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Phenotypic Expression and Clinical Outcomes in Patients With Arrhythmogenic Cardiomyopathies.
Bariani, Riccardo; Rigato, Ilaria; Celeghin, Rudy; Marinas, Maria Bueno; Cipriani, Alberto; Zorzi, Alessandro; Pergola, Valeria; Iliceto, Sabino; Basso, Cristina; Marra, Martina Perazzolo; Corrado, Domenico; Gregori, Dario; Pilichou, Kalliopi; Bauce, Barbara.
Afiliación
  • Bariani R; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Rigato I; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Celeghin R; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Marinas MB; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Cipriani A; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Zorzi A; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Pergola V; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Iliceto S; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Basso C; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Marra MP; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Corrado D; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Gregori D; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Pilichou K; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy.
  • Bauce B; Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy. Electronic address: barbara.bauce@unipd.it.
J Am Coll Cardiol ; 83(8): 797-807, 2024 Feb 27.
Article en En | MEDLINE | ID: mdl-38383094
ABSTRACT

BACKGROUND:

In recent years, it has become evident that arrhythmogenic cardiomyopathy (ACM) displays a wide spectrum of ventricular involvement. Furthermore, the influence of various clinical phenotypes on the prognosis of the disease is currently being assessed.

OBJECTIVES:

The purpose of this study was to evaluate the impact of phenotypic expression in ACM on patient outcomes.

METHODS:

We conducted an analysis of 446 patients diagnosed with ACM. These patients were categorized into 3 groups based on their phenotype arrhythmogenic right ventricular cardiomyopathy (ARVC) (right-dominant ACM), arrhythmogenic left ventricular cardiomyopathy (ALVC) (left-dominant ACM), and biventricular arrhythmogenic cardiomyopathy (BIV). We compared clinical, instrumental, and genetic findings among these groups and also evaluated their outcomes

RESULTS:

Overall, 44% of patients were diagnosed with ARVC, 23% with ALVC, and 33% with BIV forms. Subjects showing with ARVC and BIV phenotype had a significantly higher incidence of life-threatening ventricular arrhythmias compared with ALVC (P < 0.001). On the other hand, heart failure, heart transplantation, and death caused by cardiac causes were more frequent in individuals with BIV forms compared to those with ALVC and ARVC (P < 0.001). Finally, patients with an ALVC phenotype had a higher incidence of hot phases compared with those with ARVC and BIV forms (P = 0.013).

CONCLUSIONS:

The comparison of ACM phenotypes demonstrated that patients with right ventricular involvement, such as ARVC and BIV forms, exhibit a higher incidence of life-threatening ventricular arrhythmias. Conversely, ACM forms characterized by left ventricular involvement, such as ALVC and BIV, show a higher incidence of heart failure, heart transplantation, and hot phases.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Displasia Ventricular Derecha Arritmogénica / Insuficiencia Cardíaca / Cardiomiopatías Límite: Humans Idioma: En Revista: J Am Coll Cardiol Año: 2024 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Displasia Ventricular Derecha Arritmogénica / Insuficiencia Cardíaca / Cardiomiopatías Límite: Humans Idioma: En Revista: J Am Coll Cardiol Año: 2024 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Estados Unidos