Primary hyperaldosteronism is when there is too much aldosterone hormone in your bloodstream from a primary cause that can be treated.

Aldosterone tells the body to retain sodium and water and rid itself of potassium. If you have too much of this hormone, the balance of sodium, water, and potassium in your body is abnormal, which can lead to a variety of symptoms and side effects.

Causes

Causes of Primary Hyperaldosteronism

The are two main causes of primary hyperaldosteronism. It is important to differentiate the etiology because the treatment and prognosis are very different.

Solitary adrenal nodule
The most common cause (75% of the time) is a small (<2cm), benign tumor on one of your adrenal glands called an “aldosteronoma”.

Bilateral Adrenal Hyperplasia
In the other 25% of cases, both of your adrenal glands become hyperplastic, or plump, and continuously produce aldosterone.

Symptoms

Signs and Symptoms of Hyperaldosternoma / Conn’s Syndrome

Hypertension that is difficult to control.

Many patients are on multiple medications that still does not control their blood pressure. Poorly controlled blood pressure can lead to cardiac and neurologic problems.

Low potassium. This can be either without symptoms or can lead to

  • muscle weakness
  • Cramps
  • Fatigue
  • numbness.
Diagnosis

Diagnosis of Hyperaldosteronism

The first part of the diagnosis is series of blood tests to determine that you have too much aldosterone in your body and that it isn’t secondary to other causes.

Specifically, we will be measuring the levels of aldosterone, renin, and potassium (as well as some others). This may need to be repeated or done in a more sophisticated manner if the diagnosis is straightforward or you are taking certain medications for your blood pressure.

Once a hormonal diagnosis has been made, imaging will be done to try and localize the side of your adrenal tumor.

  • Adrenal vein sampling is performed by interventional radiologists usually as an outpatient procedure.
  • It involves sampling the blood in both of your adrenal veins via a large guidewire directed catheter and comparing the level of aldosterone on the right and lefts sides.
  • If the tumor cannot be localized to either the right or left side, it is presumed that you have bilateral adrenal hyperplasia.

There is no role for needle biopsy and it is contraindicated.

Risk Factors

Risk Factors of Conn’s Syndrome

Primary hyperaldoteronism is familial in up to 10% of patients. 1-2% of cases have glucocorticoid-remediable aldosteronism (GRA), which is treated by medication. Genetic testing should be done in patients with confirmed onset of primary hyperaldosteronism < 20 years of age and in patients with a family history of stroke or primary aldosteronism < 40 years of age.

Treatment

Treatment of Conn’s Syndrome

All patients need to be treated to help prevent and/or diminish potential cardiovascular complications of untreated high blood pressure.

  • Unilateral adrenal nodules that cause primary hyperaldosteronism are treated with a laparoscopic adrenalectomy. This is a minimally invasive procedure that will remove the tumor that is over-producing aldosterone hormone, allowing your body to return back to its normal state.
  • Bilateral Adrenal Hyperplasia is treated with medication that includes aldosterone-antagonist drugs and a low salt diet.

Follow Up

  • Patients treated with medication for bilateral adrenal hyperplasia can achieve stabilization of potassium levels once properly controlled, but generally remain on multiple hypertensive medications to control their blood pressure.
  • Patients should be regularly followed by their endocrinologist. Even patients that have a successful adrenalectomy should have yearly blood tests to monitor potassium, aldosterone, and renin levels.

“Cure” comes in one of 3 general forms (the rule of thirds):

  • ⅓ of patients have complete resolution of symptoms, including normalization of blood pressure and potassium levels. They are able to come off all blood pressure medications. These are usually younger patients patients with a shorter course of disease.
  • ⅓ of patients have normalization of potassium level and achieve stabilization of hypertension and are on fewer blood pressure medications than before surgery.
  • ⅓ of patients have normalization of potassium and achieve stabilization of hypertension, but remain on 3-4 blood pressure medications. These are usually older patients that have had prolonged, untreated hypertension +/-  hyperaldosteronism.