Primary hyperaldosteronism (Conn syndrome)


What is it about ?

Each of the two kidneys is surmounted by an adrenal gland. Each of the two adrenal glands is made up of a cortex, located at the periphery, and a central area called the adrenal medulla. The adrenal cortex makes different hormones called steroids.

One of the most important steroids is aldosterone. Its role is to control the amount of salt that the kidneys excrete in the urine. For example, when blood pressure is low, salt (and therefore also water) is retained, and potassium is excreted in the urine. This mechanism helps normalize blood pressure.

We talk about hyperaldosteronism when there is too much aldosterone in the blood. This excess can be primary or secondary.

  • In the primary form, the cause is in the adrenal gland herself ; it produces large amounts of aldosterone, independent of the kidneys, which causes an increase in blood pressure (hypertension). Most often, it is a benign tumor of the adrenal cortex that produces aldosterone (Conn syndrome); sometimes both adrenal glands are larger than usual. In rare cases, the cause is a malignant tumor or an inherited disease.
  • In the secondary form, the cause is outside the adrenal gland, for example a narrowing of the artery of the kidneys.

What is its frequency?

Primary hyperaldosteronism is only found in 0.5 to 2% of people with hypertension.

How to recognize it?

Primary hyperaldosteronism is manifested by general symptoms: fatigue, headache, muscle weakness and muscle cramps, thirst, urinating often (especially at night), and harder stools.

High blood pressure usually does not cause symptoms, but can lead to cardiovascular problems, such as stroke.

How is the diagnosis made?

If you are diagnosed with high blood pressure, the doctor will order a blood and urine test to look for an underlying cause. He will think about primary hyperaldosteronism especially if your blood pressure is difficult to control with antihypertensive medication or if you are under 40 years old.

Often, but not always, primary hyperaldosteronism results in decreased potassium level in the blood. Sometimes an adrenal tumor is discovered by chance, such as during an imaging test of the belly for another problem. If primary hyperaldosteronism is suspected, you will always be referred to a specialist for further examinations and treatment.

What can you do ?

If you think you havehypertension, contact your GP or measure your blood pressure yourself at home. If you have high blood pressure and one or more of the above symptoms, it is best to see your GP.

What can the doctor do?

If he suspects primary hyperaldosteronism, the general practitioner will refer you to a specialist in the disease (an endocrinologist). Additional blood tests and technical examinations will determine whether you have primary hyperaldosteronism. If so, you will receive appropriate treatment.

Surgical removal is the treatment of choice for a unilateral adrenal cortex tumor (Conn syndrome). Often after this, the blood pressure normalizes. Medication therapy combined with a low-salt diet is sometimes sufficient.

When both adrenal glands are enlarged, one will almost always opt for treatment by drugs (to be taken for life). The drug of choice is spironolactone. Side effects, however, are common and depend on the dose. These include increased breast size in men (gynecomastia) and menstrual problems in women.

Source

Foreign clinical practice guide ‘Primary hyperaldosteronism (Conn’s syndrome)’ (2000), updated 08.08.2017 and adapted to the Belgian context on 15.06.2019 – ebpracticenet