Is Occult Genetic Substrate the Missing Link Between Arrhythmic Mitral Annular Disjunction Syndrome and Sudden Cardiac Death?
Can J Cardiol
; 37(10): 1651-1653, 2021 10.
Article
en En
| MEDLINE
| ID: mdl-33933609
ABSTRACT
We present the case of a 28-year-old man with a history of unexplained syncope, frequent ventricular arrhythmias, familial LMNA-related dilated cardiomyopathy (DCM), and mitral annular disjunction (MAD). We provide the first association of a novel truncating LMNA variant serving as a potential vulnerable substrate for arrhythmogenic MAD syndrome. This could suggest a possible synergistic role between concealed genetic variants (resulting in fibrosis as a "substrate" for arrhythmogenesis) and the presence of mitral annular disjunction (the "trigger" with mechanical stretch initiating ventricular arrhythmias), which may provide a link between mitral valve prolapse and sudden cardiac death.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Músculos Papilares
/
Cardiomiopatía Dilatada
/
Prolapso de la Válvula Mitral
/
Muerte Súbita Cardíaca
/
Taquicardia Ventricular
/
Lamina Tipo A
/
Válvula Mitral
Tipo de estudio:
Diagnostic_studies
Límite:
Adult
/
Humans
/
Male
Idioma:
En
Revista:
Can J Cardiol
Asunto de la revista:
CARDIOLOGIA
Año:
2021
Tipo del documento:
Article
País de afiliación:
Italia