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Primary Hyperadosteronism
 

Primary Hyperadosteronism

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    Primary Hyperadosteronism Primary Hyperadosteronism Presentation Transcript

    • Interesting case Conference 11/13/2008 Ashish Dhungel Susan Steigerwalt
    • Case
      • History of present illness
      • 31 Yr Caucasian female
      • HTN- 2 years . No hypertension or preeclampsia with pregnancies 4 and 6 years prior to presentation. Referred by Primary due to presentation with BP 160/112 mmHg.
      • Past Medical History
      • Headache
      • Social History
      • Smoke tobacco 1 pk /day- 13 yr, no drug use, no alcohol use
      • High stress job. Sleeps 6 hours/ night
      • Family history
      • Father –DM
      • Mother- healthy
      • Medication
      • Norvasc 5 mg daily; unknown oral
      • contraceptive( intermittently for 8 years)
      • Review of symptoms
      • General-exhaustion, feels excessive hot and
      • cold
      • CVS- palpitation
      • GI- heartburn
      • Neuro- Headache, tingling of hands and feet
    • Physical exam
      • B.P 140/88 (sitting)
      • 147/91 (standing)
      • Pulse -88, Wt 146 lb, BMI- 22
      • HEENT
      • No Thyromegaly
      • Chest
      • No wheeze
      • Skin -
      • No striae, no rash
      • Cardiovascular
      • No murmur, No carotid bruit
      • Abdomen
      • No organomegaly, No bruit
      • Neurological
      • Normal exam
    • Investigation
      • CBC- Hb 12.3 ,WBC, platelets wnl
      • CMP- sodium 141, potassium 3.8, bicarb 29, creatinine 0.7
      • U/A - no proteinuria
    • Differential Diagnosis- young woman with stage 2 hypertension
    • Differential Diagnosis- young woman with stage 2 hypertension
      • Essential Hypertension
      • Medications: oral contraceptives, illicit drugs
      • PCOS
      • Primary aldosteronism
      • Fibro muscular dysplasia
      • Liddle’s syndrome
    • Additional Evaluation?
    • Additional Evaluation?
      • Aldosterone 76!!!!
      • PRA< 0.5!!!! Guess what she has!!!!
      • Best part- no invasive work up prior to lab evaluation- we saved money!!!
      • CT scan showed left adrenal adenoma
      • She was referred for AVS…
    • Guideline Approach to pt with suspected Primary Aldosteronism
    •  
    • Introduction
      • Primary aldosteronism is the syndrome from the autonomous hyper secretion of aldosterone , almost always from the adrenal cortex.
      • Conn‘s syndrome
      • was based on a thirty-four-year-old patient who entered the university hospital in 1954 complaining of seven years of episodic muscle weakness that often resulted in virtual paralysis of her lower legs. At Central Society for Clinical Research on October 29, 1954 , he present for the first time his extensive clinical investigations of this new syndrome, which he called primary aldosteronism.
      Jerome W.Conn 09 /24/1907 –06/ 11 1981
    • Incidence
      • CONN JW.
      • Primary aldosteronism.
      • J Lab Clin Med. 1955 Apr;45(4):661-4
      • In unselected patients – 0.5% of hypertensive (Gifford,1969;kaplan 1967;Sinclair 1987)
      • 20% in referred patients (Mulatero,2004;Stowasser et al 2003)
    • Incidence
      • Using ARR ,prevalence 5-13% of all hypertensives.
    • Forms of PA
      • Aldosterone producing adenoma (APA)
      • Bilateral idiopathic hyperplasia (IHA) most common!!!!
      • Primary (unilateral) adrenal hyperplasia
      • Aldosterone producing adrenocortical carcinoma
      • Familial hyperaldosteronism (FH)
      • Glucocorticoid remediable aldosteronism ( type I)
      • FH type II (APA or IHA)
    • Clinical features
      • 30- 50 years
      • Aldosteronism
      • Hypertension ↑ Na reabsorption
      • ↑ Plasma volume ↑ K excretion
      • Suppressed Hypokalemia
      • renin-angiotensin
    • Aldosterone
      • Various deleterious effects
      • Endothelial stiffness (Oberleithner,2005)
      • Vascular damage (Schmidt ,2003)
      • Myocardial fibrosis (GP Rossi,2002)
      • Proteinuria (Calhoun,2008)
    • Hypertension in PA
      • Severe hypertension
      • Aldosterone stimulate ENaC activity in apical membranes.
      • Slightly expanded plasma volume, increased total body Na content and increased peripheral resistance
    • Hypokalemia in PA
      • 9-37%
      • Normokalemic HTN – most common
      • Less common in pt diagnosed early in course of disease.
      • Diuretic induced hypokalemia.
    • Diagnosis Patients with HTN that are at risk of PA Case detection using ARR Case confirmation Adrenal CT AVS Subtype testing
    • Importance of case detection
      • Pt with PA have higher cardiovascular morbidity and mortality then age and sex matched pt with Essential HTN and the same degree of B.P control
      • Rossi GP, at el, 2006 Renal damage in primary aldosteronism: results of the PAPY Study. Hypertension 48:232–238
      • Milliez P, at el 2005 Evidence for an increased rate of cardiovascular events in patients with primary aldosteronism.J Am Coll Cardiol 45:1243–1248
      • Strong evidence linking reduction in aldosterone level to improved cardiac and cerebrovascular outcome
      • Rossi GP, Sacchetto A, Visentin P, Canali C, Graniero GR, Palatini P, Pessina AC.
      • Changes in left ventricular anatomy and function in hypertension and primary aldosteronism.
      • Hypertension. 1996 May;27(5):1039-45
    • Case detection
      • JNC Stage 2 and stage 3 HTN *(>160/110)
      • Drug Resistant hypertension
      • Hypertension and spontaneous or diuretic induced hypokalemia
      • Hypertension with adrenal incidentaloma
      • Hypertension and family history of early onset HTN or CVA at young age (< 40)
      • Hypertensive First degree relatives of patient with PA
    • Aldosterone to Renin Ratio (AAR)
      • Aldosterone 5- 20 ng /dl
      • Plasma renin activity 1-3 ng /ml per hr
      • Currently the most reliable for screening
      • Associated with False + and –ve
      • PAC:PRA ratio > 20ng/dl per ng/ml per hr
      • Should be repeated if results are inconclusive or difficult to interpret.
      • Preparation for ARR measurement
      • Attempt to correct hypokalemia
      • Encourage patient to liberalize Na intake
      • Withdraw agents affecting ARR (4wk) Spironolactone, Eplerenone, amiloride, triamterene, K wasting diuretics, tobacco, licorice
      • 4 . If results off the above agents are not diagnostic and if HTN can be controlled with relatively non interfering medication, withdraw meds that affect ARR (2wk)
      • - B blocker, clonidine, methyldopa, NSAIDs
      • - ACEI, ARB, Renin inhibitors, dihydropyridine ca ch blocker
      • 5 If necessary to maintain B.P - Verapamil, hydralazine, pazosine, terazosine
      • B) Collection of blood
      • Mid morning, after pt has been up (sitting, standing or walking) for at least 2 hr and seated for 5- 15 min
    • Factors affecting ARR
    • Case Confirmation
      • Oral Sodium loading
      • Saline infusion
      • Fludrocortisone suppression
      • Captopril challenge
      • Choice of test is determined by local expertise.
      • Meds with min or no effect in RAS should be used for B.P control
    • Oral Sodium loading test
    • Saline infusion test
    • Fludrocortisone suppression test
    • Captopril challenge test
    • Subtype classification
      • 1 CT scan
      • All pt with PA should undergo CT scan as initial study in subtype testing
      • MRI has no advantage over CT
      • CT finding along with AVS is used in combination to guide treatment decision
      • Sensitivity and specificity 78% and 75%
    • Limitation of CT
      • Small APA can be interpreted as IHA on basis of CT finding of bilateral nodularity or normal appearing adrenals
      • Adrenal micro adenomas may actually represent areas of hyperplasia
      • Non functioning unilateral adrenal macro adenoma are indistinguishable from APAs on CT.
      • CT detected fewer than 25% of APA <1 cm Gordon RD 2001 Diagnostic investigations in primary aldosteronism,Zanchetti A, ed. Clinical medicine series on hypertension
      • CT was accurate in only 53% of pt in comparision to both CT and AVS.
      • Young WF, Stanson AW, Thompson GB, Grant CS, Farley DR, van HeerdenJA 2004 Role for adrenal venous sampling in primary aldosteronism
      • Concordance between CT and AVS was found only in 54%
      • Nwariaku FE, Miller BS, Auchus R, Holt S, Watumull L, Dolmatch B, Nesbitt, 2006 Primary hyperaldosteronism: effect of adrenal vein sampling on surgical outcome.Arch Surg 141:497–502
    • AVS
      • 2 Adrenal venous sampling
      • AVS is essential in directing appropriate therapy.
      • Differentiate Uni /Bilateral disease
      • Invasive and expensive
      • Sensitivity and specificity is 95% and 100%
    • AVS Protocol
      • Unstimulated AVS
      • Unstimulated AVS followed by cosyntropin
      • Continuous cosyntropin with AVS ( used HFH )
      • - minimize stress induced fluctuation in
      • aldosterone secretion
      • Cortisol corrected aldosterone ratio - Dividing PAC by respective Cortisol concentration to correct for dilutional effect of phrenic vein flowing into adrenal vein
      • Cortisol corrected aldosterone ratio, from high side to low side > 4:1
      • indicate unilateral aldosterone excess
      • Ratio < 3:1 suggest bilateral aldosterone secretion
      • Ratio between 3:1 and 4:1 may have either ,and result should be interpreted in conjunction with CT and clinical setting.
    • Treatment
      • Unilateral PA (APA,UAH)
      • Unilateral adrenalectomy
      • -B.P, K improve in 100% pt and HTN
      • cured in 35-60%
      • - Medical management who do not go for
      • surgery
      • Spironolactone, Eplerenone
    • Treatment
      • Bilateral disease (IHA, bilateral APA,GRA)
      • 9% cure rate after uni /Bilateral adrenalectomy
      • Medical management.
    • Spironolactone
      • Starting dose 12.5 -25 mg daily
      • Eplerenone 25 mg daily/bid
    • GRA
      • Glucocorticoid to suppress Pit ACTH
      • Dexamethasone 0.125-0.25 mg daily
      • Prednisone 2.5-5 mg daily
    • Back to our patient-wow!
      • ARR - 152
      • CT scan - Lt adrenal mass
      • AVS
      1.17 26.7 315 IVC >4:1 53.51 236 12630 L adrenal vein 1.8 322 600 R adrenal vein L:R A/C ratio Cortisol corrected aldosterone ratio Cortisol Aldosterone Vein
    • Our patient
      • Unilateral ( Lt) Aldosterone Producing Adenoma (APA)
      • Pt referred for Laparoscopic Lt Adrenalectomy
    • Questions ???