dc.contributor.author
Loeper, Farina
dc.contributor.author
Oosterhof, Jantine
dc.contributor.author
van den Dorpel, Mark
dc.contributor.author
van der Linde, Denise
dc.contributor.author
Lu, Yaxin
dc.contributor.author
Robertson, Elizabeth
dc.contributor.author
Hambly, Brett
dc.contributor.author
Jeremy, Richmond
dc.date.accessioned
2018-06-08T11:11:55Z
dc.date.available
2017-02-14T08:48:56.520Z
dc.identifier.uri
https://refubium.fu-berlin.de/handle/fub188/21794
dc.identifier.uri
http://dx.doi.org/10.17169/refubium-25082
dc.description.abstract
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.
en
dc.rights.uri
http://creativecommons.org/licenses/by-nc-nd/4.0/
dc.subject
matrix metalloproteinases
dc.subject.ddc
600 Technik, Medizin, angewandte Wissenschaften::610 Medizin und Gesundheit
dc.title
Ventricular‐Vascular Coupling in Marfan and Non‐Marfan Aortopathies
dc.type
Wissenschaftlicher Artikel
dcterms.bibliographicCitation
Journal of the American Heart Association. - 5 (2016), 11, Artikel Nr. e003705
dcterms.bibliographicCitation.doi
10.1161/JAHA.116.003705
dcterms.bibliographicCitation.url
http://doi.org/10.1161/JAHA.116.003705
refubium.affiliation
Charité - Universitätsmedizin Berlin
de
refubium.mycore.fudocsId
FUDOCS_document_000000026332
refubium.note.author
Der Artikel wurde in einer reinen Open-Access-Zeitschrift publiziert.
refubium.resourceType.isindependentpub
no
refubium.mycore.derivateId
FUDOCS_derivate_000000007679
dcterms.accessRights.openaire
open access