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Broken Heart Syndrome (Takotsubo Cardiomyopathy)

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Ismail Munir

Overview

In the past, Japanese fishermen used ceramic vases, called takotsubo, to trap octopuses. The narrow neck and wide base of the pots would allow the 8-limbed sea creatures to climb inside and stay trapped within. In 1990, Dr Hikaru Sato was working at the Hiroshima City Hospital in Japan when he noticed a left ventricle, shaped like an octopus trapping pot, in a patient undergoing left ventriculography and angiography<a href="#accordion">[1]</a>.

He later observed similar findings in patients with symptoms of acute myocardial infarction, but no signs of an obstructive <a href="https://www.merisehat.pk/disease/coronary-artery-disease" target="_blank">coronary artery disease</a><a href="#accordion">[2]</a>. Soon, the cardiac abnormality became known as takotsubo cardiomyopathy (named after the pot). Later, several cases of the disease began to appear across Japan and other parts of the world. However, it wasn&rsquo;t until 2006 that the American Heart Association (AHA) included it under the classification of acquired cardiomyopathies<a href="#accordion">[3]</a>.&nbsp;

Takotsubo cardiomyopathy (TTC) is defined as a temporary dysfunction and ballooning of the left ventricular apex<a href="#accordion">[3]</a>. Therefore, it is also called apical ballooning syndrome. The prevalence of TTC is approximately 1-2% in patients with suspected acute myocardial infarction<a href="#accordion">[4]</a>. Since it normally results from a physical or emotional stressor, it&rsquo;s also called broken heart syndrome<a href="#accordion">[3]</a>.&nbsp;

Nearly 90% of cases occur in postmenopausal women<a href="#accordion">[5]</a>. However, the disease also affects men and children, particularly in Japan where the disease is most common in men<a href="#accordion">[6]</a>.&nbsp;

A study investigating the incidence of takotsubo cardiomyopathy in the United States identified a steady increase in cases between 2006 and 2017<a href="#accordion">[7]</a>. Moreover, around 88% of the cases occurred in women, mostly aged above 50 years<a href="#accordion">[7]</a>.&nbsp;

There is limited data available on the epidemiology of takotsubo cardiomyopathy in Pakistan. A 10-year retrospective study conducted at a tertiary hospital in Pakistan identified a total of 29 patients with primary and secondary TTC diagnoses<a href="#accordion">[8]</a>. Approximately 90% of these patients were female; 62% of these patients had experienced extremely stressful events such as a loved one&rsquo;s demise<a href="#accordion">[8]</a>.&nbsp;

<div>&nbsp;</div>

Takotsubo cardiomyopathy can be divided into<a href="#accordion">[9]</a>:

&nbsp;

<ul>
	<li>Primary Takotsubo Cardiomyopathy: This mainly affects women and follows an emotional stressor. No other cause for the patient&rsquo;s symptoms can be described.&nbsp;</li>
	<li>Secondary Takotsubo Cardiomyopathy: This type of takotsubo disease affects hospitalized patients. Due to a complication of their primary condition or its treatment, the patient faces a sudden rise in catecholamines and develops takotsubo cardiomyopathy. Secondary takotsubo disease affects both men and women equally and most commonly occurs after a physical stressor.&nbsp;</li>
</ul>

&nbsp;

Anatomically, takotsubo cardiomyopathy can be divided into 4 types<a href="#accordion">[10]</a>. These include:

<ul>
	<li>Typical TTC -&nbsp;Also called apical ballooning type, it is the most commonly encountered form of broken heart syndrome.</li>
	<li>Atypical TTC -&nbsp;This is further divided into:&nbsp;</li>
</ul>

<ol>
	<li>Mid-Ventricular Wall Motion Pattern</li>
	<li>Basal Wall Motion Pattern</li>
	<li>Focal Wall Motion Pattern&nbsp;</li>
</ol>

Atypical takotsubo cardiomyopathy typically occurs in younger patients and is mostly associated with underlying neurological conditions<a href="#accordion">[10]</a>.

<div>&nbsp;</div>

Signs And Symptoms

The symptoms of takotsubo cardiomyopathy are similar to that of myocardial infarction. They include:

<ul>
	<li><a href="https://www.merisehat.pk/disease/angina" target="_blank">Chest pain</a></li>
	<li>Shortness of breath</li>
	<li>Weakness</li>
	<li>Loss of consciousness</li>
</ul>

&nbsp;

In more severe cases, people may also develop an irregular heartbeat (arrhythmia) or cardiogenic shock<a href="#accordion">[11]</a>.&nbsp;

Although the symptoms are treatable, and the condition usually reverses in a few days or weeks, it is important to immediately contact a healthcare expert in the case of any sudden chest pain.&nbsp;

<div>&nbsp;</div>

Causes

Severe emotional or physical stress is believed to bring about broken heart syndrome. However, it is unclear how stressors affect the heart muscle.&nbsp;

Researchers predict that a surge in catecholamines might be to blame. They believe that the stress-induced release of catecholamines, such as adrenaline, &lsquo;stun&rsquo; the heart muscles. As a result, the left ventricle is unable to contract fully.

Moreover, the adrenaline constricts the arteries, causing a spasm of the coronary vessels, thereby, reducing the oxygen and blood flow to the muscle. The disruption in left ventricle contraction causes apical ballooning, which resembles the Japanese octopus trapping pot in imaging studies.&nbsp;

The hormone estrogen is also believed to play a role in causing takotsubo cardiomyopathy. It has a cardioprotective effect, preventing heart failure, hypertension, hypertrophy, etc<a href="#accordion">[12]</a>.&nbsp;However, in postmenopausal women, estrogen levels fall and the heart is prone to ischemia and vasoconstriction. Thus, increasing one&rsquo;s risk of developing broken heart syndrome.

&nbsp;

<h5>Physical Stressors</h5>

Examples of physical stressors that can cause takotsubo cardiomyopathy include<a href="#accordion">[10]</a>:

&nbsp;

<ul>
	<li>Medical condition (e.g., asthma, pneumothorax, COPD)</li>
	<li>Physical activity (e.g., sports)</li>
	<li>Pheochromocytoma (excessive catecholamine release from the tumor)</li>
	<li>Stroke</li>
	<li>Seizure</li>
	<li>Hypothermia</li>
	<li>Pregnancy</li>
	<li>Cesarean or vaginal delivery</li>
	<li>Alcohol</li>
	<li>Cocaine</li>
	<li>Near-death experiences, such as drowning&nbsp;</li>
	<li>Chemotherapy</li>
	<li>Medication, such as beta-agonist or epinephrine</li>
</ul>

&nbsp;

<h5>Emotional Stressors</h5>

Emotional stressors associated with takotsubo cardiomyopathy include<a href="#accordion">[10]</a>:

&nbsp;

<ul>
	<li>Loss of a loved one (e.g., friend, family member, or pet)</li>
	<li>Divorce</li>
	<li>Loss of employment</li>
	<li>Anxiety</li>
	<li>Depression</li>
	<li>Financial problems</li>
	<li>Fear or panic (e.g., during a robbery)</li>
	<li>Anger</li>
	<li>Natural disasters (e.g., earthquakes or tsunamis)</li>
</ul>

&nbsp;

Note: In nearly 30% of people in a study, there may be no identifiable triggers<a href="#accordion">[6]</a>.&nbsp;

<div>&nbsp;</div>

Although the symptoms of broken heart syndrome can resemble a heart attack, it is an entirely separate cardiac condition. A heart attack results from a complete or partial blockage of a coronary artery, whereas, in broken heart syndrome, there is no evidence of coronary artery obstruction. Moreover, in a heart attack, the blood supply to the area is obstructed, while the left ventricle in takotsubo cardiomyopathy changes shape in response to emotional or physical stressors and temporarily loses its contractility.&nbsp;

&nbsp;

Currently, there are no screening tests specifically designed to identify those at risk of takotsubo cardiomyopathy. A cardiac angiography is a definitive test to diagnose the abnormality.

&nbsp;

Diagnosis

To diagnose broken heart syndrome, doctors must first rule out all other heart conditions that can cause similar symptoms, such as myocardial infarction. After taking a detailed history, a complete physical examination is conducted. During a physical exam, doctors may report lung rales, tachycardia, low blood pressure, systolic ejection murmur, and an S3 gallop (The third heart sound (S3), also known as the &ldquo;ventricular gallop,&rdquo; occurs just after S2 when the mitral valve opens, allowing passive filling of the left ventricle).&nbsp;

&nbsp;

Moreover, the doctor may perform the following tests:

&nbsp;

<ul>
	<li>Cardiac Biomarkers: Elevated levels of cardiac enzymes, such as troponin, may be present. However, these levels are lower than ones seen in ST-elevation myocardial infarction (STEMI). Levels of creatinine kinase and brain natriuretic peptide (BNP) may also be elevated.</li>
</ul>

&nbsp;

<ul>
	<li>Echocardiography: This is an ultrasound of the heart and allows the doctor to visualize the heart chambers and their size as they pump blood. It is a quick method for diagnosing wall-motion abnormalities present in takotsubo cardiomyopathy. Moreover, echocardiography can also help estimate left ventricular ejection fraction.</li>
</ul>

&nbsp;

<ul>
	<li>Chest X-ray: An X-ray can help identify an enlarged heart and any lung abnormality.&nbsp;</li>
</ul>

&nbsp;

<ul>
	<li>Cardiac MRI Scan: MRI scans allow a more detailed view of the heart&rsquo;s anatomy, function, and identify any abnormality.&nbsp;</li>
</ul>

&nbsp;

<ul>
	<li>Electrocardiogram (ECG): In patients with takotsubo cardiomyopathy, patients often have ST-segment elevation similar to myocardial infarction. T-wave inversion may also be present in these patients.&nbsp;</li>
</ul>

&nbsp;

<ul>
	<li>Cardiac Angiography: The imaging study will show ballooning of the left ventricular apex and identify any coronary artery blockage. The absence of an artery blockage signifies takotsubo cardiomyopathy. A cardiac angiography can help confirm the diagnosis of the condition.&nbsp;</li>
</ul>

&nbsp;

The Mayo Clinic&rsquo;s revised diagnostic criteria for takotsubo cardiomyopathy require the presence of the following<a href="#accordion">[5]</a>:

<ul>
	<li>Absence of coronary artery obstruction or any angiographic evidence of acute plaque rupture</li>
	<li>ECG abnormalities or elevated troponin levels</li>
	<li>Absence of pheochromocytoma and myocarditis</li>
	<li>Temporary hypokinesis, dyskinesis, or akinesis of the left ventricular midsegments, with or without the involvement of the apex</li>
	<li>Wall motion abnormalities that extend beyond a single epicardial vascular distribution</li>
</ul>

<div>&nbsp;</div>

Treatment

There is no specific treatment for takotsubo cardiomyopathy. Treatment is mostly supportive and is continued till the left ventricle regains its function, which usually occurs within 21 days of onset<a href="#accordion">[5]</a>. Management is aimed at controlling symptoms and treating any associated complications.&nbsp;

&nbsp;

Doctors may often prescribe:

&nbsp;

<ul>
	<li>Angiotensin-Converting Enzyme (ACE) Inhibitors: Help control hypertension and reduce the workload on the heart.</li>
	<li>Angiotensin-Receptor Blockers (ARB): Help control hypertension and reduce the workload on the heart.</li>
	<li>Beta-Blockers: Help reduce the strain on the heart.</li>
	<li>Blood Thinners: Help reduce the risk of blood clots.</li>
	<li>Diuretics: Help with pulmonary congestion by decreasing the fluid buildup.</li>
	<li>Vasodilators: Help with pulmonary congestion.</li>
</ul>

&nbsp;

In patients with cardiogenic shock, the presence of left ventricular outflow tract obstruction (LVOTO) may require the administration of:

<ul>
	<li>Intravenous fluids</li>
	<li>Vasopressors</li>
	<li>Extracorporeal membrane oxygenation&nbsp;</li>
</ul>

In the absence of LVOTO, doctors may only prescribe inotropes and vasopressors to patients with cardiogenic shock.&nbsp;

&nbsp;

Approximately 95% of patients fully recover their cardiac function within a few weeks<a href="#accordion">[5]</a>. However, lifestyle changes such as stress management and physical activity along with antihypertensives may be required to keep the condition from recurring. In 5% of the patients, broken heart syndrome can recur<a href="#accordion">[13]</a>.

<div>&nbsp;</div>

Complications can occur in less than 52% of the patients<a href="#accordion">[9]</a>. These include:

<ul>
	<li>Cardiogenic shock</li>
	<li>Arrhythmia</li>
	<li>Cardiac rupture</li>
	<li><a href="https://www.merisehat.pk/disease/pulmonary-edema" target="_blank">Pulmonary edema</a></li>
	<li>Thromboembolism</li>
	<li>Heart failure</li>
	<li>Mitral regurgitation</li>
	<li><a href="https://www.merisehat.pk/disease/stroke" target="_blank">Stroke</a></li>
</ul>

&nbsp;

Moreover, the condition carries a mortality rate of 4.1%<a href="#accordion">[14]</a>.&nbsp;

<div>&nbsp;</div>

The following factors may increase one&rsquo;s risk of broken heart syndrome:

&nbsp;

<ul>
	<li>Gender: Women have a higher incidence of cardiomyopathy<a href="#accordion">[13]</a>.</li>
	<li>Age: Being 50 years and above is a risk factor for both men and women<a href="#accordion">[9]</a>.</li>
	<li>Mental Health Disorder: Both anxiety and depression are associated with broken heart syndrome</li>
	<li>Stress: Although a small proportion of cases result from no identifiable trigger, emotional and physical stress is a major cause of several cases<a href="#accordion">[6]</a>.</li>
	<li>Others: Smoking, alcohol abuse, and hyperlipidemia can also lead to broken heart syndrome.</li>
</ul>

<div>&nbsp;</div>

There are no standard prevention strategies for takotsubo cardiomyopathy. Doctors typically advise stress management. Moreover, therapy and counseling may help patients learn better coping mechanisms for any future stressors.&nbsp;

&nbsp;

Key Points

<ul>
	<li>Takotsubo cardiomyopathy or broken heart syndrome is defined as a temporary dysfunction and ballooning of the left ventricular apex. It predominantly affects post-menopausal women and is preceded by a physical or emotional stressor.</li>
	<li>The symptoms of broken heart syndrome resemble that of a heart attack. They include chest pain, shortness of breath, and sudden loss of consciousness. However, unlike a myocardial infarction, there is no obstructive coronary heart disease in patients with the broken heart syndrome.&nbsp;</li>
	<li>In more severe cases, people may also develop an irregular heartbeat (arrhythmia) or cardiogenic shock. Other complications of the disease include cardiac rupture, pulmonary edema, and stroke.&nbsp;</li>
	<li>Treatment is mostly supportive and is continued till the left ventricle regains its function. It is usually aimed at controlling symptoms and treating any associated complications. A combination of ACE inhibitors, beta-blockers, blood thinners, diuretics, and vasodilators may be prescribed. In severe cases, doctors may administer intravenous fluids, inotropes, and vasopressors.&nbsp;</li>
	<li>Although takotsubo cardiomyopathy is treatable and usually reverses in a few days or weeks, it is important to immediately contact a healthcare expert in the case of any sudden chest pain.</li>
</ul>

<div>&nbsp;</div>

<div>&nbsp;</div>

[1] Tofield A. Hikaru Sato and Takotsubo cardiomyopathy. European Heart Journal. 2016;37(37):2812-2812. doi:10.1093/eurheartj/ehw367

[2] Y-Hassan S, Tornvall P. Epidemiology, pathogenesis, and management of takotsubo syndrome. Clin Auton Res. 2018;28(1):53-65. doi:10.1007/s10286-017-0465-z

[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. doi:10.4330/wjc.v6.i7.602

[4] Prasad A, Lerman A, Rihal CS. Apical ballooning syndrome (Tako-Tsubo or stress cardiomyopathy): a mimic of acute myocardial infarction. Am Heart J. 2008;155(3):408-417. doi:10.1016/j.ahj.2007.11.008

[5] Boyd B, Solh T. Takotsubo cardiomyopathy. Journal of the American Academy of Physician Assistants. 2020;33(3):24-29. doi:10.1097/01.jaa.0000654368.35241.fc

[6] Templin C, Ghadri JR, Diekmann J, et al. Clinical features and outcomes of takotsubo (stress) cardiomyopathy. New England Journal of Medicine. 2015;373(10):929-938. doi:10.1056/nejmoa1406761

[7] Pattisapu VK, Hao H, Liu Y, et al. Sex‐ and age‐based temporal trends in takotsubo syndrome incidence in the United States. Journal of the American Heart Association. 2021;10(20). doi:10.1161/jaha.120.019583

[8] Laghari A, Khan A, Kazmi K. Takotsubo cardiomyopathy: Ten Year experience at a tertiary care hospital in Pakistan. BMC Research Notes. 2014;7(1):932. doi:10.1186/1756-0500-7-932

[9] Sheppard MN. Takotsubo Syndrome - Stress-induced Heart Failure Syndrome. Eur Cardiol. 2015;10(2):83-88. doi:10.15420/ecr.2015.10.2.83

[10] Ghadri J-R, Wittstein IS, Prasad A, et al. International expert consensus document on takotsubo syndrome (part I): Clinical characteristics, diagnostic criteria, and pathophysiology. European Heart Journal. 2018;39(22):2032-2046. doi:10.1093/eurheartj/ehy076

[11] Galiuto L, Crea F. Primary and secondary takotsubo syndrome: Pathophysiological determinant and prognosis. European Heart Journal Acute Cardiovascular Care. 2020;9(7):690-693. doi:10.1177/2048872620963493

[12] Waqar A, Jain A, Joseph C, et al. Cardioprotective Role of Estrogen in Takotsubo Cardiomyopathy. Cureus. 2022;14(3):e22845. Published 2022 Mar 4. doi:10.7759/cureus.22845

[13] Assad J, Femia G, Pender P, Badie T, Rajaratnam R. Takotsubo syndrome: A review of presentation, diagnosis and management. Clinical Medicine Insights: Cardiology. 2022;16:117954682110657. doi:10.1177/11795468211065782

[14] Ahmad SA, Brito D, Khalid N, et al. Takotsubo Cardiomyopathy. [Updated 2022 Mar 24]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-.