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Conn Syndrome (Primary Hyperaldosteronism)

Background Information

Definition

Conn syndrome (primary hyperaldosteronism) is defined by the autonomous overproduction fo aldosterone in 1 or both adrenal glands

Aetiology

There are 2 main causes: [Ref]

  • Bilateral adrenal hyperplasia (idiopathic) – most common
  • Aldosterone-producing adenoma – 2nd most common

Clinical Features

Main clinical features: [Ref]

  • Drug-resistant hypertension
    • Conn syndrome is the most common endocrine cause of secondary hypertension

 

  • Features of hypokalaemia
    • Fatigue
    • Muscle cramping / weakness
    • Paraesthesia
    • Polyuria and polydipsia
    • Constipation

Diagnosis

Non-Diagnostic Tests

Key biochemical changes: [Ref]

  • Hypernatraemia
  • Hypokalaemia
  • Metabolic alkalosis

Actions of aldosterone:

  • ↑ Sodium and water retention
  • ↑ Potassium excretion
  • ↑ Acid excretion

By learning the actions of aldosterone, you would have learned the biochemical changes in both Conn syndrome (primary hyperaldosteronism) and adrenal insufficiency.

Endocrine Tests

1. Confirming Conn Syndrome

1st line: measure serum aldosterone and renin activity [Ref]

  • ↑ Aldosterone + ↓ renin is suggestive of Conn syndrome
  • NB if both renin and aldosterone are raised, that is likely secondary hyperaldosteronism

Confirmatory test (not routinely performed): saline suppression test [Ref]

  • If saline load failed to suppress aldosterone levels → Conn syndrome is very likely

2. Identifying Underlying Cause

The 2 main causes to differentiate between are 1) unilateral aldosterone-producing adrenal adenoma and 2) bilateral adrenal hyperplasia.

Choice of test: [Ref]

  • 1st line: CT adrenals
  • Gold standard: adrenal venous sampling (definitively differentiates between unilateral and bilateral aldosterone overproduction)

Management

Management

Management depends on the underlying cause: [Ref]

Underlying cause Management
Aldosterone-producing adrenal adenoma
  • 1st line: unilateral laparoscopic adrenalectomy 
  • 2nd line (e.g. poor surgical candidate): MRA (e.g. spironolactone, eplerenone)
Bilateral adrenal hyperplasia
  • 1st line: MRA (e.g. spironolactone, eplerenone)
Bilateral adrenalectomy should be avoided if possible, as it puts the patient in permanent adrenal insufficiency, which requires lifelong steroid replacement and risk of adrenal crisis.

Therefore, it makes sense to only offer surgery in a unilateral cause (i.e. aldosterone-producing adrenal adenoma), but avoid surgery in a bilateral cause (i.e. adrenal hyperplasia).

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