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

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 mmHg*mL*HR vs. 248,595 ± 70,510 mmHg*mL*HR; 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.

Keywords: 3D speckle tracking echocardiography; Non-invasive pressure–volume loop analysis; Overweight; Turner syndrome.