Takotsubo Cardiomyopathy : Definition, Pathogenesis

Introduction

Takotsubo Cardiomyopathy is an acute reversible condition. Patients present with dyspnea, chest pain, palpitations, syncope attacks, acute heart failure.

In this disease, the left ventricular apex is dilated and hypokinetic. It resembles the Takotsubo, a Japanese octopus pot. Based on its morphology, it is also known as stress-induced cardiomyopathy. It is known as stress-induced cardiomyopathy based on its etiology. Other names include apical ballooning syndrome and broken heart syndrome. There is an Increased risk of rupture of the ballooned apex.

History of Takotsubo Cardiomyopathy

Takotsubo Cardiomyopathy was First diagnosed in a 64-year-old female. It presented with acute chest pain consistent with acute myocardial infarction. Typical ECG changes, like ST elevation, are seen in the disease. Normal coronary arteries on coronary angiogram are observed.

The left ventricle has an unusual appearance with a narrow neck and apical ballooning during systole. Abnormalities in the left ventriculography disappeared after 2 weeks.

Definition of Takotsubo cardiomyopathy

• It is an acute, transient left ventricular dysfunction.
  • Presents for 21 days.
  • Systolic dysfunction > diastolic dysfunction.
• There will be preceding emotional or stressful events.
• 2D echo - left ventricular regional wall motion abnormalities.
  • Present beyond a single epicardial coronary artery.
• Apex - Ballooned out and akinetic/hypokinetic.
• Hyperkinetic base.
• Other forms are localized within the base or mid-ventricular region.

Pathogenesis of Takotsubo cardiomyopathy

This pathology involves the brain-heart axis. Significant emotional or physical stress could also trigger neurological illness/circuit, thus inducing this cardiomyopathy.

Emotional stress can be a positive or negative event. 90% of events are negative events.

Classification of Takotsubo cardiomyopathy

Primary Takotsubo cardiomyopathy

Primary Takotsubo cardiomyopathy occurs with or without a trigger or stressful event. It is more common in postmenopausal women. The Common presentations are cardiac symptoms like Chest pain, palpitations, dyspnea, and syncope attack. Over 70% of the time, the attacks Precede emotional or physical triggers. One-fourth of patients present with no clear trigger.

Secondary Takotsubo cardiomyopathy

Secondary Takotsubo cardiomyopathy occurs in individuals with serious medical, surgical, or obstetric event.

Triggers for Takotsubo syndrome 

Clinical presentation of Takotsubo cardiomyopathy

The Most common presentation of Takotsubo cis chest pain (75%). This Chest pain is on excessive contraction, demand is more, and supply is less causing ischemia.

There is also Dyspnea (25-50%), Palpitations – increase in heart rate and Syncope attack (25-40%).

On examination there is Tachycardia, Hypotension and Bilateral basal crepitations,due to pulmonary edema.

Complications in Takotsubo Cardiomyopathy

1.2% of in-hospital patients develop rare complications.

• Irreversible cardiogenic shock – common in secondary TS.
• Left ventricular rupture.
• Thrombosis – Due to stasis of blood at apex.
• Embolization.
  • Thromboembolic events – Acute MI, acute cerebrovascular accidents.
• Ventricular tachycardia or ventricular fibrillation – Ballooned LV apex is arrhythmogenic. 
• Sudden cardiac death.
• Long QT interval can progress to Polymorphic ventricular tachycardia (Torsades de pointes).
• Complete heart block is very rare. 

Diagnosis of Takotsubo cardiomyopathy

ECG Changes in Takotsubo Cardiomyopathy

• Significant ST-T changes.
  • ST elevation - 43%.
  • ST depression 8%.
• T wave inversion.
• QTc prolongation > 500 msec.
  • Prolonged QT can lead to VT or VF.
• Polymorphic ventricular tachycardia increases risk for sudden cardiac death.
• ECG cannot differentiate apical ballooning of apex of Takotsubo cardiomyopathy and acute myocardial infarction.

Cardiac Biomarkers in Takotsubo Cardiomyopathy

• Elevated BNP and NT-proBNP.
• Elevated troponin I and troponin T.
• BNP and NT-proBNP increases 3-5 times compared to the rate of troponin increase.
• BNP and NT-proBNP are considered more reliable diagnostic biomarkers..

To read more about the Anatomical patterns of regional wall motion abnormality and Diagnostic criteria in Takotsubo cardiomyopathy, log in to the PrepLadder app and sign up to the SS medicine section.

Prognosis of Takotsubo Cardiomyopathy

There is overall good prognosis in Takotsubo cardiomyopathy. Within 21 days transient hypokinesia and akinesia will revert to normal.1.2% of hospitalized patients develop complications.

• Cardiogenic shock.
• Apical rupture.
• Arrhythmias.
• Thrombus formation.
• Sudden cardiac death secondary to PVT.

Also Read: Cardiac Resynchronization Therapy

Mortality in Takotsubo Cardiomyopathy

• In a large meta-analysis of 2120 patients out of 37 studies from 11 different countries, the in-hospital mortality of Takotsubo syndrome was 4.5% (95% CI, 3.1-6.2, 12 = 60.8%).
• In a recent study on trends in hospitalization of Takotsubo syndrome in USA, the in-hospital mortality was 1.1% in primary Takotsubo syndrome and 3.2% in secondary Takotsubo syndrome which was associated with higher incidence of cardiogenic shock, respiratory failure, and cardiac arrest.
• Mortality was higher in men compared to women in Takotsubo syndrome.

Management of Primary Takotsubo Cardiomyopathy

• No specific therapy.
• Recovery within 3-6 months.
• Supportive treatment to manage complications.

Management of Secondary Takotsubo Cardiomyopathy  

• Patients are admitted to ICU.
• Coronary angiograms cannot be performed due to critical illness.
• Treatment of underlying cause.
• Avoid catecholamine therapy e.g., dopamine, epinephrine.

Management of Complications in Takotsubo Cardiomyopathy 

Conclusion

• Takotsubo cardiomyopathy can be classified as primary or secondary depending upon the trigger and existing major medical, surgical, or psychiatric disease.
• Primary Takotsubo syndrome is diagnosed in 1-2% of patients admitted for acute coronary syndrome.
• Diagnosis based on Modified Mayo Clinic criteria or European Heart Failure Associated criteria.
• Primary Takotsubo syndrome commonly affects postmenopausal elderly women.
• Co-existing neurological or psychiatric illnesses are considered frequently in Takotsubo syndrome.
• Elevated NT-proBNP and ratio of NT-proBNP and troponin are indicative of Takotsubo syndrome.
• Echocardiography, coronary angiography, and cardiac MRI are useful tools in diagnosis of Takotsubo syndrome.
• Prognosis is generally good in 90% of patients.
• Risk stratification and proper management of life-endangering complications are essential steps in management.
• Treatment may include β-blockers, low molecular weight heparin, IABP, mechanical ventilation, LVAD and ECMO.
• In-hospital mortality varies from 1.1-4.5%.
• Takotsubo syndrome has a recurrence rate of 5-10% on 5-year follow-up.

Frequently Asked Questions:

Q: What is the Happy heart syndrome?

Answer: 10% of Takotsubo cardiomyopathy cases result from positive events, e.g., graduation. Thus, Takotsubo cardiomyopathy is also known as Happy Heart Syndrome.

Q: On what criteria the diagnosis of Takotsubo cardiomyopathy is made?

Answer: Diagnosis based on Modified Mayo Clinic criteria

Q: What is the most common presentation of Takotsubo cardiomyopathy?

Answer: The Most common presentation of Takotsubo cardiomyopathy is chest pain.

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