1 Technical Efficacy: Stage 3 J. Magn. Reson. Imaging 2018;47:372-379.

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.

Background Throughout the literature, patellofemoral instability (PI) is defined as an increased risk of re-/luxation of the patella within the patellofemoral joint (PFJ). In most patients it is caused by traumatic patella luxation or the existence of a range of predisposing anatomic risk factors leading to an unphysiological movement sequence within the PFJ also known as patellofemoral maltracking. In order to provide an individualized therapy approach, clinical and radiological evaluation of those risk factors of variable magnitude becomes essential. Diagnostic imaging such as magnetic resonance imaging (MRI), plain radiography, and computed tomography (CT) are straightforward diagnostic tools in terms of evaluation and treatment of PI. Method In this review we performed a precise analysis of today’s literature concerning the radiological evaluation of anatomic risk factors leading to PI. The purpose of the review is to present a logical compilation of the different anatomical risk factors causing PI and provide a straight overview of valuable radiological imaging techniques. Results and Conclusion PI is frequently based on a multifactorial disposition. The most relevant predisposing risk factors are trochlea dysplasia, rupture of the medial patellofemoral ligament (MPFL), patella alta, abnormal tibial tubercle to trochlea groove distance (TT-TG), femoral torsion deformities, and genu valgum. Although plain X-rays may provide basic diagnostic value, cross-sectional imaging (MRI, CT) is the standard radiological tool in terms of evaluation and detection of severity of predisposing anatomic variants leading to PI. Key Points:  Citation Format