Pathophysiology and histopathology of primary aldosteronism

Primary aldosteronism (PA) can be sporadic or familial and classified into unilateral and bilateral forms. Sporadic PA predominates with excessive aldosterone production usually arising from a unilateral aldosterone-producing adenoma (APA) or bilateral adrenocortical hyperplasia. Familial PA is rare and caused by germline variants, that partly correspond to somatic alterations in APAs. Classification into unilateral and bilateral PA determines the treatment approach but does not accurately mirror disease pathology. Some evidence indicates a disease continuum ranging from balanced aldosterone production from each adrenal to extreme asymmetrical bilateral aldosterone production. Nonetheless, surgical removal of the overactive adrenal in unilateral PA achieves highly successful outcomes and almost all patients are biochemically cured of their aldosteronism.