The role of the electrocardiographic phenotype in risk stratification for sudden cardiac death in childhood hypertrophic cardiomyopathy.

AIMS: The 12-lead electrocardiogram (ECG) is routinely performed in children with hypertrophic cardiomyopathy (HCM). An ECG risk score has been suggested as a useful tool for risk stratification, but this has not been independently validated. This aim of this study was to describe the ECG phenotype of childhood HCM in a large, international, multi-centre cohort and investigate its role in risk prediction for arrhythmic events. METHODS AND RESULTS: Data from 356 childhood HCM patients with a mean age of 10.1 years (±4.5) were collected from a retrospective, multi-centre international cohort. Three hundred and forty-seven (97.5%) patients had ECG abnormalities at baseline, most commonly repolarization abnormalities (n = 277, 77.8%); left ventricular hypertrophy (n = 240, 67.7%); abnormal QRS axis (n = 126, 35.4%); or QT prolongation (n = 131, 36.8%). Over a median follow-up of 3.9 years (interquartile range 2.0-7.7), 25 (7%) had an arrhythmic event, with an overall annual event rate of 1.38 (95% CI 0.93-2.04). No ECG variables were associated with 5-year arrhythmic event on univariable or multivariable analysis. The ECG risk score threshold of >5 had modest discriminatory ability [C-index 0.60 (95% CI 0.484-0.715)], with corresponding negative and positive predictive values of 96.7% and 6.7. CONCLUSION: In a large, international, multi-centre cohort of childhood HCM, ECG abnormalities were common and varied. No ECG characteristic, either in isolation or combined in the previously described ECG risk score, was associated with 5-year sudden cardiac death risk. This suggests that the role of baseline ECG phenotype in improving risk stratification in childhood HCM is limited.

The role of APT imaging in gliomas grading: A systematic review and meta-analysis.

PURPOSE: Gliomas are diagnosed and staged by conventional MRI. Although non-conventional sequences such as perfusion-weighted MRI may differentiate low-grade from high-grade gliomas, they are not reliable enough yet. The latter is of paramount importance for patient management. In this regard, we aim to evaluate the role of Amide Proton Transfer (APT) imaging in grading gliomas as a non-invasive tool to provide reliable differentiation across tumour grades. METHODS: A systematic search of PubMed, Medline and Embase was conducted to identify relevant publications between 01/01/2008 and 15/09/2020. Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2) was used to assess studies' quality. A random-effects model standardized mean difference meta-analysis was performed to assess APT's ability to differentiate low-grade gliomas (LGGs) from high-grade gliomas (HGGs), WHO 2-4 grades, wild-type from mutated isocitrate dehydrogenase (IDH) gliomas, methylated from unmethylated O6-methylguanine-DNA methyltransferase (MGMT) gliomas. Area under the curve (AUC) of the Receiver Operating Characteristic (ROC) meta-analysis was employed to assess the diagnostic performance of APT. RESULTS: 23 manuscripts met the inclusion criteria and reported the use of APT to differentiate glioma grades with histopathology as reference standard. APT-weighted signal intensity can differentiate LGGs from HGGs with an estimated size effect of (-1.61 standard deviations (SDs), p < 0.0001), grade 2 from grade 3 (-1.83 SDs, p = 0.005), grade 2 from grade 4 (-2.34 SDs, p < 0.0001) and IDH wild-type from IDH mutated (0.94 SDs, p = 0.003) gliomas. The combined AUC of 0.84 highlights the good diagnostic performance of APT-weighted imaging in differentiating LGGs from HGGs. CONCLUSIONS: APT imaging is an exciting prospect in differentiating LGGs from HGGs and with potential to predict the histopathological grade. However, more studies are required to optimize and improve its reliability.