Takotsubo cardiomyopathy is a diagnosis that refers to a clinical syndrome characterized by acute and transient left ventricular dysfunction. The word Takotsubo, which translates to “octopus trap,” refers to the hallmark sign of apical ballooning shape during systole which resembles the shape of an octopus trap used in Japan. This diagnosis is also referred to as stress cardiomyopathy, broken heart syndrome, and apical ballooning syndrome. Regardless of the nomenclature, this is an important cardiac diagnosis that mimics and is often mistaken for an acute myocardial infarction at presentation.
Takotsubo cardiomyopathy is often, but not always, triggered by an emotional or physical stressful event, most often occurring within the preceding five days. Emotional stressors could be sudden death of a loved one, loss of job, financial strains, or a recent unfavorable medical diagnosis. As opposed to other types of cardiomyopathies, Takotsubo usually resolves within 21 days. While the clinical course is relatively short, it is associated with a significant potential mortality rate of up to 5%. It most commonly occurs in postmenopausal women with the mean age of 66 years and is most common in the Caucasian population (Medina de Chazal, et al., 2018).
The pathophysiology of Takotsubo is not clearly understood but this diagnosis proves a true mind-body connection. The most recent theory discusses that stress hormones, such as norepinephrine, and stress-related neuropeptides, which are usually stored in the terminal synapses, spillover at the myocardial level. This event may induce a toxic effect and/or epicardial and microvascular dysfunction. Predisposed patients may have higher baseline levels of these stress hormones and neuropeptides, and perhaps baseline endothelial or microvascular dysfunction.
Clinical presentation may include symptoms of chest pain, shortness of breath, palpitations, and syncope... sounds just like a heart attack, right?! Symptoms can also be manifestations of complications of Takotsubo, including congestive heart failure, pulmonary edema, cardiogenic shock, cardiac arrest, tachyarrhythmias, and severe mitral valve regurgitation. You can expect to see ischemic changes on electrocardiogram and possibly a prolonged QT interval. Serum troponin levels, a cardiac biomarker that is also elevated in myocardial infarctions, will be elevated.
The Heart Failure Association-European Society of Cardiology Criteria is the most widely accepted diagnostic criteria. Elements of the criteria include (Medina de Chazal, et al., 2018):
- Transient regional wall motion abnormalities of left or right ventricle, which is often, but not always preceded by a stressful trigger
- Wall motion abnormalities usually extend beyond a single epicardial vascular distribution
- Absence of culprit atherosclerotic coronary artery disease or other pathological conditions to explain the left ventricular dysfunction; cardiac catheterization is essential for diagnosis
- New and reversible electrocardiogram changes during acute phase (3 months)
- Significantly elevated serum natriuretic peptide during acute phase
- Positive, but relatively small, cardiac troponin
- Recovery of ventricular systolic function on cardiac imaging at follow-up in 3 to 6 months
While Takotsubo does eventually resolve, there are several potential complications for which close monitoring is essential. These include (Ghadri, et al., 2018):
- Acute heart failure
- Cardiogenic shock
- Left ventricular outflow tract obstruction (LVOTO) with/without mitral valve systolic anterior motion (SAM)
- Arrythmias (atrial and ventricular) and heart block
- Left ventricular thrombus formation leading to system thromboembolism and stroke
- Intramyocardial hemorrhage and ventricular wall rupture (rare)
- Death (in-hospital mortality rate is as high as 5%)
So, what is the best way to manage this unusual yet potentially life-threatening diagnosis? The answer is supportive care until the myocardium recovers and to prevent/manage complications. In the acute phase, these patients require close monitoring in a cardiac care unit. Medication therapy includes the use of diuretics to relieve congestion, beta blockers, ACE inhibitors, and at times, inotropes or vasopressors, depending on the severity of the clinical scenario. In refractory cases, mechanical support (i.e. intra-aortic balloon pump or percutaneous left ventricular assist device) or mechanical ventilation may be required temporarily. While there is no solid evidence to guide long term management, most experts advocate for ACE inhibitors and beta blockers to be initiated at diagnosis and continued at discharge. This combination of therapy is generally continued for at least three months, or until left systolic function has recovered, which is monitored by periodic echocardiograms. Treatment of underlying conditions, such as non-obstructive coronary artery disease, substance abuse disorders, and anxiety/depression is also recommended.
Takotsubo cardiomyopathy is not a diagnosis you will see every day, but it is one to include on your list of differential diagnoses when a patient presents with signs and symptoms of AMI and you uncover recent emotional or physical stressor.
Ghadri, J., Wittstein, I.S., Prasad, A., Sharkey, S., Dote, K., Akashi, Y.J…Templin, C. (2018). International expert document on Takotsubo syndrome (part I): Clinical characteristics, diagnostic criteria, and pathophysiology. European Heart Journal, 39, 2032-2046. doi: 10.1093/eurheartj/ehy076
Ghadri, J., Wittstein, I.S., Prasad, A., Sharkey, S., Dote, K., Akashi, Y.J…Templin, C. (2018). International expert document on Takotsubo syndrome (part II): Diagnostic workup, outcome, and management. European Heart Journal, 39, 2047-2062. doi: 10.1093/eurheartj/ehy077
Maron, M.S. (2019). Hypertrophic cardiomyopathy: Nonpharmacologic treatment of left ventricular outflow tract obstruction. Uptodate. Retrieved April 11, 2019, from https://www.uptodate.com/contents/hypertrophic-cardiomyopathy-nonpharmacologic-treatment-of-left-ventricular-outflow-tract-obstruction
Medina de Chazal, M., Del Buono, M.G., Keyser-Marcus, L., Ma, L., Moeller, G.F., Berrocal, D., & Abbate, A. (2018). Stress cardiomyopathy diagnosis and treatment: JACC State-of-the-Art Review. Journal of American Cardiology, 72, 1955-1971. doi: 10.1016/j.jacc.2018.07.072