Healing a Broken Heart

Elaine Kamil, MD, is a busy and dedicated physician, often working 12-hour days caring for children with kidney disorders. At 71, she also finds the time to hike, travel and play with her four grandchildren.

But through her flurried days, Kamil carries with her a disease that has affected her life for years, even before she knew she had it: Takotsubo syndrome, commonly known as Broken Heart syndrome. Takotsubo is characterized by transient weakness of the heart muscle, often a “ballooning” appearance of the heart. While Takotsubo predominantly affects post-menopausal women (80–90 percent), there is evidence that among both women and men, the syndrome is preceded by an experience of severe mental or physical stress. The condition may elude diagnosis because its symptoms can be confused with heart attack and physicians may not be familiar with Takotsubo diagnosis criteria.

In 2013, after a busy weekend work trip, Kamil experienced intense chest pains and shortness of breath. She brushed off the symptoms even as they worsened; she’d seen a cardiologist a month before after similar pains, and a stress echocardiogram showed her heart to be healthy.

But this time, her husband encouraged her to seek immediate treatment. In the Emergency Department at Cedars-Sinai, her electrocardiogram and blood biomarkers showed typical signs of a heart attack. However, an angiogram showed her coronary arteries had no blockages and her echocardiogram showed severe weakness of her heart muscle.

Kamil remembered reading about Takotsubo syndrome in a medical journal but didn’t consider herself at risk. She hadn’t thought she was under too much stress before the incident, despite a stressful career of treating very sick children.

"The work can be emotionally charged," she says. "I’m kind of a mother hen with my patients."

Looking back, she realizes she probably had her first episode of Takotsubo syndrome in 2009, when her son, Adam, died unexpectedly at age 31. In the days after his death, she experienced chest pains, but in her mourning, quickly dismissed them. She suspects persistent grief may have played a role in the second, larger episode years later.

"Losing a child is an accumulation of stress," she says. "It doesn’t go away."

After her diagnosis, Kamil recuperated at home for about a month. Her cardiologist then encouraged her to return to work. She still felt “drained” and not nearly back to normal, but trusted her doctor’s insistence that she was ready to take on a full schedule. Upon starting cardiac rehab she met with Chrisandra Shufelt, MD, MS, associate director of the Preventive and Rehabilitative Cardiac Center at the Smidt Heart Institute to review her exercise prescription. Shufelt let her know a complete recovery could take three months or longer. Kamil says she wishes that her lingering fatigue could have been recognized as an indication that her body was still recovering from Takotsubo as well as the other ways the syndrome may have impacted her. If these factors had been identified as syndrome-related, she might have been given additional time to recover or the ability to come back to work on a part-time basis. In hindsight, Kamil believes that easing back into work more slowly might have assured a more full and restful recovery.

Although Takotsubo syndrome is considered “reversible” or temporary, this common perception can be problematic. Even when tests show the heart has returned to normal, the recurrence rate appears to be around 5–10 percent within five years. After learning about her risk for another episode, Kamil took a step back from her practice and cut back her schedule to limit the number of long workdays.

Even after a decade of reports in medical literature, there are no Takotsubo-specific treatment guidelines. Instead, current treatment is based on the American Heart Association/American College of Cardiology guidelines for treating heart attacks and heart failure.

“We also don’t know how to prevent recurrence or how to identify the people at risk for recurrence,” says Noel Bairey Merz, MD, director of the Barbra Streisand Women’s Heart Center and director of the Erika J. Glazer Women’s Heart Research Initiative.

Because so much about Takotsubo syndrome is still incompletely known or misinterpreted by patients and physicians alike, the Barbra Streisand Women’s Heart Center launched an initial clinical study in December 2016. The Brain-Heart Connection in Takotsubo Study evaluates the possible linkages between the sympathetic nervous system and Takotsubo Syndrome. “This research will help identify whether the hearts or brains of individuals with a history of Takotsubo Syndrome demonstrate dysregulation of the autonomic nervous system in response to emotional or physical stress,” says Janet Wei, MD, cardiologist in the Barbra Streisand Women’s Heart Center and assistant medical director of the Biomedical Imaging Research Institute.

Kamil is now part of the study, which she hopes will help shed light on what triggers the disorder and how to prevent it from recurring.

The Barbra Streisand Women’s Heart Center is developing new and expansive Takotsubo syndrome research, which builds on the Center’s long history of diagnosing, treating and studying the cardiovascular conditions that impact women’s health. To better serve patients like Kamil, the Center is launching a new U.S.-based Takotsubo Registry and Proteomics Study.  This study will build a large database of diagnosis and treatment information, patient questionnaires, along with blood samples provided by individuals who have experienced Takotsubo syndrome. The goals of the Registry are to better understand why some people experience Takotsubo syndrome and others don’t, and to identify risk factors for recurrences. Results from the Registry will help guide researchers to design clinical trials to improve the cardiovascular health of patients with or at risk for Takotsubo syndrome.

Immediately following her diagnosis, Kamil lived in fear of another incident.

“In the beginning, it was hard—every little tinge in my chest scared me,” she says. “I lost confidence in my day-to-day health.”

Five years later, she’s adjusting to the condition, and her anxiety has mostly faded into the background. She used to enjoy skiing on trips with her family, but now the altitude bothers her, so she sits it out. She continues to travel to her favorite destination, Africa (she’s been seven times; last trip to observe gorillas in the wild). But now, she brings along prescription nitroglycerin patches, which help increase blood flow to the heart and could temporarily help should the condition return while she’s away.

And in her daily life, she’s found greater balance, making time for walks through the park, where she often pauses to record bird songs.

“I think the major lesson this illness has taught me is that you should listen to your body and not push so much,” she says. “So I am becoming a better listener of my own body.”