The clinical picture of primary hyperaldosteronism is also known as Conn’s syndrome. It is characterized by increased levels of the hormone aldosterone, which increases blood pressure.
What is primary hyperaldosteronism?
In most cases, primary hyperaldosteronism is based on either hyperplasia of the adrenal cortex or an adrenal cortical adenoma. The result is an increased formation of the hormone aldosterone. This raises blood pressure, so that primary hyperaldosteronism often occurs as a result of hypertension, i.e. high blood pressure.
This type of high blood pressure is called secondary because it is caused by a hormonal imbalance. It used to be assumed that primary hyperaldosteronism is fundamental in less than one percent of all patients with hypertension. Nowadays, however, the assumption prevails that the clinical picture is one of the most common reasons for hypertension. See healthknowing for What is the Definition of Knee Injuries.
Nevertheless, it is usually recognized late, namely when high blood pressure cannot be successfully controlled even after therapy with three or more drugs. A reduced potassium level in the blood can also be measured. Although the symptoms do not differ from those of classic high blood pressure, the likelihood of complications such as stroke and heart attack is significantly higher.
Primary hyperaldosteronism is caused by a change in the adrenal cortex. This change affects a very specific zone, namely the zona glomerulosa. The steroid hormone aldosterone is produced in the zona glomerulosa of the adrenal cortex, which ensures reduced sodium excretion and thus raises blood pressure because water is also retained with the sodium. At the same time, it increases potassium excretion.
With normal function of the adrenal cortex and the superordinate glands, the blood pressure is regulated and adapted to the current need. In the case of primary hyperaldosteronism, this control loop no longer works and the adrenal cortex produces too much aldosterone. That is why high blood pressure occurs. The changes mentioned can basically be three pathologies: either bilateral adrenal hyperplasia, an aldosterone-producing adrenal adenoma, or a familial change in the adrenal cortex.
According to current information, 70 percent of cases of primary hyperaldosteronism are based on bilateral adrenal hyperplasia and less than 30 percent on an adenoma. Only in less than 1 percent of cases is the clinical picture genetic.
Symptoms, Ailments & Signs
Primary hyperaldosteronism is often symptomatic of arterial hypertension. Hypokalemia and metabolic alkalosis can also be signs of the disease. However, only rarely do all three of these classic symptoms manifest themselves at once. Serum potassium is also often in the low normal range.
The shift in the potassium level leads, among other things, to fatigue, adynamia, constipation and a mild form of diabetes insipidus, which is characterized by an increased urge to urinate and thirst. The reason for this is that hypokalemia also affects the control circuits of other hormones and these get confused. Metabolic alkalosis, i.e. a shift in blood pH to the alkaline range, is also caused by the reduced potassium level.
Due to the lack of potassium, more potassium ions are shifted from the interior of the cell to the exterior of the cell. This happens in exchange with hydrogen ions. The kidneys also give off hydrogen ions so that they can reabsorb more potassium. Overall, therefore, there is a reduction in the hydrogen ions in the exterior of the cells. As a result, the metabolic situation becomes alkaline.
Diagnosis & course of disease
Diagnosis is based on clinical symptoms and characteristic blood values. High blood pressure is likely to have secondary hypertension like primary hyperkalemia, especially if the patient is very young, i.e. under 30 years of age, or if the high blood pressure starts very suddenly in patients over 55 years of age.
Even if a significant increase in blood pressure suddenly occurs with demonstrably regular intake of medication, a secondary cause of hypertension must be expected. Furthermore, in patients with primary hyperaldosteronism, there is often no physiological drop in blood pressure at night because the hormonal control circuit is disturbed by the overproduction of aldosterone. A possible secondary cause of high blood pressure should always be checked if there is no significant improvement after the use of three antihypertensive drugs.
Blood is taken to confirm the diagnosis. The high aldosterone value, a reduced renin concentration and activity and an increased aldosterone-renin quotient are striking. Hypokalemia and metabolic alkalosis may also be present. The sodium is typically in the high normal range because hypernatraemia is avoided by hormonal counter-regulation.
The three features hypertension, hypokalemia and metabolic alkalosis are responsible for the high risk of complications in primary hyperaldosteronism. Chronic hypertension (high blood pressure) damages the cardiovascular system in the long term and can lead to arteriosclerosis, angina pectoris and heart attacks in the long term. The hypokalemia leads to muscle weakness, paralysis of the smooth muscles, cardiac arrhythmias up to ventricular fibrillation and sometimes even rapid breakdown of the striated muscles (rhabdomyolysis).
Smooth muscle paralysis can block vital body functions. If necessary, the intestinal or bladder muscles are paralyzed, which can lead to bladder paralysis with urine retention or a paralytic intestinal obstruction. Rhabdomyolysis causes severe muscle weakness, muscle pain, nausea, vomiting and fever due to muscle breakdown. In very severe cases, the ischemia can lead to extensive muscle breakdown.
This is an emergency that requires immediate medical attention. In metabolic alkalosis, the blood pH rises above 7.43. This is also a medical emergency that manifests itself in convulsions up to tetany, paresthesia, impaired consciousness and confusion. Death can also occur here if the normal PH value is not restored quickly.
Furthermore, primary hyperaldosteronism is characterized by symptoms such as polydipsia (unquenchable thirst) and polyuria (excretion of large amounts of urine). Despite drinking large amounts, polyuria can lead to exsiccosis (dehydration) of the organism.
When should you go to the doctor?
Disturbances and peculiarities of the cardiovascular system must always be clarified by a doctor. Serious illnesses that require action may be the cause. High blood pressure, tachycardia, internal heat, or persistent restlessness should be presented to a doctor. Interruptions in the night’s sleep, discoloration of the skin and hectic behavior indicate a health impairment. A doctor’s visit is necessary as soon as the symptoms persist or increase.
Digestive disorders are also considered uncommon. If there is constipation or a feeling of pressure in the intestine, a doctor’s visit is necessary. If the person concerned refuses to eat because of the symptoms, a doctor must be consulted. A feeling of pressure in the abdomen, pain or a general feeling of being unwell should be presented to a doctor. Changes in weight, feeling sick, apathy, or a loss of well-being should be evaluated further by a doctor.
A sudden increase in thirst should be understood as a warning signal from the body. If the person affected consumes significantly more liquid than usual for no apparent reason, the observation should be discussed with a doctor. Equally unusual is an increased urge to urinate. This should also be examined.
Disorders of the metabolism or irregularities in the hormonal balance indicate a disease. If there are mood swings, changes in libido, or abnormal behavior, a doctor is needed.
Treatment & Therapy
If the suspicion of primary hyperaldosteronism is confirmed, confirmatory tests are carried out. One is the saline stress test and the other is the fludrocortisone inhibition test. In the sodium chloride stress test, aldosterone secretion is increased by administering water with sodium. In healthy people, this reduces aldosterone secretion.
Fludrocortisone works similarly to aldosterone. If it is administered, the aldosterone level also decreases in healthy patients. In contrast, it remains elevated in persons with primary hyperaldosteronism. Imaging methods such as sonography and computed tomography are then used to confirm the final diagnosis, in which the enlarged adrenal cortex is detected.
Therapeutically, the drug spironolactone is given, which acts like an antagonist to aldosterone and thereby lowers the excessive aldosterone level in the blood. If adrenocortical adenomas are present, they should be surgically removed.
Effective prevention of primary hyperaldosteronism is not possible. However, if typical symptoms of the disease occur, they should be clarified as soon as possible in order to avoid consequential damage.
In the case of primary hyperaldosteronism, follow-up care depends on the therapy with which a patient has been treated. If the therapy consists of treatment with aldosterone blockers, then regular follow-up visits to the doctor are sufficient. It is checked whether the patient’s blood pressure is within a normal range and how the prescribed preparations are tolerated.
If a patient has undergone minimally invasive surgery, the healing of the surgical scar and the change in blood pressure are checked during follow-up examinations. If the doctor finds that the patient’s blood pressure has decreased significantly without medication, no further medication is necessary. The occurrence of new hyperlesions is comparatively low, allowing a patient to lead a normal life.
However, if an adrenal gland has to be completely removed as part of the therapy, a patient may have to take medication for the rest of his life. The preparations should have less of an effect on blood pressure. Instead, the patient receives cortisol preparations to normalize his hormone levels.
In the course of the follow-up examination, it can be determined whether new ulcers have formed in order to be able to remove them at an early stage. Such recurrences are very rare, so most patients can live almost normally after an adrenal gland removal.
You can do that yourself
In patients with this diagnosis, it must first be clarified whether surgical intervention makes sense. Aldosterone levels may return to normal once the adrenal gland that caused the disease is removed.
If primary hyperaldosteronism has another cause, it is usually treated with medication. Patients should ensure that they take the prescribed medication regularly according to the doctor’s instructions, otherwise they will not have the desired effect. Commercially available ACE inhibitors do not work in patients with primary hyperaldosteronism, so other painkillers are indicated for them. Here the doctors advise which medications can have an effect despite an increased aldosterone level and for which pain they are recommended.
Depending on how long it took before the diagnosis could be made, the patient may already have suffered damage from long-term elevated blood pressure or other symptoms of the disease. This damage can be compensated for by a decidedly healthy lifestyle. This includes the patient maintaining a normal weight, not smoking and drinking little alcohol. A healthy diet that includes plenty of fresh fruit, vegetables, sea fish and lean meat is also advisable. It is also important to exercise regularly in the fresh air, especially in order to permanently normalize blood pressure.