Impaired aortic distensibility and elevated central blood pressure in Turner Syndrome: a cardiovascular magnetic resonance study

作者：Jan Wen, Christian Trolle, Mette Viuff, Steffen Ringgaard, Esben Laugesen, Ephraim Gutmark, Dhananjay Radhakrishnan Subramaniam, Philippe Backeljauw, Iris Gutmark‐Little, N.H. Andersen, Kristian H. Mortensen, Claus Højbjerg Gravholt · 发表于：Journal of Cardiovascular Magnetic Resonance · 年份：2018 · DOI：10.1186/s12968-018-0497-0 · 被引用次数：35 · 研究领域：Genetic and Clinical Aspects of Sex Determination and Chromosomal Abnormalities、Congenital heart defects research、Williams Syndrome Research

BACKGROUND: Women with Turner Syndrome have an increased risk for aortic dissection. Arterial stiffening is a risk factor for aortic dilatation and dissection. Here we investigate if arterial stiffening can be observed in Turner Syndrome patients and is an initial step in the development of aortic dilatation and subsequent dissection. METHODS: Fifty-seven women with Turner Syndrome (48 years [29-66]) and thirty-six age- and sex-matched controls (49 years [26-68]) were included. Distensibility, blood pressure, carotid-femoral pulse wave velocity (PWV), the augmentation index (Aix) and central blood pressure were determined using cardiovascular magnetic resonance, a 24-h blood pressure measurement and applanation tonometry. Aortic distensibility was determined at three locations: ascending aorta, transverse aortic arch, and descending aorta. RESULTS: Mean aortic distensibility in the descending aorta was significantly lower in Turner Syndrome compared to healthy controls (P = 0.02), however, this was due to a much lower distensibility among Turner Syndrome with coarctation, while Turner Syndrome without coarctation had similar distensibility as controls. Both the mean heart rate adjusted Aix (31.4% vs. 24.4%; P = 0.02) and central diastolic blood pressure (78.8 mmHg vs. 73.7 mmHg; P = 0.02) were higher in Turner Syndrome compared to controls, and these indices correlated significantly with ambulatory night-time diastolic blood pressure. The presence of aortic coarctation (r = -...