Adrenal Vein Sampling in a Patient With Primary Hyperaldosteronism and Severe Contrast Allergy

Margaretha L.M. Prins; Bartholomeus E.P.B. Ballieux; Onno C. Meijer; Alberto M. Pereira; Michiel F. Nijhoff

Disclosures

J Endo Soc. 2021;5(10) 

In This Article

Case Presentation

A 50-year-old female patient was referred to our outpatient clinic because of biochemically confirmed PA. She had a 3-year history of difficult-to-control hypertension, along with intermittent headaches, palpitations, decreased energy, and easy fatigability for the past year. She experienced a wide range of cognitive symptoms, malaise, and was unable to work. Plasma renin concentration before saline infusion at the referring hospital was 3.2 ng/L and PAC was 514 pmol/L. The aldosterone/renin ratio was 160 pmol/ng. After salt loading the plasma renin concentration was 1.2 ng/L and the PAC was 296 pmol/L. A CT of the abdomen showed no adrenal abnormalities. To distinguish between bilateral and unilateral forms of PA, AVS was required. Unfortunately, she had a history of severe allergy to ICM, which had previously caused an episode of dyspnea and swollen throat. Due to this history of a prior severe allergic reaction to ICM, pretreatment with glucocorticoids and antihistaminic agents was required.

To avoid the potential confounding effects on the cortisol assay, we decided to use dexamethasone as anti-allergic premedication. Since it was unclear whether the adrenal response to ACTH would be intact if the patient was using high-dose dexamethasone, a high-dose ACTH stimulation test with the simultaneous use of dexamethasone was performed first.

Per protocol, the patient was administered dexamethasone 8 mg (as an equivalent of 50 mg prednisolone) orally both at 9 PM and the next morning at 7 AM. The cortisol concentration was 218 nmol/L, measured at the moment of admission at the hospital, before the administration of dexamethasone. One hour before the second dexamethasone dose, an intravenous bolus of 250 μg ACTH (Synacthen©) was administered. After 60 min, the serum cortisol concentration rose adequately to 812 nmol/L. Reference values for a normal cortisol response was >430 nmol/L after 250 μg ACTH (Roche Elecsys Cortisol gen2 ECLIA, Roche Diagnostics, Mannheim, Germany; AB_2811288).[20] This confirmed a normal cortisol response to ACTH stimulation despite the use of high dose dexamethasone. The PAC prior to dexamethasone administration was 540 pmol/L (18.53 ng/dL; reference: 110–1200 pmol/L). After the bolus ACTH and 1 dose of dexamethasone, it was 740 pmol/L. The same day, the patient underwent AVS using continuous ACTH stimulation. AVS was successful and uncomplicated; results are shown in Table 2. The mean selectivity index was 17 on the right side and 32.6 on the left side. The mean aldosterone/cortisol ratio in the adrenal veins of the 2 samples was 1.8 on the right and 2.29 on the left, amounting to a left-to-right lateralization index of 1.30. The aldosterone/cortisol ratio in each adrenal vein was greater than the corresponding ratio in the inferior vena cava, further confirming bilateral aldosterone overproduction. Altogether, the AVS produced reliable results and indicated bilateral adrenal excessive aldosterone production, with a slight dominance to the left adrenal gland.

Treatment with spironolactone was added to her baseline medication, which consisted of doxazosin 8 mg, metoprolol 100 mg, nifedipine 60 mg, and perindopril 8 mg. Her cognitive symptoms did not improve, and she experienced troubling side effects. Therefore, despite evidence of bilateral aldosterone production, she elected to undergo unilateral adrenalectomy (left side) to reduce the degree of aldosterone excess and, consequently, the severity of the PA phenotype.

She underwent uncomplicated laparoscopic unilateral adrenalectomy, and histopathology revealed micronodular adrenal hyperplasia. The postsurgical saline infusion test showed suboptimal suppression of aldosterone [nadir: 185 nmol/L (6.67 ng/dL)], and low-dose eplerenone was initiated. Cognitive functioning normalized and remained normal now up to 2 years after surgery, but hypertension persisted so she received low-dose eplerenone in addition to 2 antihypertensive drugs, consisting of amlodipine 10 mg and perindopril 8 mg.

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