Primary hyperaldosteronism resident survival guide

Primary hyperaldosteronism Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Primary Hyperaldosteronism from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic study of choice
History and Symptoms
Physical Examination
Laboratory Findings
CT scan Findings
MRI Findings
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Case Studies
Case #1
Primary hyperaldosteronism resident survival guide On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Primary hyperaldosteronism resident survival guide All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Primary hyperaldosteronism resident survival guide
CDC on Primary hyperaldosteronism resident survival guide
Primary hyperaldosteronism resident survival guide in the news
Blogs on Primary hyperaldosteronism resident survival guide
Directions to Hospitals Treating Conn syndrome
Risk calculators and risk factors for Primary hyperaldosteronism resident survival guide

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

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

The Management of Primary hyperaldosteronism including case detection, diagnosis, and treatment according to the "Endocrine Society Clinical Practice Guidlines" is as follows:

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

Preferred screening population

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

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 desired

Surgery not desired

> 35 years consider

< 35 years consider

Glucocorticoid-remediable aldosteronism (GRA) OR Idiopathic hyperaldosteronism (bilateral hyperplasia): • Preferred regimen-Spironolactone 12.5mg to 25mg QD or Eplerenone 25mg BID • Alternative regimen-Amiloride 5mg BID plus chlorthalidone/hydrochlorothiazide 12.5mg to 25mg QD

No Lateralization with Adrenal Venous Sampling

Lateralization with Adrenal Venous Sampling

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

• Preferred regimen-Spironolactone 12.5mg to 25mg QD or Eplerenone 25mg BID • Alternative regimen-Amiloride 5mg BID plus chlorthalidone/hydrochlorothiazide 12.5mg to 25mg QD