Identification and implications of new types of mineralocorticoid hypertension

Abstract

200 patients with mineralocorticoid hypertension were studied at the Clinical Study Center. The study of 150 patients with primary aldosteronism revealed five distinct subsets based on their responses to the upright posture, after administration of intravenous saline, deoxycorticosterone acetate, and spironolactone. Two new types were identified—aldosterone producing responsive adenoma (AP-RA) and primary adrenal hyperplasia (PAH). Patients with AP-RA maintained normal physiologic responses to the above maneuvers. Patients with PAH had responses similar to patients with an aldosterone producing adenoma (APA) but no tumor was identified. Both types were cured by unilateral adrenalectomy. There has been no change in subtype in up to 20 years of follow-up. The notion of a continuum from low renin hypertension to APA is not supported. Primary deoxycorticosteronism caused by a benign adrenal adenoma, malignancy and hyperplasia is described. Uniquely, overproduction of the 17-deoxysteroids of the zona fasciculata occurs with normal 17-hydroxy function. After the removal of a benign adenoma the contralateral adrenal gland revealed a delay in the 17-deoxysteroid responses to ACTH in the face of normal cortisol increases. This suggests that an independent pituitary regulator of the 17-deoxypathway may exist. Other hypertensive disorders with excessive deoxycorticosterone production are linked with increases of ACTH and cortisol levels. The hallmarks of primary deoxycorticosteronism are hypertension with hypokalemia, suppression of renin and aldosterone, and overproduction of the 17-deoxysteroids.