Genetics, prevalence, screening and confirmation of primary aldosteronism: a position statement and consensus of theWorking Group on Endocrine Hypertension of The European Society of Hypertension

Downloaded from http://journals.lww.com/jhypertension by BhDMf5ePHKbH4TTImqenVPdd2D9XRrfd2BOqrHQjqyVwrjztb9kjWnfhKQ46eiEiL/N6OPIVJr4= on 09/07/2020 Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved. Genetics, prevalence, screening and confirmation of primary aldosteronism: a position statement and consensus of theWorking Group on Endocrine Hypertension of The European Society of Hypertension Paolo Mulatero a , Silvia Monticone a, y , Jaap Deinum b,c, y , Laurence Amar d, y , Aleksander Prejbisz e, y , Maria-Christina Zennaro f,g , Felix Beuschlein h,i , Gian Paolo Rossi j , Tetsuo Nishikawa k Alberto Morganti l , Teresa Maria Seccia j , Yen-Hung Lin m , Francesco Fallo n , and Jiri Widimsky o Autonomous aldosterone overproduction represents the underlying condition of 5–10% of patients with arterial hypertension and carries a significant burden of mortality and morbidity. The diagnostic algorithm for primary aldosteronism is sequentially based on hormonal tests (screening and confirmation tests), followed by lateralization studies (adrenal CT scanning and adrenal venous sampling) to distinguish between unilateral and bilateral disease. Despite the recommendations of the Endocrine Society guideline, primary aldosteronism is largely underdiagnosed and undertreated with high between-centre heterogeneity. Experts from the European Society of Hypertension have critically reviewed the available literature and prepared a consensus document constituting two articles to summarize current knowledge on the epidemiology, diagnosis, treatment, and complications of primary aldosteronism. Keywords: aldosterone, primary aldosteronism, rennin, saline-loading test Abbreviations: ACTH, adrenocorticotropic hormone; APA, aldosterone-producing adenoma; ARR, aldosterone-to- renin ratio; DRC, direct renin concentration; GRA, glucocorticoid remediable aldosteronism; LCMS/MS, liquid chromatography and tandem mass spectrometry; MRAs, mineralocorticoid receptor antagonists; OSA, obstructive sleep apnea; PAC, plasma aldosterone concentration; PASNA, primary aldosteronism with seizures and neurologic abnormalities; PRA, plasma renin activity; RAS, renin–angiotensin system INTRODUCTION A rterial hypertension represents the leading modifi- able risk factor for cardiovascular disease, account- ing for 10.4 million deaths globally and 218 million attributable disability-adjusted life-years in 2017 [1]. Over half a century, randomized controlled trials have illus- trated the efficacy of blood pressure lowering in reducing the risk of major cardiovascular events, including coronary artery disease, stroke, and heart failure [2,3]. Despite a substantial improvement in hypertension awareness, treatment, and control since the 1980s, less than half of patients on medication have blood pressure values within the normal range [3,4]. The Lancet Commission on Hypertension recently highlighted that one of the major causes of poor blood pressure control is because of an Journal of Hypertension 2020, 38:1919–1928 a Division of Internal Medicine and Hypertension Unit, Department of Medical Scien- ces, University of Torino, Torino, Italy, b Department of Internal Medicine, Radboud University Medical Center, Nijmegen, Netherlands, c Department of Medicine III, University Hospital Carl Gustav Carus at the TU Dresden, Dresden, Germany, d Hypertension Unit, Assistance Publique-Ho ˆ pitaux de Paris, Ho ˆ pital europe ´ en Georges-Pompidou, Universite ´ de Paris, PARCC, Inserm, Paris, France, e Department of Hypertension, National Institute of Cardiology, Warsaw, Poland, f Universite ´ de Paris, PARCC, INSERM, Paris, France, g Assistance Publique-Ho ˆ pitaux de Paris, Ho ˆ pital Europe ´en Georges Pompidou, Service de Ge ´ ne ´ tique, Paris, France, h Klinik fu ¨ r Endokri- nologie, Diabetologie und Klinische Erna ¨ hrung, Universita ¨ tsspital Zu ¨ rich, Zu ¨ rich, Switzerland, i Medizinische Klinik und Poliklinik IV, Klinikum der Universita ¨ t Mu ¨ nchen, Munich, Germany, j Hypertension Unit and Specialized Center for Blood Pressure Disorders - Department of Medicine-DIMED, University of Padova, Padova, Italy, k Endocrinology and Diabetes Center, Yokohama Rosai Hospital, Yokohama, Japan, l Centro Fisiologia Clinica e Ipertensione, Ospedale Policlinico, Universita ` di Milano, Milan, Italy, m Division of cardiology, Department of Internal Medicine, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan, n Department of Medicine -DIMED, University of Padova, Padova, Italy and o 3rd Department of Medicine, Center for Hypertension, General University Hospital and First Faculty of Medicine, Charles University, Prague, Czech Republic Correspondence to Paolo Mulatero, MD, Division of Internal Medicine and Hyperten- sion, Department of Medical Sciences, University of Torino, Via Genova 3, 10126 Torino, Italy. Tel: +39 116336997; fax: +39 116336931; e-mail: [email protected] This manuscript was prepared with members of the Primary Aldosteronism Task Force for Clinical Practice Guideline of PA by Japan Endocrine Society (T.N.) and of the Taiwan Society of Aldosteronism (Y-H.L.). y Silvia Monticone , Jaap Deinum, Laurence Amar, and Aleksander Prejbisz contributed equally to the writing of this article. Received 14 January 2020 Revised 30 March 2020 Accepted 20 April 2020 J Hypertens 38:1919–1928 Copyright ß 2020 Wolters Kluwer Health, Inc. All rights reserved. DOI:10.1097/HJH.0000000000002510 Journal of Hypertension www.jhypertension.com 1919 Consensus Document

Genetics, prevalence, screening and confirmation of primary aldosteronism: a position statement and consensus of theWorking Group on Endocrine Hypertension of The European Society of Hypertension

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aldosterone

primary aldosteronism

rennin

salineloading test