Frequently Asked Questions

Diagnosis:

Who should be tested for PA?

If you have high blood pressure (hypertension), you may have primary aldosteronism. While some advocate systematic screening, the current consensus is to prioritize testing with patients who have:

  • Resistant hypertension:
    • Those patients with sustained blood pressure above 150/100 mm Hg on each of three measurements obtained on different days, or
    • Patients with hypertension (blood pressure >140/90 mm Hg) resistant to three conventional antihypertensive drugs (including a diuretic), or
    • Patients with controlled hypertension (<140/90 mg Hg) on four or more blood pressure medications.
  • Hypertension and spontaneous or diuretic-induced hypokalemia (i.e., low potassium).
  • Hypertension and an adrenal “incidentaloma.” That is, if you have high blood pressure and when you had a CT scan for some other reason, you were told that there is a nodule or bump on one of your adrenal glands.
  • Hypertension and sleep apnea. If you have high blood pressure and have been diagnosed with obstructive sleep apnea (OSA) or sleep apnea is highly suspected.
  • Hypertension and a family history of early onset hypertension or stroke at a young age (<40 years). You should also consider getting tested if you are under 40 and have been diagnosed with high blood pressure even if you are unsure about your family history.
  • Hypertension and a first degree relative with primary aldosteronism. If you have high blood pressure and someone in your immediate family has primary aldosteronism, you should be tested.
Do I have PA?

If you suspect that you have primary aldosteronism — and especially if you fall into one of the high risk categories listed on this website — you should ask your Primary Care Physician to order a blood test for aldosterone and renin. If your physician is not familiar with these tests or with primary aldosteronism, you should ask for a referral to an endocrinologist. These blood tests will allow your physician to calculate your aldosterone-to-renin ratio (ARR). A high ARR test result makes it likely that you have primary aldosteronism. In most cases, you will also need to get a separate confirmatory test.

What are the symptoms of primary aldosteronism?

The symptoms of primary aldosteronism are easily mistaken for other illnesses. The first clue that you may have primary aldosteronism is usually high blood pressure, especially hard to control blood pressure.

When primary aldosteronism also causes low potassium levels, patients usually experience:

  • Muscle cramps
  • Weakness
  • Fatigue
  • Headache
  • Excessive thirst, and
  • A frequent need to urinate.

Symptoms associated with primary aldosteronism also include sleep disorders, cognitive impairment, flushing, myalgia and bone pain. Many people go for years without knowing that they have primary aldosteronism, while their blood pressure gets steadily worse, despite taking several drugs to control it.

Do I need to be treated at a Center of Excellence for primary aldosteronism?

Our website lists some of the top medical centers in the world with experience treating primary aldosteronism. They all have an experienced team of endocrinologists, interventional radiologists, and endocrine surgeons. Because they routinely see dozens of patients with primary aldosteronism each year, they are more able to recognize and handle complex cases. These institutions have a higher success rate and a lower rate of complications than some centers with less experience. It may be impractical for you to be treated at one of these centers because of the travel distance or because your medical insurance or health care system will not cover the costs. When you select the medical facility where you will seek treatment, ask doctors about the number of patients with primary aldosteronism they treat each year, and their track record for success. You may be able to get a good referral in your area by doing a search through the Hormone Health Network, the website for patients sponsored by the Endocrine Society.

My ARR tested positive (or was high). Now what do I do?

If your physician told you that you tested positive for primary aldosteronism because you have a high aldosterone-to-renin ratio (ARR), the next step is to get a confirmatory test. Since there is no “gold standard” and various options are possible, we recommend that you learn about confirmatory testing prior to your medical appointment.

If you haven’t already done so, you should see an endocrinologist, or a nephrologist or a cardiologist who is experienced with diagnosing and treating primary aldosteronism. You should also be evaluated for the presence of other conditions that commonly coexist with primary aldosteronism, such as cardiovascular, kidney, and metabolic diseases.

How do I choose an interventional radiologist if I am considering adrenal venous sampling (AVS)?

Adrenal venous sampling (AVS) is recommended for all patients who have been diagnosed with primary aldosteronism and are considering surgery. The procedure is done by an interventional radiologist (IR). It is an invasive and expensive procedure, so you will want to select someone with experience. Only those radiologists who do AVS procedures routinely have a success rate of >95% and a very low rate of complications. All of the Centers of Excellence listed on this website have an experienced IR on staff to do AVS. There are certainly many other qualified IRs available, but you should be careful during your selection process to ask candidates about the number of procedures they have performed and their personal success rate.

My AVS showed bilateral disease. What do I do now?

If you have a bilateral form of primary aldosteronism, you should be treated with medication. The most effective drugs are mineralocorticoid receptor antagonists (MRAs) that work to inhibit the effect of excess aldosterone in the body. MRAs include Spironolactone and Eplerenone. Other medications are available for those who experience serious side effects when using those drugs. See the section on this website titled “Medical Option” for more information.

Treatment:

How do I choose an endocrinologist if I suspect I have primary aldosteronism?

Like other specialists, an endocrinologist may focus on certain practice areas such as diabetes or pituitary disorders. If you think you might have primary aldosteronism, you will want to find an endocrinologist that specializes in adrenal disorders and has experience treating patients with primary aldosteronism. We list some expert endocrinologists on our website, but you can find others through the Hormone Health Network, which is sponsored by the Endocrine Society.

How do I choose a surgeon if I am considering an adrenalectomy?

An adrenalectomy has a low complication rate in the hands of an experienced surgeon, so you should look for an endocrine surgeon who does them routinely. To be considered an expert, the American Association of Endocrine Surgeons states that a surgeon should do at least 20 adrenal surgeries each year. Our directory of PA Specialists includes surgeons, and AAES offers a list of surgeons practicing in the US and Canada. Don’t be afraid to ask potential candidates for doing your surgery how many procedures they have done and their complication rate.

What are the risks of an adrenalectomy?

An adrenalectomy is a relatively safe procedure with a low complication rate in the hands of an experienced endocrine surgeon. Most PA patients are released from the hospital 1-2 days after surgery and recover fully within a few weeks. Still, problems can occur with any surgery. Pneumonia, wound infections, bleeding, need for blood transfusions, and blood clots (deep vein thrombosis or pulmonary embolus) are rare, but may occur after adrenal surgery. Damage to other nearby organs may occur, but this is also rare.

Adrenal insufficiency is a potentially life-threatening complication that may occur in patients who have an adenoma that secretes both excess aldosterone and cortisol because the healthy adrenal gland becomes suppressed. Although still considered uncommon, the condition is increasingly found as more patients are treated for primary aldosteronism. To minimize the risk, patients considering adrenalectomy should make sure that their cortisol levels are properly tested during the diagnosis phase and prior to surgery. Symptoms of this condition include feeling generally unwell, nausea, vomiting, and confusion. You will likely be given an adrenal stimulation test following surgery to detect this problem. To address adrenal insufficiency, patients may need steroid medications, usually hydrocortisone, for several months until their adrenal gland recovers.

Can you live with only one adrenal gland?

One normal adrenal gland is able to produce sufficient amounts of cortisol, aldosterone and adrenal androgens to fulfill the body’s needs. If one adrenal gland is removed by surgery, the remaining health gland will take over completely. Your hormones levels will be tested immediately after surgery and in the following weeks to ensure that you have fully recovered. In rare cases, it may take your adrenal gland additional time for recovery because its production had been suppressed by the high levels of aldosterone produced by the gland you had removed. In that case, you might be given replacement hormones for a short period of time.

Is it possible to have both adrenal glands removed?

If you have a bilateral form of primary aldosteronism that affects both your adrenal glands, you will be treated with medication rather than surgery. In most cases, treatment with mineralocorticoid receptor antagonists (MRAs), such as Spironolactone, will be able to control your blood pressure, keep your electrolytes in balance, and prevent other organ damage. Physicians will avoid removing both adrenal glands except under very serious circumstances, such as the presence of malignant tumors. Patients who have both adrenal glands removed must take replacement hormones for life. Unfortunately, medications aren’t able to replicate the daily rhythm of hormone production made by healthy adrenal glands or respond quickly to changes in the body’s needs under stress. As a result, these patients may have poor quality of life.

What are the side effects of Eplerenone?

Tell your doctor if any of the following side effects become severe or don’t go away:

  • Dizziness
  • Tiredness
  • Mild diarrhea or stomach pain
  • Headache

Let your doctor know right away if you experience any of the following serious side effects:

  • Chest pain
  • Fast or irregular heartbeat
  • Enlarged or sore breasts in men
  • Abnormal vaginal bleeding in women
  • Severe or persistent dizziness
  • Severe or persistent diarrhea
  • Swelling of the legs, feet, or ankles
  • Vomiting
  • Tingling in the arms or legs
  • Weakness or heaviness in the legs
  • Loss of muscle tone
  • Cold, gray skin
  • Severe lack of energy
  • Confusion
  • Signs of anaphylaxis, which may include rash, hives, difficulty breathing, tightness in the chest, or swelling of the face, mouth, lips, or tongue

Consult your physician or pharmacist for more complete information on this medication.

What are the side effects of Spironolactone?

Common side effects of Spironolactone include:

  • Vomiting, diarrhea, and stomach pain or cramps
  • Dry mouth and thirst
  • Dizziness, unsteadiness, and headache
  • Gynecomastia (enlarged breast tissue) in men, and breast pain in women
  • Irregular menstrual periods and post-menopausal vaginal bleeding
  • Erectile dysfunction
  • Deepening of the voice and increased hair growth
  • Drowsiness, tiredness, and restlessness

Severe side effects of Spironolactone include:

  • Muscle pain or weakness
  • Numbness or tingling
  • Paralysis in the arms or legs
  • Arrhythmia
  • Confusion, extreme tiredness, and fainting
  • Unusual bleeding or bruising
  • Life-threatening skin reactions
  • Flu-like symptoms, pain in the upper right abdomen, loss of appetite, vomiting blood, or bloody stools
  • Difficulty breathing or swallowing
  • Decreased urination

Spironolactone has been found to cause tumor development in rats. As a result, the drug carries a black-box warning for tumor risk. Consult your physician or pharmacist for more detailed information on this medication.

For Clinicians:

Why should I test patients for PA?

According to the Endocrine Society, primary aldosteronism is a major public health issue that affects up to 10% of patients with hypertension. Most patients with hypertension are never screened for primary aldosteronism. As a result, they go untreated for years and often experience end organ damage that could be avoided with earlier detection and treatment. Evidence suggests a higher risk for cardiovascular and renal complications in patients with primary aldosteronism, including arrhythmias, myocardial infarction, stroke, chronic kidney disease and death vs. age-, sex- and BP-matched adults with hypertension. Early detection can improve cardiovascular outcomes.

Testing for primary aldosteronism begins with a test of plasma aldosterone and renin levels. If the aldosterone-to-renin ratio is high, then the patient is likely to have primary aldosteronism and should be promptly referred to a specialist for further testing.

Primary care physicians should always consider primary aldosteronism in the presence of:

  • Resistant hypertension
  • Hypertension and spontaneous or diuretic-induced hypokalemia
  • Hypertension and an adrenal incidentaloma
  • Hypertension and sleep apnea
  • Hypertension and a family history of early onset hypertension or stroke at a young age (<40 years)
  • Hypertension and a first degree relative with primary aldosteronism
Why should I treat primary aldosteronism?

Physicians are usually taught that less than 1% of patients with essential hypertension have primary aldosteronism. Not only is primary aldosteronism the most common cause of secondary hypertension, its prevalence is in fact so high that it is now believed to be a primary cause of essential hypertension. People with primary aldosteronism have a considerably higher cardiovascular risk profile,  and experience higher rates of renal disease and metabolic disorders. Untreated patients with primary aldosteronism have more serious disease and die much younger than patients with essential hypertension, even if their blood pressure is well controlled. These problems can be mitigated if patients are diagnosed and treated early.

Most cases (60%) present with bilateral disease, and should be treated with dietary sodium restriction and mineralocorticoid receptor antagonists (Spironolactone or Eplerenone), while most of the remaining cases (35%) present with unilateral disease and should undergo adrenalectomy.

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References

  • John W. Funder, Robert M. Carey, Franco Mantero, M. Hassan Murad, Martin Reincke, Hirotaka Shibata, Michael Stowasser, William F. Young, The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline, The Journal of Clinical Endocrinology & Metabolism, Volume 101, Issue 5, 1 May 2016, Pages 1889–1916, DOI: /10.1210/jc.2015-4061
  • William F. Young, Jr. Diagnosis and treatment of primary aldosteronism: practical clinical perspectives. The Journal of Internal Medicine, Volume 285, Issue 2, February 2019, DOI: 10.1111/joim.12831
  • Maurizio Iacobone, Marilisa Citton, Giovanni Viel, Gian Paolo Rossi, Donato Nitti, Approach to the surgical management of primary aldosteronism, Gland Surgery, Volume 4, No. 1, February 2015, DOI: 10.3978/j.issn.2227-684X.2015.01.05

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The Primary Aldosteronism Foundation is a registered 501(c)(3) public charity. Donations are tax deductible in the US.

©2021 Primary Aldosteronism Foundation

The Primary Aldosteronism Foundation is a registered 501(c)(3) public charity. Donations are tax deductible in the US.