title: Ventricular-Vascular Coupling in Marfan and Non-Marfan Aortopathies.
abstract: 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.
  
  
  
  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-2P<0.01). The LV Eeswas 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.
  
  
  
  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.
authors.affiliation: Charite Hospital, Berlin, Germany.
authors.forename: Farina
authors.initials: F
authors.lastname: Loeper
authors.affiliation: Erasmus University, Rotterdam, Netherlands.
authors.forename: Jantine
authors.initials: J
authors.lastname: Oosterhof
authors.affiliation: Erasmus University, Rotterdam, Netherlands.
authors.forename: Mark
authors.initials: M
authors.lastname: van den Dorpel
authors.affiliation: Sydney Medical School, University of Sydney, Australia.
authors.forename: Denise
authors.initials: D
authors.lastname: van der Linde
authors.affiliation: Sydney Medical School, University of Sydney, Australia.
authors.forename: Yaxin
authors.initials: Y
authors.lastname: Lu
authors.affiliation: Sydney Medical School, University of Sydney, Australia.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Robertson
authors.affiliation: Sydney Medical School, University of Sydney, Australia.
authors.forename: Brett
authors.initials: B
authors.lastname: Hambly
authors.affiliation: Sydney Medical School, University of Sydney, Australia richmond.jeremy@sydney.edu.au.
authors.forename: Richmond
authors.initials: R
authors.lastname: Jeremy
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D017545:Aortic Aneurysm, Thoracic / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology*; D001315:Australia / Q000453:epidemiology; D004108:Dilatation, Pathologic; D004452:Echocardiography; D015150:Echocardiography, Doppler; D005260:Female; D006333:Heart Failure / Q000453:epidemiology; D006352:Heart Ventricles; D006439:Hemodynamics; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology*; D008875:Middle Aged; D009026:Mortality; D009200:Myocardial Contraction; D012850:Sinus of Valsalva / Q000473:pathology; D059289:Vascular Stiffness; D016277:Ventricular Function, Left; D055815:Young Adult
chemical_list: D000319:Adrenergic beta-Antagonists
keywords: aneurysm; matrix metalloproteinases; mechanics; myocardium; β‐blocker