Download Primary Aldosteronism

42 year old man presents to ANW
ER with fatigue and dizziness
 Past medical history includes
 Hypertension
 Hypokalemia
 Fatty infiltration of the liver
 Allergic rhinitis
 Viral encephalitis in the 1980’s without residual deficit.
ANW ER HPI







Feeling poorly for several weeks
Feels fatigued, tired
Dizzy at times with occasional frontal headache
Chest feels tight
Also feels anxious
Has episodes of flushing and sweats
Can not recall specific provocative or palliative
associations with these symptoms but does note he
seems to have these symptoms when he has been told
his potassium is low
Additional ROS
 Occasional loose stool, up to 3x a day, not bloody
 No fevers or chills diet changes, sick contacts etc.
 Also endorses polyuria, polydypsia, nocturia, and
occasional blurred vision.
Medications
 Alprazolam prn
 Atenolol 100mg qd
 Norvasc 10mg qd
 Cozaar / HCTZ 100/25 qd
 Viagra prn
 Flonase
 Potassium Chloride 20Meq TID
 Allergy to aspirin “facial swelling”
 Mom has DM and HTN
MGM had HTN
 Never smoked, rare Etoh, no street drugs
sexually active with male partners, practices safe sex
and has multiple prior negative tests for std’s and HIV.
Exam
 BP 179/100 | Pulse 78 | Temp 98.8 | Resp 18 | Ht 6' 2"






(1.88m) | Wt 245 lbs (111.1kg) | SaO2 98% on room air
Anxious
Normal head and neck exam
Lungs clear
Normal cardiac exam, no bruits, bounding pulses, no
edema
Abdomen benign no masses or tenderness
Normal neurologic exam, normal visual acuity, no
field cuts no objective weakness, normal reflexes
Labs







Laboratory:
9/17/07 1505
WBC
8.8
HGB
16.3
MCV
85
PLT
232
INR
--
















SODIUM
POTASSIUM
CHLORIDE
CO2TOTAL
BUN
CREATININE
GLUCOSE
CALCIUM
PHOSPHORUS
ALBUMIN -BILITOTAL
BILIDIRECT
ALT
-AST
-PHOSPHORUS
PROTEIN --


TRPTQUANT
TROPTQUAL

9/10/07 1245
7.0
17.0
86
241
-9/17/07 1505
138
2.6LL
26
15
1.0
145H
--------9/17/07 1505
< 0.01
--
9/10/07 1245
139
3.0L
99
28
17
1.1
153H
10.2
----
--
9/10/07 1245
< 0.01
--
100
Imaging
 CXR normal
 Non contrast head CT normal
 EKG sinus, normal rate, normal intervals, no acute ST
changes, normal axis, t waves flat diffusely inverted in
v3-v6, no evidence of hypertrophy.
Patient admitted for evaluation of hypertension,
hypokalemia and lightheadedness
 Additional history
 Hypertension followed by MD for 5 years may have
had it before that time
 Hypokalemia dating back 3 years, on replacement for
one year
 Pt had been treated for his htn with atenolol, norvasc
and cozaar. Hyzaar substituted for cozaar 8 months
ago. Has never had good control of his blood pressure
 loose stools have been more recent 3-4 weeks.
Outpatient work up of htn
 MRI 10/2005 : IMPRESSION:
 1. Normal abdominal renal MRA with no evidence of renal







artery stenosis.
2. Bilateral normal kidney size and parenchymal thickness.
3. No other abnormalities identified.
4. No adrenal masses are identified
Labs 8/07
Plasma Renin activity level <0.1 ng/ml/hr ( 0.6 – 3 )
Aldosterone level 20 ng/dL ( 1 – 21 ng/dl)
No potassium drawn at that time, though recently 3.1
Hospital Day #1
 HCTZ stopped
 Cozaar, atenolol, norvasc continued
 Hydralazine added prn
 Urine metanephrines sent
 Potassium replaced
 Nephrology consultation obtained
 Telemetry, serial troponins, diarrhea evaluation should
pt have loose stools ordered. Hgb A1c and fasting gluc
ordered.
Hospital Day #2
 BP 157/89 , potassium 3.4 following replacement
 Renal consultation
“In august he had a simultaneous renin and aldo level
of 0.1 and 20.0 respectively when his potassium was
low at 3.1. Last year he had an abd ct scan that showed
a possible adrenal adenoma. Suggest repeating renin
and aldo levels when potassium replete. Check 24 hour
urine for aldosterone and get ct scan with special cuts
of the adrenal glands. It is suggestive of
hyperaldosteronism and it is possible that this is the
problem.”
CT abdomen 9/2006
 At the proximal medial limb of the left adrenal
gland there is a 12 mm hypodensity suggestive of,
but not diagnostic of an adrenal adenoma.
Repeat CT Abdomen 9/07
 FINDINGS: A 12 x 16 mm low density lesion in the
adrenal gland which does not enhance in the
arterial phase but it enhances at 70 seconds. This
lesion was also likely present on the MR October 21,
2005 not detected at that time due to adjacent volume
averaging.
 IMPRESSION:
 1. Fatty infiltration of the liver.
 2. Indeterminate right adrenal mass. It was present on
the MR in 2005 and is stable in size. The lesion likely
represents a lipid poor adenoma.
Hospital day #3




BP 150/88
Repeat renin and aldo levels pending
24 hour urine collection in process
Surgical consult obtained to evaluate pt for possible
resection of right adrenal adenoma
 Renal
“Suppression test complete, but this will take days to get
back. Given the pt's clinical scenario, already proven
hyperaldo state, and the appearance of this adrenal mass,
that is not c/w a simple adenoma per radiologists
description, it seems that it should be removed regardless
of hormonal activity. I would be comfortable with moving
forward with surgery if surgeon is in agreement.”
Hospital day 5
 Pt taken to OR for uneventful right laparoscopic
adrenalectomy
 2 cm soft mass noted in or sent to pathology.
 Renin and Aldosterone levels return
Renin < 0.6 ng/ml/hr ( 0.6 – 3 )
Aldosterone 18ng/dl ( 1 – 21 ng/dl)
Potassium 3.6 at time labs drawn
24 hour urine Aldosterone
ALDOSTERONE, URINE 30.2 H 2.3-21.0 MCG/24 H
24 hour metanephrines wnl
Path report
 DIAGNOSIS
RIGHT ADRENAL GLAND, LAPAROSCOPIC
ADRENALECTOMY:
1. Adrenal cortical adenoma consistent with
"aldosteronoma"
2. No evidence of malignancy
9/26/07
 Pt discharged home Hospital day 9, postop day 5
 BP 135/83
 Pt discharged on
cozaar 100 qd
atenolol 100 qd
hydralazine 50mg bid
norvasc 10 qd
KCl 20meq bid
 Serum Aldosterone 4.0
Renin activity < 0.6
Follow up
 10/2
bp 122/84
hydralazine stopped,
atenolol reduced to 50 qd
Recheck potassium 5.0
KCl supplementation stopped
 10/4
bp 100/70 atenolol stopped
 Seen in f/u by nephrology norvasc stopped
 11/15
atenolol 50mg po qd
Cozaar 100mg qd
BP 122/80
Primary Aldosteronism
 Initially felt to account for roughly 1 % of hypertensive
patients
 In a 2000 study J Clin Endocrinol Metab
Sixty-three (18%) of the 350 hypertensive patients (215
women and 135 men; age range, 23-75 yr) were
screened positive for primary aldosteronism
According to the textbook
 The presence of primary mineralocorticoid excess
(with aldosterone )should be suspected in any patient
with the triad of hypertension, unexplained
hypokalemia, and metabolic alkalosis
How does it work?
 Aldosterone is synthesized in the adrenal cortex in the




zona glomerulosa
Aldosterone acts primarily in the distal nephron to increase
the reabsorption of Na+ and Cl- and the secretion of K+
and H+
30 to 90 minute latent period before electrolyte excretion is
affected.
angiotensin II (the production of which varies inversely
with volume) and an elevation in the plasma K+
concentration are the major stimuli of aldosterone
secretion.
Aldosterone release can also be enhanced by ACTH and
hyponatremia
According to reality
 Normokalemia may be more common than
hypokalemia in patients diagnosed with primary
aldosteronism.
 In a retrospective international report less than 50 percent
of diagnosed cases were hypokalemic at presentation
 J Clin Endocrinol Metab 2000 Aug
Only 13 of these 63 subjects (21%) were hypokalemic
 J Clin Endocrinol Metab 2004 Mar
This study is a retrospective evaluation of the diagnosis of
PA from clinical centers. Only a small proportion of
patients (between 9 and 37%) were hypokalemic.
So what do you do?
 It has not been considered feasible to screen every
hypertensive patient for primary aldosteronism. However,
screening has been recommended in hypertensive
patients with one or more of the following features
 Hypokalemia (including patients treated with low-dose
diuretics as currently recommended)
 Severe, resistant, or relatively acute hypertension, which
are suggestive of some form of secondary hypertension (eg,
renal artery stenosis, pheochromocytoma)
 An adrenal incidentaloma
Screening
 Plasma aldosterone concentration to plasma renin activity




(PAC/PRA) ratio
Primary aldosteronism should be suspected when PRA is
suppressed and PAC is increased
Secondary hyperaldosteronism (eg, renovascular disease)
should be considered when both the PRA and PAC are
increased and the PAC/PRA ratio is <10 (eg, renovascular
disease)
Pt should have nl potassium at time of test and can not be
taking spironolactone or eplerenone
In a patient already receiving spironolactone, therapy
should be discontinued for at least six weeks
 The combination of a PAC above 20 ng/dL (555 pmol/L)
and a PAC/PRA ratio above 30 had a sensitivity and
specificity of 90 percent for the diagnosis of aldosteroneproducing adenoma
 To confirm pt needs to be salt loaded either po or with
saline
 24-hour urine specimen is collected for measurement of
aldosterone, sodium, and creatinine. The 24-hour urine
sodium excretion should exceed 200 meq to document
adequate sodium loading. Urine aldosterone excretion >14
µg/24 hours (39 nmol/day) in this setting is consistent with
hyperaldosteronism.
 CT or MRI to evaluate hyperplasia vs discrete adenoma
 Adenomas account for approximately 30 to 60 percent
of cases and should be considered for surgical removal.
 Adrenal hyperplasia is generally a milder disease with
less hypersecretion of aldosterone and less
hypokalemia; it should be treated with an aldosterone
receptor antagonist.
Outcomes
 Surgery





Hypokalemia improved in all patients
Hypertension is improved in all and is cured in
approximately 30 to 60 percent of patients.
Resolution of hypertension associated with
Lack of family history of hypertension
Shorter duration of hypertension and use of two or fewer
antihypertensive agents
Younger age
Higher preoperative ratio of plasma aldosterone
concentration to plasma renin activity, and higher urine
aldosterone level