Broken Heart Syndrome or Takotsubo Cardiomyopathy

Is a sudden temporary weakening of the muscular portion of the heart (Myocardium) triggered by physical or emotional stress such as;  a break-up, the death of a loved one, constant anxiety etc.

Also known as "transient apical ballooning syndrome", "apical ballooning cardiomyopathy", "stress-induced cardiomyopathy", "Gebrochenes-Herz-Syndrome", and "stress cardiomyopathy" "

Broken heart syndrome is a recently recognized heart problem. It was first described in Japan 1990, by Sato  a cardiovascular specialist.
The hallmark bulging out of the apex of the heart with preserved function of the base earned the syndrome its name "tako tsubo", meaning "Octopus trap" in Japan.

It is very Common among the Asian or Caucasian; 57.2% in Asian, 40% in Caucasian, and 2.8% in other races.

Literature reviews report a mean patient age of 67 years, although cases of TCM have occurred in children and young adults.
It is commoner in women than in men, Nearly 90% of reported cases involve postmenopausal women.
Studies reported that 1.7-2.2% of patients who had suspected acute coronary syndrome were subsequently diagnosed with takotsubo cardiomyopathy (TCM).

Signs and Symptoms
Similar symptoms to patients with a heart attack including;

• sudden, intense chest pain
• shortness of breath,
• sometime palpitations, nausea, vomiting, syncope, and rarely, cardiogenic shock have been reported.
• low blood pressure
• congestive heart failure (sudden Onset).

One of the more unique features of TCM is its association with a preceding emotionally or physically stressful trigger event, occurring in approximately two thirds of patients. Unlike acute coronary syndrome, in which peak occurrence is during the morning hours, TCM events are most prevalent in the afternoon, when stressful triggers are more likely to take place.

Risk Factors
Stressors include;

• Break up or physical separation
• divorce,
• betrayal or romantic rejection.
• arguing with a spouse
• death of a loved one
• fear from public speaking
• bad financial news
• legal problems
• natural disasters
• motor vehicle collisions
• exacerbation of a chronic medical illness,(Acute asthma, stroke etc)
• newly diagnosed, significant medical condition
• surgery
• an intensive care unit stay
• the use of or withdrawal from illicit drugs.
• chemotherapy
• It has also been reported after near-drowning episodes
• It could even happen after a good shock (like winning the lottery or a surprise party)

Hormonal;
Lack of estrogen as seen in postmenopausal women

Genetic factors;
Susceptibility to TCM in individuals may be partially related to genetic factors; L41Q  polymorphism of G protein coupled receptor kinase (GRK5) which responds to catecholamine stimulation and attenuates the response of beta-adrenergic receptors

Following a stressful event, it is believed that in Patients with broken Heart syndrome there is Increase sympathetic drive ("fight or flight" mechanism) as a result of cathecholamine mediated cardiotoxicity, which results in multi-vessle epicardial coronary artery spasm, microvascular dysfunction or spontaneous aborted myocardiac infaction.

Diagnosis

• An electrocardiogram (ST-segment elevation and T-wave inversion)
• Cardiac enzymes (troponin I and T, are elevated in 90% of patients)
• An echocardiogram (hypokinesis or akinesis of the midsegment and apical segment of the left ventricle),
• An angiogram (completely normal coronary arteries)
• Chest radiographs are often normal, but they may demonstrate pulmonary edema.
• Cardiac magnetic resonance imaging may be a diagnostic modality uniquely suited for establishing the diagnosis of TCM by accurately visualizing regional wall motion abnormalities, quantifying ventricular function, and identifying reversible injury to the myocardium by the presence of edema/inflammation and the absence of necrosis/fibrosis.

Mayo Clinic criteria for diagnosis of TCM can be applied to a patient at the time of presentation and must contain all 4 aspects:

1. Transient hypokinesis, dyskinesis, or akinesis of the left ventricular mid segments, with or without apical involvement; the regional wall-motion abnormalities extend beyond a single epicardial vascular distribution, and a stressful trigger is often, but not always, present 
2. Absence of obstructive coronary disease or angiographic evidence of acute plaque rupture.
3. New electrocardiographic abnormalities (either ST-segment elevation and/or T-wave inversion) or modest elevation in cardiac troponin level
4. Absence of pheochromocytoma or myocarditis

Differential Diagnosis
    Acute Coronary Syndromes
    Angina Pectoris
    Aortic Dissection
    Boerhaave Syndrome
    Cardiac Tamponade
    Cardiogenic Shock
    Cardiomyopathy, Cocaine
    Cardiomyopathy, Dilated
    Cardiomyopathy, Hypertrophic
    Coronary Artery Vasospasm

Complications
Pulmonary edema.

Hypotension.
Arrhythmias (irregular heartbeats).
Treatment
The treatment of takotsubo cardiomyopathy is generally supportive in nature.
Standard therapies, such as the following, may be indicated:
    Aspirin
    Beta blockers
    Nitrates
    Heparin or enoxaparin
    Platelet glycogen (GP) IIb/IIIa inhibitors
    Morphine
    Clopidogrel

Patients in acute congestive heart failure may require diuresis, and patients with cardiogenic shock may require resuscitation with intravenous fluids and inotropic agents.

The insertion of an intra-aortic balloon pump has also been reported as being a successful resuscitative intervention, due to left ventricular outflow obstruction that can result from a hyperkinetic basal segment and dyskinetic apex. Fluids and beta blockers, or calcium channel blockers, are beneficial in this situation, whereas inotropes may exacerbate the problem and should be used with caution.

Dysrhythmias and cardiopulmonary arrest should be treated using current advanced cardiac life support (ACLS) protocols. Although thrombolytics will not benefit patients with takotsubo cardiomyopathy (TCM), their use should not be withheld when percutaneous coronary intervention (PCI) is not available and patients otherwise meet criteria.

While medical treatments are important to address the acute symptoms of takotsubo cardiomyopathy, further treatment includes lifestyle changes. It is important that the individual stay physically healthy, while learning and maintaining methods to manage stress, and cope with future difficult situations.

Prevention
There's a small chance that broken heart syndrome can happen again after a first episode.
There's no proven therapy to prevent additional episodes; however, many doctors recommend long-term treatment with beta blockers or similar medications that block the potentially damaging effects of stress hormones on the heart. Recognizing and managing stress in your life also is very important.




NOTES

1. Bergman BR, Reynolds HR, Skolnick AH, Castillo D (August 2008). "A case of apical ballooning cardiomyopathy associated with duloxetine". Ann. Intern. Med. 149 (3): 218–9

2. Eshtehardi P, Koestner SC, Adorjan P, Windecker S, Meier B, Hess OM, Wahl A, Cook S (July 2009). "Transient apical ballooning syndrome--clinical characteristics, ballooning pattern, and long-term follow-up in a Swiss population". Int. J. Cardiol. 135 (3): 370–5.

3. Gianni, M, Dentali F et al. (December 2006). "Apical ballooning syndrome or takotsubo cardiomyopathy: a systematic review". European Heart Journal (Oxford University Press) 27 (13): 1523–1529.

4. Kawai S, Kitabatake A, Tomoike H. Guidelines for diagnosis of takotsubo (ampulla) cardiomyopathy. Circ J. Jun 2007;71(6):990-2

5. Medscape | article/1513631

6. Mayo Clinic | broken heart syndrome basics

7. Sato H, Tateishi H, Uchida T, et al. Kodama K, Haze K, Hon M, eds. Clinical Aspect of Myocardial Injury: From Ischaemia to Heart Failure. Tokyo: Kagakuhyouronsya; 1990:56-64.

8. Sharkey SW, Lesser JR, Garberich RF, Pink VR, Maron MS, Maron BJ. Comparison of Circadian Rhythm Patterns in Tako-tsubo Cardiomyopathy Versus ST-Segment Elevation Myocardial Infarction. Am J Cardiol. May 29 2012

9. Wikipedia | Takotsubo cardiomyopathy

10. World Journal Of Cardiology | Takotsubo cardiomyopathy: Pathophysiology, diagnosis and treatment.

11. Zamir, M (2005). The Physics of Coronary Blood Flow. Springer Science and Business Media. p. 387. ISBN 978-0387-25297-1.