Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment
Publication Date: May 1, 2016
Last Updated: March 2, 2023
Recommendations
Case detection
We recommend case detection of primary aldosteronism (PA) in patients with sustained blood pressure (BP) above 150/100 mm Hg on each of three measurements obtained on different days, with hypertension (BP >140/90 mm Hg) resistant to three conventional antihypertensive drugs (including a diuretic), or controlled BP (<140/90 mm Hg) on four or more antihypertensive drugs; hypertension and spontaneous or diuretic-induced hypokalemia; hypertension and adrenal incidentaloma; hypertension and sleep apnea; hypertension and a family history of early onset hypertension or cerebrovascular accident at a young age (<40 years); and all hypertensive first-degree relatives of patients with PA. (1-L)
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We recommend using the plasma aldosterone/renin ratio (ARR) to detect possible cases of PA in these patient groups. (1-M)
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Case confirmation
Instead of proceeding directly to subtype classification, we recommend that patients with a positive ARR undergo one or more confirmatory tests to definitively confirm or exclude the diagnosis. (1-L)
However, in the setting of spontaneous hypokalemia, plasma renin below detection levels plus plasma aldosterone concentration (PAC) >20 ng/dL (550 pmol/L), we suggest that there may is no need for further confirmatory testing.
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Subtype classification
We recommend that all patients with PA undergo adrenal computed tomography (CT) as in the initial study in subtype testing to exclude large masses that may represent adrenocortical carcinoma and to assist the interventional radiologist and surgeon where anatomically appropriate. (1-M)
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We recommend that when surgical treatment is feasible and desired by the patient, an experienced radiologist should use adrenal venous sampling (AVS) to make the distinction between unilateral and bilateral adrenal disease. (1-M)
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Younger patients (<age 35 years) with spontaneous hypokalemia, marked aldosterone excess, and unilateral adrenal lesions with radiological features consistent with a cortical adenoma on adrenal CT scan may not need AVS before proceeding to unilateral adrenalectomy.
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In patients with an onset of confirmed PA earlier than 20 years of age and in those who have a family history of PA or stroke at a young age (<40 years), we suggest genetic testing for familial hyperaldosteronism type 1 (FH-I) (glucocorticoid remediable aldosteronism [GRA]). (2-L)
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In very young patients with PA, we suggest testing for germline mutations in KCNJ5 causing familial hyperaldosteronism type 3 (FH-III). (2-VL)
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Treatment
We recommend unilateral laparoscopic adrenalectomy for patients with documented unilateral PA (ie, aldosterone-producing adenoma [APA] or unilateral adrenal hyperplasia [UAH]). (1-M)
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If a patient is unable or unwilling to undergo surgery, we recommend medical treatment including a mineralocorticoid receptor (MR) antagonist. (1-M)
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If an ARR-positive patient is unwilling or unable to undergo further investigations, we similarly recommend medical treatment including an MR antagonist. (1-L)
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In patients with PA due to bilateral adrenal disease, we recommend medical treatment with an MR antagonist. (1-L)
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We suggest spironolactone as the primary agent, with eplerenone as an alternative. (2-VL)
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In patients with GRA, we recommend administering the lowest dose of glucocorticoid to lower ACTH and thus normalize BP and potassium levels as the first-line treatment. (1-VL)
In addition, if BP fails to normalize with glucocorticoid alone, an MR antagonist may be added. For children, the glucocorticoid dosage should be adjusted for age and body weight, and BP targets should be determined from age- and gender-specific published normative data.
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Title
Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment
Endorsing Organizations
American Association of Endocrine Surgeons
American Heart Association
Publication Month/Year
May 1, 2016
Last Updated Month/Year
June 1, 2023
External Publication Status
Published
Country of Publication
US
Document Objectives
To develop clinical practice guidelines for the management of patients with primary aldosteronism.
Inclusion Criteria
Female, Male, Adolescent, Adult, Child, Older adult
Health Care Settings
Ambulatory
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Assessment and screening, Diagnosis, Management, Treatment
Diseases/Conditions (MeSH)
D000450 - Aldosterone
Keywords
primary aldosteronism, PA
Source Citation
John W. Funder, Robert M. Carey, Franco Mantero, M. Hassan Murad, Martin Reincke, Hirotaka Shibata, Michael Stowasser, William F. Young, Jr, The Management of Primary Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline, The Journal of Clinical Endocrinology & Metabolism, Volume 101, Issue 5, 1 May 2016, Pages 1889–1916, https://doi.org/10.1210/jc.2015-4061