1 research outputs found
VentricularâVascular Coupling in Marfan and NonâMarfan Aortopathies
Background: Marfan syndrome (MFS) and familial nonâsyndromal thoracic aortic
aneurysm and dissection (nsâTAAD) are genetic aortopathies causing aortic
dilatation with increased aortic stiffness. Left ventricular (LV)
contractility and ventricularâvascular coupling index (VVI) were compared
between MFS and nsâTAAD and determinants of VVI were investigated. Methods and
Results: Patients with MFS (M 57, F 47) and nsâTAAD (M 72, F 39) were studied
by echocardiography and compared with controls (M 77, F 71). Aortic geometry,
hemodynamics, LV work, LV contractility (endâsystolic elastance [Ees]), and
VVI were documented. Aortic sinuses were equally dilated in MFS (19.7±2.4) and
nsâTAAD (19.8±1.8) compared to controls (16.2±1.4 mm·mâ2, P<0.001). Aortic
stiffness index was increased in MFS (9.7±5.1) and nsâTAAD (10.8±4.7) versus
controls (5.4±2.0, P<0.01); LV stroke work was unchanged in MFS (436±74)
compared to controls (435±60) but increased in nsâTAAD (492±109 mJ·mâ2
P<0.01). The LV Ees was reduced in MFS (1.32±0.19) compared to controls
(1.65±0.29 mm Hg·mLâ1, P<0.01) but increased in nsâTAAD (1.83±0.30, P<0.01)
and VVI was abnormal in MFS (0.71±0.11) compared to controls (0.62±0.07,
P<0.01) and nsâTAAD (0.62±0.09). Treatment with ÎČâblockers was associated with
partial normalization of VVI in MFS. A VVI â„0.8 was associated with increased
risk of death and heart failure in MFS. Conclusions: Left ventricular
contractility and ventricularâvascular coupling are abnormal in MFS but
preserved in nsâTAAD, and are independent of aortic stiffness, consistent with
intrinsic impairment of myocardial contractility in MFS