Primary hyperaldosteronism resident survival guide

Template:Primary hyperladosteronism Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Syed Hassan A. Kazmi BSc, MD [2]

Overview

Causes

Common Causes

Common causes of Conn's Syndrome may be divided into:

Adrenal causes:
- Aldosterone-secreting adrenal adenoma (APA-benign tumor, 50-60%)
- Idiopathic hyperaldosteronism (IHA-Bilateral hyperplasia of the adrenal gland, 40-50%)
Extra-adrenal causes
- Ectopic secretion of aldosterone (Ovaries and Kidneys)

Less Common Causes

Familial hyperaldosteronism type I (glucocorticoid-remediable aldosteronism [GRA])
Familial hyperaldosteronism II (the familial occurrence of APA or bilateral idiopathic hyperplasia or both)
Familial hyperaldosteronism type III (associated with the germline mutation in the KCNJ5 potassium channel)
Pure aldosterone-producing adrenocortical carcinomas
Unilateral adrenal hyperplasia

Diagnosis and Treatment

Preferred screening population:

• Blood pressure > 160 / 100 particularly (< 50 years)
• Resistant hypertension or refractory hypertension (use of > 3 anti-hypertensives and poor control of blood pressure)
• Hypokalemia (provoked by diuretic therapy or unprovoked)
• Hypertension and incidentally discovered adrenal adenoma
• Hypertension with a family history of early-onset hypertension (< 20 years) or cerebrovascular accident at age less than 40 years

• Hypertensive first-degree relatives of patients with PA

Plasma Renin Activity/Aldosterone Ratio

Normal or High Renin (Plasma Renin/Aldosterone ratio <10

Suppressed Renin (Plasma Renin/Aldosterone ratio >20

•Renin-secreting tumors
•Diuretic use
•Renovascular hypertension
•Coarctation of aorta
•Malignant phase hypertension

Urinary aldosterone

Elevated

Normal

Low

Conn's syndrome (Primary aldosteronism)

Profound K+ depletion

• 17 alpha hydroxylase deficiency
• 11 beta hydroxylase deficiency
• Liddle's syndrome
• Licorice ingestion
•Deoxycorticosterone producing tumor

Do confirmatory tests:
•Fludrocortisone supression test (Positive test= Aldosterone > 6 ng / dl and simultaneous PRA levels < than 1.0 ng / ml / hour)
OR
•Intravenous saline load testing (Positive test= Aldosterone more than 10 ng / dl)
OR
•Oral sodium loading test (Positive test= 24-hour urinary aldosterone excretion > 12 μg / day
OR
•Captopril challenge test (Positive test= PAC / PRA > 30, measured two hours after the administration of 25 mg or 50 mg of captopril)

Add Mineralocrticoid antagonist for 8 weeks

BP response

No BP response

Subtype classification

• Deoxycorticosterone excess( Tumor, 17 alpha hydroxylase and 11 beta hydroxylase deficiency)
• Licorice ingestion
•Glucocorticoid resistance

Liddle's syndrome)

Adrenal CT scan

Normal, micornodularity, bilateral masses or unilateral atypical mass

Unilateral hypodense nodule >1cm and <2cm in the setting of marked primary hyperaldosteronism

Surgery not desired

Surgery desired

Surgery not desired

> 35 years consider

< 35 years consider

• Glucocorticoid-remediable aldosteronism (GRA) OR Idiopathic hyperaldosteronism:
• Pharmacologic therapy

Adrenal Venous Sampling

Aldosterone Producing Adenoma (APA) or Primary Adrenal Hyperplasia (PAH):
Unilateral laproscopic adrenalectomy

Pharmacological therapy