Abstract
The mechanisms leading to cardiac remodeling in Marfan syndrome (MFS) are a matter of debate since it could be either due to structural dysfunction of the myocardial extracellular matrix or to increased afterload caused by the dilated aorta. We aim to characterize the presence of abnormal myocardial function in MFS and to investigate its potential association with increased afterload. Aorta, left ventricle (LV) and the postsystolic thickening (PST) were analyzed in echocardiography in Fbn1C1039G/+ mice and in patients with MFS in comparison with wild type (WT) mice and healthy humans. PST was more frequent in MFS than in WT mice (p < 0.05). MFS mice with PST showed larger aorta than those without PST. Patients with MFS showed larger aorta, poorer LV function and a higher prevalence of PST (56%) than did the healthy controls (23%); p = 0.003. Blood pressure was similar. The higher prevalence of PST in an experimental murine model and in MFS patients, regardless of systemic arterial pressure, suggests an increased afterload on the LV myocardium. This finding supports the use of PST as an indicator of myocardial damage and encourage searching for novel early preventive therapy.
Highlights
The mechanisms leading to cardiac remodeling in Marfan syndrome (MFS) are a matter of debate since it could be either due to structural dysfunction of the myocardial extracellular matrix or to increased afterload caused by the dilated aorta
We hypothesize that MFS patients may be more sensitive to blood pressure, despite their basal arterial pressures being in the normal range, and they develop more segmental wall stress heterogeneity due to underlying abnormal arterial and/or myocardial wall tissue
Among the 9 mo-age mice, the aortic and left ventricle (LV) dimensions were significantly larger in MFS mice than they were in wild type (WT) mice, while LV performance, assessed through the LV ejection fraction, was lower in the MFS mice
Summary
The mechanisms leading to cardiac remodeling in Marfan syndrome (MFS) are a matter of debate since it could be either due to structural dysfunction of the myocardial extracellular matrix or to increased afterload caused by the dilated aorta. The higher prevalence of PST in an experimental murine model and in MFS patients, regardless of systemic arterial pressure, suggests an increased afterload on the LV myocardium. This finding supports the use of PST as an indicator of myocardial damage and encourage searching for novel early preventive therapy. Postsystolic thickening (PST) is an abnormal longitudinal myocardial thickening/shortening occurring after the closure of the aortic valve It has been well described as a marker of segmental heterogeneity of loading or contractility, mainly in the setting of pressure overload and myocardial ischaemia[7,8,9,10], and involves adverse functional myocardial remodeling. We have examined the presence of PST in both experimental and clinical groups and its potential relationship with blood pressure and the development of aortic aneurysm
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