The Broken Heart Syndrome

"It was a small puppy" she said, as her eyes welled up with tears. "I was walking on the sidewalk and it came out of nowhere and darted out into the street. An oncoming car could not stop in time and ran over it. It was over in less than a minute. It was then that my heart started to pound and the aching came a bit later". "So how bad is the pain now, on a scale of zero to ten" I cut her off politely, trying to keep my impatience in check.

Such is life as a cardiologist. Less than half hour ago, I was jogging at home on a lazy Saturday afternoon when I got called by the emergency physician. He explained quickly that a family member of a colleague was there with a presumed heart attack and had specifically requested that I come and see her.

Of all the ailments that we treat as cardiologists, nothing is more feared or captures the lay imagination as a heart attack. And there is a good reason for it. Not only is it very common; at 1.2 million a year, it is also a leading cause of death in industrialized societies, thus rapid diagnosis and treatment are a key to survival especially because about a third of these are true emergencies, caused by a sudden, complete closure of the coronary artery, the vessel supplies blood to the heart muscle. Unless this can be opened up quickly, the heart muscle cells literally die from a lack of oxygen, a process that starts within minutes, leading to the adage taught to medical personnel the world over -time is muscle.

Yet not every patient receives the treatment promptly. A problem though is that the diagnosis is often uncertain. Contrary to popular notions, not everyone with a heart attack have classical symptoms, patients described as clutching their chest with chest and left arm pain, cold and clammy. A quarter of the heart attacks may occur without chest pain, and typically women have more 'atypical' presentation with only mild discomfort, nausea, abdominal or jaw pain or only mild shortness of breath. The fact that some entities, like stomach acid and heartburn can cause similar symptoms adds to the diagnostic dilemmas.

Because time is of essence, we try to make a diagnosis based on a quick history, physical exam and an ECG, all of which take a few minutes, and rush the patient to the cardiac cath lab, where an angiogram is done and once a blocked artery is identified,nit is treated with a balloon angioplasty and stents. My patient seemed to meet the classic criteria with a high likelihood of an evolving heart attack. She had chest pain, and her ECG showed ST elevation in the inferior leads. Even her early blood test results were available and showed evidence of a heart muscle damage, presumably from a heart attack.

So I rushed her to the cath lab but her angiogram came as a surprise. All her coronary arteries were open! So what could be the cause of her illness? The answer came when I took pictures of her pumping chamber, the left ventricle. Rather than showing a crisp contraction and relaxation, the far end of the heart seemed to balloon out, and stayed rather sluggish while the near end contracted vigorously. And then it dawned on me; my patient did not have a heart attack at all, she was suffering from Takatsubo cardiomyopathy or the 'broken heart syndrome and suddenly it all made sense.

First described in Japan in 1990, it is named for an octopus hunting jar by the same name, that has a narrow mouth and wide body, just like what the ventricle looked on the angiographic images in my patient. Also labelled as stress induced cardiomyopathy or even the broken heart syndrome in the lay literature, it starts abruptly and unpredictably, with symptoms of chest pain usually triggered by an emotionally or physically stressful event, and with a predilection for women older than 50 years of age (only 10% in men).

Patients with takotsubo cardiomyopathy do not have significantly narrowed coronary arteries, but in the early hours takotsubo and heart attacks share many similarities in presentation, including chest pain and breathlessness, as well as abnormalities in both the electrocardiogram and blood biochemical tests. Even experienced physicians can be challenged to distinguish between the two, at least until an angiogram is performed.

In 85% of cases, takotsubo is triggered by an emotionally or physically stressful event that precedes the onset of symptoms by minutes to hours. Emotional stressors include grief (death of a loved one), fear (armed robbery, public speaking), anger (argument with spouse), relationship conflicts (dissolution of marriage), and financial problems (gambling loss, job loss). Physical stressors include acute asthma, surgery, chemotherapy, and stroke.

Because stress is such an important part of takotsubo, the terms stress cardiomyopathy and broken heart syndrome have been used frequently.

Although the basic cause of this condition is unresolved, the frequent association with stress has focused attention on the autonomic nervous system. It has been suggested that when powerful hormones such as adrenaline are released in excess, the heart muscle can be damaged in patients with takotsubo.

With proper recognition and management, nearly all patients survive an acute takotsubo episode and eventually recover. However, in approximately 5% of patients, a second (or third) stress-induced event may occur. Patients who suffer 1 episode of stress-induced cardiomyopathy should be confident that the chance of another episode is very low, even in the face of future unanticipated stressful circumstances.

About the Author Rupinder Brar

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