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Andrew J. Rosen was not surprised when he was diagnosed with high blood pressure at the age of 39. Both of his parents had been taking medications for years that effectively controlled the condition, which affects nearly half of all American adults and is common in families.
But Rosen, who lives in Carlsbad, California, was unlucky. Even at the maximum recommended doses of five drugs, his blood pressure remained high.
Rosen said he had repeatedly asked his doctors if he had been diagnosed. Each time he received the same answer: he had “essential hypertension” – high blood pressure for no apparent reason.
Doctors sometimes told him that the condition, also known as primary hypertension, could be difficult to control. Poorly controlled high blood pressure increases the risk of heart disease, stroke, irreversible kidney damage and early death.
It would be more than a decade before Rosen learned he had asked the right question but received the wrong answer. His persistent high blood pressure did have a root cause.
“He was on a bucket of drugs,” which was the first clue, said William F. Young Jr., an expert at the Mayo Clinic, whom Rosen consulted in 2019. Young said Rosen’s case was “extremely typical.” patients whose delayed diagnosis leads to preventable harm.
For a variety of reasons, says Young, a Mayo professor of medicine, “doctors often don’t seem to think about what it can cause” resistant hypertension – high blood pressure that is not controlled by three or more drugs. This is an omission he is trying to correct.
“Honestly,” Young added, “it’s one of the simplest things to diagnose.”
Already 60 years old, Rosen, senior vice president of development for a company that builds rehabilitation hospitals, suspects he developed high blood pressure in his late 20s, although there is no way to know. He hated having his blood pressure measured, so his readings were limited to periodic medical visits.
These measurements routinely exceeded 140/90 mmHg, the limit that then meant hypertension. (It has since been reduced to 130/80.) Rosen said he expressed his opinion on the diagnosis, reassuring doctors that he had “white coat syndrome,” also known as “white coat hypertension,” indications that are high in medical conditions, but otherwise normal. Due to his youth, doctors usually agree.
In fact, Rosen didn’t know if that was true; he diligently avoided measuring his blood pressure at home. “It was worrying,” he said. “It’s always been high.” He hated to feel the cuff shake his hand, and he preferred to assume he didn’t have high blood pressure.
Not all doctors were convinced. His allergist in Atlanta, where Rosen lived at the time, was skeptical about the explanation for the white apron. “He said, ‘I don’t know. You’re too young for high blood pressure, Rosen recalls.
In 2001, after his internist diagnosed hypertension, Rosen began taking a beta-blocker, a drug that blocks adrenaline. When his blood pressure did not drop, his internist added two other classes of drugs: a calcium channel blocker and an ACE inhibitor. When this trio proved ineffective, the doctor increased the doses.
In the mid-1940s, Rosen was told he had high cholesterol and type 2 diabetes, a chronic condition in which sugar processing is disrupted. Cholesterol and diabetes medications control these problems.
In 2011, after Rosen moved to the San Diego area, his new primary care physician changed his blood pressure medication. When this led to only a minimal reduction, the doctor added two more drugs.
But the increased readings remained. “It will be 148/90 when the nurse measures it on the machine several times,” Rosen said. At the end of the visit, Rosen’s doctor will make a quick manual count and announce that he has fallen to 118/69.
Rosen, whose sister is a doctor, was relieved. “I thought he was a really good doctor,” he said. “And I liked his answer.”
But over the next few years, he became increasingly restless. Although he was taking his medication correctly, too many readings were too high to make him believe his blood pressure was under control.
In 2017, after both of his parents underwent a heart bypass for clogged coronary arteries, Rosen consulted a cardiologist.
The heart specialist appointed a stress echocardiogram, a test that measures how well the heart is working. He showed that Rosen’s heart seemed normal, but the cardiologist was worried about his blood pressure, which sometimes reached 179/85, and increased the amount of the drug that Rosen did not take in the maximum dose. He suggested that Rosen’s hypertension may be the result of a kidney problem, but a kidney scan found nothing.
At this point, Rosen told his family doctor that he wanted to start looking for “zebra,” a term doctors use for a rare diagnosis. The doctor ordered tests for pheochromocytoma, a rare, typically benign tumor that originates in one or both adrenal glands that are located at the top of the kidneys.
After the tests did not reveal “feo”, Rosen was referred to an endocrinologist, a doctor who specializes in the treatment of hormone-related diseases.
‘You don’t have it’
Rosen visited the first endocrinologist in November 2018. She suspected that he may have the disorder most commonly associated with resistant hypertension – primary aldosteronism (PA), also known as Conn or Conn syndrome after University of Michigan endocrinologist Jerome W. A horse that discovered it in 1954.
PA is caused by an excess of aldosterone, a hormone produced by the adrenal glands. Excess aldosterone can cause the kidneys to retain sodium and lose potassium, which raises blood pressure.
The disease can be detected by blood tests that measure the levels of aldosterone and renin, an enzyme produced by the kidneys that helps control blood pressure, and then calculates the ratio between the two. Further tests are needed to confirm the diagnosis and determine if one or both adrenal glands are affected. In the latter case, PA is treated with medication. But in 30 percent of cases, PA is caused by a benign tumor of one gland. Surgical removal of this gland can normalize blood pressure.
While awaiting the results of his blood tests, Rosen reviewed the extensive clinical guidelines for the diagnosis and treatment of PA, issued in 2016 by the Endocrine Society, the international medical organization of endocrinologists.
He found that sleep apnea and the low potassium levels he had were linked to the disease. And he was amused when his blood tests seemed to point to him.
“It seemed like a really good thing to me because it’s treatable,” Rosen said.
But a junior doctor working with his endocrinologist ruled out this possibility. “You don’t have it. Your aldo[sterone] it’s too low, “Rosen recalled being told. Rosen said he protested that his 50-page understanding of the Endocrine Society’s guidelines, along with calculating the blood test ratio, suggested otherwise.
The man disagreed. Rosen said he had ended the conversation and immediately started looking for a new specialist.
Shortly afterwards, Rosen saw a second endocrinologist who agreed that PA seemed likely. She ordered additional tests along with a computed tomography scan. The final step was a technically complex procedure known as adrenal vein sampling, which involves inserting a catheter into the adrenal veins to determine if one or both are affected. This discovery would guide treatment.
After consulting with Young, the second endocrinologist advised Rosen to undergo a vein sampling procedure in Mayo. In April 2019, Rosen and his sister flew to Minnesota and met with Young, former chairman of the clinic’s endocrinology and former president of the Endocrine Society.
An interventional radiologist, a specialist in the vein sampling procedure, found that only one adrenal gland was affected, meaning Rosen was a candidate for surgery. (“You only need half of an adrenal gland to be completely normal,” Young said.)
In June 2019, Rosen underwent laparoscopic surgery at UCLA Medical Center. The following year he lost 35 pounds and his health improved dramatically. He now needs a low dose of just one blood pressure medication to achieve a 124/80 reading.
“I feel much better now than before,” he said. “I’m much more active, too.”
But the late diagnosis had irreversible consequences. Years of uncontrolled high blood pressure cause stage 3b kidney disease, for which Rosen takes medication and visits a kidney specialist regularly. If his illness worsens, Rosen is told he may need a kidney transplant.
Young said he saw patients like Rosen too often, the main reason he became an evangelist for PA screening.
Although doctors have long learned that the disease is rare, Young said recent studies show it is not. Researchers estimate that 5 to 10 percent of people with high blood pressure and 20 percent of those with resistant hypertension have PA. Most don’t know it because they have never been tested.
A 2020 Stanford study found that only 2.1% of patients with resistant hypertension were tested for PA; at the University of Minnesota, the figure is 4.2 percent. A 2003 study from Australia found an unexpectedly large number of cases of PA in a group of patients with high blood pressure.
“It’s very underdiagnosed, and not just in the United States,” said Young, who advocates screening anyone with hypertension at least once. “From my point of view, this comes down to the awareness of clinicians. Endocrinologists and nephrologists think about it. Primary care doctors, not so much. “
People with PA are more likely to develop heart and kidney disease and have a poor quality of life, Young notes in an article from 2018. Timely treatment can …
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