo Clinic SCAD Research Program, puts it simply: “I have sometimes described the dissection as like a blister within the layers of the artery. It can hurt as the layers of the blister split and the pressure builds up like a bruise. If it fills up and bulges enough, it can block the flow of blood going past it or, if the pressure builds up, it can burst open, leaving a free layer of artery that can also flap around to obstruct blood flow.” To mitigate the issue for Barre, whose heart function had dropped to just three percent, a mechanical pump called a left ventricular assist device (LVAD) was implanted to help her heart pump blood.

According to the Heart and Stroke Foundation, 90 percent of all SCAD cases are women between the ages of 30 and 60. Most are young and healthy. And although the cause of SCAD is still unknown and there are no warning signs, people who are most at risk include pregnant and postpartum women, those with fibromuscular dysplasia (FMD) or connective tissue disorders and those who experience considerable mental and physical stress, all of which can lead to weakened artery walls. Surgery can be high-risk, and treatments are conservative. These include taking beta blockers to reduce blood pressure or Aspirin to lower the risk of a clot forming in your torn artery. If the tear is severe, surgery can mean an angioplasty, an implanted defibrillator or a coronary artery bypass grafting. The current mortality rate is estimated to be about 70 percent, while the recurrence rate is two to 20 percent per year.

The diagnosis was difficult for Barre to grasp. It left her in the hospital for a total of eight months—the first eight months of her child’s life.

“I felt detached. I was still recovering from the Caesarean,” she says today. “It all happened so fast. How do you process that and then decide to move forward? I hadn’t experienced such a massive health challenge before, so I was in shock for most of it. By the time I got the diagnosis, I was in such disarray, mentally and physically. I went to the hospital to deliver, and here I am, weeks and weeks later, with the doctors not knowing how to manage my pain.”

Of the many numbers associated with SCAD, the one that never leaves Barre’s mind is 17. That’s her ranking in the list of Canadian patients to officially be diagnosed with SCAD, which shows just how little awareness had accrued by the time she found herself giving birth and experiencing a pain she never had before. Although uncommon, SCAD is the leading cause of heart attack among women who are pregnant or have recently given birth and women under the age of 40, and is estimated to be the cause of one to four percent of heart attacks overall. In fact, SCAD has long been under- and misdiagnosed, as it can be difficult to detect on angiograms, and blood thinners and stents can actually do more harm than good, creating more tearing in the heart’s artery walls. And because many of those who experience it are younger women—who do not often experience heart disease—their symptoms are often misread and their concerns ignored.

“SCAD is a perfect example of where we need to do so much more with respect to research,” says Dr. Paula Harvey, director of cardiovascular research and physician-in-chief of medicine at Women’s College Hospital in Toronto. “Then we can increase awareness and learn how it differs across different populations and how it’s best diagnosed and treated. Because we’ve started doing research in the last decade or so, we’re recognizing it’s probably more prevalent than we previously understood. But we still have a lot of gaps in our understanding because not only is it a different way of presenting with a heart attack, but it tends to affect younger women. This can build a lot of barriers to women being diagnosed accurately because of the tendency and unconscious bias that young women don’t have heart attacks.”

That lack of research was particularly challenging for Barre, who says the more she tried to find out about SCAD, the more questions she had. This was also the case for Risa Mallory, a 64-year-old retired psychotherapist who was diagnosed with SCAD in November 2018. For her, it wasn’t entirely a surprise, as her mother had died prematurely from atherosclerotic heart disease at the age of 59 after her second coronary artery bypass graft. Mallory thought her initial chest pains may have been due to angina, but they kept returning “with a vengeance,” she says. She eventually went to the ER, where, due to her family history, she was prepped for an urgent angiogram. She woke up intubated in the cardiac care unit 16 hours after checking in, “not knowing what had happened but being petrified.”

During her angiogram, Mallory had gone into cardiac arrest and cardiogenic shock, and was put into a medically induced coma to slow her bodily functions, with a heart pump to assist cardiac blood flow. Three stents were tenuously placed in each of the main arteries on the left side of her heart to hopefully finally restore blood flow.

“Every time I tell this story, I am astounded that I survived and did so without any major residual symptoms, organ damage or dysfunction,” recalls Mallory, adding that the other most shocking aspect came while she was still in Arizona. “My husband was doing some research to better understand what happened to me and stumbled upon a diagnosis of SCAD. When he asked the cardiologist with over 30 years’ experience who had performed the angiogram and truly saved my life about SCAD as a potential diagnosis, he said, ‘What is that?’ He had never heard the term,” she said. “I was medically cleared to [head] home six weeks later, still not knowing what my diagnosis was. Was it SCAD? Was it iatrogenic dissection caused by the catheter or dye or both? Was it microvascular disease? Not having a definitive diagnosis was very difficult for me, as I felt adrift without a compass or anchor to guide me in this uncharted territory.”

Although the research is relatively thin, in recent years, a few key figures, including Dr. Jacqueline Saw, a cardiologist at the University of British Columbia, have been making inroads. She and a team of international researchers, with support from the Heart & Stroke Foundation and a database of 3,000 Canadian patients, recently discovered a series of common genetic factors among SCAD patients. The key one is a gene called ADAMTSL4, which regulates a protein found in the wall of the heart artery, which is where SCAD typically occurs, and could help identify why the artery weakens and tears. Saw’s team has also found similar genetic risk factors between SCAD, FMD and migraine headaches, and discovered that more than 56 percent of patients studied experienced an emotionally or physically stressful event (for example,  pregnancy) sometime ahead of their SCAD.

Saw also developed a classification to analyze coronary angiograms—which help locate the tear and its measurements—to help doctors detect SCAD. Meanwhile, Hayes has created an international virtual disease registry of SCAD patients (now the largest in the world) and a DNA biobank to help identify the causes, optimal treatment and risk recurrence for SCAD.

“When I went to cardiology school, I was taught that SCAD mainly happened in women after birth, and it was like a paragraph in a textbook,” recalls Hayes. “The other thing I was taught is when I’m looking at the angiogram of somebody who has this, I’m looking for a flap and a separation between the layers of the artery.”

Since then, she and her team have discovered that most SCAD patients actually do not have a flap but a bleed or a split between the artery that is contained, which is called an intramural hematoma. That confusion means that up to 80 percent of SCADs have likely been missed—if they even got to the angiogram stage.

Hayes’s team has also shown that the majority of patients who have SCAD actually have a systemic disorder of their arteries, including FMD, which itself is quite common. That means SCAD is “maybe not completely out of the blue,” Hayes says, but something someone who already has fragile arteries is more predisposed to.

The importance of cardiac rehabilitation to improve physical and mental recovery after SCAD has also recently been spotlighted, with women not only less likely to be referred to it (due to their younger age) but less likely to complete it. Rehab typically entails exercise and education tailored to what a patient may be living with—as SCAD can alter one’s entire lifestyle, from diet to physical activity—and offers the space and resources to discover the body’s new limits.

“It can be something that can be overcome, or it can be something that can give you PTSD,” says Coutinho. “So we make sure we take care of our patients’ mental health just as carefully as we take care of their heart. In cardiac rehab, they’re going to learn what they can do, how they can do it safely, how to monitor their bodies. But what has been so great for our patients is the peer support program. Because what I tell them is, even if I was the best doctor in the world and I knew everything there was to know, there’s still a part of their recovery I cannot help with simply based on the fact that I have not had SCAD myself. I do not know what it is to walk in their shoes. This program fills those gaps, because they meet other patients like them.… They need to see others who have survived and thrived.”

Mallory, who requested to be transferred to Coutinho’s clinic soon after being diagnosed with SCAD, says, “This validation was an immense burden lifted from me. I ‘belonged’ to a group. I could research the medical papers. I could explore various points of view on the emerging literature about etiology, treatment, lifestyle management and prognosis. I could share my story, and learn from other SCAD survivors who understood the journey. I had a clear route to a more fulsome healing.”

In fact, four weeks post-discharge, Mallory joined a SCAD peer group where she listened, learned, commiserated and participated—”I moved so much further in my mental and emotional healing journey,” she says. She is now a group facilitator and a SCAD advocate. “Even without committing to anything structured, each of us can contribute and raise awareness by having conversations with friends, family and even our own medical teams about our SCAD journeys and what makes women’s heart health and disease unique.”

However, SCAD research isn’t facing only a gender gap but a race gap, with an egregious majority of research registries and studies based on white patients. “That’s because of the gap in our research,” admits Hayes, who says she has seen SCAD patients of every race over the years. “I think if you looked at the registries, you may think this is a white lady disease, but that is not my experience. If you are a racial or ethnic minority or from a disadvantaged area, you are likely to receive less care, less prompt care and less accurate diagnosis.” That’s an especially tough pill to swallow as, Barre notes, “women of colour, by nature and culture, are more likely to put our needs aside as a caregiver for everybody. You become an afterthought.”

A key part of that journey, then, is advocating—for others and for oneself. As Coutinho says, “For many years, women have been excluded from research, and we still have a lot to catch up on, because this is also a relatively rare disease. But every year that goes by, we get smarter, and we certainly know a lot more than 10 years ago.”

Hayes is especially optimistic. “I founded our women’s heart clinic in 1998. And back then, women were just being ignored. They had classic symptoms and wouldn’t even get an ECG. That’s improved hugely. The challenge still is this younger group of patients, some of whom go in literally saying, ‘I have an elephant on my chest and radiation in my jaw, which goes down my left arm, and I threw up.’ You cannot make up more classic heart attack symptoms, and they are sent home. Some of these women have been traumatized by not being taken seriously,” she says “As important as understanding this condition is validating the experience of these women, whether they were ignored, misdiagnosed or went into a cardiac rehab that was tailored more to a post-bypass 80-year-old than a 35-year-old. It’s like they’re the square peg in a round hole. And then they have their doctors scratching their heads and saying, ‘Aren’t you interesting— you’re the first one I’ve seen!’ Nobody wants to be an ‘interesting’ case. I would consider myself among the SCAD experts in the world, and I still don’t have all the answers. But if you’re treated like an oddity or a fragile egg that could break any minute, because you have symptoms that a lot of cardiologists aren’t used to dealing with, it’s very frustrating and scary.”‘

Of course no one knows your body better than you, and that’s something to hold tight when speaking to a doctor and looking for a diagnosis. Coutinho, Hayes, Harvey, Barre and Mallory all recommend the following tips when explaining what you’re feeling to a doctor: Don’t be confrontational or challenging (“because nurses and doctors do have egos!” says Hayes); don’t be afraid to have someone there with you to validate your symptoms; use the words “chest pain” or “chest pressure,” which physicians have been trained to flag; use words like “heart attack” or “stroke” if you think you might have had either; humanize yourself by sharing your and your family’s history; do your own research and ultimately choose to be “curious, not furious,” in the words of Harvey.

If you are ignored or no tests are done, say, “I’m not leaving,” says Hayes. She adds, “There is this bias that women seek care for no reason. There is data to support the fact that women are less likely to be believed. And there’s multiple studies that show that women’s diagnoses and treatments are delayed for multiple conditions and compared to those for men.”

After their own experiences, Barre and Mallory have become health advocates. Today, between speaking at heart health conferences and being an entrepreneur and a mom, Barre’s day-to-day has considerably improved, despite not being eligible for a heart transplant due to her blood type and antibodies. In 2018, after she had a minor stroke, her doctors discovered her heart was operating at 40 percent. A month and a half later, she had her LVAD removed and was physically healthy enough to play with her son and carry him without worrying. Now, Barre advocates for women’s economic independence which, she says, “is the best way for women to gain their voice back.” Many SCAD patients are women who cannot afford to take a leave of absence while in recovery. Barre herself had to quit her job as a social worker, and she’s currently developing her own business. She refers to this as “sovereignty for the self”—a tool for empowerment.

For her part, Mallory still experiences mild symptoms of fatigue, shortness of breath and occasional chest pain. But she also golfs four days a week, takes hour-long walks every other day and continues to travel the world with her family. Much like Barre, she says, “I refuse to let my health and this diagnosis dictate the experiences I have and the memories I make with whatever time I have left.” In fact, it’s worth noting that while SCAD patients have a disproportionately higher rate of symptoms after the fact, most people who survive SCAD have a good prognosis, with so many more tools and resources available than a few years ago, when Barre and Mallory were diagnosed.

Meanwhile, lead researchers are continuing to hustle and find ways to prevent SCAD before it can happen. For now, though, Hayes says it’s important to remember that life goes on after SCAD—and pretty damn well, too.

“Gaining the competence to go do the things you need to do and live your life the way you want to is everything, because there is unfortunately a risk of recurrent SCAD. It’s small, but it’s definite,” she says. “And so what I tell patients is you have to live your life like it’s never going to happen again, to exercise for your brain and your bones and your heart. You need to get back to caring for your family, your hobbies and travelling, but you also need to be prepared if it does happen again. Just remember: You will feel confident about your future again. It does get better.”