Takotsubo Cardiomyopathy

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  • Takotsubo cardiomyopathy (TCM) is a transient condition caused by the weakening of the left ventricle (LV) and can have a critical presentation. Similar to an acute myocardial infarction (AMI), the main symptoms are chest pain and dyspnea, which usually resolve completely. TCM is caused by intense emotional or physical stress, hence the reason why it may also be called broken-heart syndrome or stress-induced cardiomyopathy.

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    What is Takotsubo Cardiomyopathy?

    Takotsubo cardiomyopathy (TCM) is a transient condition caused by the weakening of the left ventricle (LV) and can have a critical presentation. Similar to an acute myocardial infarction (AMI), the main symptoms are chest pain and dyspnea, which usually resolve completely. TCM is caused by intense emotional or physical stress, hence the reason why it may also be called broken-heart syndrome or stress-induced cardiomyopathy. TCM can also be referred to as left ventricular apical ballooning syndrome. It is more prevalent in postmenopausal elderly women.1, 2, 3 

    Causes

    TCM can be caused by an intense emotional or physical situation such as a serious illness, an accident, the death of someone important, or an earthquake or other natural disaster2. Some of the stressors associated with TCM are: a sudden drop in blood pressure; intense pain, surgery, or medical procedure (e.g. cardiac stress test); domestic violence; receiving unpleasant news; and fear or other intense emotion.4

    Signs and Symptoms

    Symptoms are similar to the ones of AMI. These can include chest pain and tachypnea, but some people can also have other symptoms, including palpitations, nausea, vomiting, syncope, or cardiogenic shock.5, 3

    Diagnosis

    Takotsubo cardiomyopathy is diagnosed if an AMI is excluded in cases of myocardial damage related to changes in the left ventricle shape.5

    The Mayo Clinic published the diagnostic criteria that was most widely accepted, in 2004: The modified criteria requires for all four of the following to be observed7:

    1. Transient hypokinesis, dyskinesis, or akinesis of the LV midsegments, with or without apical involvement; the regional wall-motion abnormalities extend beyond a single epicardial vascular distribution, and a stressful trigger is often, but not always, present.
    2. Absence of obstructive coronary disease or angiographic evidence of acute plaque rupture.
    3. New ECG abnormalities (either ST-segment elevation and/or T-wave inversion) or modest elevation in the cardiac troponin level.
    4. Absence of pheochromocytoma or myocarditis.

    In 2008, a normal epicardial coronary artery was added to the criteria3.

    Electrocardiographic characteristics: Although the initial ECG of patients with TCM does not show anything specific, in half of patients, ST-segment elevation can be found mainly in the precordial leads at the onset. Inverted T waves are more frequently observed in patients with apex balloon-like dilation, in comparison with patients with base deformity, and they resolve spontaneously. TCM patients usually present transient abnormal Q waves in precordial leads.3

    Enzymes:  On admission, the blood level of catecholamines and plasma brain natriuretic peptide (BNP) is usually high. BNP secretion is similar to that in AMI patients. Basal hyperkinesia might be related to LV end-diastolic pressure and BNP effects. Cardiac enzyme levels, such as creatinine kinase and troponin T, are slightly increased, but they decrease rapidly and do not affect the prognosis.3, 6

    Coronary angiography: It is mandatory that no specific coronary lesions be detected in TCM. Usually, patients with TCM have precordial pain, ECG alterations, an elevation of cardiac enzyme levels, and wall motion abnormalities. Therefore, coronary angiography excludes the possibility of an acute coronary syndrome.3
    Balloon-like dilation of the ventricle: In contrast with AMI, in TCM patients, LV wall motion abnormalities are found beyond single coronary artery perfusion. In most cases, loss of motion or hypokinesia at the apex and an apical balloon-like dilation pattern associated with the preservation of the base is found.3 

    Cardiac Magnetic Resonance Imaging (MRI): Cardiac MRI is a great TCM diagnostic method, as it allows precise detection of reversible myocardial damage. It is able to show wall motion abnormalities in every area, the percentage of ventricular function, and the presence of inflammation or fibrosis. MRI is appropriate to evaluate wall motion and LV ejection fraction, and to check the absence of delayed gadolinium enhancement, which allows differentiation of TCM from AMI and myocarditis (the two latter conditions are associated with delayed gadolinium enhancement3).

    Treatment

    There is no standard treatment for TCM and it depends on the severity of symptoms, including if the patient has alterations in blood pressure levels or pulmonary edema. Physicians can prescribe standard heart failure drugs such as beta-blockers, ACE inhibitors, and diuretics. Aspirin can be prescribed for patients with atherosclerosis. Even if there is little evidence for long-term therapy, beta-blockers may be used continuously to avoid recurrence, by reducing the effects of stress hormones. Due to TCM etiology, it is also important to prevent any physical or emotional stress.2 
    Prognosis
    Patients with TCM usually have a good prognosis, and almost perfect recovery is observed in 96% of cases, with the mortality rate in hospitals varying at one-to-two percent.3 Patients recover from most of the abnormalities in systolic function, whilst ventricle wall movement clears up in one-to-four weeks (with most patients recovering fully within two months2). Even though death is rare, heart failure can occur in approximately 20% of the patients. Complications such as arrhythmias, LV obstructions of blood flow, and rupture of the ventricle wall are not common.2 Recurrence of TCM can occur in around 10% of patients after a 4-year follow-up.3

    References

    1. Dawson DK. Acute stress-induced (takotsubo) cardiomyopathy. Heart 2018; 104:96-102.
    2. Harvard Heart Publishing. Takotsubo cardiomyopathy (broken-heart syndrome). Jan 20.
    3. Komamura K, Fukui M, Iwasaku T, Hirotani S, Masuyama T. Takotsubo cardiomyopathy: Pathophysiology, diagnosis, and treatment. World J Cardiol. 2014;6(7):602‐609. 
    4. Prasad A, et al. American Heart Journal (2008), Vol. 155, No. 3, pp. 408–17.
    5. Takotsubo cardiomyopathy, British Heart Foundation. Jan. 20.
    6. Yoshihiro et al. Takotsubo Cardiomyopathy, A New Form of Acute, Reversible Heart Failure.Circulation. 2008; 118:2754–2762.
    7. Kawai S; Kitabatake A; Tomoike H. Guidelines for diagnosis of takotsubo (ampulla) cardiomyopathy. Circ J.  2007; 71(6):990-2.
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