Ventricular-Vascular Coupling in Marfan and Non-Marfan Aortopathies

Background--marfan syndrome (mfs) and familial non-syndromal thoracic aortic aneurysm and dissection (ns-taad) are genetic aortopathies causing aortic dilatation with increased aortic stiffness. left ventricular (lv) contractility and ventricular-vascular coupling index (vvi) were compared between mfs and ns-taad and determinants of vvi were investigated. methods and results--patients with mfs (m 57,f 47) and ns-taad (m 72,f 39) were studied by echocardiography and compared with controls (m 77,f 71). aortic geometry,hemodynamics,lv work,lv contractility (end-systolic elastance [ees]),and vvi were documented. aortic sinuses were equally dilated in mfs (19.7 2.4) and ns-taad (19.8 1.8) compared to controls (16.2 1.4 mm·m-2,p<0.001). aortic stiffness index was increased in mfs (9.7 5.1) and ns-taad (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 ns-taad (492 109 mj·m-2 p<0.01). the lv ees was reduced in mfs (1.32 0.19) compared to controls (1.65 0.29 mm hg·ml-1,p<0.01) but increased in ns-taad (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 ns-taad (0.62 0.09). treatment with β-blockers was associated with partial normalization of vvi in mfs. a vvi =0.8 was associated with increased risk of death and heart failure in mfs. conclusions--left ventricular contractility and ventricular-vascular coupling are abnormal in mfs but preserved in ns-taad,and are independent of aortic stiffness,consistent with intrinsic impairment of myocardial contractility in mfs. © 2016 the authors.