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特纳综合征患者的左心室重构:来自无创 3D 超声心动图衍生压力-容积环分析的见解。

Left ventricular remodelling among Turner syndrome patients: insights from non-invasive 3D echocardiography-derived pressure-volume loop analysis.

机构信息

Department of Pediatric Cardiology, Saarland University Hospital, Kirrberger Straße, Geb. 9, 66421, Homburg/Saar, Germany.

Department of Pediatric Endocrinology, Saarland University Hospital, Homburg/Saar, Germany.

出版信息

Clin Res Cardiol. 2020 Jul;109(7):892-903. doi: 10.1007/s00392-019-01579-8. Epub 2019 Nov 30.

Abstract

BACKGROUND

Turner syndrome (TS) is a X-chromosomal disease affecting one in 2500-3000 female newborns. TS individuals are at high cardiovascular risk and more likely to be overweight or obese. The aim of this study was to assess left ventricular performance in TS patients through three-dimensional speckle tracking echocardiography (3DSTE) and non-invasive left ventricular pressure-volume loop (PVL) analysis. Moreover, this study focused on the impact of excess weight on the left ventricular efficiency in TS patients.

METHODS

Thirty-six TS patients and 19 healthy age-matched controls were included in this study. 3DSTE and non-invasive left ventricular PVL analysis were performed and left ventricular efficiency parameters were calculated.

RESULTS

TS patients had significantly lower values than controls in longitudinal strain (- 16.67 ± 3.23% vs. - 18.47 ± 1.87%; p = 0.029), but significantly higher values for arterial elastance (BSA) (3.31, 1.87-5.88 mmHg/mL vs. 2.99, 2.31-4.61 mmHg/mL; p = 0.011) and cardiac work (BSA) (292,070 ± 71,348 mmHgmLHR vs. 248,595 ± 70,510 mmHgmLHR; p = 0.036). Compared with normal weight patients, overweight and obese TS subjects demonstrated worse left ventricular efficiency (175.08 ± 17.73 mmHg vs. 157.24 ± 26.75 mmHg; p = 0.037). Even after excluding TS patients with cardiovascular morbidity, arterial elastance (BSA) was compared to healthy peers, significantly increased in TS patients.

CONCLUSIONS

3DSTE and non-invasive left ventricular PVL analysis might be useful tools to detect early cardiac changes in TS. Arterial elastance seems to be significantly increased in TS patients, independent of cardiovascular morbidity. Compared with normal weight TS patients, overweight/obese TS patients displayed lower left ventricular efficiency.

摘要

背景

特纳综合征(TS)是一种影响每 2500-3000 名女新生儿中 1 人的 X 染色体疾病。TS 个体存在较高的心血管风险,并且更容易超重或肥胖。本研究的目的是通过三维斑点追踪超声心动图(3DSTE)和非侵入性左心室压力-容积环(PVL)分析评估 TS 患者的左心室功能。此外,本研究重点关注超重对 TS 患者左心室效率的影响。

方法

本研究纳入了 36 名 TS 患者和 19 名年龄匹配的健康对照者。进行了 3DSTE 和非侵入性左心室 PVL 分析,并计算了左心室效率参数。

结果

与对照组相比,TS 患者的纵向应变明显较低(-16.67±3.23%比-18.47±1.87%;p=0.029),但动脉弹性(BSA)明显较高(3.31,1.87-5.88mmHg/mL 比 2.99,2.31-4.61mmHg/mL;p=0.011)和心脏工作量(BSA)(292070±71348mmHgmLHR 比 248595±70510mmHgmLHR;p=0.036)。与体重正常的患者相比,超重和肥胖的 TS 患者的左心室效率更差(175.08±17.73mmHg 比 157.24±26.75mmHg;p=0.037)。即使排除了有心血管病史的 TS 患者,TS 患者的动脉弹性(BSA)与健康同龄人相比仍显著增加。

结论

3DSTE 和非侵入性左心室 PVL 分析可能是检测 TS 患者早期心脏变化的有用工具。动脉弹性似乎在 TS 患者中显著增加,与心血管疾病无关。与体重正常的 TS 患者相比,超重/肥胖的 TS 患者的左心室效率较低。

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