The heart like a pot, that is, about the takotsubo syndrome

The symptoms of takotsubo syndrome resemble those of a heart attack, how are the two diseases different?
Prof. Marcin Grabowski: Takotsubo syndrome is very similar to myocardial infarction. Patient experiences chest pain, shortness of breath, and palpitations may occur. On the other hand, during the examination, it turns out that there are no changes in the coronary vessels. Or they are insignificant, minor changes. Left ventricular wall motion abnormalities appear. They also resemble a heart attack. In contrast, it is characteristic that in most patients with takotsubo syndrome, cardiac changes normalize quickly.

Does this mean the disease is less dangerous than a heart attack?
Monika Budnik, MD, PhD: On the surface it may seem so. However, the data show that the mortality in takotsubo syndrome may even be similar to a heart attack.
M.G.: Takotsubo syndrome can have a benign course, but it can also cause numerous complications. The most common is acute heart failure. Pulmonary edema, cardiogenic shock, cardiac arrhythmias may also occur and, in extreme situations, the left ventricular wall can rupture.

Takotsubo syndrome is a relatively new disease. When were the first cases described?
M.G.: The first description is from 1990. Its author is Japanese scientist Hikuro Sato from Hiroshima City Hospital.

And when were the first cases of this disease reported in Poland?
M.G.: Those were the years 2003-2004. It is worth noting that the first Polish descriptions of the disease came from our Department. In 2006, a case of a 57-year-old female patient with takotsubo syndrome during anesthesia was published. Then our Department, headed by Professor Grzegorz Opolski, participated in a large multicenter project on this disease. The findings were published in the top medical journal The New England Journal of Medicine (NEJM). As you can see, we have been interested in this topic for many years. However, not only our Department. Professor Agnieszka Cudnoch-Jędrzejewska from the Chair and  Department of Experimental and Clinical Physiology together with Dr Agnieszka Kołodzińska, Renata Główczyńska, MD, PhD, and Monika Budnik, MD, PhD from our Department conducted experiments on an animal model. The clinical part is run by Monika Budnik, MD, PhD, and Radosław Piątkowski, MD, PhD, in the Department of Cardiology, who have started a Polish registry of takotsubo disease patients, "Pol-tako".

Where did the idea to start such a registry come from?
M.B.: It started when our Department was invited to join a worldwide registry of people with takotsubo syndrome. Only two centers from Poland participate in it. This is not enough for us to check the prevalence of this disease in the Polish population. That is why we have taken the initiative to connect all patients in Poland and create a national registry under the auspices of the Heart Failure Association of the Polish Cardiac Society and the Association of  Cardiovascular Interventions of the PCS. We invited leading cardiology centers to participate. The earliest data from our Department are from 2006. Whereas, the registry itself launched in 2020. We collect data both retrospectively and prospectively.

How many people are on this registry today?
M.B.: In our Department we have 300 patients included in the registry. They undergo regular follow-up.

And what is the prevalence of takotsubo syndrome in the population?
M.B.: About 3 percent of patients with suspected myocardial infarction are thought to have takotsubo syndrome. However, we think that these figures are severely underestimated because the disease is often not recognized. To make a correct diagnosis, an echocardiogram should be performed within the first 24 hours of the onset of symptoms. And that doesn't always happen.

Who gets sick most often: older people, younger people, women, men?
M.B.: The youngest case of the disease described so far concerned a premature infant born at 28 weeks' gestation. The oldest was a 98-year-old woman hospitalized in our Department. Postmenopausal women are most commonly affected, accounting for 90 percent of all patients with the syndrome.

Why is it that postmenopausal women get sick most often?
M.B.: We don't know that exactly. It could be due to psychological factors, but it could also have to do with the termination of the protective effects of estrogen.

The disease we're talking about has a very interesting name, what does it come from?
M.B.: The word takotsubo means a Japanese vessel used for catching octopuses. It somewhat resembles a pot in that it has a narrow neck and wide bottom. The left ventricle looks similar in the acute phase of the disease. That's why the condition has been called takotsubo syndrome. But it also has two other names: broken heart disease and happy heart disease.

This nomenclature suggests that emotions play a large role in the development of takotsubo syndrome...
M.B.: Yes, and these can be both negative and positive emotions. Takotsubo syndrome can develop as a result of such traumatic experiences as the loss of a loved one, fear for one's health, interpersonal conflicts, involvement in an accident. But many clinical cases have also been described in which the cause was a strong positive experience, e.g., winning the lottery, a wedding, a surprise party. A slightly modified name for the disease has been proposed for these latter situations: "happy heart syndrome."

Is it stress hormones that harm the heart?
M.B.: Stress hormones certainly play a role here. What exactly is the mechanism - this is what we will want to clarify, among other things, by analyzing the registry data.

During the COVID-19 pandemic, we are all more vulnerable to stress. Does this translate into an increase in the number of patients with takotsubo syndrome?
M.G.: Recently there has been reports in the literature about the increase in cases of Takotsubo syndrome. This may be related to COVID-19. In the era of the pandemic, there have been unprecedented restrictions on social isolation. Schools and many jobs have been closed. This resulted in serious emotional consequences such as stress, fear, depression, insomnia, anger, irritability, frustration and boredom. These emotions remained with us even after the quarantine was lifted. An additional source of stress is the fear for the future compounded by constant and alarming media reports on the epidemiological situation. Thus, a possible increase in the incidence of takotsubo syndrome in the era of the COVID-19 pandemic may be related, on the one hand, to psychological, social, and economic factors accompanying the pandemic and, on the other hand, with the direct effect of the virus and the consequences of infection.

How is takotsubo syndrome treated?
M.G.: There are no specific recommendations. In takotsubo syndrome, we use medications such as those used in heart failure. Anticoagulant therapy is also required until contractile dysfunction subsides. In the first phase of the disease, i.e. within the first 48-72 hours, the patient requires hospitalization. After that, the treatment depends on how quickly left ventricular function returns.

And do relapses of this disease occur?
M.B.: Yes, it is estimated that about 5 percent of patients will have a recurrence or relapses. There was a patient in our Department who developed takotsubo syndrome as many as five times. She had her first recurrence after several years. Another one four years later. And another one every few months. Each time it happened after some stressful event, and each time her heart returned to normal.

Interviewed by Iwona Kołakowska, WUM