The role of advanced echocardiography and cardiovascular magnetic resonance in the assessment of myocardial function in Marfan syndrome-An update.
Cardiovascular assessment of patients with Marfan syndrome has normally focused on the aortic root and vascular manifestations of the disease due to the high risk of aortic dissection. Although primary myocardial impairment has long been suspected in these patients, the evidence has been controversial. Advanced echocardiography and cardiovascular magnetic resonance imaging have proven to be effective, accurate, and more sensitive in the detection of subtle cardiac dysfunction. The application of these techniques to Marfan syndrome over the last 10 years has made significant progress in demonstrating the presence of primary myocardial impairment in these patients, but further work is still required to obtain confirmatory molecular, pathophysiological, and prognostic clinical data. Phenotypic expression of the disease has prognostic value, also suggesting potential effective medical therapy.
Department of Clinical Physiology, University Hospital of Örebro, Örebro, Sweden.
Anatoli
http://orcid.org/0000-0002-8336-9430
A
Kiotsekoglou
Department of Medical Physics and Bioengineering, University College Hospital, London, United Kingdom.
James C
JC
Moggridge
Cardiovascular and Cell Sciences Research Institute, St George's, University of London, London, United Kingdom.
Anne H
AH
Child
Department of Clinical Physiology, University Hospital of Örebro, Örebro, Sweden.
Peter
P
Rask
D057791:Cardiac Imaging Techniques / Q000379:methods*; D004452:Echocardiography / Q000379:methods*; D019317:Evidence-Based Medicine; D006801:Humans; D007089:Image Enhancement / Q000379:methods; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008382:Marfan Syndrome / Q000000981:diagnostic imaging*; D064847:Multimodal Imaging / Q000379:methods*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D018754:Ventricular Dysfunction / Q000000981:diagnostic imaging*
Marfan syndrome; cardiac magnetic resonance imaging; echocardiography; left atrium; left ventricular function; right atrium; right ventricular function