Maladaptive response to pressure overload in Marfan subjects with no valvular disease

Abstract Background Postsystolic shortening (PSS), abnormal longitudinal myocardial shortening occurring after the closure of the aortic valve, is a marker of segmental heterogeneity of loading or contractility. It occurs mainly in the setting of pressure overload and myocardial ischemia and is a sign of adverse functional myocardial remodeling. Hand grip (HG) results in increased left ventricular afterload by elevating heart rate and blood pressure and can unmask the presence of adverse functional myocardial remodeling through revealing PSS. Purpose We aimed to look for PSS presence as a sign of pressure overload intolerance at baseline and during the HG maneuver in Marfan patients assessed by echocardiography. Methods Forty Marfan subjects without a history of valvular disease or cardiac surgery and 10 control subjects were scanned with echocardiography at rest and during the HG maneuver. PSS was assessed with Tissue Doppler imaging (TDI), ensuring high frame rate acquisitions (> 150 fps) at rest and after 1 minute HG maneuver in the basal septum. PSS was considered to be present when the systolic myocardial velocity wave in the spectral TDI had a double-peak appearance with the second peak higher than the first one. Results No significant differences in the prevalence of PSS were observed at rest between Marfan and control subjects. Baseline blood pressure was similar in both groups. However, during isometric effort, PSS appeared in 33% of Marfan and 0% of control subjects (p=0,036). One control subject with PSS at rest normalized the deformation during HG. Conclusion A HG challenge might be useful to detect PSS as an early marker of cardiomyopathy in Marfan subjects, promoting a closer follow up in these subjects.