Endocrine Hypertension

Transcription

1 Endocrine Hypertension 1

2 No Disclosures

3 Endocrine Hypertension Objectives: 1. Understand Endocrine disorders causing hypertension 2. Understand clinical presentation of Pheochromocytoma and Hyperaldosteronism 3. Understand clinical detection testing for Pheochromocytoma and hyperaldosteronism

4 Principle Centered Medicine Framework for patient care centered in the whole patient Consider body, mind, spirit in the pathway to health Seeking the full health potential of the patient For this lecture: focus on getting the the bottom of the problem of Hypertension, looking for an endocrine cause Seeking a diagnosis to apply Rational Therapy based in scientific endocrine principles

5 Resistant Hypertension Blood pressure above goal on 3 medi-cations of different classes as per ACC/AHA 2017 One of these medications should be a diuretic If control on 4 drugs, this is also resistant hypertension 5

6 Pseudoresistant Hypertension Insufficient treatment Incorrect measurement Poor adherence to medications Poor adherence to dietary & lifestyle measures White coat hypertension 6

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8 Factors that could be adjusted in Resistant Hypertension Medications/herbals Diet & Lifestyle Insufficient treatment Volume expansion Secondary etiologies 8

9 Endocrine Etiologies Pheochromocytoma & Paraganglioma Primary Aldosteronism Mineralocorticoid Excess Secondary hyperaldosteronism and Renovascular Cushing syndrome Hypothyroidism Hyperthyroidism Hyperparathyroidism & hypercalcemis Acromegaly 9

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11 Pheochromocytoma & Paraganglioma (PPargls) In addition to hypertension, Headache Diaphoresis Palpitations Sometimes no symptoms, particularly paragangliomas 11

12 Pheochromocytoma & Paraganglioma (PPargls) Tumors produce Metanephrines But, only intermittent exocytotic parent values Poorly diagnostic results: urine VMA, Catecholamines, Chromogranin A Arise from adrenal medulla up to 85 % and otherwise arise from sympathetic paraganglia 1.6 per 10,000 people, But at autopsy 5 per 10,000 people Incidental adrenal lesion 500 per 10,000 people 12

13 Imaging last Not first Common endocrine rule. Incidentalomas are common, and, finding a lesion, may result in inappropriate testing or procedures

14 PPargls measurement parameters Serum testing positional, supine 30 minutes at rest, cannula in place, no dietary requirement Urine 24 hour test; Detail to pt how to collect 14

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16 Case detection testing High sensitivity labs Serum free metanephrines Urine 24 hour fractionated metanephrines Liquid chromatography with Tandem mass spect abnormal values 2 to 3 X normal

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22 Primary Hyperaldosteronism Increased aldosterone Decrease renin Urine potassium loss Hypokalemia with cramps, paresthesia, weakness But, Hypokalemia is not predominate 5 to 10 % of hypertension 20 % resistent hypertension patients Obstructive sleep apnea common 22

23 Primary Hyperaldosteronism Increases CV events over essential hypertension Non blood pressure effects reversed with aldosterone directed treatment Only 20 % hypokalemia Hypokalemia provokes aldosterone Screen most patients with hypertension Case detection for 1st degree hypertensive relatives 23

24 Primary Hyperaldosteronism Case Detection Many patients are Not hypokalemic, normal electrolytes do not exclude diagnosis Aldosterone/Renin ratio (ARR) is the preferred screen No diuretics for 4 weeks prior to ARR testing Normalize potassium, hypokalemia provokes potassium Liberal sodium diet 24

25 Primary Hyperaldosteronism Case Detection Plasma aldosterone > 10 ng/ml with Plasma renin < 1 ng/ml This values indicate a positive screen Then, proceed to confirmation testing 25

26 Adrenal Venous Sampling Summary RE: Male DOB: 1942 Aldosterone (ng/dl) Cortisol (ug/dl) Basal: Right adrenal vein Left adrenal vein Peripheral Arm POST ACTH: Right adrenal vein Left adrenal vein >150 Peripheral Vena Cava Peripheral Femoral Vein

27 Other Mineralocorticoid Excess

28 Congenital Adrenal Hyperplasia Adrenal enzyme defect ACTH drives precursors Not in deficiency 21 hydroxylase = not htn Low Renin and Aldosterone in hypertension 28

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32 Enzymatic defects in steroidogensis that yield androgen over cortisol Most common cases are 21-hydroxylase deficiency If this is complete enzymatic block, the result is ambiguous genitalia in infant girls This presents a crisis of Adrenal Insufficiency, which can be unrecognized in male infants with screening for 17-OH progesterone Variations include late onset CAH in which adult women present with hirsutism

33 CAH and Hypertension 11 beta hydroxylase deficiency 2/3 with Hypertension Hypokalemia Aldosterone low and Renin Low Girls = ambiguous genitalia Boys = precocious puberty 1 in 100,000 caucasian, 5% of CAH

34 Case Detection in 11 beta hydroxylase deficiency Deoxycorticosterone increased Increased 11-deoxycortisol Other steroidogenesis precursors If abnormal, germline mutation testing

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36 CAH and Hypertension 17 alpha hydroxylase deficiency Hypertension Estimated 1 in 1,000,000 live births Decreased cortisol and sex hormones Girls primary amenorrhea Boys present female body habitus or pseudohermaphrodite

37 Case Detection in 17 alpha hydroxylase deficiency Low blood testing for cortisol, aldosterone, DHEAS, 17-hydroxyprogesterone and others High DOC and corticosterone These labs with hypertension and hypokalemia should have genetic testing, additional diagnostic testing to confirm this deficiency

38 Renovascular Hypertension Renin angiotensin aldosterone system associated Abnormally high aldosterone 1 to 5 % of hypertensives Case detection by renal artery duplex ultrasound and CT angiography highly sensitive Peripheral renin, aldosterone and renal vein renin vary as per conditions of testing Focus for endovascular stents is less on BP and more on saving kidney function and treating pulmonary edema 38

39 Other endocrine HTN presentations Cushing Syndrome: Urine 24 hr free cortisol, 1 mg overnight dexamethasone suppression, midnight salivary cortisol Hyperthyroidism: TSH, Free T4 Hypothyroidism: TSH, Free T4 Hypercalcemia/hyperparathyroidism: PTH, urine 24 hr calcium Acromegaly: IGF-1 Obstructive sleep apnea: Sleep study

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57 References Endocrine Hypertension: I: Clinical disorders of hypertension Chapter 298 Online Harrisons: Kotchen, Theodore A.. "Hypertensive Vascular Disease." Harrison's Principles of Internal Medicine, 19e Eds. Dennis Ka II: Endocrine Reviews Endocrine hypertension screening: William F. Young, David A. Calhoun, Jacques W.M. Lenders, Michael Stowasser, Stephen C. Textor; Screening for Endo III: Endocrine Society Guidelines for Primary Hyperaldosteronism: John W. Funder, Robert M. Carey, Franco Mantero, M. Hassan Murad, Martin Reincke, Hirotaka Shibata, Michael Stow IV: Endocrine Society Guidelines for Pheochromocytoma and Paraganglioma: Jacques W. M. Lenders, Quan-Yang Duh, Graeme Eisenhofer, Anne-Paule Gimenez-Roqueplo, Stefan K. G. Grebe, Moh

58 John Sutton, DO, FACOI, FACE, CCD Carson Tahoe Endocrinology Carson City, NV Phone: