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Silvia Giuliana Priori

Full Professor of Cardiology at the University of Pavia, director of the Molecular Cardiology Unit at the IRCCS ICS Maugeri Pavia


Phone: +39 0382 592040 / +39 0382 592051

Lab members
  • Silvia G. Priori, Full Professor of Cardiology at the University of Pavia, director of the Molecular Cardiology Unit at the IRCCS ICS Maugeri Pavia
  • Carlo Napolitano, assistant professor at the University of Pavia, cardiologist
  • Andrea Mazzanti, researcher, cardiologist
  • Raffaella Bloise, geneticist
  • Maira Marino, research nurse
  • Mirella Memmi, genetic biologist
  • Patrick Gambelli, molecular biologist
  • Barbara Colombi, molecular biologist
  • Jannì Nastoli, molecular biologist
  • Alessio Guarracino, molecular biologist
  • Marco Denegri, molecular biologist
  • Rossana Bongianino, molecular biologist
  • Elisa Tavazzani, cellular electrophysiologist
  • Chiara Marabelli, molecular and structural biologist
  • Deni Kukavica, post-doctoral student and cardiology resident
  • Alessandro Trancuccio, medical doctor and PhD student
SG Priori Molecular Cardiology lab

Molecular Cardiology Unit, founded and directed by Prof. Silvia G. Priori, is the national referral center for patients with inherited arrhythmia syndromes, and for the past twenty years, it has successfully conducted both clinical and basic science research.


Clinical Research

The availability of clinical and genetic data collected from over ten thousand patients has permitted the elucidation of some of the crucial aspects of management of patients with cardiomyopathies and channelopathies. Focusing on some recent examples in the field of cardiomyopathies, the team led by Prof. Priori has described the natural history of arrhythmogenic cardiomyopathy, a disease of the heart muscle characterized by an uncanny arrhythmic potential. Similarly, Priori lab has recently proposed a novel classification system for Catecholaminergic Polymorphic Ventricular Tachycardia, a channelopathy characterized by exercise- or emotion-induced life-threatening ventricular arrhythmias.

Since its foundation, clinical research on the stratification of the arrhythmic risk in the Long QT Syndrome, a group of genetic diseases characterized by prolongation of the QT interval and predisposition to suffer life-threatening arrhythmic events, has been a cornerstone of the lab’s activity. Twenty years of research culminated in the recent development and external validation of a first-in-class risk prediction model capable of accurately predicting the risk of life-threatening arrhythmias in patients with Long QT Syndrome, which has been endorsed by the 2022 European Society of Cardiology guidelines: 1-2-3 LQTS Risk Calculator

Basic Science Research

Basic science research in the laboratories of Prof. Priori assembles in vivo, in vitro and in silico techniques to dissect the arrhythmogenic mechanisms at the heart of inherited arrhythmia syndromes and to develop game-changing therapies. Following the development of the RYR2 gene as the principal cause of the autosomal dominant form of Catecholaminergic Polymorphic Ventricular Tachycardia in 2001, Priori lab has developed and extensively characterized different murine models of the disease, which have been instrumental for the development of novel RNA- and DNA-based gene therapies, for the cure of the autosomal dominant and the autosomal recessive form of the disease.

More recently, within the framework of European Research Council grant, Prof. Priori’s laboratory has developed a first porcine knock-in model of Long QT Syndrome type 8 and has developed an RNA-based gene therapy.

1-2-3 LQTS Risk Calculator
LQTS risk calculator
1-2-3 LQTS Risk Calculator

Mazzanti A, Ng K, Faragli A, Maragna R, Chiodaroli E, Orphanou N, Monteforte N, Memmi M, Gambelli P, Novelli V, Bloise R, Catalano O, Moro G, Tibollo V, Morini M, Bellazzi R, Napolitano C, Bagnardi V, Priori SG. Arrhythmogenic Right Ventricular Cardiomyopathy: Clinical Course and Predictors of Arrhythmic Risk. J Am Coll Cardiol. 2016 Dec 13;68(23):2540-2550. doi: 10.1016/j.jacc.2016.09.951. PMID: 27931611.

Priori SG, Mazzanti A, Santiago DJ, Kukavica D, Trancuccio A, Kovacic JC. Precision Medicine in Catecholaminergic Polymorphic Ventricular Tachycardia: JACC Focus Seminar 5/5. J Am Coll Cardiol. 2021 May 25;77(20):2592-2612. doi: 10.1016/j.jacc.2020.12.073. PMID: 34016269.

Mazzanti A, Trancuccio A, Kukavica D, Pagan E, Wang M, Mohsin M, Peterson D, Bagnardi V, Zareba W, Priori SG. Independent validation and clinical implications of the risk prediction model for long QT syndrome (1-2-3-LQTS-Risk). Europace. 2022 Apr 5;24(4):614-619. doi: 10.1093/europace/euab238. PMID: 34505884.

Denegri M, Avelino-Cruz JE, Boncompagni S, De Simone SA, Auricchio A, Villani L, Volpe P, Protasi F, Napolitano C, Priori SG. Viral gene transfer rescues arrhythmogenic phenotype and ultrastructural abnormalities in adult calsequestrin-null mice with inherited arrhythmias. Circ Res. 2012 Mar 2;110(5):663-8. doi: 10.1161/CIRCRESAHA.111.263939. Epub 2012 Jan 31. PMID: 22298808.

Bongianino R, Denegri M, Mazzanti A, Lodola F, Vollero A, Boncompagni S, Fasciano S, Rizzo G, Mangione D, Barbaro S, Di Fonso A, Napolitano C, Auricchio A, Protasi F, Priori SG. Allele-Specific Silencing of Mutant mRNA Rescues Ultrastructural and Arrhythmic Phenotype in Mice Carriers of the R4496C Mutation in the Ryanodine Receptor Gene (RYR2). Circ Res. 2017 Aug 18;121(5):525-536. doi: 10.1161/CIRCRESAHA.117.310882. Epub 2017 Jun 15. PMID: 28620067.