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Adrenal Diseases - Primary Hyperaldosteronism
The Facts You Need To Know

On This Page:

What is Primary Hyperaldosteronism?

What causes Primary Hyperaldosteronism?

How is Primary Hyperaldosteronism diagnosed?

How is Primary Hyperaldosteronism treated?

Primary Hyperaldosteronism Fact Sheet (pdf)


 

    Primary hyperaldosteronism is a disease caused by an excess production of the normal adrenal hormone aldosterone. This hormone is responsible for sodium and potassium balance, which then directly controls water balance to maintain appropriate blood pressure and blood volume.
    People with a deficiency of aldosterone, especially found in association with cortisol deficiency in Addison's disease, have low blood volume and therefore low blood pressure, low sodium and high potassium. Just the opposite is seen in hyperaldosteronism. Primary hyperaldosteronism causes high blood pressure and a low serum potassium. The serum sodium is usually in the normal range. A separate disorder called secondary hyperaldosteronism is the result of abnormal fluid shifts seen in congestive heart failure, liver disease and certain kidney diseases.
    Primary hyperaldosteronism used to be considered a rare disease, but recent studies show it to be a fairly common cause of hypertension. It is usually considered by physicians if they find an unexpectedly low potassium in a person being treated for hypertension. However, many people with this disease have potassium levels in the normal range. There is a growing awareness that many people with hypertension that is difficult to control or have progressively worsening hypertension may have primary hyperaldosteronism. While most individuals have no specific symptoms, some may have fatigue, headaches, muscle weakness and numbness. The physical examination is usually normal except for the elevated blood pressure.
    There are two major types of primary hyperaldosteronism and three rare forms. The most common form is due to hyperplasia in both adrenal glands (about 60% of cases). A benign tumor of one of the adrenal glands is the cause in 35%. When the cause is a single adrenal tumor, it is labeled Conn's Syndrome. A malignant tumor is a very rare cause. Other rare forms are unilateral hyperplasia and a familial glucocorticoid remediable form.
    When primary hyperaldosteronism is considered by a physician, tests are done to look for an excess of the hormone aldosterone in the blood and urine, and also a suppressed plasma renin. The initial screening test is the aldosterone/renin ratio. To be certain that the value is accurate, serum potassium must be corrected first, and blood pressure medications that can affect aldosterone or renin must be discontinued for a few weeks. Borderline test results are common, so repetition of the ratio is often needed. Other tests, looking at other adrenal steroid hormones, can be very useful as well as tests looking for the normal physiologic changes in hormones in the morning and evening, as well as responses to sodium challenge or sodium restriction. Once primary hyperaldosteronism is confirmed, it is important to try to differentiate a single adenoma from hyperplasia. A CT of the adrenals will often show a small tumor in one of the adrenals. Typically, the adrenal tumors that cause hyperaldosteronism are very small, usually less than 2 cm. The presence of a tumor on CT is not adequate proof of an adenoma as the cause of the disease. Often it is misleading, with a tiny adenoma on the other side and a non-functioning tumor that is seen on the CT. The gold standard for confirming unilateral disease is the use of adrenal vein sampling. This is a special procedure performed in major medical centers with experienced radiologists. Catheters are inserted in both adrenal veins and blood samples are obtained before and after ACTH infusion looking for a significant difference in levels of aldosterone between the two sides. If there is a significant difference, an adenoma is confirmed. Unfortunately, this prodedure is not always diagnostic because it is difficult to get a catheter in the right adrenal vein.
    The treatment of primary hyperaldosteronism depends on the cause. If there is a single tumor, surgical removal of that tumor can cure the disease. The remaining adrenal gland is usually normal and individuals with this form of the disease will have enough adrenal hormone production from the remaining gland to live normally. Unfortunately, quite often there is still some residual hypertension even after the surgery, so sometimes antihypertensive medication is still necessary.
    If bilateral hyperplasia is the cause of hyperaldosteronism, or if surgery for an adenoma is not appropriate or safe, the disorder is treated with specific medications that block the effect of aldosterone. There are three such medications: spironolactone (Aldactone), eplerenone (Inspra) and amiloride (Midamor). These medications are very effective, but it is common to need to combine them with other antihypertensive medications for the management of hypertension in individuals with hyperaldosteronism.
revised 3/2013

Q&A with NADF Medical Director Paul Margulies, MD, FACE, FACP on the subjects of hyperaldosteronism and Conn's Syndrome:

Q. "3 years ago I had a left adrenalectomy for Conn's disease and was symptom free and feeling great. Within the past two weeks my symptoms reappeared and my diastolic pressure is high. Weakness and muscle aches have returned as well. My right adrenal gland is enlarged and I wonder if hyperaldosteronism could reoccur on the remaining adrenal gland. I went to my endocrinologist and he wants me to have a postural test. However, he is rather slow to schedule this and meanwhile I feel awful. Any suggestions? Thanks,"

A. "It is quite possible that the hyperaldosteronism was from bilateral hyperplasia. It is also possible that there is a second adenoma. You need a full evaluation of the function of the remaining adrenal gland. Most likely medical therapy will be successful. There is no useful suggestion I can make for your symptoms pending your work-up."

Q. I had a left adrenalectomy November 2006 for Conn's Syndrome (primary aldosteronism). I did very well after surgery UNTIL I caught a virus in December 2007. I now have symptoms of adrenal insufficiency, periods of nausea, weight loss (20 lbs in two months) inability to handle any stress, muscle weakness and shakiness and fatigue. The quality of my life has been greatly affected. The 'spells' come and go and can last from a few hours to a few days. I can feel a little better at times only to have it start all over again. I am being followed by Endocrinologists in Syracuse for the Conn's diagnosis, but they state that the above problems are not related to my having only one adrenal gland. My primary admitted that I could have a temporay adrenal insufficiency BUT ordered only one serum cortisol which was normal and pronounced that I am ok. I AM NOT OK

A. I certainly agree that further testing is appropriate. Although the remaining adrenal should be adequate after surgery for Conn's syndrome, the symptoms should prompt at least a full Cortrosyn stimulation test with baseline and 1 hour cortisol levels, plus a baseline plasma ACTH just to be sure. You should be able to get this done with an endocrinologist in Syracuse. A.Hyperaldosteronism due to an adrenal tumor does not necessarily require surgery. It is usually managed fairly well with medications like spironolactone (Aldactone), sometimes in combination with other blood pressure medications. In fact, older patients who may have had the tumor as a cause of hypertension for many years are less likely to be cured with removal of the tumor, and usually continue to need blood pressure drugs. The tumor is almost never malignant. By the way, the change in size seen on imaging was probably an artifact of the angle of the slices on CT, rather than a real change. In any case, it is important to stress that medical management is a safe and effective way to treat her.

Q. "Dear NADF: I was diagnosed with Conn's Syndrome four years ago, as of now Aldactone is part of my medication (30 mg). How come symptoms like severe headache, dizziness, palpitation, muscle cramps, flushing are still present although it had lessen. Is this expected? Three years ago I had my heart attack then November of last year a confinement due to blood clotting. Is this considered a complication of this condition?
Thank You and God Bless!

A. "The symptoms listed are not typical of Conn's syndrome or the management with spironolactone. They are more suggestive of pheochromocytoma, a tumor of the adrenal medulla. I would discuss the symptoms with your doctor, and make sure that further tests be done for pheo, such as plasma metanephrines and a 24 hour urine for metanephrines and fractionated catecholamines. Blood clots can occur from bed rest, but are not a feature of Conn's syndrome."

Q. "My endocrinologist is out of the country and gets back next month. I did convey your suggestion to her assistant doctor and she prefer to wait for my principal doctor's decision when further lab. test is concern. Guided by the words of Dr. Paul Margulies I'll submit for a plasma metanephrine test. Is there any preparation for this? What particular time of the day does it be taken to come up a reliable result? This is the only adrenal hormone not tested in my previous lab. Work. Please don't misunderstand my questions, it's just that I want to know the process because when I ask about this kind of test, I can sense that it's quite remote for them. They are telling me that it's their first time to encounter this test. Give my thanks to Dr. Paul Margulies, to you and all people behind NADF.
God Bless You All!
Thank you."

A. "The plasma metanephrines and 24 hour urine test can be performed without any special preparation as long as she is not taking any beta blocker medication for the blood pressure. If she is, then it should be withdrawn for 2 weeks first. The blood and urine tests are most sensitive if she actually has symptoms at the time of the test."

Q. "Any suggestion for those of us who produce too much aldosterone? I have adrenal hyperplasia.
Thanks,"

A. "Hyperaldosteronism from adrenal hyperplasia can usually be managed with one of the aldosterone blocking drugs: spironolactone, amiloride or eplerenone. Quite often the hypertension requires additional medications, such as diuretics, calcium channel blockers or beta blockers. The objective is normal blood pressure and normal potassium."

Q. "This lady has had one adrenal gland removed and the other is not producing cortisol. She says it produces too much aldosterone, though. Anyway....Her concern is that when her internal prednisone levels are too low (dwindling, in other words), she gets noise in her ears and a weird sensation in her head. And that tells her she needs to up her meds a bit."

A. "I need to point out that prednisone is a drug, not a natural hormone. So, what she apparantly means by a low prednisone level is that the dose of prednisone she is taking needs to increase. This may be an indication that she has glucococortioid deficiency as a feature of her adrenal physiology and she is experiencing sensations in her head as a symptom of this deficiency. That may be the case. However, her situation cannot be applied to most people because she seems to have a rare type of adrenal disease. Without knowing why her adrenal gland was removed, if she does have hyeraldosteronism from the remaining adrenal, she might have a form of glucocorticoid suppressible hyperaldosteronism. The symptoms she gets should be assessed by her endocrinologist to sort out how they might relate to her blood pressure or electrolytes"

Q. "Dear NADF,
I am writting due to an adrenal adenoma That was found during a CT scan for another reason it was 1.3 cm..I had to go to er for another reason they did CT and results said adrenals unremarkable.Do these adenomas go away or are their different levels of CT scans?I was told they do not go away and they can cause problems sometimes but most are benign .I am having some problems and would like to know if there are any studies showing they do go away on their own. Thank You,"

A. "Adrenal nodules as an incidental finding on CT and MRI of the chest and abdomen are very common. Most are non-functioning adenomas that do not grow and cause no harm. I think it is appropriate for an internist or endocrinologist to clinically evaluate anyone with this finding. Most of the functioning adenomas will cause signs and symptoms. The major syndromes are pheochromocytoma, Cushing's syndrome and Conn's syndrome. Each of these can be ruled out by basic history, physical exam and some basic laboratory tests. Even in the absence of any findings, it is useful to do an overnight dexamethasone test to rule out subclinical Cushing's. At least one follow up imaging 6 to 12 months later is useful to rule out significant growth of the nodule. In the case of a large nodule (over 3.5 cm) or a rapidly growing nodule, surgical removal is necessary because of the risk of cancer. Generally, adrenal adenomas do not go away. However, different techniques used in imaging the area might miss a tiny adenoma or missjudge the size."

Q. "I was diagnosed many years ago with hyperaldosteronism. I have had symptom control with Spironolactone, hydrochlorothiazide and Potassium supplements. My endocrinologist wants me to discontinue Spironolactone for 6-8 weeks and then have blood work and possible AVS. She wants to replace the hormone blocker with Lisinopril and keep me on the HCTZ and potassium.Though I have great respect for my doctor, she does not specialize in adrenal disorders. Are these medications good choices to give accurate results in blood work/ renal tests? Thank you,
Sincerely,"

A. "The basic question is why her doctor wants to do further work up if blood pressure and symptoms are currently well controlled. The presumption I would have is that surgery is contemplated, aiming for a cure. If that is not the case, I don't see the need for the invasive test of adrenal venous sampling. If surgery is the goal, venous sampling remains the gold standard for localization before surgery, and can be done on the proposed medications. A recent study showed that in people with classic laboratory studies diagnostic of hyperaldosteronism (high aldo, suppressed renin and low potassium) a CT showing a unilateral tumor is as accurate as adrenal venous sampling."

We have a few of Q&A items regarding hyperaldosteronism with Dr. Margulies that have yet to be printed in the NADF News. Here they are:

Q. '3 years ago I had a left adrenalectomy for Conn's disease and was symptom free and feeling great. Within the past two weeks my symptoms reappeared and my diastolic pressure is high. Weakness and muscle aches have returned as well. My right adrenal gland is enlarged and I wonder if hyperaldosteronism could reoccur on the remaining adrenal gland. I went to my endocrinologist and he wants me to have a postural test. However, he is rather slow to schedule this and meanwhile I feel awful. Any suggestions? Thanks,"

A. "It is quite possible that the hyperaldosteronism was from bilateral hyperplasia. It is also possible that there is a second adenoma. You need a full evaluation of the function of the remaining adrenal gland. Most likely medical therapy will be successful. There is no useful suggestion I can make for your symptoms pending your work-up."

Q. "I had a left adrenalectomy November 2006 for Conn's Syndrome (primary aldosteronism). I did very well after surgery UNTIL I caught a virus in December 2007. I now have symptoms of adrenal insufficiency, periods of nausea, weight loss (20 lbs in two months) inability to handle any stress, muscle weakness and shakiness and fatigue. The quality of my life has been greatly affected. The "spells" come and go and can last from a few hours to a few days. I can feel a little better at times only to have it start all over again. I am being followed by Endocrinologists in Syracuse for the Conn's diagnosis, but they state that the above problems are not related to my having only one adrenal gland. My primary admitted that I could have a temporay adrenal insufficiency BUT ordered only one serum cortisol which was normal and pronounced that I am ok. I AM NOT OK"

A. "I certainly agree that further testing is appropriate. Although the remaining adrenal should be adequate after surgery for Conn's syndrome, the symptoms should prompt at least a full Cortrosyn stimulation test with baseline and 1 hour cortisol levels, plus a baseline plasma ACTH just to be sure. You should be able to get this done with an endocrinologist in your area of residence."

Q. "Any suggestion for those of us who produce too much aldosterone? I have adrenal hyperplasia. Thanks,"

A. "Hyperaldosteronism from adrenal hyperplasia can usually be managed with one of the aldosterone blocking drugs: spironolactone, amiloride or eplerenone. Quite often the hypertension requires additional medications, such as diuretics, calcium channel blockers or beta blockers. The objective is normal blood pressure and normal potassium."

The answers above were provided by
Dr. Paul Margulies, MD, FACP, FACE, NADF Medical Director
Clinical Associate Professor of Medicine, New York University Medical School

 

NADF does not engage in the practice of medicine, is not a medical authority, and does not claim medical knowledge.
In all cases, NADF recommends that you consult your own physician regarding any course of treatment or medication.

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