Hyperaldosteronism Treatment & Management

  • Author: George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London); Chief Editor: Stephen Kemp, MD, PhD   more...
 
Updated: Mar 4, 2009
 

Medical Care

Surgical excision of the affected adrenal gland is recommended for all patients with hyperaldosteronism who have a proven aldosterone-producing adenoma (APA).

Ensuring good control of blood pressure and replenishment of potassium levels preoperatively is important. The literature on adults indicates that removal of an APA by unilateral adrenalectomy results in normotension in approximately 70% of cases and improves blood pressure control and restores normokalemia in most of the remainder. These rates are likely to be even better in children who have fewer independent factors that predispose to hypertension.

Persistent hypertension despite control of hyperaldosteronism may be the result of coexistent essential hypertension, hypertensive vascular damage secondary to the hyperaldosteronism, or, rarely, another cause of secondary hypertension. Pheochromocytoma and renal artery stenosis have been reported in association with APA.

Postoperative hypoaldosteronism is common. Potassium replacement may produce hyperkalemia in this period. Patients may need supplementation with mineralocorticoids for several months after successful surgery. Immediate postoperative declines in blood pressure may not be sustained.

Idiopathic hyperaldosteronism (IHA)

Although bilateral adrenalectomy corrects hypokalemia in patients with IHA, it has not been shown to be effective at controlling blood pressure. This may be because this condition is typically insidious in its onset, allowing time for chronic hypertension to cause secondary damage. Furthermore, bilateral adrenalectomy commits the patient to lifelong replacement therapy with glucocorticoids and mineralocorticoids.

Control of hypokalemia and hypertension in IHA can be achieved with sodium restriction (to < 2 g/d) and spironolactone or amiloride, but additional antihypertensives are often needed to achieve good control in this patient group. Pediatric drug doses are outlined in the table below. Although spironolactone is an effective aldosterone antagonist, it antagonizes testosterone synthesis and action and can cause hypogonadism with gynecomastia and reduction in libido and erectile dysfunction in pubertal and adult males. Menstrual irregularities are also common in females. For this reason, it should be used with caution in peripubertal children.

Newer alternatives are being produced with better specificity for the mineralocorticoid receptor. Amiloride and triamterene may be used instead of spironolactone. They have a direct effect on the renal tubule to impair sodium reabsorption in exchange for potassium and hydrogen.

Table 1. Drugs Used in the Management of Hyperaldosteronism (Open Table in a new window)

DrugClassPediatric Dose
SpironolactoneAldosterone antagonist0-10 kg: 6.25 mg/dose PO q12h



11-20 kg: 12.5 mg/dose PO q12h



21-40 kg: 25 mg/dose PO q12h



>40 kg: 25 mg PO q8h



Potassium canrenoateAldosterone antagonist3-8 mg/kg IV qd; not to exceed 400 mg
AmiloridePotassium-sparing diuretic0.2 mg/kg q12h
TriamterenePotassium-sparing diuretic2 mg/kg/dose q8-24h
NifedipineDihydropyridine calcium channel antagonist0.25-0.5 mg/kg PO q6-8h
AmlodipineCalcium channel antagonist0.05-0.2 mg/d PO
DoxazosinAlpha1-specific adrenergic antagonist0.02-0.1 mg/d; not to exceed 4 mg
PrazosinAlpha1-specific adrenergic antagonist0.005 mg/kg test dose, then 0.025-0.1 mg/kg/dose q6h; not to exceed 0.5 mg/dose

Glucocorticoid-remediable hyperaldosteronism (GRA)

In adult patients with glucocorticoid-remediable aldosteronism (GRA), control of hypertension can be achieved by treatment with physiologic doses of dexamethasone. However, in children, avoiding dexamethasone is best because of its adverse effects on growth and bone density. Hydrocortisone is a better choice because of its short half-life (typical dose is 10-12 mg/m2), but it is not as efficient at reducing mineralocorticoid levels.

Children receiving long-term treatment with glucocorticoids require consultation by a pediatric endocrinologist. GRA is associated with intracranial aneurysm and hemorrhagic stroke, and screening for intracranial aneurysms in patients with proven GRA is recommended. Amiloride and spironolactone have also been used as monotherapy for treating GRA.

Familial hyperaldosteronism type II (FH-II)

Patients with FH-II should be regularly observed, and treatment should be started when they develop hypertension. Treatment is with the same agents as for IHA. In the event that patients develop an adenoma, adrenal venous sampling should be considered to confirm lateralization of aldosterone hypersecretion before surgical removal.

In cases where gradient is lacking, medical treatment is recommended, with regular monitoring. Because patients with FH-II are not at increased risk of carcinoma, nonsurgical management may be worth considering.

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Surgical Care

Laparoscopic adrenalectomy significantly reduces operative morbidity with a substantially shorter hospital stay and reduced blood loss compared with an open approach.

A limited number of cases of isolated adenomectomy with preservation of the remaining normal adrenal tissue have been reported.

Transcatheter arterial ablation with high-concentration ethanol injection of APA has been reported.

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Consultations

Endocrinology

Once screening indicates a possible diagnosis of hyperaldosteronism, referral to an endocrinologist is recommended for further assessment and management because numerous causes of primary aldosteronism in children and adolescents can be managed medically.[7]

Cardiology

Patients with severe or long-standing hypertension may require assessment by a cardiologist because hyperaldosteronism may lead to myocardial fibrosis. This problem is more likely to occur in adults in whom the duration of disease is much greater.

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Diet

Patients being evaluated for hyperaldosteronism should be receiving a high-sodium intake as described above. Adult recommendations are for a sodium intake of 10 g/d or more. This amount can be reduced proportionately for children, depending on their size. Regular monitoring of potassium is important when increasing sodium in patients with suspected hyperaldosteronism because this may unmask hypokalemia.

Medical management of patients with established hyperaldosteronism should include salt restriction. This should include not adding salt to cooking and not having salt on the table. Ideally, patients should receive fewer than 2 g of sodium chloride a day. Problems with compliance may occur because this degree of restriction is often unpalatable to children.

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Activity

Patients with significant hypertension should be advised to avoid strenuous activity until blood pressure is under control because strenuous activity may further exacerbate their problem.

The type of surgery that has been performed governs postoperative activity. Patients should avoid bathing or wetting their wounds until they have healed. Patients who have undergone laparotomy must avoid heavy lifting for 6 weeks after their operation. Patients who have undergone laparoscopic adrenalectomy need only restrict their activity while they are sore or the wound has not healed.

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Contributor Information and Disclosures
Author

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London)  Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece; UNESCO Chair on Adolescent Health Care, University of Athens, Athens, Greece

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) is a member of the following medical societies: American Academy of Pediatrics, American College of Endocrinology, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Antony Lafferty, MB ChB, FRACP  Senior Lecturer of Pediatric Endocrinology, Monash University Department of Pediatrics, National Institutes of Health, Bethesda, MD, and Princess Margaret Hospital for Children, Perth, Western Australia

Antony Lafferty, MB ChB, FRACP is a member of the following medical societies: Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Thomas A Wilson, MD  Professor of Clinical Pediatrics, Chief and Program Director, Division of Pediatric Endocrinology, Department of Pediatrics, The School of Medicine at Stony Brook University Medical Center

Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Pediatric Endocrine Society, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Lynne Lipton Levitsky, MD  Chief, Pediatric Endocrine Unit, Massachusetts General Hospital; Associate Professor of Pediatrics, Harvard Medical School

Lynne Lipton Levitsky, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Diabetes Association, American Pediatric Society, Endocrine Society, Pediatric Endocrine Society, and Society for Pediatric Research

Disclosure: Pfizer Grant/research funds P.I.; Tercica Grant/research funds Other; Eli Lily Grant/research funds PI; NovoNordisk Grant/research funds PI

Merrily P M Poth, MD  Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences

Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Pediatric Endocrine Society

Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD  Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas College of Medicine and Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, and Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

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Steroid biosynthetic pathway.
Physiologic regulation of the renin-angiotensin-aldosterone axis.
Table 1. Drugs Used in the Management of Hyperaldosteronism
DrugClassPediatric Dose
SpironolactoneAldosterone antagonist0-10 kg: 6.25 mg/dose PO q12h



11-20 kg: 12.5 mg/dose PO q12h



21-40 kg: 25 mg/dose PO q12h



>40 kg: 25 mg PO q8h



Potassium canrenoateAldosterone antagonist3-8 mg/kg IV qd; not to exceed 400 mg
AmiloridePotassium-sparing diuretic0.2 mg/kg q12h
TriamterenePotassium-sparing diuretic2 mg/kg/dose q8-24h
NifedipineDihydropyridine calcium channel antagonist0.25-0.5 mg/kg PO q6-8h
AmlodipineCalcium channel antagonist0.05-0.2 mg/d PO
DoxazosinAlpha1-specific adrenergic antagonist0.02-0.1 mg/d; not to exceed 4 mg
PrazosinAlpha1-specific adrenergic antagonist0.005 mg/kg test dose, then 0.025-0.1 mg/kg/dose q6h; not to exceed 0.5 mg/dose
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