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Conn’s Syndrome: Causes, Symptoms, Treatment
Conn’s Syndrome is a rare disease in which the adrenal glands make too much of a hormone (chemical messenger) called aldosterone.
Aldosterone is a hormone that controls sodium and potassium levels in the blood. Too much aldosterone leads to high blood pressure and low blood potassium levels (hypokalaemia). It is also known as primary hyperaldosteronism.
Some cases require surgery. It is one of the most treatable causes of high blood pressure; and most people have an excellent outlook with treatment.
What You Should Know about Adrenal Glands
The adrenal glands are located on top of each kidney. They are a small, triangular-shaped organs, about 5 cm long, 2-3 cm wide, and 1 cm thick – and weigh 4-5 grams.
The left adrenal gland is often slightly larger than the right. Each adrenal gland has two layers:
- Adrenal medulla (inner part) makes epinephrine (also called adrenaline) and many other similar hormones.
- Adrenal cortex (outer part) makes steroid hormones (such as cortisol and aldosterone).
Here are 10 key facts about Conn’s Syndrome.
1. Definition
- Conn’s Syndrome, also known as primary aldosteronism, is a rare endocrine disorder characterised by excessive production of aldosterone, a hormone produced by the adrenal glands.
- This abnormality is a paraneoplastic syndrome.
- 5-10% of all secondary cases of high blood pressure (i.e. where there is an underlying cause) are caused by Conn’s Syndrome.
- It is more common in females than in males. It can happen at any age, but more often in people in their 30s and 40s.
- Adenomas tend to occur in younger women, and bilateral hyperplasia in older men.
2. Causes
- Benign (noncancerous) tumour in one or both adrenal glands (unilateral aldosterone-producing adenoma). This is the commonest cause (35%).
- Bilateral adrenal hyperplasia (overgrowth of both adrenal glands)
- Familial hyperaldosteronism, e.g. like congenital adrenal hyperplasia (CAH). This is rare type that can run in families.
- Adrenal cancer (rare).
3. Risk Factors
Anyone can develop primary aldosteronism. But it’s more common in people with:
- Low blood potassium levels.
- High blood pressure starting before age 30.
- High blood pressure requiring three or more medications to manage.
- An adrenal tumour.
- Family history of hypertension or endocrine disorders, and hypokalaemia (low potassium levels).
4. Symptoms
- Common symptoms include hypertension, headaches, muscle weakness, fatigue; and potassium deficiency symptoms like muscle cramps and palpitations.
- Alternatively, there may be no symptoms. The diagnosis being picked up in the investigation of someone with high blood pressure – especially a young patient.
- There may be nothing to find on examination, other than high blood pressure.
5. Diagnosis
- Excess production of the hormone aldosterone from the adrenal glands, results in high blood pressure, low renin and potassium levels in the blood, and metabolic alkalosis (high alkali levels).
- Some patients will have high sodium levels as well.
- Aldosterone levels (and aldosterone-to-renin ratio) are needed to confirm primary aldosteronism; some require saline suppression testing and/or other confirmatory tests.
- Imaging inlcuding ultrasound, CT and MRI may be necssary.
- If patients with an adrenal mass are found to have a high aldosterone level on a blood test, a special test known as adrenal vein sampling must be done before the adrenal gland with the mass is surgically removed; since in upto 1/3 of patients the problem can stem from both adrenal glands or just the opposite adrenal gland.
6. Treatment
- Treatment options include surgical removal of an aldosterone-producing adenoma.
- This is usually done using a minimally invasive (laparoscopic) approach.
- Other patients require medical management with mineralocorticoid receptor antagonists (e.g. spironolactone or eplerenone, which block the effects of aldosterone), e.g. for bilateral adrenal hyperplasia or those not suitable for surgery.
7. Complications
- Untreated Conn’s Syndrome can lead to cardiovascular complications like stroke, heart failure, and chronic kidney disease (CKD) due to prolonged hypertension and hypokalemia.
8. Prognosis
- With appropriate treatment, blood pressure can often be controlled, and complications can be minimised. Surgical intervention can be curative in cases of unilateral adenoma.
9. Prevention
- There’s no specific prevention strategy, but early detection and treatment of hypertension and regular monitoring of potassium levels can help identify cases earlier.
10. Referral
- Referral to an endocrinologist is recommended for patients with suspected Conn’s Syndrome for further evaluation and management.
Interesting History
Dr Jerome Conn described a young man with hypertension and low potassium levels caused by excess aldosterone secretion from a small adrenal tumour.
