We’ve probably all heard of a story like this. One half of a long-term married couple dies. Shortly thereafter, the spouse, who was apparently healthy, is found dead. Family and friends are shocked. Some say the surviving spouse just couldn’t live without their partner of 50+ years. And they might just be right. I’m here to tell you that you can quite literally die of a broken heart.
The syndrome was first discovered in Japan in the 1990s. Some cardiologists reported finding five patients who presented with symptoms very much like heart attacks: chest pain, shortness of breath and other physical symptoms. Most had EKG changes suggesting that they had a heart attack. But the biochemical tests that can indicate a heart attack, instead of being significantly elevated as they often are in cases of heart attack, were only increased a little above normal.
When the cardiologists did coronary angiograms (similar to a cardiac catheterization), injecting dye into their coronary arteries, these patients had normal coronary arteries – no excessive plaque causing blockages. So no evidence on a heart attack on imaging either.
The doctors went further and injected dye into the atria and ventricles of the heart. This time there was an abnormality and a major one (look at the photos below to see what the ventricle should look like, as compared to the video):
Compare the video with this:
The left ventricle (the major pumping chamber of the heart) was bulging abnormally, and not contracting as strongly as it should. In some cases it was only pumping out about 1/3 of the blood that it should be ejecting from the heart.
Some of these patients needed external cardiac support for brief periods because of this inefficient pumping. Because the characteristic bulging of the ventricle resembled the fishing pot that is used in Japan to trap octopi, this cardiomyopathy (heart muscle disease) was named tako-tsubo (octopus pot) cardiomyopathy.
Interestingly, most cases (~85%) of takotsubo cardiomyopathy occur in women, often older women from post-menopause age upwards. Though much younger patients of both sexes have developed it. It can be severe enough to cause death from shock or other causes. But if recognized early, and treated appropriately, patients survive and the ability of their ventricles to pump normally usually returns over a period of several days.
At first, it was thought that takotsubo was quite rare and might be limited to just Japan. But since the initial report, many more cases have been found in several countries in Europe, the United States, South America and elsewhere.
Takotsubo cardiomyopathy can be triggered by several things: e.g., a severe emotional/psychological shock (death of a family member, financial ruin, being in intensive care, etc.), or physical trauma (like choking). But in about one-third of the cases, there is no known emotional or physical trauma that precipitates it. The mechanism of how it happens may have to do with spasm of the coronary arteries. Or perhaps, the very tiny vessels in the heart muscle are part of the problem. It may be due, in part, to the arterial supply to the left ventricle or other causes or a combination of the above.
Initially, since the first patients were all women who were post-menopausal, it was thought that it was due to decreased estrogen in women in that age group. But as younger women and men developed it, this theory was discarded.
So the cause and mechanism are unclear. But it is indeed a syndrome that causes a broken heart, and one that can be deadly.