In the middle of his wedding speech to his daughter and son-in-law, a middle-aged man experienced chest pain and difficulty to breathe. A heart attack, was the first thought of the people at the wedding. The man was rushed to the nearest ER. Some minor changes in the electrocardiogram (ECG) were seen and a small elevation in blood tests indicating some type of heart muscle damage. Unexpectedly angiography, an x-ray in which a contrast media is injected into the coronary arteries in order to visualize them and evaluate their condition, showed all of the coronaries to be open. So a cardiac stroke was not the cause of the man’s symptoms. However, the tip of the left heart ventricle was paralyzed and the muscle nearer to the base of the ventricle was hyperactive causing ballooning of the tip of the heart ventricle. It seemed like the area at the base was desperately trying to get the left ventricle of the heart to pump oxygenated blood into the circulation.
The malfunctioning heart was like a totally through soaked limp wet sock. Have you have ever gotten your socked feet totally wet? If so, you probably also remember that being out of a spare pair of socks you had to use quite a lot of grip force to squeeze and wring the socks to get all the water out of them before pulling them on your feet again. With this experience in mind you get quite a good idea about what is happening with the heart in a medical condition called Takotsubo (stress) cardiomyopathy. The heart is a heavy limp lump soaked with fluid. In an X-ray the shape of the heart is similar to that of a round-bottomed, narrow-necked vessel – tako-tsubo – Japanese fishermen use to trap octopuses. Hence the name Takotsubo. The cardiac disorder is also known as the “apical ballooning syndrome”.
This acutely starting dysfunction of the heart mimicking an ischemic heart attack was first described in Japan in 1990. First it was considered to be a rare condition triggered by a strong emotional state the patient experienced just prior to the attack. The middle-aged man above told the doctors that he had been anxiously waiting all through the wedding dinner for the time point at which he would give his speech. He wasn’t into speaking publicly. So the situation was even somewhat frightening for him. And to top it all he was also extremely happy that the newly-weds had finally tied the knot and at the same time worried that he soon stammers and mixes words; forgets what he had planned to say. All through dinner his mind had been full of questions like: will people think the speech boring, will anybody laugh at the preplanned jokes, will his daughter and wife like the speech? So this poor man was in the clutches of a turmoil of positive and negative emotions. His heart couldn’t take it. Today Takotsubo cardiomyopathy is also know as the broken-heart syndrome.
In medicine case reports are published on clinical rarities. This is how it all started with the Takotsubo syndrome. Case reports in medical research articles also alert clinical doctors to new possible causes underlying their patients’ symptoms. Since the early 1990’s medical data has steadily accumulated on Takotsubo. We now know that this acute stress cardiomyopathy is not that rare. In ERs and Cardiology Units patient cases are seen on a weekly basis. Doctors nowadays also know how to look for them.
In 2005 I was unnecessarily, during a small medical procedure, treated for too low blood pressure. Unnecessarily because the blood pressure measuring device was out of order. When this was discovered I had, due to fast hydration and blood elevating drugs, a blood pressure (BP) of 250/170! Soon after this my lungs filled with fluid and I felt like suffocating. A knowledgeable doctor in the ICU performed a heart ultrasound on me and diagnosed Takotsubo. I remember saying to the doctors that the root cause of Takotsubo in my case had to be the severe physiological stress caused by the unnecessary treatment of low (BP) that resulted in a hypertensive crisis. We had this discussion because at the time Takotsubo was linked to severe emotional stress. (Of course after the BP crisis I was also emotionally stressed!)
Today, 25 years after the first case report from Japan, we know that in only one third of the patients the attack can be linked to an emotional stressor such as grief, a panic attack or a big quarrel. In another third of patients Takotsubo is triggered by extreme physical strain, a medical procedure like an operation or treatment in an intensive care unit. In one third of patients no underlying cause can be found. A recent large international study (reference at the end of this article) on stress cardiomyopathy reports that Takotsubo is more common in patients with an underlying neurologic or psychiatric disease.
The current train of thought based on a lot of medical research on the underlying etiology of Takotsubo is that it is triggered by abnormal activity of the sympathetic part of the autonomous nervous system. Also the autonomic and central nervous system are in constant interaction. So the functions of the heart, the blood circulation, bodily reactions to emotions and mental states are all intertwined together and several different neuro-hormonal pathways are in play in a complex way. Luckily in most cases (I’m one of the lucky), patients recover fully from the acute temporary loss of cardiac (most probably) neural based dysfunction.
The first stress cardiomyopathy cases I happened to learn about from my medical colleagues were men. The middle-aged man at his daughter’s wedding and an older man (retired taxi-driver) who was involved in a three-car-collision accident. In this heated situation, in the middle of the traffic jam, a young man shouted at the older man accusing him of being a lousy old driver. The old man felt totally humiliated and wrongly accused and immediately experienced crushing pain in his chest due to Takotsuba. Currently cumulative patient data on stress cardiomyopathy, however, indicates that a “typical” Takotsubo –patient is a female in her later middle-age. So the possible beneficial effect of estrogen for preventing stress cardiomyopathy is currently being studied.
We may not yet have the whole picture of Takotsubo. In my opinion, milder cases of Takotsubo-like cardiac distress triggered by harmful stress, may not seek medical attention at ERs. They recover spontaneously after experiencing some chest discomfort and shortness of breath. Another interesting and important medical question has to do with the role of harmful stress in the development of progressing cardiomyopathy, a medical condition in which the heart muscle gradually degenerates loosing its pumping power. The development of cardiomyopathy is also seen in extreme sports. Stress during physical activity can also result in abnormal activity of the sympathetic nervous system. Every now and then we read about professional athletes experiencing heart arrhythmias.
Matters of the heart matter in all areas of human life. Some time ago, during a tram ride in Helsinki, I heard two, about ten-year old, girls talking. “The brain is the most important organ in our body”, one of the girls said. “You cannot do without the heart”, said the other. For human health the heart-brain axis is in the core of many things. How to be heart smart? You need to use your brain and listen to your heart.
Eitel I. et al. Clinical characteristics and cardiovascular magnetic resonance findings in stress (Takotsubo) cardiomyopathy. JAMA 2011:306:277-86
Templin C et al. Clinical features and outcomes of Takotsubo (Stress) Cardiomyopathy. N Engl J Med 2015:373:929-38