Can you actually die of a broken heart?

Takotsubo cardiomyopathy is a diagnosis that refers to a clinical syndrome characterised by acute and transient left ventricular dysfunction. The word takotsubo, which will be translated as “octopus trap,” refers back to the characteristic symptom of a ballooned apex during systole that resembles the form of the octopus trap utilized in Japan. This diagnosis can also be known as stress cardiomyopathy, broken heart syndrome, and ballooned apex syndrome. Regardless of the nomenclature, it’s a very important cardiology diagnosis that mimics acute myocardial infarction and is commonly confused with it at presentation.

Takotsubo cardiomyopathy is commonly, but not at all times, triggered by a stressful emotional or physical event, most frequently occurring throughout the previous five days. Emotional stressors may include the sudden death of a loved one, job loss, financial problems, or a recent adversarial medical diagnosis. Unlike other sorts of cardiomyopathy, Takotsubo normally resolves inside 21 days. Although the clinical course is comparatively short, it’s related to a major potential mortality rate of as much as 5%. It most frequently occurs in postmenopausal women with a mean age of 66 years and is commonest within the white population (Medina de Chazal et al., 2018).

Pathophysiology

The pathophysiology of Takotsubo will not be fully understood, but this diagnosis demonstrates a real mind-body connection. The latest theory is that stress hormones corresponding to norepinephrine and stress-related neuropeptides, that are normally stored in terminal synapses, are transmitted to the extent of the myocardium. This event could cause toxic effects and/or epicardial and microvascular dysfunction. Predisposed patients can have higher baseline levels of those stress hormones and neuropeptides, and maybe baseline endothelial or microvascular dysfunction.

Clinical presentation

The clinical picture may include symptoms of chest pain, shortness of breath, palpitations, and fainting…appears like a heart attack, right?! Symptoms may additionally be a manifestation of Takotsubo complications, including congestive heart failure, pulmonary edema, cardiogenic shock, cardiac arrest, tachyarrhythmia, and severe mitral valve regurgitation. You can expect ischemic changes on the electrocardiogram and possibly a protracted QT interval. Serum troponin levels, a cardiac biomarker that can also be elevated in myocardial infarction, can be elevated.

Diagnosis

The Heart Failure Association-European Society of Cardiology criteria (also often called InterTAK diagnostic criteria) include (Medina de Chazal et al., 2018):

• Transient regional abnormalities of left or right ventricular wall motion which might be often, but not at all times, accompanied by a stress factor
• Wall motion abnormalities normally extend beyond a single epicardial vessel system.
• Absence of atherosclerotic coronary artery disease or other pathological conditions explaining left ventricular dysfunction; cardiac catheterization is required to make the diagnosis
• New and reversible electrocardiogram changes within the acute phase (3 months)
• Significantly elevated serum natriuretic peptide levels within the acute phase
• Positive but relatively low cardiac troponin level
• Restoration of ventricular systolic function in cardiac imaging on the follow-up visit after 3–6 months

The updated Mayo Clinic criteria may also be used to diagnose Takotsubo cardiomyopathy and include the next (Medina de Chazal et al., 2018):

• Transient hypokinesia, akinesia, or dyskinesia of the center segments of the left ventricle with or without apical involvement; regional wall motion abnormalities extend beyond a single epicardial vascular distribution; a stress trigger is commonly, but not at all times, present
• No obstructive coronary artery disease or angiographic evidence of acute plaque rupture
• New ECG abnormalities (ST segment elevation and/or T wave inversion) or mild cardiac troponin elevation
• No pheochromocytoma or myocarditis

Complications

Although Takotsubo eventually resolves, there are several potential complications for which close monitoring is crucial. These include (Ghadri et al., 2018):

• Acute heart failure
• Cardiogenic shock
• Left ventricular outflow tract obstruction (LVOTO) with/without systolic anterior mitral valve tour (SAM)
• Arrhythmias (atrial and ventricular) and heart blocks
• Formation of clots within the left ventricle of the center resulting in systemic embolism and stroke
• Intramyocardial hemorrhage and ventricular wall rupture (rare)
• Death (hospital mortality rate as high as 5%)

Management

So what’s the very best option to manage this unusual but potentially life-threatening diagnosis? The answer is supportive care until the myocardium recovers and prevention/treatment of complications. In the acute phase, these patients require close monitoring in a cardiology department resulting from potential complications, including shock and decompensated heart failure. Drug therapy includes standard guidelines for heart failure with treatment of reduced ejection fraction, with particular attention to avoiding volume depletion or vasodilation if LVOT obstruction is present (Reeder, 2023). In refractory cases, inotropic drugs, vasopressors, or mechanical support (i.e. intra-aortic balloon pump or percutaneous left ventricular assist device) and/or mechanical ventilation could also be required temporarily. Although there isn’t a solid evidence to guide long-term treatment, most experts favor starting ACE inhibitors and beta-blockers at diagnosis and continuing them at discharge. This combination of therapies is often continued for a minimum of three months or until left systolic function recovers, as monitored by periodic echocardiograms. Treatment of comorbidities corresponding to nonobstructive coronary artery disease, substance use disorders, and anxiety/depression can also be really helpful.

Takotsubo cardiomyopathy will not be a diagnosis you hear on daily basis, however it is price considering in your differential diagnosis list when you have got symptoms of a heart attack and also you detect a recent emotional or physical stressor.

Ghadri, J. R., Wittstein, I. S., Prasad, A., Sharkey, S., Dote, K., Akashi, Y. J., Cammann, V. L., Crea, F., Galiuto, L., Desmet, W., Yoshida, T., Manfredini, R., Eitel, I., Kosuge, M., Nef, H. M., Deshmukh, A., Lerman, A., Bossone, E., Citro, R., Ueyama, T., … Templin, C. (2018). International expert consensus document on takotsubo syndrome (part I): clinical characteristics, diagnostic criteria, and pathophysiology. , (22), 2032–2046. https://doi.org/10.1093/eurheartj/ehy076

Ghadri, J. R., Wittstein, I. S., Prasad, A., Sharkey, S., Dote, K., Akashi, Y. J., Cammann, V. L., Crea, F., Galiuto, L., Desmet, W., Yoshida, T., Manfredini, R., Eitel, I., Kosuge, M., Nef, H. M., Deshmukh, A., Lerman, A., Bossone, E., Citro, R., Ueyama, T., … Templin, C. (2018). International expert consensus document on Takotsubo syndrome (Part II): diagnostic studies, outcomes, and treatment. , (22), 2047–2062. https://doi.org/10.1093/eurheartj/ehy077

Maron, MS (2023, September 13). Hypertrophic cardiomyopathy: treatment for patients with left ventricular outflow tract obstruction. https://www.uptodate.com/contents/hypertrophic-cardiomyopathy-management-of-patients-with-outflow-duct-obstruction

Medina de Chazal, H., Del Buono, M. G., Keyser-Marcus, L., Ma, L., Moeller, F. G., Berrocal, D., and Abbate, A. (2018). Diagnosis and treatment of stress cardiomyopathy: a review of recent advances. JACC. , (16), 1955–1971. https://doi.org/10.1016/j.jacc.2018.07.072

Reeder, G. (2023, October 4). Treatment and prognosis of stress cardiomyopathy (Takotsubo). https://www.uptodate.com/contents/management-and-prognosis-of-stress-takotsubo-cardiomyopathy