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Baseline characteristics of the patients with Marfan syndrome
Source publication
Background: Aortic root dilatation and -dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality.
Methods: To characterize myocardial dysfunction and arrhythmia in MFS we conducted a pros...
Context in source publication
Context 1
... of the study patients declined AECG (Fig. 1). Table 1 shows the baseline characteristics of the MFS group. a Marfan systemic score is a scoring system which takes into consideration several characteristic features of MFS and assigns each of them a value between 1-3, 3 being the most specic for the disease. ...Similar publications
Background
Aortic root dilatation and—dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality.
Methods
To characterize myocardial dysfunction and arrhythmia in MFS we conducted a prospe...
Background: Aortic root dilatation and -dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality.
Methods: To characterize myocardial dysfunction and arrhythmia in MFS we conducted a pros...
Citations
... In addition to aortic complications and cardiomyopathy, arrhythmia should be recognized as a relevant manifestation of the cardiac phenotype observed in MFS [14,52,54]. Several studies have associated MFS with an increased risk of arrhythmia, as summarized in Table 4. Studies based on data from ambulatory ECG in adults have demonstrated the presence of significant ventricular ectopy (defined as >10 premature ventricular contractions per hour) in 20-35% [14,77,78]. In children with MFS, the reported frequency of ventricular arrhythmia is much lower (7% demonstrating ventricular ectopy) [79]. ...
... In children with MFS, the reported frequency of ventricular arrhythmia is much lower (7% demonstrating ventricular ectopy) [79]. Similarly, non-sustained ventricular tachycardia (NSVT) is reported in 10-20% of the adult patients with MFS and appears to be very rare in children [14,77,78,80]. However, ventricular tachycardia (VT) and SCD have been reported in both adults and children with MFS [14,81]. ...
... Patients with mitral valve prolapse, mitral valve regurgitation and previous aortic surgery have been reported to have higher risk of ventricular arrhythmia in several studies [14,[52][53][54]77,78]. Furthermore, decreased LV EF, increased LV dimensions, prolonged QTc/QTu interval and high levels of N-terminal pro b-type natriuretic peptide (NTproBNP) have also been associated with ventricular ectopy independently of valvular disease and aortic surgery [14,77,82]. ...
jats:p>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.</jats:p
