Takotsubo Syndrome (Broken Heart Syndrome)

A patient arrives with chest pain and breathlessness. Their electrocardiogram (ECG) is abnormal and their troponin is raised. Myocardial infarction has to be considered, because Takotsubo syndrome can look very similar during the first few hours.

The difference begins to emerge when the coronary arteries and the movement of the left ventricle are examined. In myocardial infarction, an acutely blocked coronary artery causes ischaemia and injury in the myocardium supplied by that vessel. In Takotsubo syndrome, part of the ventricular muscle suddenly becomes severely hypokinetic or akinetic, meaning it contracts very little or not at all, but the pattern usually extends beyond the territory supplied by a single coronary artery.

This is a real episode of acute cardiac dysfunction. The term broken heart syndrome is widely recognised because emotional stress can be a trigger, but it can be misleading. Takotsubo can follow physical illness as well as emotional distress, and sometimes there is no obvious trigger.

What happens to the left ventricle?

Think about what the left ventricle normally does during systole. The myocardium contracts and the ventricular cavity becomes smaller as blood is ejected into the aorta. In the classic form of Takotsubo syndrome, the basal segments continue to contract while the mid and apical segments contract poorly. The base therefore narrows during systole while the apex remains relatively expanded. This produces the characteristic apical ballooning appearance.

The name takotsubo comes from the resemblance of this ventricular shape to a Japanese pot traditionally used to trap octopuses. Apical ballooning is the best-known pattern, but it is not the only one. Mid-ventricular, basal and focal patterns also occur.

The sudden loss of normal contraction reduces the amount of blood the ventricle can eject. Depending on how much myocardium is affected, the patient may have only modest impairment or develop acute heart failure and haemodynamic instability.

Exactly why the myocardium behaves this way is still being investigated. Excess catecholamine activity and an abnormal myocardial response to stress hormones are strongly implicated, and changes in the coronary microcirculation may also contribute. Current evidence does not support reducing Takotsubo to one simple mechanism.

Why does it look so much like a myocardial infarction?

Chest pain and breathlessness are common in both conditions. The ECG in Takotsubo may show ST-segment elevation, T-wave inversion or QT interval prolongation, and troponin can rise because the myocardium has been injured. None of those findings can safely distinguish Takotsubo from an acute coronary syndrome by themselves.

That is why the initial assessment takes possible myocardial infarction seriously. Depending on the presentation, coronary angiography may be required to look for an acute culprit coronary lesion. Echocardiography shows how the ventricle is moving and may reveal a wall-motion pattern that extends beyond one coronary territory. Cardiac magnetic resonance imaging can provide further information when myocarditis or another cause of myocardial injury remains possible.

Coronary artery disease can also coexist with Takotsubo syndrome. Finding coronary disease does not automatically mean that it explains the ventricular dysfunction. The coronary anatomy and the pattern of myocardial contraction need to make sense together.

Bleepbook's Myocardial Infarction: What Actually Happens During a Heart Attack? follows what happens when coronary blood flow is acutely interrupted.

Stress does not always mean emotional stress

Takotsubo is strongly associated with stress, but the word needs some explanation. Bereavement, fear and other intense emotional experiences can precede an episode. So can major physical stress such as surgery, severe infection, acute respiratory illness or neurological disease. Some people cannot identify a trigger at all.

Most recognised cases occur in postmenopausal women, although Takotsubo can affect men and younger people too. A recent stressful event should never be used to dismiss cardiac symptoms as anxiety or an emotional reaction. A person who has recently been bereaved can still have a myocardial infarction, and someone with Takotsubo has genuine ventricular dysfunction that may cause serious complications.

What can happen while the ventricle is recovering?

Takotsubo is usually reversible, but the acute phase can make a patient very unwell. If a substantial part of the left ventricle is barely contracting, stroke volume falls. Blood and pressure can then build behind the left ventricle into the pulmonary circulation, causing breathlessness, pulmonary congestion or pulmonary oedema. A severe fall in cardiac output can lead to cardiogenic shock.

Some patients develop left ventricular outflow tract obstruction. This can happen when the basal myocardium contracts strongly while other ventricular segments are impaired, changing the shape and haemodynamics of the ventricle during systole. Identifying this on echocardiography is important because it affects the way circulatory instability is managed.

There can be electrical complications too. QT prolongation and ventricular arrhythmias may occur. Severe apical akinesia can also allow blood to remain relatively static in the ventricular apex, creating conditions in which a thrombus can form. If part of that thrombus embolises into the systemic circulation, stroke is one possible consequence.

At the bedside, improvement in chest pain does not necessarily mean ventricular function has recovered. Increasing breathlessness, a new oxygen requirement, falling blood pressure, cool peripheries, reduced urine output, palpitations, syncope or a change in cardiac rhythm all need attention because they may indicate that the ventricular dysfunction is affecting the circulation.

Treatment depends on what the heart is doing

There is no single medicine that reverses Takotsubo syndrome. During the initial presentation, care is shaped by the possibility of acute coronary syndrome until the diagnosis becomes clearer. Once Takotsubo is established, treatment is guided by the patient's ventricular function, haemodynamics and any complications.

A patient with pulmonary congestion may require diuretic treatment. Significant systolic dysfunction may be managed with appropriate heart failure therapies while the ventricle recovers, although the evidence for specific long-term drug treatment in Takotsubo remains limited. Cardiogenic shock, ventricular arrhythmias and left ventricular outflow tract obstruction require specialist management because the treatment has to match the underlying haemodynamics. Anticoagulation may be considered when a ventricular thrombus is present or when severe ventricular akinesia creates substantial thromboembolic risk.

For many patients, left ventricular systolic function improves over days to weeks. Follow-up imaging can demonstrate that recovery. Recurrence is possible, however, and recovery of the ejection fraction does not mean that every symptom disappears immediately. Some people continue to experience fatigue, breathlessness, chest discomfort or reduced exercise tolerance after the acute episode.

The way the diagnosis is explained to the patient is important. Calling it “just stress” suggests that nothing physical happened to the heart. A more accurate explanation is that part of the heart muscle temporarily stopped contracting normally. The trigger may have been emotional, physical or unclear, but the cardiac dysfunction itself was real and required assessment, treatment and follow-up.

References

Ghadri, J-R. et al. (2018) International Expert Consensus Document on Takotsubo Syndrome (Part I): Clinical Characteristics, Diagnostic Criteria, and Pathophysiology. European Heart Journal, 39(22), pp. 2032–2046.

Ghadri, J-R. et al. (2018) International Expert Consensus Document on Takotsubo Syndrome (Part II): Diagnostic Workup, Outcome, and Management. European Heart Journal, 39(22), pp. 2047–2062.

Citro, R. et al. (2020) Multimodality imaging in takotsubo syndrome: a joint consensus document of the European Association of Cardiovascular Imaging and the Japanese Society of Echocardiography. European Heart Journal – Cardiovascular Imaging, 21(11), pp. 1184–1207.

Lyon, A.R. et al. (2016) Current state of knowledge on Takotsubo syndrome: a position statement from the Taskforce on Takotsubo Syndrome of the Heart Failure Association of the European Society of Cardiology. European Journal of Heart Failure, 18(1), pp. 8–27.

British Heart Foundation Takotsubo cardiomyopathy.