In my years practicing as a cardiac surgery PA, I have only come across two patients with Takotsubo cardiomyopathy (TTC). Perhaps large teaching centers have higher volumes of this type of patient. When I came across my most recent patient, I was reminded that it is a fascinating, if not terrifying, disease process. Here is a brief review of the epidemiology, causes and clinical presentation, and outcomes after diagnosis. I have also included a few articles I found useful or reference.
TTC derives its name from the Japanese word takotsubo, which means “octopus pot”. This is in reference to the balloon-like appearance of the left ventricular (LV) apex, which is a hallmark of the disease. This strange appearance is caused by akinesis of the apex, with preserved function at the base of the LV. While this type of TTC is the most common presentation, there are also subtypes that affect the midventricular, or basal region of the LV, as well as subtypes which effect only a focal region of the LV. The disease itself was first reported in Japan in the early 1990s. At that time, it was almost unheard of here in the United States. With increased discussion about the disease, the early 2000s brought an increase in diagnosis here, as well as in Europe. It has now been reported in 6 continents and in over 50 countries. In 2006, TTC was recognized by the American Heart Association as an acquired cardiomyopathy.
The most common population affected by TTC is older, often postmenopausal, women in the 65-70 age range. Even so, there have been cases seen in children as young as 2 years of age, as well as younger women in the perinatal period. Males are affected, but in much lower percentages. The interesting thing about this cardiomyopathy is that there is often an associated stressor that brings on symptoms. This is where the disease got one of its nicknames, broken heart syndrome. It can be precipitated by the death of a loved one, having to go through a stressful medical procedure, having a heated argument, or by natural disasters such as earthquakes. All of these situations involve high levels of circulating catecholamines, thus, allowing researchers to find a link between these physiologic chemicals, and TTC onset. Because of this finding, the medical community also initially referred to TTC as stress induced cardiomyopathy. But as information about this disease became more widespread, it was found that in a small percentage of patients there is no precipitating stressful event.
Initial presentation can vary, but onset is most often acute. It is frequently confused with acute coronary syndrome (ACS) in its presenting stage. Patients also have EKG changes that would indicate an ST elevation myocardial infarction, as well as a troponin leak. Because TTC cannot be distinguished from ACS, emergent coronary angiography is required, along with left ventriculogram imaging. Angiography will reveal clean, or nearly clean, coronaries. Left ventriculogram will reveal a decrease in ejection fraction, often to as low as 30 or 40%, as well as the regional wall motion abnormalities described above, and/or left ventricular outflow obstruction. The fact that the wall motion abnormalities go beyond a single coronary artery distribution is another way to distinguish TTC from ACS. Patients who are unstable from these symptoms may need vasopressor and inotropic support, preferably from non-catecholamine medications when possible, since symptoms can worsen when additional catecholamines are introduced. Some patients may even need intra-aortic balloon pump support.
The good news about TTC is that it is most often temporary. The majority of patients experience complete recovery of their left ventricular function. Arrhythmias and embolic events in the acute phase have been well documented and need to be monitored for. Anticoagulation until the heart recovers normal contractility should be considered. TTC is a complex and fascinating disease, and this summary has only touched on its clinical features. In the cardiac surgery world, we are often not on the front lines diagnosing TTC, but we may be taking care of the patients afterward. The idea that emotional trauma can cause such physical damage to the body, is a frightening one. That triggered my interest in learning more about TTC. For any readers who have also had their interest sparked, I have included a few interesting papers.
“Epidemiology and Clinical Profile of Takotsubo Cardiomyopathy”, published in Circulation Journal (Focus Issue of Takotsubo Cardiomyopathy), Official Journal of the Japanese Circulation Society, Vol. 78, September 2014, p.2119-2128
“Clinical Features and Outcomes of Takotsubo (Stress) Cardiomyopathy”, published in the New England Journal of Medicine 373;10, September 2015, p.929-938
“Diagnosis of Takotsubo Cardiomyopathy”, published in Circulation Journal (Focus Issue of Takotsubo Cardiomyopathy), Official Journal of the Japanese Circulation Society, Vol. 78, September 2014, p.2129-2139