Takotsubo (Stress) Cardiomyopathy
Whenever a new and previously unrecognized medical condition enters our consciousness, considerable confusion and uncertainly can ensue. If a patient is afflicted by such a disease state, the natural reaction is, “I have never heard of what I have,” triggering considerable personal and family anxiety. Such is the case with the condition we now call takotsubo cardiomyopathy, which has received considerable attention from the media and has been assigned a myriad of names in the literature.
Historical Background and Increasing Visibility
Initial recognition of takotsubo cardiomyopathy occurred in Japan in 1990, with the first report emerging from the United States in 1998. Thereafter, scientific interest in this condition has increased steadily and dramatically. For example, in 2000, only 2 publications were recorded, compared with nearly 300 in 2010 (Figure 1). Now, takotsubo is widely recognized, with reports from 6 continents and diverse countries, including France, Belgium, Mexico, Australia, Spain, South Korea, China, Brazil, Germany, Israel, South Africa, Turkey, and Iceland.
What Is Takotsubo?
The Acute Illness
Takotsubo cardiomyopathy starts abruptly and unpredictably, with symptoms of chest pain and, often, shortness of breath, 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). Most patients go to the emergency department because of concern they are experiencing a heart attack, a much more common acute heart condition caused by a blocked coronary artery (the Table). Although patients with takotsubo do not have significantly narrowed coronary arteries, 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 x-ray study of the coronary arteries and left ventricle with dye establishes the absence of severe plaque buildup in a coronary artery as well as the unusual shape of the left ventricle (ie, pumping chamber) that has given takotsubo its unique name (Figure 2).
This distinctive shape includes a narrow upper portion (neck) in contrast with the lower enlarged portion that contracts poorly and closely resembles the ceramic Japanese takotsubo pot used to collect an octopus. Because a large proportion of the heart muscle is injured, heart failure can occur (ie, low blood pressure and fluid buildup in the lungs).
Hospital Stay and Recuperation
An echocardiogram (ultrasound) and sometimes a magnetic resonance imaging study are performed to evaluate the heart's pumping ability. Patients are in the intensive care unit for at least 24 hours, during which time vital signs are monitored and blood is tested for troponin (a protein released by injured heart muscle). Commonly used medications include β-blockers and angiotensin-converting enzyme inhibitor drugs, both of which promote recovery of heart muscle. Drugs that interfere with blood clotting (anticoagulants) may be administered briefly to avoid a stroke caused by a blood clot traveling from the heart to the brain.
Major life-threatening complications are infrequent. Low blood pressure (hypotension) requires administration of intravenous drugs or, in extreme cases, a temporary internal blood pressure support device (intra-aortic balloon pump). Fluid buildup in the lungs (congestive heart failure) necessitates drugs to promote urine production (diuretics) or occasionally the temporary use of a breathing support device (mechanical ventilator). A chaotic heart rhythm may require rhythm-stabilizing drugs or rarely electric shock (cardioversion).
Fortunately, with timely recognition and supportive therapy, takotsubo events are reversible, and recovery is usually rapid and complete. Heart function (contraction) gradually improves over several days and is usually normal by hospital discharge (3–7 days). The term stunned heart muscle is commonly used to indicate that injury in takotsubo, although initially profound, is only temporary. Drugs are discontinued once heart contraction has returned to normal.
Importance of Stress
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.
Identifying the stress trigger is occasionally challenging, and in 15% of cases cannot be elicited by careful history. Whether chronic stress can trigger this condition is unknown. Because stress is such an important part of takotsubo, the terms stress cardiomyopathy and broken heart syndrome have been used frequently.
What Causes Takotsubo?
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. In fact, events have been reported in patients after accidental overdose of adrenaline or associated with adrenaline-producing tumors (pheochromocytoma).
What Is the Long-Term Outlook?
With proper recognition and management, nearly all patients survive an acute takotsubo episode. 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. Although there is no evidence that drugs used in this syndrome prevent recurrences, multiple episodes do not produce cumulative damage. It is not understood why a specific stressful event will, on one occasion, trigger this condition, whereas at another time, a similar circumstance (even more stressful) does not.
Sources of Funding
This work was supported in part by a grant from The Hearst Foundations, New York, NY.
The information contained in this Circulation Cardiology Patient Page is not a substitute for medical advice, and the American Heart Association recommends consultation with your doctor or healthcare professional.
- © 2011 American Heart Association, Inc.
- Sharkey SW,
- Lesser JR,
- Zenovich AG,
- Maron MS,
- Lindberg J,
- Longe TF,
- Maron BJ
- Bybee KA,
- Prasad A