Key result
A systematic review of the literature highlights that Marfan syndrome is associated with primary myocardial impairment and an increased susceptibility to ventricular arrhythmia and heart failure.
Why the study?
Heart failure and ventricular arrhythmia have emerged as extra-aortic cardiovascular manifestations and causes of death in Marfan syndrome, prompting a review of myocardial function, heart failure, and arrhythmia characteristics and pathophysiology.
Systematic Review
This systematic review highlights that Marfan syndrome is associated with primary myocardial impairment and increased risk of ventricular arrhythmias, independent of valvular or aortic disease.
Extra-aortic manifestations like heart failure and ventricular arrhythmia merit monitoring in Marfan syndrome; leaves open optimal screening and therapies for prospective study.
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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Demolder et al. (2020) conducted a systematic review in Marfan syndrome. A systematic review of the literature highlights that Marfan syndrome is associated with primary myocardial impairment and an increased susceptibility to ventricular arrhythmia and heart failure.
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