CSANZ 2021

Silvia G Priori

University of Pavia, Italy

Silvia G Priori, M.D., PhD was born in Torino in 1960. She graduated in Medicine in 1986 at the University of Milan (Italy) and she became specialist in Cardiology in 1990. In 1995 she obtained a PhD in Cardiac Pathophysiology. Currently, she is Full Professor of Cardiology and Director of Cardiology Specialization School at the University of Pavia (Italy), http://molecularmedicine.unipv.it/reseach-groups/cardiology/priori/ , Head of the Molecular Cardiology Unit and Director of General Medicine III Unit (April 2018-present) at the IRCCS Istituti Clinici Scientifici Maugeri SpA SB of Pavia (Ex IRCCS Fondazione S Maugeri).

She was the Director of Cardiac Rehabilitation (2010-2018) and Scientific Director of the Istituti Clinici Scientifici (ICS) of Pavia (2008-2019) and Global Scientific Director of the ICS Maugeri (2013-2018).

Dr. Priori is also Director of the Cardiovascular Genetics Program, at the Centro National de Investigaciones Cardiovasculares, Carlos III (CNIC), Madrid, Spain. The Clinic for Inherited Arrhythmogenic Disorders directed by Prof Priori at the ICS Maugeri, is a referral center for patients with Long QT Syndrome, Short QT Syndrome, Brugada Syndrome, Catecholaminergic Ventricular Tachycardia, Dilated Cardiomyopathy, Hypertrophic Cardiomyopathy and Right Ventricular Cardiomyopathy as well as for Competitive Athletes and young patients with "idiopathic" ventricular tachyarrhythmias. The Molecular diagnostic laboratories, accredited with the National Healthcare System (SSN) is tightly linked to the clinics and performs genetic screening for patients with inherited arrhythmias.

During the last 20 years Professor Priori has been involved in the study of the mechanisms of cardiac arrhythmias and has contributed to the definition of the relationship between the autonomic nervous system and triggered activity. She has implemented the Unexplained Cardiac Arrest Registry of Europe (UCARE) that has collected the world largest series of patients with a normal heart surviving an episode of ventricular

fibrillation. Since 1995 Prof. Priori has been primarily involved in the study of the genetic mechanisms of the inherited arrhythmogenic syndromes. This activity allowed her to achieve important results in the understanding of the genotype phenotype correlation both in vivo and in vitro and in the definition of the electrophysiological consequences of the cardiac ion channel mutations.

Among the major achievements in the study of genetic bases of arrhthymias are the identification of the first gene (RyR2) for a highly malignant form of cardiac arrhythmias and sudden death called catecholaminergic VT (CPVT), the discovery that gain of function mutations of the KCNJ2 gene cause short QT syndrome type 3 and the publication in the New England Journal of Medicine and JAMA of the first genotype-based risk stratification algorhythm for the patients with Long QT Syndrome. In 2012 the results of the PRELUDE multicenter clinical study has contributed to redefine the algorhythm for risk stratification in Brugada Syndrome.

The basic science research laboratories directed by Professor Priori at the ICS Maugeri have produced several transgenic mouse models that have contributed to the characterization of fundamental aspects of the pathophysiology of catecholamiergic VT (2005-2011). A new "Gene therapy Unit" has been activated in the research laboratories and in 2012 one of the transgenic animal models of CPVT has been used to develop the first viral gene transfer strategy that has been able to revert all the clinical manifestations of the disease.

Professor Priori has actively contributed to scientific societies in the field of cardiology and electrophysiology holding leadership positions listed in the honours and awards section. Between 2008-2016 Professor Priori directed the inherited arrhythmias program at New York University.

Professor Priori is Vice-President of the European Society of Cardiology and has been/ and is in the Board of a lot of Scientific Societies (i.e. Heart Rhythm, European Society of Cardiology, Italian Society of Cardiology; in 2015 she was Member of the Scientific Panel for Health of the European Community, that serves as a consulting body for the identification of area of research and for the identification of gaps that impair translation of discoveries in science to clinical practice.

Prof Priori has been the president of the European Heart Rhythm Association and has served as Past-President on the Board of Directors of the European Heart Rhythm Association. Professor Priori is Associate Editor of Circulation Research and Member of the Editorial Board of several scientific journals including Circulation, European Heart Journal, Journal of Cardiovascular Electrophysiology, Pacing and Clinical Electrophysiology, Journal of Cardiovascular Pharmacology and Therapeutics.

Professor Priori has published 485 original articles in international journals and has an H-index of Wos 110/SCOPUS 131/Goole Scholar 144 - that contributed to the nomination of one of the 2014 ''Highly Cited Researchers'', by ISI Thomson-Reuters (cf. http://highlycited.com).

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