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SCAD: What’s Overlooked Can Be Fatal

SCAD

As she glided down the mountain, skiing alongside her husband and children, Brianne Callahan felt discomfort in her chest.

“It felt like an elephant was sitting on me,” she recalls.

Since the birth of her fourth child four months earlier, in September 2017, Callahan had felt more fatigued than usual. The 37-year-old – who frequently participated in high-intensity workouts, including sprint triathlons – attributed the fatigue to her recent lack of exercise and the constant grind of raising four children. Now, standing at the bottom of the slope, Callahan jokingly told her husband that this new feeling was yet another sign of how out-of-shape she was.

In reality, the chest discomfort was a symptom of decreased blood flow to her heart. Callahan was suffering a heart attack.

Three days later – after another full day of skiing, and a 300-mile drive back to her home in New Jersey – Callahan lost vision in one of her eyes. At that point, she sought medical care at a local hospital, where doctors revealed she was having a heart attack from spontaneous coronary artery dissection (SCAD) – a condition that occurs when a tear forms in one of the blood vessels in the heart. The tear separates the inner layer of the artery wall from the outer layer, creating a flap that can slow or block blood flow to the heart, potentially causing heart attack, cardiac arrest or, in some cases, sudden death. The tear can also cause blood to pool and get trapped between the layers, leading to a blood clot.

Lewey
Jennifer Lewey, MD

“I never even considered it was heart-related,” Callahan said. “I was thinking, ‘I’m active and healthy. I have perfect cholesterol.’ I was completely dumbfounded.”

Like Callahan, SCAD patients are generally healthy, and have few conventional cardiovascular risk factors, such as high blood pressure, unhealthy cholesterol levels and smoking.

While the condition is responsible for a small percentage (about 1 to 4 percent) of all heart attacks, SCAD is the cause of about one-third of heart attacks in women younger than 50. And, it’s the leading cause of pregnancy-associated heart attacks.

Yet, until recently, little was understood about SCAD. For years, the condition was described as a rare and almost universally fatal cause of acute coronary syndrome, heart attack and sudden cardiac arrest in women during and shortly after pregnancy. As a result, SCAD was often underdiagnosed or, in some cases, managed as atherosclerosis – an approach that could potentially cause more harm for the patients.

In February 2018, the American Heart Association published a new scientific statement on SCAD in its journal, Circulation. The 35-page statement revealed that SCAD is not only far more common than was previously thought, but that SCAD patients may benefit most from conservative treatment that allows the body to heal on its own. Treating a SCAD patient with a stent – one of the most important initial steps in treating a heart attack caused by atherosclerosis – can make the dissection worse, says Jennifer Lewey, MD, director of the Women’s Cardiovascular Center and co-director of the Pregnancy and Heart Disease Program at Penn Medicine.

“If you try to put a stent in, it can cause the hematoma to spread in the vessel,” Lewey said. “Some patients will need a stent or bypass surgery, but that’s the exception – in most cases, the dissection will heal within weeks to months with medication alone.”

Fortunately for Callahan, one of the physicians at her local hospital had attended a conference two days earlier and joined an educational session on SCAD. When examining the X-rays from her cardiac catheterization, he recognized the dissection and immediately instructed other physicians how to manage the treatment.

Upon discharge, Callahan was prescribed medication to lower her blood pressure and decrease the amount of stress on the blood vessel wall. She continued to have chest discomfort and, eight days later, visited the Hospital at the University of Pennsylvania, where physicians saw imaging from her cardiac catheterization and referred her directly to Lewey.

Lewey altered the medications, and advised Callahan on how to manage her recovery.

For the first three months, Callahan spent most days in bed. She bought a monitor to track her heart rate to ensure it remained within a certain range. She couldn’t hold any of her children, including her five-month-old, or walk up the stairs because of the potential stress it could put on her body.

“It was really difficult,” Callahan said. “I probably would’ve tried to go back to my old high-intensity exercise regimen, but Lewey was very specific. She said, ‘You don’t have a choice.’ She helped me understand the magnitude of what just happened and what I needed to do to reduce the risk of it reoccurring.”

One in five patients will experience a recurrence, so it’s critical for patients to adhere to a heart healthy plan that helps them mitigate their risk.

While Lewey advises against strenuous exercise, she encourages patients to participate in cardiac rehabilitation. Rehab programs often include monitored exercise, nutritional counseling, emotional support and education. Staying physically active is important for a patient’s heart health and mental health, Lewey says.

Connecting patients to behavioral health counseling is another critical step in the recovery process, as depression and anxiety are prevalent among SCAD patients. There is an active online community, including support groups for survivors. The online community, spearheaded by patients, has not only provided other patients with social support, but has helped to increase awareness of SCAD and fuel new research efforts, including the Mayo Clinic’s SCAD Research Program. Lewey encourages her patients to check out and, if interested, join the program, which is currently the largest SCAD registry.

Despite the heightened awareness, questions about SCAD still exist – including what causes the condition to develop. Common risk factors for SCAD are female sex, pregnancy and underlying blood vessel conditions, such as fibromuscular dysplasia. Fibromuscular dysplasia, which occurs more often in women, causes an irregular growth of cells that can weaken the artery walls, leading to blockages, dissections or aneurysms. Half of SCAD patients have fibromuscular dysplasia, but not all patients with fibromuscular dysplasia with develop SCAD.

“We suspect there is a hormonal component that leads to SCAD, but we don’t know what that mechanism is yet,” Lewey said. “The most important advice I can offer is to pay attention to your body and pay attention to any new symptoms. It doesn’t matter how old you are, and it doesn’t matter if you are otherwise healthy. If you think you are having symptoms of a heart attack, seek care.”

Callahan remembers her first appointment at Penn, and recognizes how much progress she has made since then. During her first visit, she used a wheelchair to get from her car to the physician’s office. Now, she often walks a couple miles around her home.

“I’m always chomping at the bit to do more, but I have gradually expanded my activities in the past few months,” said Callahan, who has worked with Chris Kusmiesz, MS, an exercise physiologist in the Penn Sports Cardiology & Fitness Program, to develop a customized plan. “As an athlete, any time you suffer an injury or feel pain, you’re trained to think, ‘suck it up, you will be fine.’ That’s the mindset that got me to ski for two days while I was having a heart attack. But I hope my story helps to raise awareness of this condition and encourage anyone who experiences similar symptoms to seek medical care.”

 

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