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3 results on '"Zafeirenia Xylouri"'

1. Cardiomyopathies in children: An overview

Author

Adalena Tsatsopoulou, Ioannis Protonotarios, Zafeirenia Xylouri, Ioannis Papagiannis, Aris Anastasakis, Ioannis Germanakis, Alexandros Patrianakos, Evangelia Nyktari, Christoforos Gavras, Georgios Papadopoulos, Soultana Meditskou, Emilia Lazarou, Antigoni Miliou, and George Lazaros

Subjects
Inherited cardiomyopathies, Paediatric cardiomyopathies, Diagnosis, Genetics, Outcome, Diseases of the circulatory (Cardiovascular) system, RC666-701
Abstract

Paediatric cardiomyopathies form a heterogeneous group of disorders characterized by structural and electrical abnormalities of the heart muscle, commonly due to a gene variant of the myocardial cell structure. Mostly inherited as a dominant or occasionally recessive trait, they might be part of a syndromic disorder of underlying metabolic or neuromuscular defects or combine early developing extracardiac abnormalities (i.e., Naxos disease). The annual incidence of 1 per 100,000 children appears higher during the first two years of life. Dilated and hypertrophic cardiomyopathy phenotypes share an incidence of 60% and 25%, respectively. Arrhythmogenic right ventricular cardiomyopathy (ARVC), restrictive cardiomyopathy, and left ventricular noncompaction are less commonly diagnosed. Adverse events such as severe heart failure, heart transplantation, or death usually appear early after the initial presentation. In ARVC patients, high-intensity aerobic exercise has been associated with worse clinical outcomes and increased penetrance in at-risk genotype-positive relatives. Acute myocarditis in children has an incidence of 1.4–2.1 cases/per 100,000 children per year, with a 6–14% mortality rate during the acute phase. A genetic defect is considered responsible for the progression to dilated cardiomyopathy phenotype. Similarly, a dilated or arrhythmogenic cardiomyopathy phenotype might emerge with an episode of acute myocarditis in childhood or adolescence. This review provides an overview of childhood cardiomyopathies focusing on clinical presentation, outcome, and pathology.

Published
2023
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2. Importance of genotype for risk stratification in arrhythmogenic right ventricular cardiomyopathy using the 2019 ARVC risk calculator

Author

Alexandros Protonotarios, Riccardo Bariani, Chiara Cappelletto, Menelaos Pavlou, Alba García-García, Alberto Cipriani, Ioannis Protonotarios, Adrian Rivas, Regitze Wittenberg, Maddalena Graziosi, Zafeirenia Xylouri, José M Larrañaga-Moreira, Antonio de Luca, Rudy Celeghin, Kalliopi Pilichou, Athanasios Bakalakos, Luis Rocha Lopes, Konstantinos Savvatis, Davide Stolfo, Matteo Dal Ferro, Marco Merlo, Cristina Basso, Javier Limeres Freire, Jose F Rodriguez-Palomares, Toru Kubo, Tomas Ripoll-Vera, Roberto Barriales-Villa, Loizos Antoniades, Jens Mogensen, Pablo Garcia-Pavia, Karim Wahbi, Elena Biagini, Aris Anastasakis, Adalena Tsatsopoulou, Esther Zorio, Juan R Gimeno, Jose Manuel Garcia-Pinilla, Petros Syrris, Gianfranco Sinagra, Barbara Bauce, Perry M Elliott, Protonotarios, Alexandro, Bariani, Riccardo, Cappelletto, Chiara, Pavlou, Menelao, García-García, Alba, Cipriani, Alberto, Protonotarios, Ioanni, Rivas, Adrian, Wittenberg, Regitze, Graziosi, Maddalena, Xylouri, Zafeirenia, Larrañaga-Moreira, José M, de Luca, Antonio, Celeghin, Rudy, Pilichou, Kalliopi, Bakalakos, Athanasio, Lopes, Luis Rocha, Savvatis, Konstantino, Stolfo, Davide, Dal Ferro, Matteo, Merlo, Marco, Basso, Cristina, Freire, Javier Limere, Rodriguez-Palomares, Jose F, Kubo, Toru, Ripoll-Vera, Toma, Barriales-Villa, Roberto, Antoniades, Loizo, Mogensen, Jen, Garcia-Pavia, Pablo, Wahbi, Karim, Biagini, Elena, Anastasakis, Ari, Tsatsopoulou, Adalena, Zorio, Esther, Gimeno, Juan R, Garcia-Pinilla, Jose Manuel, Syrris, Petro, Sinagra, Gianfranco, Bauce, Barbara, and Elliott, Perry M

Subjects
Arrhythmogenic Right Ventricular Dysplasia/genetics, Arrhythmogenic right ventricular cardiomyopathy, Genotype, Risk stratification, Sudden cardiac death, Ventricular arrhythmia, Arrhythmias, Cardiac, Risk Assessment, Death, Sudden, Cardiac, Risk Factors, Humans, Cardiology and Cardiovascular Medicine, Death, Sudden, Cardiac/epidemiology, Arrhythmogenic Right Ventricular Dysplasia
Abstract

Aims To study the impact of genotype on the performance of the 2019 risk model for arrhythmogenic right ventricular cardiomyopathy (ARVC). Methods and results The study cohort comprised 554 patients with a definite diagnosis of ARVC and no history of sustained ventricular arrhythmia (VA). During a median follow-up of 6.0 (3.1,12.5) years, 100 patients (18%) experienced the primary VA outcome (sustained ventricular tachycardia, appropriate implantable cardioverter defibrillator intervention, aborted sudden cardiac arrest, or sudden cardiac death) corresponding to an annual event rate of 2.6% [95% confidence interval (CI) 1.9–3.3]. Risk estimates for VA using the 2019 ARVC risk model showed reasonable discriminative ability but with overestimation of risk. The ARVC risk model was compared in four gene groups: PKP2 (n = 118, 21%); desmoplakin (DSP) (n = 79, 14%); other desmosomal (n = 59, 11%); and gene elusive (n = 160, 29%). Discrimination and calibration were highest for PKP2 and lowest for the gene-elusive group. Univariable analyses revealed the variable performance of individual clinical risk markers in the different gene groups, e.g. right ventricular dimensions and systolic function are significant risk markers in PKP2 but not in DSP patients and the opposite is true for left ventricular systolic function. Conclusion The 2019 ARVC risk model performs reasonably well in gene-positive ARVC (particularly for PKP2) but is more limited in gene-elusive patients. Genotype should be included in future risk models for ARVC.

Published
2022

3. Clinical and Molecular Aspects of Naxos Disease

Author

Alexandros Protonotarios, Zafeirenia Xylouri, Angeliki Asimaki, Ioannis Protonotarios, and Adalena Tsatsopoulou

Subjects
Pathology, medicine.medical_specialty, Myocarditis, business.industry, Cardiomyopathy, Plakoglobin, Context (language use), General Medicine, medicine.disease, Penetrance, Naxos disease, Palmoplantar keratoderma, Keratoderma, Palmoplantar, Heart failure, medicine, Humans, Cardiology and Cardiovascular Medicine, business, Cardiomyopathies, Hair Diseases, Arrhythmogenic Right Ventricular Dysplasia
Abstract

Naxos disease is a recessively inherited pattern of arrhythmogenic cardiomyopathy with palmoplantar keratoderma and woolly hair. The causative mutation identified in plakoglobin protein gene indicated a potential role of the desmosomal protein complex as culprit for cardiomyopathy. In the context of a family, the early evident cutaneous features may serve as a clinical screening tool to spot arrhythmogenic cardiomyopathy in subclinical stage. "Myocarditis-like episodes" may step up the disease evolution or mark a transition from concealed to symptomatic cardiomyopathy phase. Arrhythmogenic cardiomyopathy in Naxos disease shows increased penetrance and phenotypic expression but its arrhythmic risk is analogous to dominant forms.

Published
2021

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