Primary aldosteronism: diagnostic and treatment strategies

Abstract

Primary aldosteronism is caused by bilateral idiopathic hyperplasia in approximately two-thirds of cases and aldosterone-producing adenoma in one-third. Most patients with primary aldosteronism are normokalemic. In the clinical setting of normokalemic hypertension, patients who have resistant hypertension and hypertensive patients with a family history atypical for polygenic hypertension should be tested for primary aldosteronism. The ratio of plasma aldosterone concentration to plasma renin activity has been generally accepted as a first-line case-finding test. If a patient has an increased ratio, autonomous aldosterone production must be confirmed with an aldosterone suppression test. Once primary aldosteronism is confirmed, the subtype needs to be determined to guide treatment. The initial test in subtype evaluation is CT imaging of the adrenal glands. If surgical treatment is considered, adrenal vein sampling is the most accurate method for distinguishing between unilateral and bilateral adrenal aldosterone production. Optimal treatment for aldosterone-producing adenoma or unilateral hyperplasia is unilateral laparoscopic adrenalectomy. The idiopathic bilateral hyperplasia and glucocorticoid-remediable aldosteronism subtypes should be treated pharmacologically. All patients treated pharmacologically should receive a mineralocorticoid receptor antagonist, a drug type that has been shown to block the toxic effects of aldosterone on nonepithelial tissues.

Aldosterone-to-renin ratio and home blood pressure in subjects with higher and lower sodium intake: the Ohasama Study

“…At enrollment, 62 participants (12.1%) were current smokers, 182 (35.4%) reported intake of alcohol, 139 (27.0%) had hypercholesterolemia, 33 (6.4%) had diabetes mellitus and 19 (3.7%) had a history of cardiovascular disease. Two subjects (0.4%) had ARR X20 ng per 100 ml per ng ml -1 h -1 in the presence of PAC X15 ng per 100 ml, which was the cutoff value to screen for primary aldosteronism; 24,25 however, neither subject had hypertension or hypokalemia. The mean number of measurements for HBP was 23.2±6.0, and the mean systolic/diastolic HBP and CBP values of subjects were 117.2±13.6/73.2±8.8 and 128.9±13.1/ 72.1 ± 8.9 mm Hg, respectively.…”

Increasing Incidence of Primary Aldosteronism in Patients Investigated for Resistant Hypertension: An Observational Study from a Nephrology Unit

“…Also, there is a lack of consensus on the types of patients' populations in which the ARR should be used as screening test for PA and on clear cut-off values to adopt. For example, Montori and Young, in a systematic review of 16 studies (for a total of 3,136 patients), reported that the thresholds for the ARR used varied between 7.2 and 100.0 ng/dL per ng/mL per hour [34,35].…”

“…Less common forms are the Unilateral Adrenal Hyperplasia (UAH), that is the micro-or macronodular hyerplasia of the zona glomerulosa of the adrenal cortex, mainly involving one of the adrenal glands (3%); and the bilateral adrenal hyperplasia, micro-or macronodular (1%) [3,4] Even if sporadic, PA may be familial and manifests in at least two forms [5,6]: the Familial Hyperaldosteronism type I (FH-I), or Glucocorticoid-Remediable Aldosteronism (GRA), very rare (< 1%), which has an autosomal dominant mode of transmission and is caused by an adrenocorticotropic hormone-regulated, hybrid CYP11B1/ CYP11B2 gene [7,8]; and the familial hyperaldosteronism type II (FH-II), which is the familial form of APA or BAH or both. FH-II relative frequency is unknown, but it is considered to be at least 5 times higher than that of FH-I.…”

Diagnosis dan Tata Laksana Sindrom Conn/Hiperaldosteronisme Primer: Sebuah Studi Kasus

“…5 Bentuk yang umum dari hiperaldosteronisme primer adalah idiopathic bilateral hyperplasia (IHA) dan aldosterone-producing adenoma (APA). 6 Hiperplasia adrenal unilateral jarang ditemui, namun diagnosis ini dapat dipertimbangkan bila tes lateralisasi, seperti adrenal venous sampling (AVS) positif, tetapi pemeriksaan histologi dan radiologi tidak mendeteksi adanya suatu adenoma. Sekitar 1% pasien hiperaldosteronisme primer merupakan kasus karsinoma adrenal yang memproduksi aldosteron.…”