Please find something wrong with me, the 28-year-old woman pleaded. For nearly a year, shed been looking for a reason for the strange symptoms that now dominated her life. Dr. Raphael Sung, a cardiologist specializing in finding and fixing abnormal heart rhythms at National Jewish Health hospital in Denver, was surprised by her reaction to the news that her heart was normal. Most patients are happy to get that report. For this patient, it seemed like just one more dead end.
The patients symptoms started right after her baby was born 10 months earlier. Out of nowhere, her heart would start beating like crazy. At first, she assumed that these were anxiety attacks, triggered by the stress of bringing her premature daughter home. Her baby spent her first week of life in the newborn intensive care unit. When she was big enough to come home, she still weighed only four pounds, nine ounces. The new mother worried that without the doctors and nurses and equipment that had kept her alive, her tiny baby might die. But she didnt. She seemed to thrive at home. Despite that, her mothers heart continued to take off like a spooked horse several times a day.
After a couple of weeks, her symptoms worsened. Sometimes her racing heart would set off terrible headaches, the worst shed ever had. It was as if someone had thrust a sharp stick deep into her brain. The knife of pain quickly turned into a sense of pressure so intense it felt as if the back of her skull would blow off. Minutes later, she would feel the blood drain from her face; shed be suddenly drenched in sweat. Her hands would curl into tight fists, and vomit would shoot out of her mouth like a geyser. Her husband joked (though only once) that she looked like the girl in The Exorcist.
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Vomiting somehow relieved the pressure in her skull, but for the rest of the day, shed have a dull, pounding ache behind her eyes. Light and noise were intolerable. Shed have to lie in a darkened room for hours. When her husband had to go back to work after his paternity leave, they moved in with her parents in Colorado Springs so that someone would be able to look after the baby whenever she was prostrate with pain.
Her primary-care doctor focused on her headaches, she recalled. They sounded like migraines. Nearly one in four new mothers will have a first migraine in the weeks after delivery. He prescribed propranolol, a medication designed to slow the heart and often used to prevent migraines; it didnt help.
A second internist focused on her racing heart. She ordered an echocardiogram, which was normal. An event monitor, a device worn on the chest that looks for abnormal rhythms, showed a couple of brief episodes of rates up to 140 beats per minute. Although that was twice the normal speed, having the occasional episode of a rapid heart rate isnt worrying as long as the rhythm is normal which hers was. The device also had a button that the patient was told to push whenever she felt her heart pounding wildly. But most of the time when she hit the button, the recorded beats picked up by the monitor were normal.
Seeing this, her doctor told the patient she probably did have anxiety just as she initially thought. But, the patient objected, it was her body racing, not her mind. The doctor gently reminded her that the test showed that her heart was beating normally when she felt it was not. The doctor suggested a medication to treat this new anxiety. The patient agreed, reluctantly, but couldnt bring herself to try it.
Over the next several months, the woman was in and out of the emergency room more than a dozen times. She had blood tests, a CT scan of her head and another of her chest. No one could come up with a reason for her racing heart, knifelike head pain or episodes of vomiting.
She had a second normal cardiac monitoring test, then saw two cardiologists. They had theories and offered therapies, but nothing worked.
A friend told her about a woman she knew with similar symptoms. That woman had gone to Dr. Sung, who found an arrhythmia and fixed it; she was fine now. His office was in Denver an hour away. The woman called as soon as she got home.
To Sung, the heart episodes the young woman described the way they came and went abruptly without warning sounded like the episodes his other patients described, the kind of abnormal rhythms he treated. He arranged for her to come back a few weeks later for a test in which he would try to trigger her heart into the odd behavior she described.
A normal heart beats when its organic pacemaker sends out regular, timed electrical signals through the cardiac muscle. These signals travel down established pathways delivering the message, and the heart contracts in response. Sung an electrophysiologist, as this kind of cardiologist is called looked for abnormalities in this living electrical system. The patient had none of them. Yet as Sung was stimulating the heart, trying to make it reveal its abnormality, the patient recognized the sensation. It was the kind of feeling shed been having these past several months. Still, she never developed any abnormal rhythms.
It was when Sung reported these normal results that the patient began to cry. If she didnt have what he was looking for, couldnt he look for some other causes of her upsetting symptoms? The patients request prompted the doctor to take off his cardiology hat and dust off his internist cap, as he explained to me, and think back to his internal-medicine training. What else, besides the heart, could cause these abrupt surges that tormented the patient?
Although Sung still suspected her heart was the issue, he broadened his thinking. One possibility was that her body was inappropriately releasing one of several hormones that stepped up the activity of her body. Another could be a problem in the part of the nervous system that managed blood pressure and heart rate. He ordered lab tests and put in a referral to a neurologist. And if all that came back normal as he suspected it would he would try an implantable cardiac monitor to look for more subtle abnormalities.
The results of the lab studies came back before she went to see the neurologist. Her thyroid was normal. And she didnt have a serotonin-secreting tumor another possible, but rare, cause of erratic surging sensations. But one test was telling. And it wasnt directly related to her heart. One type of the hormones he checked the so-called fight-or-flight hormones was sky-high. The most likely cause of that result was a rare tumor called a pheochromocytoma. Up to 90 percent of these abnormal growths develop on the adrenal glands a pair of small organs situated on top of the kidneys where hormones, like epinephrine and its counterpart, cortisol, are made.
A CT scan of her abdomen revealed a tumor the size of a lime on the left adrenal gland. Sung was surprised but pleased. The patient was ecstatic. A diagnosis, even of a tumor, was an answer, and thats what she needed.
Sung referred her to an endocrinologist, and the tumor was removed a few weeks later. The crazy spells, the racing heart, the terrible headaches disappeared.
The patients endocrinologist told her that she probably had this disorder during her pregnancy. It may even have played a role in her early delivery. But the combination of a rare disorder and common symptoms are the very hardest diagnoses to make. The vast majority of people who experience a racing heart and debilitating headaches dont actually have a pheochromocytoma.
The patients daughter is now 3 years old and doing great. So is the patient. She and her husband are planning for their next child.
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