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Magnetic resonance imaging 4-D flow-based analysis of aortic hemodynamics in Turner syndrome

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Abstract

Background

Cardiovascular surveillance is important in Turner syndrome because of the increased risk of aortic dilation and dissection with consecutively increased mortality.

Objective

To compare 4-D flow MRI for the characterization of aortic 3-D flow patterns, dimensions and vessel wall parameters in pediatric patients with Turner syndrome and age-matched controls.

Materials and methods

We performed 4-D flow MRI measuring in vivo 3-D blood flow with coverage of the thoracic aorta in 25 patients with Turner syndrome and in 16 female healthy controls (age mean ± standard deviation were 16 ± 5 years and 17 ± 4 years, respectively). Blood flow was visualized by time-resolved 3-D path lines. Visual grading of aortic flow in terms of helices and vortices was performed by two independent observers. Quantitative analysis included measurement of aortic diameters, quantification of peak systolic wall shear stress, pulsatility index and oscillatory shear index at eight defined sites.

Results

Patients with Turner syndrome had significantly larger aortic diameters normalized to BSA, increased vortices in the ascending aorta and elevated helix flow in the ascending and descending aorta compared to controls (all P<0.03). Patients with abnormal helical or vortical flow in the ascending aorta had significantly larger diameters of the ascending aorta (P<0.03). Peak systolic wall shear stress, pulsatility index and oscillatory shear index were significantly lower in Turner patients compared to controls (p=0.02, p=0.002 and p=0.01 respectively).

Conclusion

Four-dimensional flow MRI provides new insights into the altered aortic hemodynamics and wall shear stress that could have an impact on the development of aortic dissections.

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Acknowledgement

Raoul Arnold and Marie Neu have contributed equally to this work and are both first authors.

Author information

Authors and Affiliations

  1. Department of Congenital Heart Disease and Pediatric Cardiology, University Medical Center Heidelberg, Im Neuenheimer Feld 430, D-69120, Heidelberg, Germany

    Raoul Arnold

  2. Department of Pediatric Hematology/Oncology/Hemostaseology, University Medical Center, Mainz, Germany

    Marie Neu

  3. Department of Congenital Heart Defects and Pediatric Cardiology, Heart Center, University of Freiburg, Freiburg im Breisgau, Germany

    Daniel Hirtler

  4. Department of General Pediatrics, Adolescent Medicine and Neonatology, Center for Pediatrics, Medical Center – University of Freiburg, Freiburg im Breisgau, Germany

    Charlotte Gimpel

  5. Department of Radiology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA

    Michael Markl & Julia Geiger

  6. Department of Biomedical Engineering, McCormick School of Engineering, Northwestern University, Chicago, IL, USA

    Michael Markl

  7. Department of Radiology, University Children’s Hospital, Zürich, Switzerland

    Julia Geiger

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  1. Raoul Arnold
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  2. Marie Neu
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  3. Daniel Hirtler
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  4. Charlotte Gimpel
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  5. Michael Markl
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  6. Julia Geiger
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Corresponding author

Correspondence to Raoul Arnold.

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Conflicts of interest

Dr. Markl receives funding from the National Institutes of Health (grant number R01HL115828).

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Arnold, R., Neu, M., Hirtler, D. et al. Magnetic resonance imaging 4-D flow-based analysis of aortic hemodynamics in Turner syndrome. Pediatr Radiol 47, 382–390 (2017). https://doi.org/10.1007/s00247-016-3767-8

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  • DOI: https://doi.org/10.1007/s00247-016-3767-8

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