Takotsubo cardiomyopathy (TCM), also known as stress cardiomyopathy or broken heart syndrome, is an acute, rapidly reversible, heart failure syndrome that's usually associated with identifiable emotional, psychological, or physical stress. Although TCM can be misdiagnosed as a myocardial infarction (MI) because it mimics the symptoms (chest pain, ST-segment elevation, and elevated cardiac enzyme levels), it presents with a distinctive left ventricle contraction pattern, with virtual apical ballooning and the absence of obstructive coronary artery disease. The term takotsubo cardiomyopathy was first used in Japan in 1990 to describe this syndrome. The shape of the left ventricle during systole appeared to have similarities with Japanese octopus trapping pots called takotsubo, which have round bottoms and narrow necks.
TCM is considered an important part of the differential diagnosis in acute coronary syndrome because of its close similarities with ST-segment elevation MI. About 1.7% to 2.2% of patients who were suspected to have acute coronary syndrome were diagnosed with TCM in reported studies. A literature review reported that 57.2% of patients affected were Asian, 40% were Caucasian, and 2% to 8% were other races. A mean patient age of 67 was reported, but some cases of TCM occurred in children and young adults. About 90% of reported cases were female and most of the women were postmenopausal, which is believed to be due to the loss of estrogen's protective action in response to stress.
Last December, the death of actress Debbie Reynolds a day after her daughter, actress Carrie Fisher, generated a public interest in the science behind broken heart syndrome.
What's going on with the heart?
Existing literature identifies serum catecholamine levels as being two to three times higher in patients with TCM within 1 to 2 days after the initial symptoms compared with MI patients with evidence of heart failure, and 20 times higher than in normal adults. Epinephrine concentration remains elevated and reaches MI level after 7 to 9 days. TCM can be classified into four groups based on the ballooning pattern (see TCM classification).
Transient epicardial coronary artery spasms lead to a transient myocardial stunning without any lasting injury. The reduction of parameters that indicate myocardial perfusion, such as the thrombosis in MI, or TIMI, frame count or myocardial perfusion imaging, is observed in the majority of TCM cases.
Transient left ventricular dysfunction can be caused by plaque rupture not clearly seen on coronary angiography. Oxidative stress response to excess catecholamines may be the underlying mechanism of left ventricular dysfunction in TCM, but there's a lack of evidence as to how oxygen-free radicals are released in response to an increased concentration of catecholamines—as a result of microvascular changes or myocyte injury caused by other mechanisms. Dynamic left ventricular outflow tract obstruction (LVOTO) may cause a rapid increase of left ventricular intracavity pressure and dilatation of the left ventricular apex.
No infective agent has been successfully isolated, even though mononuclear lymphocyte and macrophage infiltration was observed upon histologic examination of patients with TCM.
Causes to consider
The exact cause of TCM isn't known, but research suggests that the sudden release of stress hormones (norepinephrine, epinephrine, and dopamine) stuns the heart and triggers changes in the heart muscle and coronary blood vessels. The left ventricle weakens, preventing it from contracting effectively and pumping oxygen-rich blood throughout the body.
TCM can be caused by significant emotional or physical stressors, including:
- the unexpected loss of, or news of the illness or injury of, a spouse/partner, relative, friend, or pet
- receiving bad financial news or exacerbation of legal problems
- a newly diagnosed, significant medical condition or exacerbation of a chronic medical condition
- a fierce argument or domestic violence situation
- intense fear, such as with public speaking, a motor vehicle accident or other accident, during a near-drowning experience, or during a natural disaster
- sudden surprise
- severe pain
- physiologic stressors, such as noncardiac surgery, an asthma attack, withdrawal from illicit drugs, seizures, sepsis, or subarachnoid hemorrhage.
Certain pharmacologic therapies can also cause a surge of stress hormones, including epinephrine, duloxetine, venlafaxine, and levothyroxine.
TCM events are most prevalent in the afternoon, when stressful triggers are more likely to take place.
Patients are at a higher risk for TCM if one of their family members once had it. Although there are reported familial associations, there are no genetic studies to support the genetic basis of TCM.
Patient risk increases with age (over age 60), and more women are affected than men. Those with neurologic disorders, such as head injury or seizure disorder (epilepsy), are also at greater risk. Previous or current psychiatric disorders, as well as anxiety or depression, can also increase the risk of developing TCM.
Avenues to diagnosis
TCM is diagnosed in several ways. One method is to use an ECG to measure the heart's electrical activity and rhythm. ST-segment elevation (67% to 75%) and T wave inversion (61%) are the most common abnormalities seen on the ECG. Blood testing for cardiac enzymes that indicate damage to the heart muscles is another method. An echocardiogram may be done. Coronary angiograms allow healthcare professionals to view the heart and blood vessels to check for blockages restricting blood flow; the left ventricle has the syndrome's characteristic shape and no blockages are found in the blood vessels.
A chest X-ray can be used to see if the heart is enlarged or has the shape that's typical of TCM. It can also be used to check if there are any problems in the lungs that may be causing symptoms. Cardiac magnetic resonance imaging can be used to accurately visualize regional wall motion abnormalities, quantify ventricular function, and identify reversible injury to the myocardium due to the presence of edema or inflammation and the absence of necrosis or fibrosis.
A medical history will typically reveal that the patient with TCM didn't have any heart disease symptoms before diagnosis. Experiencing recent major stresses also points to TCM.
Supportive care in the hospital setting is required until left ventricle function is restored, with ICU monitoring for at least 24 hours due to the risk of ventricular arrhythmias, cardiogenic shock, and hemodynamic instability requiring more aggressive and invasive intervention. A wearable cardioverter defibrillator for patients with left ventricular systolic dysfunction in the acute care setting must be considered. Electrolytes must be closely monitored and replaced as needed to prevent torsades de pointes. QT-prolonging medications should be used with caution.
Medications. Beta-blockers, angiotensin-converting enzyme inhibitors, and diuretics can be used to reduce the heart's workload while a patient recovers and may help prevent further complications. Anticoagulants maybe administered briefly to avoid stroke secondary to risk of thromboembolism. Antianxiety or beta-blocker therapy may be given for a longer period to help control stress hormone release. Dobutamine and dopamine may be used in patients without evidence of LVOTO if they're intolerant and refractory to fluid resuscitation. Use these medications with caution in patients with LVOTO because agents with positive inotropic and chronotropic effects may worsen the obstruction and decrease cardiac output.
Other therapies. Intra-aortic balloon counterpulsation for patients with cardiogenic shock is another treatment. However, this may worsen cardiac output for TCM patients with LVOTO by reducing afterload; the adrenergic receptor antagonist phenylephrine must be selected for hypotensive patients because it has less inotropic effect and is less likely to increase the obstruction. Oxygen therapy can increase oxygen in the blood. Psychological therapy can address anxiety and stress. Treatment of triggering medical conditions such as asthma may be helpful. I.V. fluids may be needed if dehydration is suspected.
Patients may be discharged with long-term beta-blocker therapy or similar medications that block the potentially damaging effects of stress hormones on the heart. Patients should be encouraged to find ways to reduce stress and cope with upsetting situations, such as sharing their feelings with a supportive family member, friend, or therapist. Physical activity and relaxation therapy can also help relieve stress. Patients should also consider stress-management programs.
Close follow-up care with a cardiologist is necessary because the long-term effects of TCM are still unknown. Echocardiography may be recommended for about a month after the diagnosis of TCM. Instruct patients to call 911 or have someone take them to the ED immediately if they have chest pain, a rapid or irregular heart rate, or shortness of breath after a stressful event.
TCM has a good prognosis, with near-perfect recovery in 96% of patients. Mortality in the hospital is about 1% to 2%. Recurrence of symptoms, usually complaints of chest pain, occurred in 11% of TCM patients after 4 years' follow-up.
Timely recognition of this syndrome, supportive therapy, and medical follow-up are needed for quick recovery and to prevent sustaining long-term heart damage for patients with TCM.
Signs and symptoms
- Anxiety and diaphoresis
- Tachydysrhythmias and bradyarrhythmias
- Hypotension secondary to reduction in stroke volume due to acute left ventricular systolic dysfunction or LVOTO
- Murmurs and crackles secondary to pulmonary edema
- Shortness of breath
- Chest pain, pressure, or tightness
- Throat tightness
- Pain in the arm or back
- Loss of consciousness or fainting
- Takotsubo type: apical akinesia and basal hypercontraction
- Reverse takotsubo: basal akinesia and apical hypercontraction
- Mid ventricular type: mid ventricular ballooning and basal/apical hypercontraction
- Localized type: any other segmental ballooning when takotsubo-like left ventricle dysfunction is present
Source: Shimizu M, Kato Y, Masai H, Shima T, Miwa Y. Recurrent episodes of Takotsubo-like transient left ventricular ballooning occurring in different regions: a case report. J Cardiol. 2006;48(2):101-107.
- Left-sided heart failure with or without pulmonary edema
- Torsades de pointes
- Left ventricular thrombus formation
- Left ventricular free wall rupture
- Mitral valve regurgitation
- Rare cases: stroke, pneumothorax, ventricular septal defect, or death
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