Supported by The Richard A. and Susan F. Smith Foundation, President's Innovation Award, Boston Children's Hospital; Michael B. Klein and Family; the Sidman Family Foundation; the Bulens/Capozzi Foundation; and the Kenneth C. Griffin Charitable Research Fund. The authors attest that they had full freedom to explore the data and analyze the results independently of any sponsor and that they had sole authority to make the final decision to submit the material for publication. Disclosures: J.D.M. and P.J.dN. have patents pending for the isolation and usage of mitochondria have patents pending for the isolation and usage of mitochondria. All other authors have nothing to disclose with regard to commercial support.

This is the first study to report normal fetal LV circumferential strain. These data may be useful as a reference for assessing fetal cardiac function. The retrospective study design and relatively low frame rates used in this study were important limitations.

Background Pulmonary valve replacement (PVR) in repaired tetralogy of Fallot (TOF) reduces pulmonary regurgitation and decreases right ventricular (RV) dilation, but its long-term impact on ventricular tachycardia (VT) and mortality is unknown. This study aimed to determine the incidence of death and VT in TOF following PVR and test the hypothesis that PVR leads to improvement in these outcomes. Methods and Results A total of 98 patients with TOF and late PVR for RV dilation were identified. Matched controls were identified for 77 of these; controls had TOF with RV dilation but no PVR. Matching was done by age (±2y) and baseline QRS duration (±30msec). There were no significant differences in age, QRS duration, type or decade of initial repair, age at TOF repair, or presence of pre-PVR VT between the two groups; limited echocardiographic and MRI data showed no difference in left ventricular function but more RV dilation among PVR patients than controls. In the PVR group, there were 13 events over 272 patient-years. There was no significant change in QRS duration for any group. Overall 5- and 10-year freedom from death and/or VT was 80% and 41%. In the matched comparison, there were no significant differences seen in VT, death, or combined VT and/or death; p = 0.32, 0.06 (nearly favoring controls), 0.21. Conclusions This cohort experienced either VT or death every 20 patient-years. In a matched comparison with a similar TOF group, late PVR for symptomatic PR/RV dilation did not reduce the incidence of VT or death.

Background Cardiac manifestations in multisystem inflammatory syndrome in children (MIS-C) occur in ∼80% of patients. Left ventricular (LV) systolic dysfunction is the most frequent cardiac finding. Methods In this single-center, retrospective cohort study we report on detailed assessment of LV function in MIS-C patients using strain and strain rate analysis. We compare those with normal peak systolic strain z-scores (both longitudinal and circumferential strain) to those with abnormal peak systolic strain z-scores (decreased circumferential and/or longitudinal strain). Results Among 25 patients, 14 (56%) were male, 20 (80%) were Black or Hispanic, 13 (52%) were overweight/obese, and median age was 11.4 years (IQR, 7.5 to 16). Median ejection fraction (EF) was 55.2% (IQR, 48.3 to 58%) with the abnormal strain patients having lower EF (p<0.01). Demographics were similar between groups. The abnormal strain patients had more organ systems involved and were more likely to require inotropic support. When comparing MIS-C patients with normal EF (n=15) to controls, MIS-C patients had lower peak systolic strain as well as lower early diastolic strain rates. In patients with initially depressed function, EF normalized in 8/10 (80%) but 4/11 (36%) patients had persistently abnormal systolic strain after discharge. Conclusion Left ventricular systolic dysfunction is common in the acute phase of MIS-C and detection may be improved with strain imaging. Longitudinal cardiac follow-up is imperative as some patients may be at risk for persistent LV dysfunction.

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