Abstract
Primary aldosteronism was first described by Dr. Jerome Conn in 1955 as a clinical entity of potassium depletion and hypertension caused by an adrenocortical adenoma [1]. Patients often had episodes of severe muscle weakness and paralysis, tetany and paraesthesias, and hypertension was the rule. At that time, it was believed that it accounted for <1% of hypertension cases [2].
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Conn JW, Louis LH. Primary aldosteronism, a new clinical entity. Ann Intern Med. 1956;44(1):1–15.
Ganguly A. Primary aldosteronism. N Engl J Med. 1998;339(25):1828–34.
Milliez P, Girerd X, Plouin PF, Blacher J, Safar ME, Mourad JJ. Evidence for an increased rate of cardiovascular events in patients with primary aldosteronism. J Am Coll Cardiol. 2005;45(8):1243–8.
Chao C-T, Wu V-C, Kuo C-C, Lin Y-H, Chang C-C, Chueh SJ, et al. Diagnosis and management of primary aldosteronism: An updated review. Annals of Medicine 2013;45(4):375–383.
Rossi GP, Bernini G, Caliumi C, Desideri G, Fabris B, Ferri C, et al. A prospective study of the prevalence of primary aldosteronism in 1,125 hypertensive patients. J Am Coll Cardiol. 2006;48(11):2293–300.
Funder JW, Carey RM, Mantero F, Murad MH, Reincke M, Shibata H, et al. The management of primary aldosteronism: case detection, diagnosis, and treatment: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2016;101(5):1889–916.
Funder JW, Carey RM, Fardella C, Gomez-Sanchez CE, Mantero F, Stowasser M, et al. Case detection, diagnosis, and treatment of patients with primary aldosteronism: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2008;93(9):3266–81.
Monticone S, Viola A, Tizzani D, Crudo V, Burrello J, Galmozzi M, et al. Primary aldosteronism: who should be screened? Horm Metab Res. 2012;44(3):163–9.
Calhoun DA, Nishizaka MK, Zaman MA, Harding SM. Aldosterone excretion among subjects with resistant hypertension and symptoms of sleep apnea. Chest. 2004;125(1):112–7.
Di Murro A, Petramala L, Cotesta D, Zinnamosca L, Crescenzi E, Marinelli C, et al. Renin-angiotensin-aldosterone system in patients with sleep apnoea: prevalence of primary aldosteronism. J Renin-Angiotensin-Aldosterone Syst. 2010;11(3):165–72.
Gonzaga CC, Gaddam KK, Ahmed MI, Pimenta E, Thomas SJ, Harding SM, et al. Severity of obstructive sleep apnea is related to aldosterone status in subjects with resistant hypertension. J Clin Sleep Med. 2010;6(4):363–8.
Pratt-Ubunama MN, Nishizaka MK, Boedefeld RL, Cofield SS, Harding SM, Calhoun DA. Plasma aldosterone is related to severity of obstructive sleep apnea in subjects with resistant hypertension. Chest. 2007;131(2):453–9.
Sim JJ, Yan EH, Liu IL, Rasgon SA, Kalantar-Zadeh K, Calhoun DA, et al. Positive relationship of sleep apnea to hyperaldosteronism in an ethnically diverse population. J Hypertens. 2011;29(8):1553–9.
Yang L, Zhang H, Cai M, Zou Y, Jiang X, Song L, et al. Effect of spironolactone on patients with resistant hypertension and obstructive sleep apnea. Clin Exp Hypertens. 2016;38(5):464–8.
Gaddam K, Pimenta E, Thomas SJ, Cofield SS, Oparil S, Harding SM, et al. Spironolactone reduces severity of obstructive sleep apnoea in patients with resistant hypertension: a preliminary report. J Hum Hypertens. 2010;24(8):532–7.
Rossi GP. A comprehensive review of the clinical aspects of primary aldosteronism. Nat Rev Endocrinol. 2011;7(8):485–95.
Ahmed AH, Cowley D, Wolley M, Gordon RD, Xu S, Taylor PJ, et al. Seated saline suppression testing for the diagnosis of primary aldosteronism: a preliminary study. J Clin Endocrinol Metab. 2014;99(8):2745–53.
Magill SB, Raff H, Shaker JL, Brickner RC, Knechtges TE, Kehoe ME, et al. Comparison of adrenal vein sampling and computed tomography in the differentiation of primary aldosteronism. J Clin Endocrinol Metab. 2001;86(3):1066–71.
Kloos RT, Gross MD, Francis IR, Korobkin M, Shapiro B. Incidentally discovered adrenal masses. Endocr Rev. 1995;16(4):460–84.
Young WF, Stanson AW, Thompson GB, Grant CS, Farley DR, van Heerden JA. Role for adrenal venous sampling in primary aldosteronism. Surgery. 2004;136(6):1227–35.
Rossi GP, Auchus RJ, Brown M, Lenders JW, Naruse M, Plouin PF, et al. An expert consensus statement on use of adrenal vein sampling for the subtyping of primary aldosteronism. Hypertension. 2014;63(1):151–60.
Webb R, Mathur A, Chang R, Baid S, Nilubol N, Libutti SK, et al. What is the best criterion for the interpretation of adrenal vein sample results in patients with primary hyperaldosteronism? Ann Surg Oncol. 2012;19(6):1881–6.
Young WF, Stanson AW. What are the keys to successful adrenal venous sampling (AVS) in patients with primary aldosteronism? Clin Endocrinol. 2009;70(1):14–7.
Miotto D, De Toni R, Pitter G, Seccia TM, Motta R, Vincenzi M, et al. Impact of accessory hepatic veins on adrenal vein sampling for identification of surgically curable primary aldosteronism. Hypertension. 2009;54(4):885–9.
Matsuura T, Takase K, Ota H, Yamada T, Sato A, Satoh F, et al. Radiologic anatomy of the right adrenal vein: preliminary experience with MDCT. AJR Am J Roentgenol. 2008;191(2):402–8.
Ota H, Seiji K, Kawabata M, Satani N, Omata K, Ono Y, et al. Dynamic multidetector CT and non-contrast-enhanced MR for right adrenal vein imaging: comparison with catheter venography in adrenal venous sampling. Eur Radiol. 2016;26(3):622–30.
Omura K, Ota H, Takahashi Y, Matsuura T, Seiji K, Arai Y, et al. Anatomical variations of the right adrenal vein: concordance between multidetector computed tomography and catheter venography. Hypertension. 2017;69:428.
Reardon MA, Angle JF, Abi-Jaoudeh N, Bruns DE, Haverstick DM, Matsumoto AH, et al. Intraprocedural cortisol levels in the evaluation of proper catheter placement in adrenal venous sampling. J Vasc Interv Radiol. 2011;22(11):1575–80.
Rossi E, Regolisti G, Perazzoli F, Negro A, Grasselli C, Santi R, et al. Intraprocedural cortisol measurement increases adrenal vein sampling success rate in primary aldosteronism. Am J Hypertens. 2011;24(12):1280–5.
Stowasser M. Improving the success and reliability of adrenal venous sampling: focus on intraprocedural cortisol measurement. Clin Chem. 2012;58(9):1275–7.
Rossi GP, Barisa M, Allolio B, Auchus RJ, Amar L, Cohen D, et al. The Adrenal Vein Sampling International Study (AVIS) for identifying the major subtypes of primary aldosteronism. J Clin Endocrinol Metab. 2012;97(5):1606–14.
Kupers EM, Amar L, Raynaud A, Plouin PF, Steichen O. A clinical prediction score to diagnose unilateral primary aldosteronism. J Clin Endocrinol Metab. 2012;97(10):3530–7.
Riester A, Fischer E, Degenhart C, Reiser MF, Bidlingmaier M, Beuschlein F, et al. Age below 40 or a recently proposed clinical prediction score cannot bypass adrenal venous sampling in primary aldosteronism. J Clin Endocrinol Metab. 2014;99(6):E1035–9.
Sze WC, Soh LM, Lau JH, Reznek R, Sahdev A, Matson M, et al. Diagnosing unilateral primary aldosteronism—comparison of a clinical prediction score, computed tomography and adrenal venous sampling. Clin Endocrinol. 2014;81(1):25–30.
Venos ES, So B, Dias VC, Harvey A, Pasieka JL, Kline GA. A clinical prediction score for diagnosing unilateral primary aldosteronism may not be generalizable. BMC Endocr Disord. 2014;14:94.
Mendichovszky IA, Powlson AS, Manavaki R, Aigbirhio FI, Cheow H, Buscombe JR, et al. Targeted molecular imaging in adrenal disease—an emerging role for metomidate PET-CT. Diagnostics (Basel). 2016;6(4):42.
Burton TJ, Mackenzie IS, Balan K, Koo B, Bird N, Soloviev DV, et al. Evaluation of the sensitivity and specificity of (11)C-metomidate positron emission tomography (PET)-CT for lateralizing aldosterone secretion by Conn’s adenomas. J Clin Endocrinol Metab. 2012;97(1):100–9.
Duncan JL 3rd, Fuhrman GM, Bolton JS, Bowen JD, Richardson WS. Laparoscopic adrenalectomy is superior to an open approach to treat primary hyperaldosteronism. Am Surg. 2000;66(10):932–5; discussion 5–6
Haveran LA, Novitsky YW, Czerniach DR, Kaban GK, Kelly JJ, Litwin DE. Benefits of laparoscopic adrenalectomy: a 10-year single institution experience. Surg Laparosc Endosc Percutan Tech. 2006;16(4):217–21.
Meria P, Kempf BF, Hermieu JF, Plouin PF, Duclos JM. Laparoscopic management of primary hyperaldosteronism: clinical experience with 212 cases. J Urol. 2003;169(1):32–5.
Lubikowski J, Uminski M, Andrysiak-Mamos E, Pynka S, Fuchs H, Wojcicki M, et al. From open to laparoscopic adrenalectomy: thirty years’ experience of one medical centre. Endokrynol Pol. 2010;61(1):94–101.
Muth A, Ragnarsson O, Johannsson G, Wangberg B. Systematic review of surgery and outcomes in patients with primary aldosteronism. Br J Surg. 2015;102(4):307–17.
Steichen O, Amar L, Chaffanjon P, Kraimps JL, Menegaux F, Zinzindohoue F. SFE/SFHTA/AFCE consensus on primary aldosteronism, part 6: adrenal surgery. Ann Endocrinol (Paris). 2016;77(3):220–5.
Lim PO, Jung RT, MacDonald TM. Raised aldosterone to renin ratio predicts antihypertensive efficacy of spironolactone: a prospective cohort follow-up study. Br J Clin Pharmacol. 1999;48(5):756–60.
Parthasarathy HK, Menard J, White WB, Young WF Jr, Williams GH, Williams B, et al. A double-blind, randomized study comparing the antihypertensive effect of eplerenone and spironolactone in patients with hypertension and evidence of primary aldosteronism. J Hypertens. 2011;29(5):980–90.
Karagiannis A, Tziomalos K, Papageorgiou A, Kakafika AI, Pagourelias ED, Anagnostis P, et al. Spironolactone versus eplerenone for the treatment of idiopathic hyperaldosteronism. Expert Opin Pharmacother. 2008;9(4):509–15.
Born-Frontsberg E, Reincke M, Rump LC, Hahner S, Diederich S, Lorenz R, et al. Cardiovascular and cerebrovascular comorbidities of hypokalemic and normokalemic primary aldosteronism: results of the German Conn’s Registry. J Clin Endocrinol Metab. 2009;94(4):1125–30.
Mulatero P, Monticone S, Bertello C, Viola A, Tizzani D, Iannaccone A, et al. Long-term cardio- and cerebrovascular events in patients with primary aldosteronism. J Clin Endocrinol Metab. 2013;98(12):4826–33.
Savard S, Amar L, Plouin PF, Steichen O. Cardiovascular complications associated with primary aldosteronism: a controlled cross-sectional study. Hypertension. 2013;62(2):331–6.
Catena C, Colussi G, Nadalini E, Chiuch A, Baroselli S, Lapenna R, et al. Cardiovascular outcomes in patients with primary aldosteronism after treatment. Arch Intern Med. 2008;168(1):80–5.
Marzano L, Colussi G, Sechi LA, Catena C. Adrenalectomy is comparable with medical treatment for reduction of left ventricular mass in primary aldosteronism: meta-analysis of long-term studies. Am J Hypertens. 2015;28(3):312–8.
Rossi GP, Bernini G, Desideri G, Fabris B, Ferri C, Giacchetti G, et al. Renal damage in primary aldosteronism: results of the PAPY Study. Hypertension. 2006;48(2):232–8.
Sechi LA, Novello M, Lapenna R, Baroselli S, Nadalini E, Colussi GL, et al. Long-term renal outcomes in patients with primary aldosteronism. JAMA. 2006;295(22):2638–45.
Catena C, Colussi G, Sechi LA. Mineralocorticoid receptor antagonists and renal involvement in primary aldosteronism: opening of a new era. Eur J Endocrinol. 2013;168(1):C1–5.
Fallo F, Pilon C, Urbanet R. Primary aldosteronism and metabolic syndrome. Horm Metab Res. 2012;44(3):208–14.
Catena C, Lapenna R, Baroselli S, Nadalini E, Colussi G, Novello M, et al. Insulin sensitivity in patients with primary aldosteronism: a follow-up study. J Clin Endocrinol Metab. 2006;91(9):3457–63.
Fagart J, Hillisch A, Huyet J, Barfacker L, Fay M, Pleiss U, et al. A new mode of mineralocorticoid receptor antagonism by a potent and selective nonsteroidal molecule. J Biol Chem. 2010;285(39):29932–40.
Colussi G, Catena C, Sechi LA. Spironolactone, eplerenone and the new aldosterone blockers in endocrine and primary hypertension. J Hypertens. 2013;31(1):3–15.
Choi M, Scholl UI, Yue P, Bjorklund P, Zhao B, Nelson-Williams C, et al. K+ channel mutations in adrenal aldosterone-producing adenomas and hereditary hypertension. Science. 2011;331(6018):768–72.
Beuschlein F, Boulkroun S, Osswald A, Wieland T, Nielsen HN, Lichtenauer UD, et al. Somatic mutations in ATP1A1 and ATP2B3 lead to aldosterone-producing adenomas and secondary hypertension. Nat Genet. 2013;45(4):440–4. 444e1–2
Tauber P, Aichinger B, Christ C, Stindl J, Rhayem Y, Beuschlein F, et al. Cellular pathophysiology of an adrenal adenoma-associated mutant of the plasma membrane Ca(2+)-ATPase ATP2B3. Endocrinology. 2016;157(6):2489–99.
Scholl UI, Goh G, Stolting G, de Oliveira RC, Choi M, Overton JD, et al. Somatic and germline CACNA1D calcium channel mutations in aldosterone-producing adenomas and primary aldosteronism. Nat Genet. 2013;45(9):1050–4.
Piaditis G, Markou A, Papanastasiou L, Androulakis II, Kaltsas G. Progress in aldosteronism: a review of the prevalence of primary aldosteronism in pre-hypertension and hypertension. Eur J Endocrinol. 2015;172(5):R191–203.
Mulatero P, Stowasser M, Loh KC, Fardella CE, Gordon RD, Mosso L, et al. Increased diagnosis of primary aldosteronism, including surgically correctable forms, in centers from five continents. J Clin Endocrinol Metab. 2004;89(3):1045–50.
Stowasser M, Sharman J, Leano R, Gordon RD, Ward G, Cowley D, et al. Evidence for abnormal left ventricular structure and function in normotensive individuals with familial hyperaldosteronism type I. J Clin Endocrinol Metab. 2005;90(9):5070–6.
Gouli A, Kaltsas G, Tzonou A, Markou A, Androulakis II, Ragkou D, et al. High prevalence of autonomous aldosterone secretion among patients with essential hypertension. Eur J Clin Investig. 2011;41(11):1227–36.
Piaditis GP, Kaltsas GA, Androulakis II, Gouli A, Makras P, Papadogias D, et al. High prevalence of autonomous cortisol and aldosterone secretion from adrenal adenomas. Clin Endocrinol. 2009;71(6):772–8.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2018 Springer Science+Business Media Singapore
About this chapter
Cite this chapter
Soh, L.M. (2018). Conn’s Syndrome. In: Parameswaran, R., Agarwal, A. (eds) Evidence-Based Endocrine Surgery. Springer, Singapore. https://doi.org/10.1007/978-981-10-1124-5_27
Download citation
DOI: https://doi.org/10.1007/978-981-10-1124-5_27
Published:
Publisher Name: Springer, Singapore
Print ISBN: 978-981-10-1123-8
Online ISBN: 978-981-10-1124-5
eBook Packages: MedicineMedicine (R0)