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Primary Aldosteronism: an update on the management

Primary Aldosteronism: an update on the management. Dr Man Chi Mei Vivian Queen Mary Hospital. Content. Background information Diagnostic algorithms Localization and subtype differentiation Management. Case Scenario. 60/M , good past health Blood test confirmed primary aldosteronism.

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Primary Aldosteronism: an update on the management

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  1. Primary Aldosteronism: an update on the management Dr Man Chi Mei Vivian Queen Mary Hospital

  2. Content • Background information • Diagnostic algorithms • Localization and subtype differentiation • Management

  3. Case Scenario • 60/M , good past health • Blood test confirmed primary aldosteronism 17mm right adrenal nodule 7mm left adrenal nodule

  4. What should be the management? • Adrenalectomy • Right adrenalectomy? • Left adrenalectomy? • Bilateral adrenalectomy? • Medical therapy with aldosterone receptor antagonist

  5. Background • First described in 1954 • Group of disorders in which aldosterone production is inappropriately high, relatively autonomous, and non-suppressible by sodium loading1 • Estimated prevalence of 11.2% in hypertensives2 • Hypokalemia not always present • 52% aldosterone-producing adenoma • 83.1% bilateral adrenal hyperplasia Jerome W. Conn(1907-1994) 1 J Clin Endocrinol Metab 93:3266-3281, 2008 2 Rossi et al. JACC vol. 48, No. 11. 2006: 2293-300

  6. Types of Primary Aldosteronism Role for adrenal venous sampling in primary aldosteronism. Young WF et al. Surgery. December 2004.

  7. Diagnosis of primary aldosteronism • Screening • Aldosterone/ renin ratio (ARR) • Plasma aldosterone concentration (PAC) • Elevated ARR >20ng/dl per ng/ml/h and PAC >10ng/dL • Confirmation • Intravenous saline load/ oral salt load • Captopril challenge test • Fludro-cortisone suppression test

  8. Lateralization and subtype differentiation • Computed tomography (CT) • Adrenal scintigraphy • Adrenal venous sampling (AVS)

  9. Computed tomography Young WF et al. The incidentally discovered adrenal mass. New England Journal of Medicine 2007; 356: 601-10

  10. Adrenocortical carcinoma Adrenocortical adenoma Young WF et al. The incidentally discovered adrenal mass. New England Journal of Medicine 2007; 356: 601-10

  11. Lateralization and subtype differentiation • Computed tomography (CT) • Adrenal scintigraphy • Adrenal vein sampling (AVS)

  12. Adrenal Scintigraphy • Provides functional information • 131I-6β-iodomethyl-19-norcholesterol (NP-59) • Marker of adreno-cortical uptake Huang YE et al. Role of 131I-NP-59 Adrenal Imaging in Patients of ACTH-Independent Cushing’s Syndrome. Ann Nucl Med Sci 2001;14:75-83

  13. Chen YC et al. Use of NP-59 SPECT/ CT imaging in atypical primary aldosteronism. Q J Med (2013)

  14. Lateralization and subtype differentiation • Computed tomography (CT) • Adrenal scintigraphy • Adrenal venous sampling (AVS)

  15. Adrenal vein sampling • First proposed in 1967 as a test to distinguish between aldosterone-producing adenoma and idiopathic hyperaldosteronism • Gold standard for lateralization of disease • Femoral venous access • Simultaneously left and right adrenal venous sampling • Blood aldosterone and cortisol level Kahn SL et al. Adrenal vein sampling. Techniques in vascular and interventional radiology 13: 110-125

  16. Interpretation • Confirmation of correct position of adrenal catheters: • Cortisol level from adrenal catheters being 5-10 times the value obtained from peripheral sheath • Asymmetrical aldosterone: cortisol values on the affected side being 3-5 times the value obtained from the unaffected side Kahn SL et al. Adrenal vein sampling. Techniques in vascular and interventional radiology 13: 110-125

  17. Left adrenal vein catheterization Right adrenal vein catheterization Kahn SL et al. Adrenal vein sampling. Techniques in vascular and interventional radiology 13: 110-125 Lau JHG et al . Clinical Endocrinology (2010) 76; 182-188

  18. Diagnostic values and accuracy • 203 patients were selected for AVS from 1990-2003 • 194 patients underwent successful adrenal vein cannulation • Success rate 95.6% • Computed tomography, AVS and histopathological findings were compared Young WF et al. Role of adrenal venous sampling in primary aldosteronism. Surgery 2004; 136: 1227-35

  19. Discordant between CT and AVS • 110 patients (56.7%) had unilateral source for aldosterone hyper-secretion Young WF et al. Role of adrenal venous sampling in primary aldosteronism. Surgery 2004; 136: 1227-35

  20. Discordant between CT and AVS • 21.7% (42 patients) would have been incorrectly excluded as candidates for adrenalectomy • 24.7% (48 patients) might have unnecessary or inappropriate adrenalectomy Young WF et al. Role of adrenal venous sampling in primary aldosteronism. Surgery 2004; 136: 1227-35

  21. Discordant between CT/MRI and AVS • Systematic review of 38 studies, 950 patients included • 37.8% discordance between CT/MRI and AVS • 14.6% inappropriate adrenalectomy • 19.1% inappropriate exclusion from adrenalectomy • 3.9% adrenalectomy on wrong side Kempers MJE et al. Systematic Review: Diagnostic Procedures to Differentiate Unilateral From Bilateral Adrenal Abnormality in Primary Aldosteronism. Annals of Internal Medicine, 09/2009, Volume 151, Issue 5, pp. 329 - 337

  22. Lau JHG et al . Clinical Endocrinology (2010) 76; 182-188 • Yen RF et al. The Journal of Nuclear Medicine (2009) 50; 10: 1631-1637 • Young WF et al. Surgery (2004) 136; 6; 1227-1234 • Tsai YS et al. Formos J Endocrin Metab 2011; 2(2): 38-43

  23. Kahn SL et al. Adrenal vein sampling. Techniques in vascular and interventional radiology 13: 110-125

  24. Management • Bilateral idiopathic hyperaldosteronism • Medical treatment: aldosterone receptor antagonist • Intolerance/ refractory cases: bilateral adrenalectomy • Aldosterone producing adenoma • Primary adrenal hyperplasia • Adrenalectomy (laparoscopic/ open) • Emerging therapies: • Acetic acid injection • Radiofrequency ablation Moo TA et al. Prediction of Successful Outcome in Patients with Primary Aldosteronism. Current treatment options in oncology (2007) 8:314-321

  25. Back to our patient • Adrenal to peripheral cortisol level: • Right side:3.6 versus left side: 3.2 • Lateralization index 7.4 • Right side: 42 versus left side: 5.7 • Right adrenalectomy performed and Conn’s adenoma confirmed • Improvement in ARR one month after operation

  26. Conclusion • Primary aldosteronism is frequently under-diagnosed in hypertensives • Aldosterone producing adenoma and bilateral idiopathic hyperaldosteronism are two commonest causes of primary aldosteronism • Lateralization is important for identification of surgically-amendable causes

  27. Reference • Galati SJ et al. Primary aldosteronism: emerging trends. Trends in Endocrinology and Metabolism. September 2013, Vol.24, No. 9 • Role for adrenal venous sampling in primary aldosteronism. Young WF et al. Surgery. December 2004. • Schwatz et al. Screening for primary aldosteronism in essential hypertension: Diagnostic accuracy of the ratio of plasma aldosterone concentration to plasma renin activity. Clinical Chemistry. 51:2. 386-394 • Young WF et al. The incidentally discovered adrenal mass. New England Journal of Medicine 2007; 356: 601-10 • Kahn SL et al. Adrenal vein sampling. Techniques in vascular and interventional radiology 13: 110-125 • Huang YE et al. Role of 131I-NP-59 Adrenal Imaging in Patients ofACTH-Independent Cushing’s Syndrome. Ann Nucl Med Sci 2001;14:75-83 • Chen YC et al. Use of NP-59 SPECT/ CT imaging in atypical primary aldosteronism. Q J Med (2013)

  28. Rossi GP et al. A Prospective Study of the Prevalence of Primary Aldosteronism in 1125 Hypertensive Patients. JACC vol 48, No 11, 2006 • Moo TA et al. Prediction of Successful Outcome in Patients with Primary Aldosteronism. Current treatment options in oncology (2007) 8:314-321 • Stowasser M et al. Update in Primary aldosteronism. J Clin Endocrinol Metab (2009) 94: 3623-3630 • Liu SYW et al. Radiofrequency ablation for benign aldosterone-producing adenoma: a scarless technique to an old disease. Annals of Surgery 256(6): 1058-1064 • Kempers MJE et al. Systematic Review: Diagnostic Procedures to Differentiate Unilateral From Bilateral Adrenal Abnormality in Primary Aldosteronism. Annals of Internal Medicine, 09/2009, Volume 151, Issue 5, pp. 329 - 337 • American Association of Clinical Endocrinologists and American Association of Endocrine Surgeons Medical Guidelines for the Management of Adrenal Incidentalomas • The Endocrine Society’s Clinical Guidelines. J Clin Endocrinol Metab 93: 3266-3281, 2008

  29. Thank you Questions?

  30. Who should be screened? • Moderate to severe hypertension • Resistant hypertension • Hypertension with a family history of early-onset disease • Hypertension with an adrenal incidentaloma • History of cerebrovascular accident occurring before age 40 years • First-degree relative with primary aldosteronism Galati SJ et al. Primary aldosteronism: emerging trends. Trends in Endocrinology and Metabolism. September 2013, Vol.24, No. 9

  31. Sequelae of primary aldosteronism Galati SJ et al. Primary aldosteronism: emerging trends. Trends in Endocrinology and Metabolism. September 2013, Vol.24, No. 9

  32. Galati SJ et al. Primary aldosteronism: emerging trends. Trends in Endocrinology and Metabolism. September 2013, Vol.24, No. 9

  33. Schwatz et al. Screening for primary aldosteronism in essential hypertension: Diagnostic accuracy of the ratio of plasma aldosterone concentration to plasma renin activity. Clinical Chemistry. 51:2. 386-394

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