Interatrial block as a first clinical presentation of atrial cardiomyopathy related to a novel LMNA variant: a case report.
Eur Heart J Case Rep
; 7(12): ytad532, 2023 Dec.
Article
en En
| MEDLINE
| ID: mdl-38130860
ABSTRACT
Background:
Interatrial block (IAB) is a conduction delay in Bachmann's bundle with a well-described association with structural heart disease, supraventricular arrhythmias, and cardiovascular events. Casesummary:
We report the case of an asymptomatic 35-year-old man in whom the presence of IAB at electrocardiogram led to a comprehensive evaluation including speckle-tracking echocardiography, 24â h Holter monitoring, and genetic testing. Speckle-tracking echocardiography demonstrated a decrease in the longitudinal strain of interventricular septum, a typical feature of LMNA-related cardiomyopathy, and decreased indices of left atrial deformation. A diagnosis of cardiac laminopathy related to the frame shift variant c.1367 (p.Asn456Thrfs*24) of the LMNA gene was made. A dual-chamber implantable cardioverter defibrillator implantation was performed for the high risk of life-threatening ventricular tachyarrhythmias.Discussion:
This case demonstrates that IAB could be a rare presentation of a life-threatening laminopathy. Strain echocardiography is an essential tool to evaluate the deposition of fibrosis tissue in subclinical cardiomyopathies. Our report describes a novel variant of LMNA gene associated with a high risk of sudden cardiac death.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Contexto en salud:
6_ODS3_enfermedades_notrasmisibles
Problema de salud:
6_cardiovascular_diseases
/
6_other_circulatory_diseases
Idioma:
En
Revista:
Eur Heart J Case Rep
Año:
2023
Tipo del documento:
Article
País de afiliación:
Italia