Takotsubo Cardiomyopathy

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Original Editors - Megan Kaiser & Briana Ulanowski from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Megan Kaiser, Briana Ulanowski, Kim Jackson, Lucinda hampton, Elaine Lonnemann, Wendy Walker, WikiSysop, 127.0.0.1 and Adam Vallely Farrell  

Definition/Description

Takotsubo cardiomyopathy occurs when there is an abnormal contraction of the transient left ventricle, creating a balloon shape appearance initially during systole. The Japanese first described the heart condition around 1991. The shape of the heart resembles a Japanese octopus trapping pot with a rounded bottom and narrow neck; hence the name tako-tsubo.

 

Image depicting resemblance between heart during contraction phase in patient with TC (on left) and Japanese takotsubo (on right)

Typically an intense emotional stress can trigger this type of event. High levels of catecholamines are present in these patients and can cause the heart to be stunned temporarily. This is a reversible cardiomyopathy and clinically presents as a myocardial infarction. Individuals that experience this may or may not have a cardiovascular disease.
Other names include:

•  Broken heart syndrome
• Ampulla cardiomyopathy
• Stress cardiomyopathy
• Apical ballooning syndrome (ABS)
• Acute left ventricular ballooning
  

Prevalence

Takotsubo cardiomyopathy is a very rare condition and has to be differentiated from myocardial infarct. The incidence of takotsubo cardiomyopathy is 1-2% in patients diagnosed with MI. These individuals are usually postmenopausal females (90%). The average age is 62-75 years old. There are approximately 7,000-14,000 cases of takotsubo cardiomyopathy in the U.S.

Characteristics/Clinical Presentation

Usually takotsubo cadiomyopathy is triggered by emotional stress, physical stress, non-cardiac surgery or procedure (refer to table). The onset is insidious and presents with chest pain at rest.

Table I. Stressors reported to trigger ABS Emotional stress Death or severe illness or injury of a family member, friend, or pet Receiving bad news—diagnosis of a major illness, daughter's divorce, spouse leaving for war Severe argument Public speaking Involvement with legal proceedings Financial loss—business, gambling Car accident Surprise party Move to a new residence Physical stress Non-cardiac surgery or procedure—cholecystectomy, hysterectomy Severe illness—asthma or chronic obstructive airway exacerbation, connective tissue disorders, acute cholecystitis, pseudomembranous colitis Severe pain—fracture, renal colic, pneumothorax, pulmonary embolism Recovering from general anesthesia Cocaine use Opiate withdrawal Stress test—dobutamine stress echo, exercise sestamibi Thyrotoxicosis

 Signs and Symptoms of Takotsubo include:

• Chest pain
• Dyspnea
• Hypotension
• Syncope
• Mild to moderate HF
• Pulmonary edema
• Lab Changes
• Modest increase in troponin T levels
• Increase in creatine phosphokinase (CPK)
• Elevation in pBNP
• High levels of serum catecholamines

The most common signs and symptoms are chest pain and dyspnea; resembling a myocardial infarction.

Associated Co-morbidities

Research has not shown any comorbidities linked with TC.

Medications

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Diagnostic Tests/Lab Tests/Lab Values

Diagnostic tests used for TC include lab values, echocardiography, cardiac angiography, and electrocardiography.

Lab Values
Troponin T level:

• Mean value of healthy individuals = 0.49 ng/mL,
• Mean peak value of patients with TC = 0.64 ng/mL

Troponin I level:

• Mean value of healthy individuals = 4.2 ng/mL
• Mean peak value of patients with TC = 8.6 ng/mL

Brain natriuretic peptide level is elevated
Catecholamines are elevated in the acute phase

Echocardiography
An echocardiography is used when diagnosing wall-motion abnormalities. The heart will show hypokinesis or akinesis of the middle and apical segment of the left ventricle if the diagnosis is TC.

Cardiac Angiography
The test will show normal coronary arteries in patients with TC.

Electrocardiography (EKG) Changes

Generally, patients could have 4 different EKG changes:

• ST-segment elevation
• T-wave inversion
• Abnormal Q waves
• QT segment prolongation

In a study conducted by Mitsuma et al, they showed that EKG changes could occur in phases:

1. ST-segment elevation (immediately)
2. T-wave inversion (days 1-3)
3. Improvement in T-wave inversion (days 2-6)
4. Deeper T-wave inversion
   

Diagnostic Criteria
Must have all 4 criteria to be diagnosed with ABS
• Transient hypokinesis, akinesis, or dyskinesis of the LV midsegments
• Absence of obstructive coronary artery or angiographic evidence of acute plaque rupture
• New EKG abnormalities or elevated cardiac troponin
• Absence of recent head trauma, intracranial bleeding, pheochromocytoma, myocarditis, and hypertrophic cardiomyopathy

Etiology/Causes

Research has shown three possible causes of takotsubo cardiomyopathy: increased levels of catecholamines, left ventricular outflow tract (LVOT) obstruction, and microcirculation dysfunction.

1. Catecholamines (norepinephrine, epinephrine, and dopamine): In individuals with suspected TC, catecholamines are 7 to 34 times higher than the values of normal individuals.  The catecholamine levels are also 2 to 3 times greater than with individuals diagnosed with a myocardial infarction.  These levels stay elevated for 7 to 9 days following the episode. TC can develop due to high doses of epinephrine.  There are also more catecholamine-sensitive receptors in the apex of the heart, making it more sensitive to increased levels.
2. LVOT obstruction: It could be linked to the increased catecholamine levels and occurs in approximately 25% of patients with TC.
   —Mid-ventricular septal thickening is a common cardiac abnormality in elderly women
   —Increases ventricle wall stress, increased filling pressure, and decreased systemic BP2
   —Leads to myocardial ischemia and “stunning”
   Stunning due to increased O2 demand and decrease coronary perfusion pressure
   Still uncertainty in its relationship to TC 
   2.Nef HM, Mollmann H, Akashi YJ. Mechanisms of Stress (Takotsubo) cardiomyopathy. Cardiol 2010; 7: 187-193.

Microcirculation Dysfunction
—Either vasospasms or a decrease in blood flow through the coronary arteries without evidence of atherosclerosis
—Involvement of many vessels

—Majority of patients do have microvascular dysfunction at time of presentation

Dissipates after 3-5 days
TIMI frame counts were prolonged (TIMI flow was reduced in patients with ABS)
Vasospasms could be a result of increased stimulation at B1 receptors.
Some studies used a chemical to induce vasospasms, and found that < 30% of patients had vasospasms therefore not large vessel spasms
Provokable vasospasms ranged from 0-43% depending on the studies that you look at. This leads us to continue to question the significant contribution of microcirculation dysfunction to the development of ABS.
1.Antionopoulos A, Kyriacou C. Apical boallooning syndrome or Takotsubo cardiomyopathy: A new challenge in acute cardiac care. Cardiol J 2008; 15: 572-577.
2.Nef HM, Mollmann H, Akashi YJ. Mechanisms of Stress (Takotsubo) cardiomyopathy. Cardiol 2010; 7: 187-193.

Systemic Involvement

There are a number of possible complications in patients who present with takotsubo cardiomyopathy. Left-sided congestive heart failure with pulmonary edema, cardiogenic shock, ventricular fibrillation, left ventricular thrombus formation, and left ventricular free wall rupture are all possibilities. Death is included in this list; however, the mortality rate is only 0-8%, and generally less than 2%.

Medical Management (current best evidence)

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Physical Therapy Management (current best evidence)

Preferred Practice Pattern:

Pattern 6D: Impaired Aerobic Capacity/Endurance Associated With Cardiovascular Pump Dysfunction or Failure

Alternative/Holistic Management (current best evidence)

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Differential Diagnosis

The following are possible diagnoses that present similarly to TC:

• Esophageal spasm
• Gastroesophageal reflux disease
• Myocardial infarction
• Myocardial ischemia
• Myocarditis
• Acute pericarditis
• Pneumothorax
• Cardiogenic pulmonary edema
• Pulmonary embolism
• Unstable angina
• Acute Coronary Syndromes
• Angina Pectoris
• Aortic Dissection
• Boerhaave Syndrome
• Cardiac Tamponade
• Cardiogenic Shock
• Cardiomyopathy, Cocaine
• Cardiomyopathy, Dilated
• Cardiomyopathy, Hypertrophic
• Coronary Artery Vasospasm

Case Reports/ Case Studies

Obunai K, Misra D, Van Tosh A, Bergmann SR. Metabolic evidence of myocardial stunning in takotsubo cardiomyopathy: a positron emission tomography study. Jour Nuc Cardiol. 2005;12(6):742-4.

Resources

American Heart Association HeartHub for Patients

http://www.hearthub.org/

Recent Related Research (from Pubmed)

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References

see adding references tutorial.