Myocardial Function, Heart Failure and Arrhythmia in Marfan Syndrome: A Systematic Literature Review.
Diagnostics (Basel)
; 10(10)2020 Sep 25.
Article
em En
| MEDLINE
| ID: mdl-32992882
ABSTRACT
Marfan syndrome (MFS) is a heritable systemic connective tissue disease with important cardiovascular involvement, including aortic root dilatation and mitral valve prolapse. Life expectancy in patients with MFS is mainly determined by cardiovascular complications, among which aortic dissection or rupture are most dreaded. In recent years, heart failure and ventricular arrhythmia have drawn attention as extra-aortic cardiovascular manifestations and as additional reported causes of death. Imaging studies have provided data supporting a primary myocardial impairment in the absence of valvular disease or cardiovascular surgery, while studies using ambulatory ECG have demonstrated an increased susceptibility to ventricular arrhythmia. In this paper, current literature was reviewed in order to provide insights in characteristics, pathophysiology and evolution of myocardial function, heart failure and ventricular arrhythmia in MFS.
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Base de dados:
MEDLINE
Tipo de estudo:
Systematic_reviews
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article