Initially, takotsubo cardiomyopathy resembles a myocardial infarction: there is chest pain and dyspnoea.
The symptoms usually begin after an episode of severe emotional or physical stress. In about 1 out of 4 patients, no clear triggering factor can be identified.
The patients are typically ≥ 60-year-old women.
They should be hospitalized.
Initial treatment should be given according to the guidelines for myocardial infarction because the two are difficult to distinguish.
Once the diagnosis of takotsubo cardiomyopathy has been confirmed, treatment is similar to that of cardiac failure.
Most patients recover completely within about a month but slightly less than 10% have complications, i.e. die or have a cerebral infarction.
Prevalence
Of all patients with symptoms of myocardial infarction, about 2% have takotsubo cardiomyopathy;
90% of these are women.
In Finland (population 5.5 million), about 350 new cases of takotsubo cardiomyopathy are diagnosed per year.
Aetiology and pathophysiology
The exact aetiology of takotsubo cardiomyopathy is unknown.
Acute stress or excessive physical exertion is believed to activate the central and autonomic nervous systems. This elevates cortisol and catecholamine levels in the body.
The myocardium is damaged by the direct toxic effect of the catecholamines and, on the other hand, by signals mediated by adrenergic receptors.
Microvascular spasm is believed to be involved.
The patient typically develops acute akinesis in the apex of the left ventricle, producing a shape resembling a squid trap (tako tsubo in Japanese). This results in acute cardiac failure.
The akinesis leads to insufficient pumping, predisposing to formation of a blood clot in the apex of the heart, which may later lead to cerebral infarction.
Symptoms
The symptoms resemble myocardial infarction.
Three in four patients have chest pain and one in two suffer from dyspnoea.
Syncope occurs in a small share of patients.
Patients may be asymptomatic at first.
Diagnosis
Diagnostic criteria
As takotsubo cardiomyopathy cannot be reliably distinguished from myocardial infarction, it must be treated like myocardial infarction.
The patients are usually postmenopausal women lacking the typical risk factors of coronary artery disease.
ECG changes (ST elevation, ST depression, T inversion) resemble those seen in myocardial infarction or, sometimes, myocarditis.
In 2 cases out of 3, the ECG resembles that seen in ST elevation MI. T inversions can be seen in 2 of 3 cases and Q waves in 1 out of 3 cases.
Extensive ST changes may resemble those seen in myocarditis.
In some cases, ECG changes may be minor.
Troponin concentration is similarly increased as in myocardial infarction.
Echocardiography may reveal typical akinesis in the apical area but this may be difficult to distinguish from myocardial infarction.
Confirming the diagnosis
Contrast study of the coronary arteries and the left ventricle is needed to confirm the diagnosis.
This usually confirms the diagnosis
Constrictive coronary artery disease does not rule out takotsubo cardiomyopathy, since it can be found in about 1 out of 4 patients with takotsubo cardiomyopathy.
In some cases, the diagnosis can only be confirmed after cardiac MRI.
Treatment
Initial treatment
Patients should be hospitalized.
The symptoms resemble those of myocardial infarction - initial treatment should be chosen based on symptoms and ECG findings.
If the symptoms resemble non-ST-elevation myocardial infarction, treatment should be chosen accordingly (ASA, LMWH and haemodynamic stabilization).
If ECG shows an ST elevation MI, proceed according to the relevant guidelines (ASA, LMWH, and, according to local guidelines, ADP receptor blocker, haemodynamic stabilization and immediate referral to a unit performing emergency angiography).
Drug treatment after the initial phase
Once the diagnosis of takotsubo cardiomyopathy has been confirmed, treatment is similar to that of cardiac failure.
LMWH should be continued at least until the end of hospital treatment, preferably for at least 10 days.
If there are signs of a blood clot at the apex of the heart or if the ventricular apex is severely akinetic, longer-term anticoagulant therapy (warfarin/DOAC) should be started.
MRI may be useful for making the decision.
Follow-up
An appointment should be made for a check-up 4 to 6 weeks after the attack to confirm by echocardiography that cardiac failure can no longer be seen.
If this is so, the beta-blocker, ACE inhibitor and any anticoagulant therapy can be withdrawn.
References
Templin C, Ghadri JR, Diekmann J et al. Clinical Features and Outcomes of Takotsubo (Stress) Cardiomyopathy. N Engl J Med 2015;373(10):929-38. [PubMed]
Scally C, Rudd A, Mezincescu A et al. Persistent Long-Term Structural, Functional, and Metabolic Changes After Stress-Induced (Takotsubo) Cardiomyopathy. Circulation 2018;137(10):1039-1048. [PubMed]
Lyon AR, Citro R, Schneider B ym. Pathophysiology of Takotsubo Syndrome: JACC State-of-the-Art Review. J Am Coll Cardiol 2021;77(7):902-921. [PubMed]