What is the role of pharmacologic therapy for familial hyperaldosteronism type III (FH-III)?

Updated: Oct 19, 2018
  • Author: George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London); Chief Editor: Robert P Hoffman, MD  more...
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Answer

The clinical spectrum of FH-III widely varies. Hence, some patients may benefit from medical treatment, whereas others require bilateral adrenalectomy due to resistance to aggressive antihypertensive therapy, including aldosterone receptor blockade and amiloride.

Patients with APAs and gain of function mutations in CACNA1D can respond to treatment with a calcium channel blocker. Approved calcium channel blockers are weak antagonists of wild type CaV1.3, although potent and specific CaV1.3 inhibitors have been identified. [48] This type of compound might be useful in patients with KCNJ5 mutations because the latter leads to aldosterone production through increased calcium influx. [16] Data have shown that a number of dihydropyridine calcium channel blockers also have mineralocorticoid receptor antagonist activity at high doses, suggesting that these agents may target multiple mechanisms in control of hypertension.


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