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Background Cardiac involvement is common and is the leading cause of mortality in Fabry disease (FD). We explored the association between cardiovascular magnetic resonance (CMR) myocardial strain, T1 mapping, late gadolinium enhancement (LGE) and left ventricular hypertrophy (LVH) in patients with FD. Methods In this prospective study, 38 FD patients (45.0 ± 14.5 years, 37% male) and 8 healthy controls (40.1 ± 13.7 years, 63% male) underwent 3 T CMR including cine balanced steady-state free precession (bSSFP), LGE and modified Look-Locker Inversion recovery (MOLLI) T1 mapping. Global longitudinal (GLS) and circumferential (GCS) strain and base-to-apex longitudinal strain (LS) and circumferential strain (CS) gradients were derived from cine bSSFP images using feature tracking analysis. Results Among FD patients, 8 had LVH (FD LVH+, 21%) and 17 had LGE (FD LGE+, 45%). Nineteen FD patients (50%) had neither LVH nor LGE (FD LVH- LGE-). None of the healthy controls had LVH or LGE. FD patients and healthy controls did not differ significantly with respect to GLS (− 15.3 ± 3.5% vs. − 16.3 ± 1.5%, p  = 0.45), GCS (− 19.4 ± 3.0% vs. -19.5 ± 2.9%, p  = 0.84) or base-to-apex LS gradient (7.5 ± 3.8% vs. 9.3 ± 3.5%, p  = 0.24). FD patients had significantly lower base-to-apex CS gradient (2.1 ± 3.7% vs. 6.5 ± 2.2%, p  = 0.002) and native T1 (1170.2 ± 37.5 ms vs. 1239.0 ± 18.0 ms, p  < 0.001). Base-to-apex CS gradient differentiated FD LVH- LGE- patients from healthy controls (OR 0.42, 95% CI: 0.20 to 0.86, p  = 0.019), even after controlling for native T1 (OR 0.24, 95% CI: 0.06 to 0.99, p  = 0.049). In a nested logistic regression model with native T1, model fit was significantly improved by the addition of base-to-apex CS gradient (χ 2 (df = 1) = 11.04, p  < 0.001). Intra- and inter-observer agreement were moderate to good for myocardial strain parameters: GLS (ICC 0.849 and 0.774, respectively), GCS (ICC 0.831 and 0.833, respectively), and base-to-apex CS gradient (ICC 0.737 and 0.613, respectively). Conclusions CMR reproducibly identifies myocardial strain abnormalities in FD. Loss of base-to-apex CS gradient may be an early marker of cardiac involvement in FD, with independent and incremental value beyond native T1.

Background Investigation of the myocardial strain characteristics of the left ventricular non-compaction (LVNC) phenotype with cardiovascular magnetic resonance (CMR) feature tracking. Methods CMR cine balanced steady-state free precession data sets of 59 retrospectively identified LVNC phenotype patients (40 years, IQR: 28–50 years; 51% male) and 36 healthy subjects (39 years, IQR: 30–47 years; 44% male) were evaluated for LV volumes, systolic function and mass. Hypertrabeculation in patients and healthy subjects was evaluated against established CMR diagnostic criteria. Global circumferential strain (GCS), global radial strain (GRS) and global longitudinal strain (GLS) were evaluated with feature-tracking software. Subgroup analyses were performed in patients ( n  = 25) and healthy subjects ( n  = 34) with normal LV volumetrics, and with healthy subjects ( n  = 18) meeting at least one LVNC diagnostic criteria. Results All LVNC phenotype patients, as well as a significant proportion of healthy subjects, met morphology-based CMR diagnostic criteria: non-compacted (NC): compacted myocardial diameter ratio > 2.3 (100% vs. 19.4%), NC mass > 20% (100% vs. 44.4%) and > 25% (100% vs. 13.9%), and NC mass indexed to body surface area > 15 g/m 2 (100% vs. 41.7%). LVNC phenotype patients demonstrated reduced GRS (26.4% vs. 37.1%; p  < 0.001), GCS (− 16.5% vs. -20.5%; p  < 0.001) and GLS (− 14.6% vs. -17.1%; p  < 0.001) compared to healthy subjects, with statistically significant differences persisting on subgroup comparisons of LVNC phenotype patients with healthy subjects meeting diagnostic criteria. GCS also demonstrated independent and incremental diagnostic value beyond each of the morphology-based CMR diagnostic criteria. Conclusions LVNC phenotype patients demonstrate impaired strain by CMR feature tracking, also present on comparison of subjects with normal LV volumetrics meeting diagnostic criteria. The high proportion of healthy subjects meeting morphology-based CMR diagnostic criteria emphasizes the important potential complementary diagnostic value of strain in differentiating LVNC from physiologic hypertrabeculation.

ObjectiveThe National Institute for Health and Care Excellence (NICE) clinical guidelines ‘chest pain of recent onset: assessment and diagnosis’ (update 2016) state CT coronary angiography (CTCA) should be offered as the first-line investigation for patients with stable chest pain. However, the current provision in the UK is unknown. We aimed to evaluate this and estimate the requirements for full implementation of the guidelines including geographical variation. Ancillary aims included surveying the number of CTCA-capable scanners and accredited practitioners in the UK.MethodsThe number of CTCA scans performed annually was surveyed across the National Health Service (NHS). The number of percutaneous coronary interventions performed for stable angina in the NHS in 2015 was applied to a model based on SCOT-HEART (CTCA in patients with suspected angina due to coronary heart disease: an open-label, parallel-group, multicentre trial) data to estimate the requirement for CTCA, for full guideline implementation. Details of CTCA-capable scanners were obtained from manufacturers and formally accredited practitioner details from professional societies.ResultsAn estimated 42 340 CTCAs are currently performed annually in the UK. We estimate that 350 000 would be required to fully implement the guidelines. 304 CTCA-capable scanners and 198 accredited practitioners were identified. A marked geographical variation between health regions was observed.ConclusionsThis study provides insight into the scale of increase in the provision of CTCA required to fully implement the updated NICE guidelines. A small specialist workforce and limited number of CTCA-capable scanners may present challenges to service expansion.

Aortic valve replacement is the second most common cardiothoracic procedure in the UK. With an ageing population, there are an increasing number of patients with prosthetic valves that require follow-up. Imaging of prosthetic valves is challenging with conventional echocardiographic techniques making early detection of valve dysfunction or complications difficult. CT has recently emerged as a complementary approach offering excellent spatial resolution and the ability to identify a range of aortic valve replacement complications including structural valve dysfunction, thrombus development, pannus formation and prosthetic valve infective endocarditis. This review discusses each and how CT might be incorporated into a multimodal cardiovascular imaging pathway for the assessment of aortic valve replacements and in guiding clinical management.

ObjectivesNoncardiac findings are common on coronary computed tomography angiography (CCTA). We assessed the clinical impact of noncardiac findings, and potential changes to surveillance scans with the application of new lung nodule guidelines.MethodsThis substudy of the SCOT-HEART randomized controlled trial assessed noncardiac findings identified on CCTA. Clinically significant noncardiac findings were those causing symptoms or requiring further investigation, follow-up or treatment. Lung nodule follow-up was undertaken following the 2005 Fleischner guidelines. The potential impact of the 2015 British Thoracic Society (BTS) and the 2017 Fleischner guidelines was assessed.ResultsCCTA was performed in 1,778 patients and noncardiac findings were identified in 677 (38%). In 173 patients (10%) the abnormal findings were clinically significant and in 55 patients (3%) the findings were the cause of symptoms. Follow-up imaging was recommended in 136 patients (7.6%) and additional clinic consultations were organized in 46 patients (2.6%). Malignancy was diagnosed in 7 patients (0.4%). Application of the new lung nodule guidelines would have reduced the number of patients undergoing a follow-up CT scan: 68 fewer with the 2015 BTS guidelines and 78 fewer with the 2017 Fleischner guidelines; none of these patients subsequently developed malignancy.ConclusionsClinically significant noncardiac findings are identified in 10% of patients undergoing CCTA. Application of new lung nodule guidelines will reduce the cost of surveillance, without the risk of missing malignancy.Key Points• Clinically significant noncardiac findings occur in 10% of patients undergoing CCTA.• Noncardiac findings may be an important treatable cause of chest pain• Further imaging investigations for noncardiac findings were recommended in 8% of patients after CCTA.• New lung nodule follow-up guidelines will result in cost savings.

This randomized, controlled trial compared standard renal-replacement therapy with more intensive therapy in critically ill patients with acute kidney injury and failure of at least one nonrenal organ or sepsis. Intensive renal support did not decrease mortality, improve recovery of kidney function, or reduce the rate of nonrenal organ failure as compared with thrice-weekly intermittent hemodialysis. Intensive renal support did not decrease mortality, improve recovery of kidney function, or reduce the rate of nonrenal organ failure as compared with thrice-weekly intermittent hemodialysis. Acute kidney injury is a common complication of acute illness, affecting approximately 2 to 7% of hospitalized patients 1 – 4 and more than 35% of critically ill patients. 5 – 8 Renal-replacement therapy is the mainstay of supportive treatment of patients with severe acute kidney injury; its use is required in 5 to 6% of critically ill patients and is associated with in-hospital mortality rates of 50 to 80%. 5 , 9 – 12 The optimal timing for the initiation, method, and dosing of renal-replacement therapy remains uncertain more than 60 years after the first clinical use of hemodialysis in patients with acute kidney injury. 13 – . . .