CHS is associated with decreased in-hospital mortality in T18 and T13. These results suggest CHS may be beneficial in select cases.
Multisystem inflammatory syndrome in children (MIS-C) is one of the most significant sequela of coronavirus disease 2019 in children. Emerging literature has described myocardial dysfunction in MIS-C patients using traditional and two-dimensional speckle tracking echocardiography in the acute phase. However, data regarding persistence of subclinical myocardial injury after recovery is limited. We aimed to detect these changes with deformation imaging, hypothesizing that left ventricular global longitudinal (GLS) and circumferential strain (GCS) would remain impaired in the chronic phase despite normalization of ventricular function parameters assessed by two-dimensional echocardiography. A retrospective, single-institution review of 22 patients with MIS-C was performed. Fractional shortening, GLS, and GCS, along with regional longitudinal (RLS) and circumferential strain (RCS) were compared across the acute, subacute, and chronic timepoints (presentation, 14-42, and > 42 days, respectively). Mean GLS improved from − 18.4% in the acute phase to − 20.1% in the chronic phase (p = 0.4). Mean GCS improved from − 19.4% in the acute phase to − 23.5% in the chronic phase (p = 0.03). RCS and RLS were impaired in the acute phase and showed a trend towards recovery by the chronic phase, with the exception of the basal anterolateral segment. In our longitudinal study of MIS-C patients, GLS and GCS were lower in the acute phase, corroborating with left ventricular dysfunction by traditional measures. Additionally, as function globally recovers, GLS and GCS also normalize. However, some regional segments continue to have decreased strain values which may be an important subclinical marker for future adverse events. KeywordsCoronavirus disease 2019 (COVID-19) • Multisystem inflammatory syndrome in children (MIS-C) • Twodimensional speckle tracking echocardiography (2D-STE) • Longitudinal strain • Circumferential strain Abbreviations MIS-C Multisystem inflammatory syndrome in children COVID-19 Coronavirus disease 2019 2D-STE Two-dimensional speckle tracking echocardiography GLS Global longitudinal strain GCS Global circumferential strain RLS Regional longitudinal strain RCS Regional circumferential strain FS Fractional shortening KD Kawasaki disease PICU Pediatric intensive care unit * Michael He
Objectives Congenital diaphragmatic hernia (CDH) can cause a significant mass effect in the fetal thorax, displacing the heart into the opposite hemithorax. In left‐sided CDH (L‐CDH), this is associated with smaller left‐sided cardiac structures and reduced left‐ventricular cardiac output (LVCO). The effect of these physiologic changes on cerebral blood flow is not well understood. We sought to describe the middle cerebral artery (MCA) pulsatility index (PI), a measure of cerebrovascular impedance, in fetuses with L‐CDH and those with right‐sided CDH (R‐CDH) compared with unaffected fetuses, and the relationship between MCA‐PI and LVCO. We hypothesized that MCA‐PI would be lower in fetuses with L‐CDH and similar in those with R‐CDH compared to controls, and that MCA‐PI would be correlated with LVCO. Methods We identified all fetuses with CDH evaluated at The University of California San Francisco, San Francisco, CA, USA from 2011 to 2018. Fetal echocardiograms and ultrasound scans were reviewed. Umbilical artery and MCA Doppler examinations were assessed to calculate pulsatility indices. Ventricular outputs were calculated using Doppler‐derived stroke volume and fetal heart rate. Lung‐to‐head ratio (LHR), estimated fetal weight, biparietal diameter (BPD) and head circumference (HC) were obtained from fetal sonograms. Measurements in fetuses with CDH, according to the side of the defect, were compared with those in unaffected, gestational age‐matched controls. A subset of CDH survivors had available data on neurodevelopmental outcome, as assessed using the Bayley Scales of Infant Development, 3rd edition. Results A total of 64 fetuses with CDH (L‐CDH, n = 53; R‐CDH, n = 11) comprised the study groups, with 27 unaffected fetuses serving as controls. Mean gestational age at evaluation was similar between the three groups. Compared to controls, fetuses with L‐CDH had significantly lower LVCO expressed as a percentage of combined cardiac output (CCO) (32%; 95% CI, 29–35% vs 38%; 95% CI, 33–42%; P = 0.04) and lower MCA‐PI Z‐score (−1.3; 95% CI, −1.7 to −1.0 vs 0.08; 95% CI, −0.5 to 0.6; P < 0.001), while they did not differ between the R‐CDH group and controls. There was a strong positive association between LVCO as a percentage of CCO and MCA‐PI Z‐score in the overall cohort of CDH and control fetuses (P = 0.01). BPD and HC were similar between the three groups. At neurodevelopmental follow‐up, mean cognitive, motor and language scores in the CDH group were within 1 SD of those in the general population. Conclusion MCA‐PI values are significantly lower in fetuses with L‐CDH as compared to controls, and lower LVCO was correlated with lower MCA vascular impedance. The neurodevelopmental effect of changes in MCA‐PI in response to decreased LVCO is unknown, although, on average, CDH survivors had neurodevelopmental scores in the normal range. This may reflect a fetal compensatory mechanism in response to diminished antegrade cerebral blood flow. © 2020 International Society of Ultrasound in Obstetrics and Gynecol...
Congenital heart disease is exceedingly prevalent in trisomy 13 and 18. Improved survival following congenital heart surgery has been reported, however, mortality remains significantly elevated. Utilizing inpatient data on trisomy 13 and 18 from the 2003–2016 Pediatric Health Information System database, a survival model was developed and validated using data from the California Perinatal Quality Care Collaborative and the California Office of Statewide Health Planning and Development. The study cohort included 1,761 infants with trisomy 13 and 18. Two models predicting survival to 6 months of age were developed and tested. The initial model performed excellently, with a c‐statistic of 0.87 and a c‐statistic of 0.76 in the validation cohort. After excluding procedures performed on the day of death, the revised model's c‐statistic was 0.76. Certain variables, including cardiac surgery, gastrostomy, parenteral nutrition, and mechanical ventilation, are predictive of survival to 6 months of age. This study presents a model, which potentially can inform decision‐making regarding congenital heart surgery.
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