Impaired myocardial deformation and aortic distensibility by cardiac MRI in girls with Turner syndrome

TL;DR

This study finds that girls with Turner syndrome have reduced heart muscle deformation and aortic flexibility, suggesting early cardiovascular issues detectable by MRI.

Contribution

The study introduces cardiac MRI-based measurements of myocardial deformation and aortic distensibility as potential early markers for cardiovascular disease in Turner syndrome.

Findings

Girls with Turner syndrome showed significantly impaired left ventricular global longitudinal strain compared to controls.

Aortic strain and distensibility were significantly lower in Turner syndrome patients in the ascending aorta region.

Inter-observer agreement for MRI measurements was substantial to perfect, supporting their reliability.

Abstract

Girls and women with Turner syndrome (TS) suffer from increased risk of cardiovascular diseases. We hypothesized that left ventricular (LV) myocardial strain and aortic elasticity will be impaired in girls with TS. Cardiac MRI of 45 girls with TS and 14 healthy control girls was performed. Tissue tracking was used to assess LV Global Longitudinal (GLS), circumferential (GCS), radial short and long axes (GRS SAX and GRS LAX) in patients compared to controls. Maximal and minimal aortic areas were measured in ascending aorta, proximal descending, and aorta at diaphragm. Regional strain and distensibility were calculated using previously validated formulas. Comparisons were made between patients with and without bicuspid aortic valve (BAV). Inter-observer agreement was assessed for myocardial strain and aortic strain and distensibility. Results of the study showed GLS was significantly impaired in TS patients − 15.6 ± 1.8% compared to the control group − 17.2 ± 1%, p value = 0.013. No significant differences were observed in other strain parameters. Aortic diameter was similar in patients and control groups. Ascending aorta strain and distensibility were significantly lower in TS patients (33 ± 19% and 9.1 ± 5.5 mm Hg− 1) compared to control group (55 ± 17% and 13.9 ± 4.9 10− 3 mm Hg− 1), p values 0.004 and 0.013. No significant differences were found in aortic strain and distensibility in the other 2 regions. No significant differences were observed between TS patients with and without BAV in myocardial strain and aortic strain and distensibility. Substantial to perfect inter-observer agreement was found for myocardial strain and aortic strain and distensibility. Cardiac MRI measurements of LV deformation and aortic function carry potential value as markers for early detection of myocardial and aortic disease in TS patients. Validation of the clinical impact and prognostic role require further longitudinal studies.

The online version contains supplementary material available at 10.1038/s41598-024-75312-5.

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