Risk predictors in a Spanish cohort with cardiac laminopathies. The REDLAMINA registry.
Rev Esp Cardiol (Engl Ed)
; 74(3): 216-224, 2021 Mar.
Article
em En, Es
| MEDLINE
| ID: mdl-32616434
ABSTRACT
INTRODUCTION AND OBJECTIVES:
According to sudden cardiac death guidelines, an implantable cardioverter-defibrillator (ICD) should be considered in patients with LMNA-related dilated cardiomyopathy (DCM) and ≥ 2 risk factors male sex, left ventricular ejection fraction (LVEF) <45%, nonsustained ventricular tachycardia (NSVT), and nonmissense genetic variants. In this study we aimed to describe the clinical characteristics of carriers of LMNA genetic variants among individuals from a Spanish cardiac-laminopathies cohort (REDLAMINA registry) and to assess previously reported risk criteria.METHODS:
The relationship between risk factors and cardiovascular events was evaluated in a cohort of 140 carriers (age ≥ 16 years) of pathogenic LMNA variants (54 probands, 86 relatives). We considered a) major arrhythmic events (MAE) if there was appropriate ICD discharge or sudden cardiac death; b) heart failure death if there was heart transplant or death due to heart failure.RESULTS:
We identified 11 novel and 21 previously reported LMNA-related DCM variants. LVEF <45% (P=.001) and NSVT (P <.001) were related to MAE, but not sex or type of genetic variant. The only factor independently related to heart failure death was LVEF <45% (P <.001).CONCLUSIONS:
In the REDLAMINA registry cohort, the only predictors independently associated with MAE were NSVT and LVEF <45%. Therefore, female carriers of missense variants with either NSVT or LVEF <45% should not be considered a low-risk group. It is important to individualize risk stratification in carriers of LMNA missense variants, because not all have the same prognosis.Palavras-chave
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Laminopatias
Tipo de estudo:
Etiology_studies
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Guideline
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Prognostic_studies
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Risk_factors_studies
Limite:
Adolescent
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Female
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Humans
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Male
Idioma:
En
/
Es
Revista:
Rev Esp Cardiol (Engl Ed)
Ano de publicação:
2021
Tipo de documento:
Article