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Last updated: 07/22/2024
Years published: 2020, 2024
NORD gratefully acknowledges Leslie T. Cooper Jr., MD, Chair, Cardiovascular Department, Mayo Clinic in Florida, Professor of Medicine, Mayo Clinic and Etienne Leveille, MD, Yale University School of Medicine, for assistance in the preparation of this report.
Summary
Takotsubo cardiomyopathy is a heart disease characterized by transient dysfunction and ballooning of the left ventricle of the heart. It mostly affects elderly women and is often triggered by severe physical or emotional stress. The symptoms are similar to those of a heart attack (myocardial infarction) and include chest pain, difficulty breathing (dyspnea), and sometimes transient loss of consciousness (syncope). Although complications can occur, most individuals fully recover within one month. The treatment of takotsubo cardiomyopathy is focused on symptom control and the prevention and treatment of associated complications.
Introduction
The role of the heart is to act as a pump to make sure blood is appropriately oxygenated and able to feed the body. The heart has four chambers: two small atria (left and right), which are located on top of the heart and two larger ventricles (also left and right), which are located below the atria and act as the main pump of the heart. Blood flows in the body the following way: 1) Blood in the left ventricle is ejected into the aorta, which redistributes oxygenated blood throughout the body 2) Oxygen in the blood is extracted by organs and deoxygenated blood comes back in the right atrium of the heart via the veins 3) Deoxygenated blood in the right atrium falls into the right ventricle 3) Deoxygenated blood is ejected from the right ventricle into the pulmonary arteries, where it will be able to take oxygen from the lungs 4) Oxygenated blood comes back to the left atrium via the pulmonary veins 5) Oxygenated blood in the left atrium falls into the left ventricle of the heart where the cycle can start again.
Takotsubo cardiomyopathy is a type of disease of cardiac muscle (cardiomyopathy) characterized by dysfunction and ballooning of a portion of the left ventricle of the heart, most often its tip (apex). It was first identified in Japan in the 1990s and was named after Japanese octopus traps (takotsubo) that are shaped similarly to the heart of affected individuals. Since that time, the condition has been recognized in individuals all over the world.
Takotsubo cardiomyopathy can occur in adults of any age, but most often develops in elderly females. The most common symptoms are the abrupt (acute) onset of crushing chest pain and difficulty breathing (dyspnea). Anxiety, sweating (diaphoresis), nausea, vomiting, palpitations and temporary loss of consciousness (syncope) can also occur.
Most individuals that develop takotsubo cardiomyopathy fully recover within one month and recurrence is rare. However, complications occur in about 20% of patients. In some individuals, the heart might be unable to pump blood efficiently (heart failure), which can lead to fatigue, dyspnea and leg and abdomen swelling due to fluid accumulation (edema). In severe cases, cardiac dysfunction might prevent organs from receiving adequate oxygen supply (cardiogenic shock). Takotsubo cardiomyopathy can also lead to an irregular, too fast, or too slow heartbeat (arrhythmia). Another possible complication is pooling of blood in the heart, which promotes clot formation. These clots can be expulsed from the heart and lodge into arteries of the brain, which can lead to a stroke. Despite the possibility of complications, more than 90% of affected individuals survive takotsubo cardiomyopathy.
The mechanism by which takotsubo cardiomyopathy develops is not entirely understood. In about 2/3 of cases, the disease is preceded by intense emotional stress (such as divorce, death of a loved one or bankruptcy) or physical stress (such as a stroke, fracture or infection). A hypothesis is that these stressors lead to the release of large amounts of adrenaline (epinephrine) and other stress-related hormones collectively known as catecholamines. This surge of hormones might lead to spasms of the blood vessels and disruption of the ventricles of the heart, which would be responsible for the ventricular dysfunction and ballooning seen in takotsubo cardiomyopathy.
Takotsubo cardiomyopathy can develop in males and females of any age and ethnic background. However, about 90% of affected individuals are females, and 80% are females over the age of 50. The exact frequency of the disease is not known and might be underestimated but is thought to comprise approximately 2% of all individuals (and up to 5% of females) with suspected ST-segment elevation myocardial infarction, which is the most severe type of heart attack. This represents more than 4,000 individuals in the United States.
Individuals with takotsubo cardiomyopathy usually present with symptoms undistinguishable from a myocardial infarction and will, therefore, be considered to have a heart attack until proven otherwise. The two most common and useful initial tests for an individual with symptoms suggesting an acute MI are an electrocardiogram (ECG), which measures electrical activity of the heart and blood troponin levels, which is a marker of damage to the heart. In takotsubo cardiomyopathy, the ECG shows changes that are typically seen in a ST-segment elevation myocardial infarction (the most severe type of heart attack), and troponin levels are elevated, as they also are in an MI. However, when coronary angiography (a medical imagining technique in which a dye is injected into the coronary arteries to visualize them with radiographs) is performed in individuals with takotsubo cardiomyopathy, no significant coronary artery occlusion is seen. Ventricular dysfunction and ballooning can be identified with ventriculography (which uses the same principles as coronary angiography but allows visualization of the ventricles) or echocardiography (which uses ultrasound waves to make visualization of the heart possible). Cardiac magnetic resonance imaging (MRI) is a specialized imaging technique that can occasionally be used to diagnose takotsubo cardiomyopathy and assess the extent of ventricular dysfunction and ballooning.
The InterTAK Diagnostic Score predicts the probability of the diagnosis of a takotsubo cardiomyopathy event and differentiates these patients from those who have acute coronary syndrome. The InterTAK Diagnostic Score is based on data from the International Takotsubo Registry and includes seven clinical variables that can be easily applied without using invasive imaging tools. The maximum score is 100 points.
Treatment & Management
Although there is no standard treatment for takotsubo cardiomyopathy, most individuals fully recover within 1 month. Research has shown that the use of angiotensin-converting–enzyme (ACE) inhibitors or angiotensin-receptor blockers (ARB), which are two classes of medication used to treat high blood pressure, might improve survival after the condition has resolved. Many patients are prescribed beta-blockers, a class of medication that decreases the activating effects of catecholamines on the heart. However, there is no scientific evidence proving the use of beta-blockers improves survival in takotsubo cardiomyopathy.
The management of the disease is mostly centered on symptom control and prevention and treatment of associated complications (supportive therapy). Patients who develop heart failure are usually treated with medication that stimulate the production of urine to eliminate excess fluid (diuretics) and medication that dilate blood vessels (vasodilators) to decrease the blood pressure the heart has to pump against. Fluid resuscitation might be required in those that develop cardiogenic shock. In some patients, blood thinners (anticoagulants) might be given to prevent the formation of blood clots (thrombus). Pain control and addressing emotional and physical stressors that might have played a role in the development of the condition are two other important factors to consider.
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JOURNAL ARTICLES
Ghadri JR, Wittstein IS, Prasad A, et al. International expert consensus document on takotsubo syndrome (part I): clinical characteristics, diagnostic criteria, and pathophysiology. Eur Heart J. 2018 Jun 7;39(22):2032-2046. doi: 10.1093/eurheartj/ehy076. https://www.ncbi.nlm.nih.gov/pubmed/29850871
Templin C, Ghadri JR, Diekmann J, et al. Clinical features and outcomes of takotsubo (stress) cardiomyopathy. N Engl J Med 2015; 373:929-938. https://www.nejm.org/doi/full/10.1056/NEJMoa1406761
INTERNET
Broken heart syndrome. Mayo Clinic. Last updated: Nov. 11, 2023.
https://www.mayoclinic.org/diseases-conditions/broken-heart-syndrome/diagnosis-treatment/drc-20354623 Accessed May 2, 2024.
Cammann VL, Templin C, Templin-Ghadri J-R. Tako-Tsubo cardiomyopathy. Orphanet. Last updated: October 2019.
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=66529 Accessed May 2, 2024.
Cooper LT. Definition and classification of the cardiomyopathies. UpToDate. Last updated: Aug 16, 2022. https://www.uptodate.com/contents/definition-and-classification-of-the-cardiomyopathies Accessed May 2, 2024.
Meyer TE. Approach to acute decompensated heart failure in adults. UpToDate. Last updated: May 09, 2023. https://www.uptodate.com/contents/approach-to-acute-decompensated-heart-failure-in-adults Accessed May 2, 2024.
Beltrame JF and Crea F. Vasospastic angina. UpToDate. Last updated: Aug 10, 2022. https://www.uptodate.com/contents/vasospastic-angina/print Accessed May 2, 2024.
Reeder GS, Awtry E, Mahler SA. Initial evaluation and management of suspected acute coronary syndrome (myocardial infarction, unstable angina) in the emergency department. UpToDate. Feb 19, 2024. https://www.uptodate.com/contents/initial-evaluation-and-management-of-suspected-acute-coronary-syndrome-myocardial-infarction-unstable-angina-in-the-emergency-department Accessed May 2, 2024.
Reeder GS, Kennedy HL. Diagnosis of acute myocardial infarction. UpToDate. Last updated: Oct 05, 2022. https://www.uptodate.com/contents/diagnosis-of-acute-myocardial-infarction Accessed May 2, 2024.
Reeder GS, Kennedy HL. Overview of the acute management of ST-elevation myocardial infarction. UpToDate. Last updated: Apr 26, 2024.
https://www.uptodate.com/contents/overview-of-the-acute-management-of-st-elevation-myocardial-infarction Accessed May 2, 2024.
Reeder GS, Prasad A. Clinical manifestations and diagnosis of stress (takotsubo) cardiomyopathy. UpToDate. Last updated: Aug 17, 2023.
https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-stress-takotsubo-cardiomyopathy Accessed May 2, 2024.
Reeder GS, Prasad A. Management and prognosis of stress (takotsubo) cardiomyopathy. UpToDate. Last updated: Aug 17, 2023. https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-stress-takotsubo-cardiomyopathy Accessed May 2, 2024.
Tomich EB, Takotsubo (Stress) Cardiomyopathy (Broken Heart Syndrome). Medscape. Last updated: Jul 31, 2019. https://emedicine.medscape.com/article/1513631-overview Accessed May 2, 2024.
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