Expert Consensus on Takotsubo Syndrome, Part I

Ghadri JR, Wittstein IS, Prasad A, et al.
International Expert Consensus Document on Takotsubo Syndrome (Part I): Clinical Characteristics, Diagnostic Criteria, and Pathophysiology. Eur Heart J 2018;May 29:[Epub ahead of print].

The following are key points to remember from part I of this International Expert Consensus Document on Takotsubo Syndrome (TTS):

  1. TTS is a poorly recognized heart disease that was initially regarded as a benign condition. Recently, it has been shown that TTS may be associated with severe clinical complications including death, and that its prevalence is probably underestimated.
  2. TTS derived its name from the Japanese word for octopus trap, due to the shape of the left ventricle at the end of systole, and has been described under a remarkable number of different names in the literature including “broken heart syndrome,” “stress cardiomyopathy,” and “apical ballooning syndrome.”
  3. The most common symptoms of TTS are acute chest pain, dyspnea, or syncope and thus indistinguishable from acute myocardial infarction (AMI) at the first glance.
  4. TTS is estimated to represent approximately 1–3% of all and 5–6% of female patients presenting with suspected ST-segment elevation MI.
  5. The diagnosis of TTS is often challenging because its clinical phenotype may closely resemble AMI regarding electrocardiographic abnormalities and biomarkers. While a widely established noninvasive tool allowing a rapid and reliable diagnosis of TTS is currently lacking, coronary angiography with left ventriculography is considered the “gold standard” diagnostic tool to exclude or confirm TTS.
  6. The precise pathophysiological mechanisms of TTS are incompletely understood, but there is considerable evidence that sympathetic stimulation is central to its pathogenesis. An identifiable emotionally or physically triggering event precipitates the syndrome in most cases, and TTS has been associated with conditions of catecholamine excess (e.g., pheochromocytoma, central nervous system disorders) and activated specific cerebral regions.
  7. Physical triggers are more common than emotional stress factors. Of note, male patients are more often affected from a physical stressful event, while in women, an emotional trigger can be observed more frequently.
  8. Current evidence suggests that TTS is caused by an acute release of catecholamines from either sympathetic nerves, the adrenal medulla, or as drug therapy, and occurs primarily in subjects with increased susceptibility of the coronary microcirculation and of cardiac myocytes to the stress hormones leading to prolonged but transient left ventricular dysfunction with secondary myocardial inflammation.
  9. Although several anatomical TTS variants have been described, four major types can be differentiated based on the distribution of regional wall motion abnormalities. The most common TTS type and widely recognized form is the (i) apical ballooning type, also known as the typical TTS form, which occurs in the majority of cases. Over the past years, atypical TTS types have been increasingly recognized. These include the (ii) midventricular, (iii) basal, and (iv) focal wall motion patterns.
  10. A growing body of evidence reveals that acute cardiovascular events are not distributed randomly over time, but instead depend on the time of day, day of the week, and months/season of the year. Most conducted studies reported a summer preference for TTS, while one study reported a winter peak.

Clinical Topics: Acute Coronary Syndromes, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Prevention, ACS and Cardiac Biomarkers, Acute Heart Failure, Heart Failure and Cardiac Biomarkers, Interventions and ACS, Interventions and Imaging, Angiography, Nuclear Imaging, Stress

Keywords: Acute Coronary Syndrome, Biomarkers, Cardiomyopathies, Catecholamines, Central Nervous System Diseases, Chest Pain, Coronary Angiography, Dyspnea, Electrocardiography, Heart Failure, Inflammation, Microcirculation, Myocardial Infarction, Myocytes, Cardiac, Pheochromocytoma, Stress, Psychological, Syncope, Systole, Takotsubo Cardiomyopathy

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