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At a glance

It is a rare disorder characterized by non-ischemic cardiomyopathy induced by sudden emotional stress such as the death of a loved one, break-up and/or rejection from a partner, or sudden anguish. The clinical feature is an acute dysfunctional portion of the heart leading to, most often, left ventricular failure. It is also associated with lethal ventricular arrhythmias, ventricular muscle rupture, and sudden death. This medical condition is often called the “Broken Heart Syndrome.”

Synonyms

Broken Heart Syndrome; Stress-Induced Cardiomyopathy Syndrome; Stress Cardiomyopathy Syndrome; Takotsubo Cardiomyopathy Syndrome; Apical Ballooning Syndrome; Acute Stress-Induced Cardiomyopathy Syndrome; Acute Stunned Left Ventricle Syndrome; Transient Left Ventricular Apical Ballooning Syndrome.

History

It refers to a Japanese word defining a kind of octopus trap named “Takotsubo” since the left ventricle takes on a shape resembling a fishing pot. The first case was reported in Japan in 1991 by Sato et al. However, Cebelin and Hirsch had written about human stress cardiomyopathy in 1980 after reviewing cases of homicidal assaults that happened in Cuyahoga County, Ohio, over a period of 30 years. When they studied individual who showed no injury at autopsy but had died of physical assaults, they noticed in 11 of 15 cases severe left ventricular myofibrillar degeneration similar to what was observed in animal stress studies. They suggested a theory of catecholamine-surge induced to explain these myocardial changes and determined that acute stress was responsible for the death of these individuals. The syndrome was finally described in 1997 when Pavin et al wrote about two cases of “reversible left ventricular dysfunction precipitated by acute emotional stress.” Few years later, a report from the Japanese literature confirmed Pavin’s observation in 2001 by mentioning a “transient LV apical ballooning.” The syndrome reached international audiences through the media in 2005 when the New England Journal of Medicine wrote about the syndrome.

Incidence

The true incidence remains unknown. It is rare, affecting between 1.2 and 2.2% of people in Japan and 2 to 3% in western countries that suffer a myocardial infarction and acute left heart failure (sudden death). It is reported more frequent in women than men. Of all cases reported in the literature, 90% were women, especially postmenopausal. It has been suggested scientifically that estrogen increase the amount of catecholamine and glucocorticoid in response to mental stress. It is not likely that a patient who recovered once from this sudden attack will experience it a second time, although it has been reported. The average ages at onset are between 58 and 75 years. Less than 3% of cases occurred in patients under age 50 years. Besides Japan, several cases have been reported more recently in the United States and Belgium.

Genetic inheritance

This is an enigmatic disease with multifactorial and still unresolved pathogenesis. There is no clear ...

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