Open AccessVentricular‐Vascular Coupling in Marfan and Non‐Marfan Aortopathies
Author(s) -
Loeper Farina,
Oosterhof Jantine,
Dorpel Mark,
Linde Denise,
Lu Yaxin,
Robertson Elizabeth,
Hambly Brett,
Jeremy Richmond
Publication year - 2016
Publication title -
journal of the american heart association
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.494
H-Index - 85
ISSN - 2047-9980
DOI - 10.1161/jaha.116.003705
Subject(s) - medicine , cardiology , contractility , marfan syndrome , aortic dissection , hemodynamics , aortic aneurysm , aorta
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 [E es ]), 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 E es 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.