What is the treatment for Familial Hyperaldosteronism (Primary Aldosteronism)?

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From the Guidelines

The treatment of familial hyperaldosteronism should focus on controlling blood pressure and normalizing potassium levels, with the specific approach depending on the type of familial hyperaldosteronism, as outlined in the 2024 ESC guidelines for the management of elevated blood pressure and hypertension 1.

Treatment Approach

The management of familial hyperaldosteronism depends on its subtype. For familial hyperaldosteronism type 1, also known as glucocorticoid-remediable primary aldosteronism, low-dose dexamethasone is the primary treatment, as it suppresses ACTH production and reduces aldosterone synthesis.

Medical Treatment

For other types of familial hyperaldosteronism, mineralocorticoid receptor antagonists (MRAs) are the mainstay of treatment, with spironolactone being the most widely available MRA 1. The effective dose of spironolactone is usually 50–100 mg once daily and can be titrated up to 300–400 mg once daily if necessary. Eplerenone is also used, particularly if spironolactone causes side effects like gynaecomastia and erectile dysfunction in men.

Surgical Intervention

In cases with unilateral adrenal hyperplasia or adenoma, laparoscopic adrenalectomy may be curative, unless the patient is older or has co-morbidities of concern 1.

Monitoring and Counseling

Regular monitoring of blood pressure, serum potassium, and aldosterone levels is essential to assess treatment efficacy. Genetic counseling should be offered to affected families, as these conditions are inherited in an autosomal dominant pattern.

Additional Considerations

Additional antihypertensive medications such as calcium channel blockers or ACE inhibitors may be needed to achieve blood pressure control. Potassium supplementation is often required initially to correct hypokalemia. Newer agents, such as the non-steroidal MRAs finerenone and exarenone, and the aldosterone synthase inhibitor baxdrostat, are also being tested for treating primary aldosteronism 1.

From the FDA Drug Label

2.5 Treatment of Primary Hyperaldosteronism Administer spironolactone tablets in doses of 100 mg to 400 mg daily in preparation for surgery. For patients who are considered unsuitable for surgery, spironolactone tablets can be used as long-term maintenance therapy at the lowest effective dosage determined for the individual patient.

1.4 Primary Hyperaldosteronism Spironolactone tablets are indicated in the following settings: Short-term preoperative treatment of patients with primary hyperaldosteronism. Long-term maintenance therapy for patients with discrete aldosterone-producing adrenal adenomas who are not candidates for surgery Long-term maintenance therapy for patients with bilateral micro or macronodular adrenal hyperplasia (idiopathic hyperaldosteronism).

The treatment of Familial hyperaldosteronism is not directly addressed in the provided drug labels. However, Primary Hyperaldosteronism is mentioned, and it can be inferred that spironolactone may be used in similar cases.

  • The recommended dosage for Primary Hyperaldosteronism is 100 mg to 400 mg daily.
  • Spironolactone can be used for short-term preoperative treatment or long-term maintenance therapy. However, since Familial hyperaldosteronism is not explicitly mentioned, this answer should be taken with caution and considered as not directly supported by the FDA drug label [2] [3].

From the Research

Treatment Options for Familial Hyperaldosteronism

  • The treatment of familial hyperaldosteronism depends on the specific type of the disease 4.
  • Glucocorticoid-remediable aldosteronism (GRA) or familial hyperaldosteronism type I (FH-I) can be treated with exogenous glucocorticoids such as dexamethasone in physiologic range doses 4.
  • Familial hyperaldosteronism type II (FH-II) is characterized by autosomal dominant inheritance of autonomous aldosterone hypersecretion which is not suppressible by dexamethasone, and its treatment is similar to that of bilateral primary aldosteronism, mainly with mineralocorticoid receptor antagonists 4, 5.
  • Mineralocorticoid receptor antagonists, such as spironolactone or eplerenone, are used to treat bilateral adrenal hyperplasia and familial hyperaldosteronism type II 6, 5, 7.
  • New therapeutic approaches, including the use of finerenone, a novel mineralocorticoid receptor antagonist, are being explored for the treatment of primary aldosteronism and may be applicable to familial hyperaldosteronism 8.

Surgical Treatment

  • Unilateral disease, such as aldosterone-producing adenoma, can be treated with adrenalectomy, which offers the possibility of cure 6, 5, 7.
  • Adrenal venous sampling is key for reliable subtype identification and can help determine the best course of treatment 5, 7.

Medical Management

  • The goals of treatment are to normalize both blood pressure and excessive aldosterone production, and to reduce associated comorbidities, improve quality of life, and reduce mortality 5.
  • Treatment should be closely monitored by serum electrolyte and creatinine controls, especially when starting medication 6.
  • Additional treatment with potassium-sparing diuretics or calcium channel antagonists may be necessary if blood pressure is not normalized by first-line treatment 6.

References

Research

Familial hyperaldosteronism.

Brazilian journal of medical and biological research = Revista brasileira de pesquisas medicas e biologicas, 2000

Research

Diagnosis and treatment of primary aldosteronism.

The lancet. Diabetes & endocrinology, 2021

Research

Treatment of primary aldosteronism.

Best practice & research. Clinical endocrinology & metabolism, 2010

Research

Primary aldosteronism: Treatment of the disease, and new therapeutic approaches.

Best practice & research. Clinical endocrinology & metabolism, 2020

Research

Real-world outcomes of finerenone in primary aldosteronism.

European journal of endocrinology, 2025

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.