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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 M...

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Autores principales: Loeper, Farina, Oosterhof, Jantine, van den Dorpel, Mark, van der Linde, Denise, Lu, Yaxin, Robertson, Elizabeth, Hambly, Brett, Jeremy, Richmond
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5210366/
https://www.ncbi.nlm.nih.gov/pubmed/27852591
http://dx.doi.org/10.1161/JAHA.116.003705
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author Loeper, Farina
Oosterhof, Jantine
van den Dorpel, Mark
van der Linde, Denise
Lu, Yaxin
Robertson, Elizabeth
Hambly, Brett
Jeremy, Richmond
author_facet Loeper, Farina
Oosterhof, Jantine
van den Dorpel, Mark
van der Linde, Denise
Lu, Yaxin
Robertson, Elizabeth
Hambly, Brett
Jeremy, Richmond
author_sort Loeper, Farina
collection PubMed
description 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.
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spelling pubmed-52103662017-01-05 Ventricular‐Vascular Coupling in Marfan and Non‐Marfan Aortopathies Loeper, Farina Oosterhof, Jantine van den Dorpel, Mark van der Linde, Denise Lu, Yaxin Robertson, Elizabeth Hambly, Brett Jeremy, Richmond J Am Heart Assoc Original Research 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. John Wiley and Sons Inc. 2016-11-16 /pmc/articles/PMC5210366/ /pubmed/27852591 http://dx.doi.org/10.1161/JAHA.116.003705 Text en © 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell. This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial‐NoDerivs (http://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Original Research
Loeper, Farina
Oosterhof, Jantine
van den Dorpel, Mark
van der Linde, Denise
Lu, Yaxin
Robertson, Elizabeth
Hambly, Brett
Jeremy, Richmond
Ventricular‐Vascular Coupling in Marfan and Non‐Marfan Aortopathies
title Ventricular‐Vascular Coupling in Marfan and Non‐Marfan Aortopathies
title_full Ventricular‐Vascular Coupling in Marfan and Non‐Marfan Aortopathies
title_fullStr Ventricular‐Vascular Coupling in Marfan and Non‐Marfan Aortopathies
title_full_unstemmed Ventricular‐Vascular Coupling in Marfan and Non‐Marfan Aortopathies
title_short Ventricular‐Vascular Coupling in Marfan and Non‐Marfan Aortopathies
title_sort ventricular‐vascular coupling in marfan and non‐marfan aortopathies
topic Original Research
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5210366/
https://www.ncbi.nlm.nih.gov/pubmed/27852591
http://dx.doi.org/10.1161/JAHA.116.003705
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