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

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.

Ultrasound in legal medicine—a missed opportunity or simply too late? A narrative review of ultrasonic applications in forensic contexts

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Quantification of Myocardial Deformation Applying CMR-Feature-Tracking—All About the Left Ventricle?

“…In other relatively rare diseases like Marfan syndrome, Fabry disease or systemic sclerosis that can potentially affect myocardial structures, the use of CMR-FT including biventricular strain assessments and myocardial dyssynchrony was also demonstrated to sensitively detect myocardial dysfunction [87][88][89]. For example, subtle attenuations of LV and RV longitudinal function were found in patients with Marfan syndrome, while RV GCS values were increased reflecting a potential compensation mechanism and indicating the existence of a Marfan-related cardiomyopathy that is detectable by applying CMR-FT deformation analyses [90]. Furthermore, CMR-FT detected lower RV strain in patients with convalescent Kawasaki disease and RV functional impairment was more pronounced in those with persisting coronary artery lesion [91].…”

Acute impact of an endurance race on biventricular and biatrial myocardial strain in competitive male and female triathletes evaluated by feature-tracking CMR

“…1a), whereas LV global circumferential strain (GCS) and radial strain (GRS) were measured on three short-axis cine series (apical, midventricular, and basal) (Fig. 1b) [17]. Long-axis 4-chamber and three short-axis series were used to calculate RV GLS and GCS (Fig.…”

“…Long-axis 4-chamber and three short-axis series were used to calculate RV GLS and GCS (Fig. 1c, d); long-axis 4-chamber series were applied for RV free wall (FW) GLS [17]. LA and RA endocardial contours were manually delineated on end-diastolic images and propagated throughout the cardiac cycle generating longitudinal strain (Fig.…”