Journal Reading

Conn’s Syndrome
(Primary Hyperaldosteronism)
Ahmad Nur Aulia

Supervisor : dr. Andra Aswar,SpPD-KEMD

Introduction
• Primary aldosteronism —> considered one of the
more common causes of
secondary hypertension (HTN).
• How common ? —> less tahn 1% of all patients with
Hypertension.
• Hyperaldosteronism is the most common cause of
drug-resistant hypertension.
• Conn was the first to well characterize the disorder,
in 1956 —> Presence of an adrenal aldosteronoma
(aldosterone-secreting benign adrenal neoplasm).
Viera AJ, Neutze DM. Diagnosis of secondary hypertension: an age-based approach. Am Fam Physician. 2010;82(12):
1474.
 Journal Reading

Funder, J. W., Carey, R. M., Mantero, F., Murad, M. H., Reincke, M., Shibata, H., … Young, W. F. (2016). The Management of Primary
Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline. The Journal of Clinical
Endocrinology & Metabolism, 101(5), 1889–1916. doi:10.1210/jc.2015-4061
Pathophysiology
Synthesis Glucocorticoid and Mineralocorticoid

Stowasser M, Gordon RD.

Primary Aldosteronism: Changing
Definitions and New Concepts of
Physiology and Pathophysiology
Both Inside and Outside the Kidney.
Physiol Rev 96: 1327– 1384, 2016.
Published August 17, 2016; doi:
10.1152/physrev.00026.2015.
Stowasser M, Gordon RD. Primary Aldosteronism: Changing Definitions and New Concepts of Physiology and Pathophysiology Both Inside and Outside the Kidney. Physiol Rev 96:
1327– 1384, 2016. Published August 17, 2016; doi:10.1152/physrev.00026.2015.
Stowasser M, Gordon RD.
Primary Aldosteronism: Changing
Definitions and New Concepts of
Physiology and Pathophysiology
Both Inside and Outside the Kidney.
Physiol Rev 96: 1327– 1384, 2016.
Published August 17, 2016; doi:
10.1152/physrev.00026.2015.
 • 1st hyper-aldosteronism —> excess production of aldosterone
independent of the renin-angiotensin system
• Caused by : Adrenal adenoma, unilateral or bilateral adrenal
hyperplasia, or adrenocortical carcinoma.
• Hypokalemia —> urinary potassium wasting is the most prominent
feature of hyper-aldosteronism.

Stowasser M, Gordon RD. Primary Aldosteronism: Changing Definitions and New Concepts of Physiology and Pathophysiology Both Inside and Outside the Kidney.
Physiol Rev 96: 1327– 1384, 2016. Published August 17, 2016; doi:10.1152/physrev.00026.2015.
Clinical Presentation
 Sign and symptoms
•Clinical presentation of primary aldosteronism is not
distinctive —> Require High Suspicion
•Classic —> Spontaneous or unprovoked hypokalemia (seen
ini 9 to 37% patients), especially if the patient is also
hypertensive and metabolic alkalosis
•Develop severe and/or persistent hypokalemia in the setting
of low to moderate doses of potassium-wasting diuretics
•Patients with treatment-refractory/-resistant hypertension
(HTN)
• Additional : muscle cramps or weakness,
Diagnosis
 Case Detection
(Using plasma ARR to detect possible case)

•Sustained BP above 150/100 on each of three measurements

obtained on different days, with hypertension (BP 140/90)
resistant to 3 conventional antihypertensive drugs (including a
diuretic)
•Controlled BP ( 140/90) on four or more antihypertensive drugs
•Hypertension and adrenal incidentaloma
•Hypertension and spontaneous hypokalemia
•Hypertension and a family history of early onset hypertension
or cerebrovascular ac- cident at a young age ( 40 years)
• First Degree Relatives of patients with PA
Confirmatory
Test Pathway
When aldosterone is measured in ng/dL and PRA is measured in ng/
mL/h, a plasma aldosterone/PRA ratio of greater than 20-25 has 95%
sensitivity and 75% specificity for primary aldosteronism. When
aldosterone is measured in pmol/L, a ratio greater than 900 is
consistent with primary aldosteronism.
* Patient out of bed
for at least two hours
and seated for at
least 5 to 15 minutes
before sample is
drawn.
¶ Direct PRC can be
measured instead of
PRA. However,
UpToDate authors
prefer PRA. Refer to
topic text for
interpretation of PRC
cutoffs and normal
values.
Δ Oral sodium loading
over three days is
one confirmation test
that many experts
use. An alternative is
the saline infusion
test.
Young WF. Primary aldosteronism: renaissance of a syndrome. Clin Endocrinol (Oxf) 2007; 66:607.
Adrenal vein sampling
(AVS) can be completed
with simultaneous or
sequential cannulation
of the adrenal veins.
Management
 Goals
• (1) normalization of blood pressure,
• (2) normalization of levels of serum potassium and other
electrolytes,
• (3) normalization of serum aldosterone levels

• Appropriate treatment for primary aldosteronism depends on its

cause.
• Aldosteronoma —> Medical therapy used for decreasing surgical
risk or poor surgical candidate and who refused surgery
• Heart Failure —> Secondary aldosteronism —> Spironolactone
and eplerenone

Carey RM. Primary aldosteronism. Horm Res. 2009 Jan. 71 Suppl 1:8-12.

 Mineralocorticoid Antagonist
• Hypertension is the rule in patients diagnosed with PA,
• Cured or improved by unilateral adrenalectomy in
patients with unilateral disease
• Improved by MR antagonists in the remaining patients. —
> MR antagonists appear to be effective at controlling BP
and protecting target organs independent of effects on BP.
ean
• Reduction in systolic BP of 25% and diastolic BP of 22%
in response to spironolactone 50 – 400 mg per day for 1
to 96 months.
Funder, J. W., Carey, R. M., Mantero, F., Murad, M. H., Reincke, M., Shibata, H., … Young, W. F. (2016). The Management of Primary
Aldosteronism: Case Detection, Diagnosis, and Treatment: An Endocrine Society Clinical Practice Guideline. The Journal of Clinical
Endocrinology & Metabolism, 101(5), 1889–1916. doi:10.1210/jc.2015-4061
Thank
You