Hyperaldosteronism: types, clinical characteristics, and treatment

Cases with hyperaldosteronism might be asymptomatic in many events. Therefore, the diagnosis can be missed. However, the usual presentation for many patients has been reported to be a refractory elevation in the blood pressure which might be mild to severe. Based on the type of hyperaldosteronism and the diagnosis, the treatment of these conditions should be established. Therefore, it can be concluded that the treatment is specific to the management of the underlying etiology, and managing the clinical characteristics and associated complications. This present literature review aims to provide evidence regarding the types, clinical characteristics, and treatment of aldosterone based on data from the current investigations in the literature. Different clinical phenotypes have been reported for the condition. Nevertheless, the disease can be broadly classified into primary and secondary hyperaldosteronism based on the pathophysiology and etiology of the condition. Clinical characteristics might not be diagnostic since they are very non-specific, despite being common in these patients, as hypokalemia and hypertension. Therefore, clinicians should be aware of conducting the necessary diagnostic approaches before establishing the diagnosis. Management of these patients requires the integration of different approaches, including surgical and medical treatment. Perioperative care is important because it may lead to unfavorable consequences if neglected.

Prognostic value of secondary hyperaldosteronism in patients with chronic heart failure with preserved ejection fraction

Purpose: to investigate the prognostic value of secondary hyperaldosteronism patients with heart failure with preserved ejection fraction. Materials and methods: prospective cohort study included 158 patients with hyperaldosteronism and heart failure with preserved ejection fraction. Baseline blood aldosterone levels were determined in all patients. Hyperaldosteronemia was diagnosed when the plasma aldosterone level was > 160 pg/ml. The primary endpoint was all-cause mortality. Results: at baseline, hyperaldosteronemia was detected in 59 of 158 patients (37.3%). Hyperaldosteronemic patients were younger, had higher functional class and NT-proBNP level, and a higher rate of comorbidity (all Ps <0.05). Over a median follow‐up of 32 (28-38) months, a total of 50 (37.6%) patients died. Cardiovascular death occurred in 32 (20.3%) cases, non-cardiovascular – in 18 (11.4%) cases. A total of 65 (41.1%) patients were hospitalized for HF. High aldosterone levels were associated with a significant (p <0.05) increase in the risk of hospitalization for HF (adjusted odds ratio (OR) 2.14, 95% confidence interval (CI) 1.34-9.68), all-cause death (OR 1.64; 95% CI 1.23-7.65, P = 0.033) and HF death (OR 1.56; 95 % CI 1.14-11.3, P = 0.021). Conclusion: Hyperaldosteronism in patients with heart failure with preserved ejection fraction secondary hyperaldosteronism is an independent predictor of hospitalization for heart failure, all-cause, and cardiovascular mortality. The inclusion of plasma aldosterone level in the existing prognosis models of heart failure with preserved ejection fraction will help improve their predictive value and optimize the management of high-risk patients.

Adrenoleukodystrophy and Secondary Hyperaldosteronism; A Clinical Demonstration of the Zona Glomerulosa Persistence

Adrenoleukodystrophy (ALD) is a peroxisomal disorder which leads to the accumulation of very long-chain fatty acids in all tissues. Age at onset of symptoms vary depending on the phenotype severity. It can present with progressive symptoms of neurological defects and/or primary adrenal insufficiency. We report a case of 31 yo man diagnosed in childhood with ALD and treated with hydrocortisone and fludrocortisone who eventually developed resistant hypertension due to secondary hyperaldosteronism. He was diagnosed with ALD at 9-year-old and have received an allogenic hematopoietic cell graft 4 years later. After transplantation, he developed a bronchiolitis obliterans which was treated with high dose of glucocorticoids for 3 years. In 2014, at first evaluation with our team, he was on hydrocortisone 10mg/m2 and fludrocortisone 0.05 mg daily. Fludrocortisone was started at 14 yo for a clinical suspicion of mineralocorticoids deficiency. At that time, the patient was normokaliemic (3.8 mmol\L), his aldosterone was, 2 days apart, 245pmol/L lying down and 68 pmol/L (N 138 - 413) sitting, his renin activity was 0.11 ng/L/s (N 0.14 - 0.31) and no orthostatic hypotension was documented. In July 2015, high blood pressure (BP) was noticed, and fludrocortisone was decreased at 0.05 mg every other day. However, his BP continued to increase progressively, thereby fludrocortisone was discontinued in June 2018. Thereafter, amlodipine 10 mg daily, hydrochlorothiazide 12.5 mg daily and terazosin 2 mg daily were progressively introduced. Even with those three anti-hypertensive drugs his BP wasn’t controlled (190/100 mmHg) and he also developed persistent hypokalemia (3.0 - 3.3 mmol/L). Furthermore, significant aortic atheromatosis was described on abdominal computed tomography. In that context, his serum aldosterone/renin ratio was measured. The aldosterone was 632 pmol/L (N &lt; 350) and the renin mass was 93.4 ng/L (N 3 - 16). An assessment of renal arteries was done by doppler ultrasonography, which was compatible with a hemodynamically significant right renal artery stenotic lesion. The patient had never smoke, had no diabetes (HbA1c 5.1%) or dyslipidemia (LDL 2.02 mmol/L). Based on the diagnosis of secondary aldosteronism, spironolactone was introduced in June 2020. Spironolactone was titrated to 37.5 mg daily and BP significantly improved with values around 136/75 and potassium return in normal range (3.8 mmol/L). This interesting case illustrates 1) the persistence of a functioning zona glomerulosa in some patient with ALD and the possible development of secondary hyperaldosteronism in response to renovascular disorder and 2) the particularly high burden of atherosclerosis in this young patient, without classic risk factor, raises questions on the effect of the metabolic defect of ALD itself on the development of atheromatosis.

Secondary hyperaldosteronism and hypertension

Introduction: Arterial hypertension is a major cardiovascular risk factor affecting about 10-40% of the adult population. Secondary endocrine hypertension most often results from excessive aldosterone secretion. Complications related to excessive aldosterone secretion include atrial fibrillation, myocardial infarction, myocardial fibrosis, left ventricular hypertrophy, stroke, and increased cardiovascular mortality. Case report: This report presents a hypotensive woman with hypertensive reactions, newly diagnosed unilateral hyperplasia of the left adrenal gland and secondary hyperaldosteronism. Due to good blood pressure and normalized electrolyte status as a result of antihypertensive drug therapy and absence of damage to target organs, surgical treatment of unilateral adrenal hyperplasia was postponed. Conclusion: In case of midlife and late-life hypertension, it is necessary to consider a cause in the patient's endocrine system. AUTHORS SUMMARY SRPSKI 2021; 50 (1,2) 51-54

Reninoma: Uma Causa Rara de Hipertensão Endócrina

Juxtaglomerular tumours are rare causes of secondary hypertension. They typically present with difficult-to-manage hypertension, hypokalemia, hyperreninemia and secondary hyperaldosteronism. The authors describe a clinical case of a 45 years old female patient, with personal history of difficult-to-manage hypertension and hypokalemia since age 35, medicated with four types of anti-hypertensive agents. An analytical study was performed, which revealed secondary hyperaldosteronism [aldosterone 44.3 ng/dL (4 – 28 ng/dL), renin > 1000 mIU/mL (4.4 – 46.2 mIU/mL)]. Abdominal computed tomography scan identified a heterogeneous nodule located in the middle third of the right kidney, with 3.7 cm. Partial nephrectomy was performed and histological analysis confirmed the diagnosis of reninoma. After surgery, the patient had normal levels of aldosterone (9.2 ng/dL) and renin (1.20 mIU/mL), as well as normal blood pressure. The authors want to highlight this potentially curable cause of endocrine hypertension. Surgical resection is the treatment of choice and leads to normalization of blood pressure.

Gitelman Syndrome: A Rare Cause of Persistent Hypokalemia

Gitelman syndrome is a rare autosomal recessive renal tubular disease, caused by mutations in the SLC12A3 gene, which encodes the renal thiazide-sensitive sodium-chloride cotransporter (NCCT) in the distal renal convoluted tubule. We present a 48-years-old male referred to our observation after being considered not suitable to a previous proposed surgery due to persistent hypokalemia. No valued symptoms were described. Laboratory tests showed metabolic alkalosis, hypomagnesemia, hypokalemia and secondary hyperaldosteronism. Genetic test was performed and sequence analysis of the SLC12A3 gene revealed a homozygous mutation confirming this disease. The aim of this report is to remind and increase awareness of the existence of GS, manage the condition properly and consider the risk of disease recurrence to the next generations.

Secondary hyperaldosteronism and medullary nephrocalcinosis caused by self-administered and uncontrolled laxative use in an adolescent patient

Secondary hyperaldosteronism is respondent aldosterone secretion increase, occurring due to some diseases or drug use. It may be accompanied by normal arterial pressure with/without water retention or arterial hypertension without water retention. Secondary hyperaldosteronism without arterial hypertension and without water retention is usually caused by the use of laxative and diuretic drugs. This condition is characterized by the lack of salt wasting symptoms, presence of myalgia and muscle weakness resulting from hypokalemia, calcium oxalate crystalluria and sonographic signs of medullary nephrocalcinosis. Such characteristics of water-salt exchange and presence of nephrocalcinosis in combination with hypercalciuria are defined as Bartter-like syndrome. Peculiarity of the given clinical case is determined not by a diagnostic difficulty of secondary hyperaldosteronism but concealment of long term self-administered use of laxatives 2 years without medical indications in a female patient, resulting in medullary nephrocalcinosis. A well-informed patient may endanger medical practice, because it is impossible to foresee everything including the uncontrolled self-administered drug use leading to the undesirable consequences.