Phenotypic Expression and Clinical Outcomes in Patients With Arrhythmogenic Cardiomyopathies.
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 outcomesRESULTS:
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.Palabras clave
Texto completo:
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Bases de datos:
MEDLINE
Asunto principal:
Displasia Ventricular Derecha Arritmogénica
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Insuficiencia Cardíaca
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Cardiomiopatías
Límite:
Humans
Idioma:
En
Revista:
J Am Coll Cardiol
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J. Am. Coll. Cardiol
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Journal of the American College of Cardiology
Año:
2024
Tipo del documento:
Article
País de afiliación:
Italia