Can someone literally be scared to death or die of a broken heart?
"You can, and this has been described in biblical literature, and it's something that's been passed on -- 'died of a broken heart' -- these kinds of things," said Hunter Champion, a heart failure cardiologist and director of the pulmonary vascular disease program at UPMC.
Dr. Champion is a longtime researcher of the cardiac condition properly known as stress cardiomyopathy but commonly called broken heart syndrome because of the emotional events that apparently trigger it.
Srinivas Murali, director of the division of cardiovascular medicine, West Penn Allegheny Health System, knows of similar cases. He said, "You can also suddenly be frightened to death.
"This is a sudden weakening of the heart muscle, which is triggered by a sudden, unexpected mental stress, so for example if there is a death of a loved one, a spouse or a child."
He said WPAHS typically sees two to three cases a year.
Dr. Champion, who has seen 200 to 250 cases over his career, believes the syndrome "is much more common than we think."
Physical events such as a head injury or stroke apparently also can spark it, researchers say.
The condition strikes women much more often than men, and most of those women are beyond menopause.
Besides the sudden death of a loved one, Dr. Champion has seen the condition follow such events as a family argument, armed robbery, fear of giving a public speech, fear during a medical procedure and, his favorite case: "A woman went to a surprise birthday and truly was surprised."
Symptoms mimic those of a heart attack -- chest pain, shortness of breath, arm or facial numbness -- but there are a couple of big differences between the two conditions besides the emotional stress that precedes stress cardiomyopathy: Heart muscle dies during a heart attack, but in broken heart syndrome there usually is complete recovery if proper treatment is undertaken immediately. Also in stress cardiomyopathy, the patient has much higher levels of the stress hormones, including adrenaline, or epinephrine, and norepinephrine, in the bloodstream.
Dr. Champion was senior author of a New England Journal of Medicine-published study in 2005 that pointed out the direct link between the body's response to emotional stress and the syndrome. Nineteen patients who sought treatment for symptoms of heart attacks after a shock were compared to a smaller group who had heart attacks without preceding emotional events.
The 19 experiencing shocks had "levels of adrenaline far higher than even the worst of heart attacks," he said.
The study also showed that the physical results of the shocks, left ventricular dysfunction, were present on hospital admission but quickly went away over the next few days.
Stress cardiomyopathy also is sometimes called stress-induced cardiomyopathy, stunning, stunned myocardium, takotsubo or takotsubo-like cardiomyopathy, or apical ballooning syndrome. The last terms come from the appearance of the heart when the condition strikes (a takotsubo is a Japanese octopus pot that balloons out toward the top), although Dr. Champion said his research has shown the heart does not actually balloon out. Rather he said it simply looks that way because the top of the heart beats normally while the end of it doesn't.
"There are three theories of stress cardiomyopathy," said Martin Samuels, a neurologist and Harvard Medical School professor who studies links between the nervous system and heart function. He recently spoke about his research into stress cardiomyopathy at the scientific meeting of the Heart Failure Society of America in Boston, then summarized his presentation in an interview.
The three, he said, are stress hormones toxicity, spasm of the coronary arteries, and myocarditis or inflammation of the heart.
"All are correct," he said, "but they are all connected by the fact that the cascade of events is triggered from the central nervous system. These events occur when there is a stress or a brain disease like a seizure, head injury or stroke. The brain causes the surge in the stress hormones."
Spasm of the arteries may occur but isn't the central cause, and the heart inflammation is caused by the stress hormones, he said.
"I emphasized to [the society] that the nervous system was capable of causing malfunction of the heart and an actual structural change in the heart, and it could cause sudden cardiac death."
Dr. Champion said he believes the cause "is more likely a direct toxic effect of the adrenaline on the heart muscle."
Dr. Murali said, "Adrenaline alone is not the explanation here because the level goes up during physical stress, too, and people generally don't develop takotsubo during physical stress."
Dr. Champion said, however, that there are physical triggers.
"We see a lot of different things. Most common are anything involving the head and neck." They include head and neck tumors, insertion of a breathing tube during surgery, blood clots in the lungs, blows to the head, and either typical or atypical migraine.
Asked how those physical events might trigger the syndrome, he noted that a lot of nerves go from the brain into the lower part of the body. "[Anything that can impinge] on a nerve can change the neural outflow of the body."
The UPMC researcher believes genetics are involved.
"My personal opinion: Our patients we see are somehow genetically predisposed, and then there is something that then makes it [happen] because this isn't the first time they've had something emotional happen to them."
He is actively genotyping patients for a research study on the question.
The subject of still another study by Dr. Champion and his colleagues is why women, particularly postmenopausal women, get stress cardiomyopathy more often than men.
One possible reason is the fact that postmenopausal women are more likely to seek medical attention for heart attack symptoms than a younger woman who believes she's too young for such an event and does nothing. "It's called selection bias," he said.
"My second [potential] reason is there's something specific about heart chemistry that [occurs] when you lose the sex hormones, estrogen and progesterone, that it predisposes someone to react differently to those elevated adrenaline levels."
Drs. Champion and Murali agree on what to do if you or someone you know has symptoms of a heart attack after a stressful event: Seek medical help right away.
"After getting the news, one should take immediate action by getting to an emergency room and getting evaluated quickly [so] proper treatment can be established," Dr. Murali said. "They're treated no differently than anyone with a heart attack."
That includes taking a complete history with the patient telling the doctor about the stressful event and ordering bloodwork to check for enzymes released by the body because of a heart attack. Doctors need a diagnostic test -- an ultrasound, electrocardiogram or a heart catheterization, which is more likely -- to evaluate these patients.
"The most important thing I can say about this syndrome is it is a diagnosis of exclusion," Dr. Champion said. "You have to diagnose it after you've ruled out other potential problems.
"Time really is a great healer if you can support them during the initial period, which usually is the first 24 to 48 hours," he said. "Then you have a good chance of [the patient] surviving."
Of the hundreds of patients he has taken direct care of, he has not lost a life. Dr. Champion, however, said he has consulted on cases "where I believe cause of death was [stress cardiomyopathy]."
For more information or for self-referral to his studies, call 412-864-2089 and ask for nurse practitioner Jennifer Kelly.
Pohla Smith: email@example.com or 412-263-1228. First Published October 31, 2011 4:00 AM