Hy Van Lam1, Michael Groth1, Thomas Mir2, Peter Bannas3, Gunnar K Lund3, Charlotte M Jahnke4, Malte Warncke3, Kai-Jonathan Maas3, Gerhard Adam3, Jochen Herrmann1, Enver Tahir5. 1. Department of Pediatric Radiology, University Hospital Hamburg Eppendorf, Hamburg, Germany. 2. Department of Pediatric Cardiology, University Heart Center, Hamburg, Germany. 3. Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Hospital Hamburg Eppendorf, Martinistr. 52, 20246, Hamburg, Germany. 4. Department of General and Interventional Cardiology, University Heart Center, Hamburg, Germany. 5. Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Hospital Hamburg Eppendorf, Martinistr. 52, 20246, Hamburg, Germany. e.tahir@uke.de.
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.
OBJECTIVES: To evaluate systolic cardiac dysfunction in paediatric MFSpatients with chest wall deformity using cardiac magnetic resonance (CMR) imaging and feature-tracking strain analysis. METHODS: Forty paediatric MFSpatients (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 MFSpatients 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 MFSpatients. A subgroup of MFSpatients 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 MFSpatients 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 MFSpatients compared to controls, but not GRS (p = 0.31). RV GLS was attenuated in MFSpatients compared to controls (- 21 ± 3 vs. - 23 ± 3%, p < 0.05). CONCLUSION:Chest wall deformity in paediatric MFSpatients is associated with reduced RV volume, ejection fraction and GLS. Feature-tracking CMR also indicates impairment of systolic LV function in paediatric MFSpatients. 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.
Authors: Hang Chen; Malte L Warncke; Kai Muellerleile; Dennis Saering; Antonia Beitzen-Heineke; Anna Kisters; Monika Swiderska; Ersin Cavus; Charlotte M Jahnke; Gerhard Adam; Gunnar K Lund; Enver Tahir Journal: Eur Radiol Date: 2021-12-13 Impact factor: 7.034
Authors: Dustin Möbius; Antonia Fitzek; Niels Hammer; Axel Heinemann; Alexandra Ron; Julia Schädler; Johann Zwirner; Benjamin Ondruschka Journal: Int J Legal Med Date: 2021-07-22 Impact factor: 2.686