In previously asymptomatic children, myocarditis can present with symptoms of acute chest pain suspicious for coronary ischemia. Cardiac MRI and echocardiographic strain imaging are noninvasive, radiation-free tests of immense diagnostic utility in these situations. Long-term studies are needed to assess prognostic significance of these findings.

Background Aortic arch obstruction can be evaluated by catheter peak-to-peak gradient or by Doppler peak instantaneous pressure gradient. Previous studies have shown moderate correlation in discrete coarctation, but few have assessed correlation in patients with more complex aortic reconstruction. Methods We carried out retrospective comparison of cardiac catheterisations and pre- and post-catheterisation echocardiograms in 60 patients with native/recurrent coarctation or aortic reconstruction. Aortic arch obstruction was defined as peak-to-peak gradient ≥25 mmHg in patients with native/recurrent coarctation and ≥10 mmHg in aortic reconstruction. Results Diastolic continuation of flow was not associated with aortic arch obstruction in either group. Doppler peak instantaneous pressure gradient, with and without the expanded Bernoulli equation, weakly correlated with peak-to-peak gradient even in patients with a normal cardiac index (r=0.36, p=0.016, and r=0.49, p=0.001, respectively). Receiver operating characteristic curve analysis identified an area under the curve of 0.61 for patients with all types of obstruction, with a cut-off point of 45 mmHg correctly classifying 64% of patients with arch obstruction (sensitivity 39%, specificity 89%). In patients with aortic arch reconstruction who had a cardiac index ≥3 L/min/m2, a cut-off point of 23 mmHg correctly classified 69% of patients (71% sensitivity, 50% specificity) with an area under the curve of 0.82. Conclusion The non-invasive assessment of aortic obstruction remains challenging. The greatest correlation of Doppler indices was noted in patients with aortic reconstruction and a normal cardiac index.

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Evaluation of myocardial mechanics after heart transplant is important in monitoring allograft function and identifying rejection. Speckle tracking global longitudinal strain (GLS) may be more sensitive to early regional changes from rejection. This study aimed to determine feasibility of GLS in pediatric hearts during surveillance echocardiograms, compare their GLS to published norms (-18% to -22%), and assess association of GLS with other indices of graft function. Retrospective review of transplant echocardiograms from 2013 to 2014. Philips QLAB was used for post-acquisition GLS analysis. Multiple linear regression was used to assess the association of GLS with echocardiographic/catheterization indices, and B-type natriuretic peptide (BNP). Forty-seven patients (84 studies) were included. Calculation of GLS was feasible in 82 studies (97%) with inter- and intra-observer variability of 0.71 and 0.69. Patients (n=9) with rejection had GLS of -16.4% (SD=3.5%) compared to those without [-16.8% (SD=3.7%)]. GLS worsened linearly with increasing Ln(BNP) (P=<.001), left ventricular volume in diastole (P=<.001), septal a' wave (P=<.001), and pulmonary capillary wedge pressure (P=<.001). Speckle tracking-based GLS is feasible and reproducible in pediatric heart recipients and is reduced at baseline. The role of GLS and BNP in detecting early systolic dysfunction warrants further investigation.

What is known and objective: Epstein-Barr virus (EBV) viraemia and autoimmune cytopenias (AICs) are significant complications that occur following paediatric solid organ transplantation. A variety of treatment methods have been investigated but limited research has focused on the utilization of rituximab in paediatric cardiac transplant recipients for these indications. Rituximab is a monoclonal antibody that binds the CD20 antigen on the surface of B-type lymphocytes resulting in B-cell cytotoxicity. It is considered a second-line therapy for treatment of autoimmune cytopenias and EBV viraemia following adult solid organ transplant (SOT) and haematopoietic stem cell transplant (HSCT). However, data for its use in the paediatric population for treatment of autoimmune cytopenias are lacking. Dosing is based on adult studies, and the frequency and length of therapy associated with resolution of EVB viraemia and AICs in paediatric cardiac transplant recipients is unknown. The objective of this retrospective study was to describe the dosing and length of therapy of expanded off-label use of rituximab for the management of refractory EBV viraemia and AICs, specifically in paediatric cardiac transplant patients.Methods: A retrospective chart review was conducted evaluating children <18 years of age who underwent cardiac transplantation, were diagnosed with EBV viraemia or autoimmune cytopenia, and subsequently received treatment with rituximab between June 1995 and October 2018. Data were analysed descriptively.Results and discussion: Of all (n = 188) paediatric cardiac transplant recipients since 1995, 10 patients met the inclusion/exclusion criteria. Primary diagnoses were EBV viraemia (n = 6), immune haemolytic anaemia (n = 3) and immune thrombocytopenic purpura (n = 1). Complete responses were observed in 83.3% and 100% of patients with EVB viraemia and AICs treated with rituximab, respectively. All patients (n = 10) received rituximab 325 mg/m 2 at weekly intervals. The number of total doses associated with complete resolution was 4-6 doses for EBV viraemia and 2-4 doses for AICs. The most common adverse events reported were neutropenia (n = 3), thrombocytopenia (n = 4), infusion reactions (n = 1) and significant anaemia (n = 2). What is new and conclusion:Although the efficacy of rituximab for treatment of EBV viraemia and autoimmune cytopenia in the paediatric cardiac transplant population How to cite this article: Kiskaddon AL, Landmesser K, Carapellucci J, Wisotzkey B, Asante-Korang A. Expanded utilization of rituximab in paediatric cardiac transplant patients.