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Open Access 01-06-2021 | Marfan Syndrome | Cardiac

Impact of chest wall deformity on cardiac function by CMR and feature-tracking strain analysis in paediatric patients with Marfan syndrome

Authors: Hy Van Lam, Michael Groth, Thomas Mir, Peter Bannas, Gunnar K. Lund, Charlotte M. Jahnke, Malte Warncke, Kai-Jonathan Maas, Gerhard Adam, Jochen Herrmann, Enver Tahir

Published in: European Radiology | Issue 6/2021

Abstract

Objectives

To evaluate systolic cardiac dysfunction in paediatric MFS patients with chest wall deformity using cardiac magnetic resonance (CMR) imaging and feature-tracking strain analysis.

Methods

Forty paediatric MFS patients (16 ± 3 years, range 8−22 years) and 20 age-matched healthy controls (16 ± 4 years, range 11−24 years) were evaluated retrospectively. Biventricular function and volumes were determined using cine sequences. Feature-tracking CMR was used to assess global systolic longitudinal (GLS), circumferential (GCS) and radial strain (GRS). A dedicated balanced turbo field echo sequence was used to quantify chest wall deformity by measuring the Haller index (HI).

Results

LV volumes and ejection fraction (EF) were similar in MFS patients and controls. There was a trend for lower right ventricular (RV) volume (75 ± 17 vs. 81 ± 10 ml/m², p = 0.08), RV stroke volume (41 ± 12 vs. 50 ± 5 ml/m², p < 0.001) and RVEF (55 ± 10 vs. 62 ± 6%, p < 0.01) in MFS patients. A subgroup of MFS patients had an increased HI compared to controls (4.6 ± 1.7 vs. 2.6 ± 0.3, p < 0.001). They demonstrated a reduced RVEF compared to MFS patients without chest wall deformity (50 ± 11% vs. 58 ± 8%, p = 0.01) and controls (p < 0.001). LV GLS was attenuated when HI ≥ 3.25 (- 16 ± 2 vs. - 18 ± 3%, p = 0.03), but not GCS and GRS. LV GLS (p < 0.01) and GCS (p < 0.0001) were attenuated in MFS patients compared to controls, but not GRS (p = 0.31). RV GLS was attenuated in MFS patients compared to controls (- 21 ± 3 vs. - 23 ± 3%, p < 0.05).

Conclusion

Chest wall deformity in paediatric MFS patients is associated with reduced RV volume, ejection fraction and GLS. Feature-tracking CMR also indicates impairment of systolic LV function in paediatric MFS patients.

Key Points

• Paediatric Marfan patients demonstrate reduced RV volume and ejection fraction compared to healthy controls.

• A concordant attenuation in RV global longitudinal strain was observed in Marfan patients, while the RV global circumferential strain was increased, indicating a possible compensatory mechanism.

• Subgroup analyses demonstrated alterations in RV ejection fraction and RV/LV global strain parameters, indicating a possible association of severe chest wall deformity with biventricular dysfunction in paediatric Marfan patients.

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Title: Impact of chest wall deformity on cardiac function by CMR and feature-tracking strain analysis in paediatric patients with Marfan syndrome
Authors: Hy Van Lam
Michael Groth
Thomas Mir
Peter Bannas
Gunnar K. Lund
Charlotte M. Jahnke
Malte Warncke
Kai-Jonathan Maas
Gerhard Adam
Jochen Herrmann
Enver Tahir
Publication date: 01-06-2021
Publisher: Springer Berlin Heidelberg
Keyword: Marfan Syndrome
Published in: European Radiology / Issue 6/2021
Print ISSN: 0938-7994
Electronic ISSN: 1432-1084
DOI: https://doi.org/10.1007/s00330-020-07616-9

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Impact of chest wall deformity on cardiac function by CMR and feature-tracking strain analysis in paediatric patients with Marfan syndrome

Abstract

Objectives

Methods

Results

Conclusion

Key Points

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objectives

Methods

Results

Conclusion

Key Points

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