Takayasu's disease is a rare chronic vasculitis of unknown aetiology. Initial symptoms and signs are non-specific, and a high index of suspicion is needed to make the correct diagnosis. The disease is associated with a high incidence of morbidity, and a significant risk of premature death. Serological tests have proved unreliable in distinguishing active from quiescent disease, with non-invasive imaging currently offering the best option for early diagnosis, and monitoring the response to treatment. In this review, we detail the epidemiology, pathophysiology, clinical features, imaging characteristics, and currently available treatments.

BackgroundReal-time cardiovascular magnetic resonance (CMR) assessment of ventricular volumes and function enables data acquisition during free-breathing. The requirement for high spatiotemporal resolution in children necessitates the use of highly accelerated imaging techniques.MethodsA novel real-time balanced steady state free precession (bSSFP) spiral sequence reconstructed using Compressed Sensing (CS) was prospectively validated against the breath-hold clinical standard for assessment of ventricular volumes in 60 children with congenital heart disease. Qualitative image scoring, quantitative image quality, as well as evaluation of biventricular volumes was performed. Standard BH and real-time measures were compared using the paired t-test and agreement for volumetric measures were evaluated using Bland Altman analysis.ResultsAcquisition time for the entire short axis stack (~ 13 slices) using the spiral real-time technique was ~ 20 s, compared to ~ 348 s for the standard breath hold technique. Qualitative scores reflected more residual aliasing artefact (p < 0.001) and lower edge definition (p < 0.001) in spiral real-time images than standard breath hold images, with lower quantitative edge sharpness and estimates of image contrast (p < 0.001).There was a small but statistically significant (p < 0.05) overestimation of left ventricular (LV) end-systolic volume (1.0 ± 3.5 mL), and underestimation of LV end-diastolic volume (− 1.7 ± 4.6 mL), LV stroke volume (− 2.6 ± 4.8 mL) and LV ejection fraction (− 1.5 ± 3.0%) using the real-time technique. We also observed a small underestimation of right ventricular stroke volume (− 1.8 ± 4.9 mL) and ejection fraction (− 1.4 ± 3.7%) using the real-time imaging technique. No difference in inter-observer or intra-observer variability were observed between the BH and real-time sequences.ConclusionsReal-time bSSFP imaging using spiral trajectories combined with a compressed sensing reconstruction showed good agreement for quantification of biventricular metrics in children with heart disease, despite slightly lower image quality. This technique holds the potential for free breathing data acquisition, with significantly shorter scan times in children.Electronic supplementary materialThe online version of this article (10.1186/s12968-018-0500-9) contains supplementary material, which is available to authorized users.

CMR can provide an effective measurement of SPC flow prior to TCPC. Young age at BCPC, high preoperative oxygen saturation and smaller PAs are associated with increased SPC flow, which may promote increased postoperative pleural drainage and lengthen recovery.

BackgroundAnomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary artery anomaly. This study shows the role of cardiovascular magnetic resonance (CMR) in assessing young patients following surgical repair of ALCAPA.Methods6 patients, aged 9-21 years, with repaired ALCAPA (2 Tackeuchi method, 4 direct re-implantation) underwent CMR because of clinical suspicion of myocardial ischemia. Imaging used short and long axis cine images (assess ventricular function), late-gadolinium enhancement (LGE) (detect segmental myocardial fibrosis), adenosine stress perfusion (detect reversible ischaemia) and 3D whole-heart imaging (visualize proximal coronary arteries).ResultsThe left ventricular (LV) global systolic function was preserved in all patients (mean LV ejection fraction = 62.7% ± 4.23%). The LV volumes were within the normal ranges, (mean indexed LVEDV = 75.4 ± 3.5 ml/m2, LVESV = 31.6 ± 9.4 ml/m2). In 1 patient, hypokinesia of the anterior segments was visualized. Five patients showed sub-endocardial LGE involving the basal, antero-lateral wall and the anterior papillary muscle. Three patients had areas of reversible ischemia. In these 3, 3D whole-heart MRA showed that the proximal course of the left coronary artery was occluded (confirmed with cardiac catheterisation).ConclusionsCMR is a good, non-invasive, radiation-free investigation in the post-surgical evaluation of ALCAPA. In referred patients we show that basal, antero-lateral sub-endocardial myocardial fibrosis is a characteristic finding. Furthermore, stress adenosine CMR perfusion, can identify reversible ischemia in this group, and was indicative of left coronary artery occlusion.

BackgroundAortic arch geometry is linked to abnormal blood pressure (BP) response to maximum exercise. This study aims to quantitatively assess whether aortic arch geometry plays a role in blood pressure (BP) response to exercise.Methods60 age- and BSA-matched subjects – 20 post-aortic coarctation (CoA) repair, 20 transposition of great arteries post arterial switch operation (ASO) and 20 healthy controls – had a three-dimensional (3D), whole heart magnetic resonance angiography (MRA) at 1.5 Tesla, 3D geometric reconstructions created from the MRA. All subjects underwent cardiopulmonary exercise test on the same day as MRA using an ergometer cycle with manual BP measurements. Geometric analysis and their correlation with BP at peak exercise were assessed.ResultsArch curvature was similarly acute in both the post-CoA and ASO cases [0.05 ± 0.01 vs. 0.05 ± 0.01 (1/mm/m2); p = 1.0] and significantly different to that of normal healthy controls [0.05 ± 0.01 vs. 0.03 ± 0.01 (1/mm/m2), p < 0.001]. Indexed transverse arch cross sectional area were significantly abnormal in the post-CoA cases compared to the ASO cases (117.8 ± 47.7 vs. 221.3 ± 44.6; p < 0.001) and controls (117.8 ± 47.7 vs. 157.5 ± 27.2 mm2; p = 0.003). BP response to peak exercise did not correlate with arch curvature (r = 0.203, p = 0.120), but showed inverse correlation with indexed minimum cross sectional area of transverse arch and isthmus (r = -0.364, p = 0.004), and ratios of minimum arch area/ descending diameter (r = -0.491, p < 0.001).ConclusionTransverse arch and isthmus hypoplasia, rather than acute arch angulation plays a role in the pathophysiology of BP response to peak exercise following CoA repair.