It had been a tough hike, even for the experienced 74-year-old hiker. She and her husband finally made their way to the top of Jefferson Rock, outside Harpers Ferry, W.Va. The rolling hills that stretched out forever were as spectacular as she had been told, and she tried to capture the vista in a photograph. Suddenly she was hit with an overwhelming wave of fatigue. She made her way to the shade of a large pine tree, sat down and tore into a protein bar. She felt a little disoriented. There was a dull ache in her chest and she felt strangely out of breath. Her stomach turned anxiously. Her husband called her over to admire the view they both worked so hard to get to on that sweltering June day. “I can’t,” she called out. “We have to leave now,” she added moments later, as she struggled to her feet. “I don’t feel well.”
Her husband looked at her inquiringly. His wife never complained. Could she make it back down? he asked. They both knew she had to; there was no other way. She leaned heavily on her walking stick and held onto his shoulder for balance. It took the couple more than twice as long to get down the rocky slope as it had getting up. The woman had to stop every few steps to catch her breath. When they finally got to the road, her husband suggested they ask for a ride to get them to their car, which was still maybe half a mile farther.
“I think it’s just the heat,” the woman told her husband once they were back in their rented room. She drank a couple of swallows of water, grateful for the coolness, when she was hit with a wave of intense nausea. It was so sudden she knew she couldn’t make it to the bathroom; she grabbed a trash can and heaved.
As she lay on the bed, the woman, a retired physician, tried to make sense of her symptoms. Could this be heat stroke? She had felt a little confused, just for a minute or two, when she reached the peak. She wasn’t sure. Her husband pressed her to go to the emergency room — he was worried she was having a heart attack. But she refused. She was pretty sure she’d feel OK if she just had a chance to rest. The next day she was a little better. The nausea had disappeared, though the ache in her chest persisted. She was still tired, though, and got a little out of breath just walking down to breakfast. Her husband again suggested they go to the emergency room. No, she insisted. It was the last day of their trip. She’d be fine, and tomorrow they’d be home.
Heart Attack Concerns
It was during their drive home to Connecticut that the woman decided to call her new internist, Dr. Lara Colabelli. She described the funny feeling in her chest — not really pressure, not quite pain, just kind of an ache. Then the nausea and vomiting and the strange sense of being just slightly out of breath when she walked. Her doctor suggested that she come in the following day.
The next morning, Colabelli greeted her patient in the waiting room. The woman looked well and moved with a vigor that she didn’t often see in her older patients. Her exam was completely normal. The EKG, however, was not. Looking at the abnormal peaks and valleys of the image, Colabelli had a flashback to a different patient she’d seen years before who had the same jagged pattern; it was another woman, but older and sicker than this one. That patient had a rare heart disorder called takotsubo cardiomyopathy, or broken heart syndrome. In this disease, a sudden shock — in her case, the rapid onset of a serious illness — causes a surge of adrenaline and other stress hormones to be released, which in turn cause the pumping part of the heart, the left ventricle, to balloon outward and lose its strength. Could this new patient have that same rarity? It didn’t quite fit. This patient wasn’t nearly as sick as that first one and didn’t report a sudden shock. But to Colabelli’s experienced eye, the similarity between the EKGs was striking.
She sent the results to two cardiologist friends. They replied with matching assessments: This was a heart attack unless proved otherwise. She wasn’t sure exactly what was going on, Colabelli told the woman, but she was sure that the patient needed to go to the E.R. to get evaluated. She sent her by ambulance to Yale New Haven Hospital, half an hour away.
In the E.R., she was seen by the cardiologist on duty, Dr. Lisa Freed. Another EKG showed the same abnormalities. She also had elevated levels of the enzymes that are released when the heart is injured. Freed’s immediate concern was that the patient was having an ongoing heart attack, or had had one recently. She was taken to the cardiac catheterization lab, where she was sedated and a tiny catheter was introduced to her radial artery, the largest vessel in her wrist, and fed up the vessel into the heart. From there, contrast was injected and the coronary arteries were assessed for signs of a blockage that would starve the cardiac muscle of oxygen — the cause of most heart attacks. This is what causes the typical pain signaling a heart attack. The images were surprising: There was only minimal narrowing of the arteries and no blockages at all. This was not a heart attack. Yet the study also showed the heart was not pumping well. Her ejection fraction, the percentage of blood that is in the heart and pumped into the circulation with each heartbeat, was lower than it had been.
A Rare Diagnosis
To find out why, Freed ordered an ultrasound, called an echocardiogram, of her heart. The next morning the patient found herself on yet another procedure table, though this time she was awake. Grainy images of her heart in motion glowed on a black screen. The patient had been a doctor for 25 years, but she felt too weak and tired to even look at the images. Freed came by later that day to tell her what the study showed: The lower part of her heart, known as the apex, had started to bulge and was not moving at all, while the rest of the organ was hard at work, so that with each beat the heart looked like a round-bottomed, narrow-necked jar. She had takotsubo cardiomyopathy.
This disorder was first described in 1990 by a Japanese cardiologist who observed that the distended shape of the heart resembled a takotsubo, the clay pot that fishermen used to catch octopuses. It is most commonly seen in postmenopausal women. Indeed, although considered rare, up to 6 percent of women who present with what looks like a heart attack have takotsubo cardiomyopathy. Initially it was thought that the trigger for this disorder was a psychological or emotional shock, either good or bad, which causes a dramatic release of stress hormones that in turn stun the muscle so that it doesn’t move and often balloons outward. More recently it has become clear that the disorder is often set off by illness or some other physical event.
The other remarkable quality of this unusual disorder is that, although the heart looks badly weakened, it can recover rapidly with time and the right medicines. Most patients recover completely within a few weeks. But though the heart resumes its normal shape and movement, the stretching and thinning of the bulging part of the heart muscle can cause scarring, making normal pumping less effective. This patient was started on a medication to prevent scarring of the heart. She was already on the other mainstay of treatment: a cholesterol-lowering statin. She was discharged from the hospital the next day. Her shortness of breath got better over the next couple of weeks, and by the time she got her next echocardiogram, five weeks later, her heart had recovered fully.
I asked the patient recently if she knew what caused her heart to react this way. No idea, she told me. Her best theory was that it was just the effort of that tough hike in the terrible heat. In studies, as many as one-third of patients can’t identify a typical trigger. Freed’s advice to the patient was for her to “take it down a notch” in her physical activity. And the patient tells me she’s trying. Hiking, biking and swimming are some of the greatest pleasures of her retired life, but she’ll try to ease up a little, she said. Her heart has gotten her this far, and she wants to take care of it so she will continue to be able to go the distance.
Lisa Sanders, M.D., is a contributing writer for the magazine. Her latest book is “Diagnosis: Solving the Most Baffling Medical Mysteries.” If you have a solved case to share, write to her at [email protected].
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