Racial and Ethnic Disparity in Multisystem Inflammatory Syndrome in Children Associated With SARS-CoV-2 in Mississippi, USA.

We aimed to study the disparity in the clinical profile and outcomes of hospitalized Multisystem Inflammatory Syndrome in Children (MIS-C) patients at our center. The second goal was to examine the temporal association with preceding SARS-CoV-2 infection by race/ethnicity in our community in Mississippi. We found the racial disparity in the prevalence of MIS-C exceeded its temporal association with SARS-CoV-2 infections. We included 51 consecutive MIS-C patients hospitalized, whose median age was 9 (interquartile range [IQR] 5-12) years, 58% were male, 71% were black, 25% were white, and 4% belonged to other groups. We found a delay between onset of symptoms and hospitalization in black patients compared with white patients with a median of 2 (IQR 0-7) vs median of 0 (0-5) urgent care visits (P = .022), respectively. Black patients were hospitalized longer (median 8, IQR 2-39 days) than whites (median 5, IQR 3-14 days), P = .047. A total of 38.9% of blacks and 23.1% of whites were admitted to intensive care unit (P = .498); 36.1% of blacks had severe cardiac involvement vs 23.1% of white patients, P = .531. Future studies of MIS-C are required to improve health equity for children.

Heart Failure with Preserved Ejection Fraction in Children.

Diastolic dysfunction (DD) refers to abnormalities in the mechanical function of the left ventricle (LV) during diastole. Severe LVDD can cause symptoms and the signs of heart failure (HF) in the setting of normal or near normal LV systolic function and is referred to as diastolic HF or HF with preserved ejection fraction (HFpEF). Pediatric cardiologists have long speculated HFpEF in children with congenital heart disease and cardiomyopathy. However, understanding the risk factors, clinical course, and validated biomarkers predictive of the outcome of HFpEF in children is challenging due to heterogeneous etiologies and overlapping pathophysiological mechanisms. The natural history of HFpEF varies depending upon the patient's age, sex, race, geographic location, nutritional status, biochemical risk factors, underlying heart disease, and genetic-environmental interaction, among other factors. Pediatric onset HFpEF is often not the same disease as in adults. Advances in the noninvasive evaluation of the LV diastolic function by strain, and strain rate analysis with speckle-tracking echocardiography, tissue Doppler imaging, and cardiac magnetic resonance imaging have increased our understanding of the HFpEF in children. This review addresses HFpEF in children and identifies knowledge gaps in the underlying etiologies, pathogenesis, diagnosis, and management, especially compared to adults with HFpEF.

SARS-CoV-2 infection induced thyroid storm and heart failure in an adolescent girl.

We report a case of thyroid storm precipitated by SARS-CoV-2 infection in an adolescent girl with a history of Graves disease and dilated cardiomyopathy. This case highlights that SARS-CoV-2 infection can potentially trigger a thyrotoxicosis crisis and acute decompensated heart failure in a patient with underlying thyroid disease and myocardial dysfunction even in the absence of multi-system inflammatory syndrome in children. We systematically reviewed the thyrotoxicosis cases with SARS-CoV-2 infection and described its impact on pre-existing dilated cardiomyopathy.

Rheumatic Heart Disease in the United States: Forgotten But Not Gone: Results of a 10 Year Multicenter Review.

Background Recent evaluation of rheumatic heart disease (RHD) mortality demonstrates disproportionate disease burden within the United States. However, there are few contemporary data on US children living with acute rheumatic fever (ARF) and RHD. Methods and Results Twenty-two US pediatric institutions participated in a 10-year review (2008-2018) of electronic medical records and echocardiographic databases of children 4 to 17 years diagnosed with ARF/RHD to determine demographics, diagnosis, and management. Geocoding was used to determine a census tract-based socioeconomic deprivation index. Descriptive statistics of patient characteristics and regression analysis of RHD classification, disease severity, and initial antibiotic prescription according to community deprivation were obtained. Data for 947 cases showed median age at diagnosis of 9 years; 51% and 56% identified as male and non-White, respectively. Most (89%) had health insurance and were first diagnosed in the United States (82%). Only 13% reported travel to an endemic region before diagnosis. Although 96% of patients were prescribed secondary prophylaxis, only 58% were prescribed intramuscular benzathine penicillin G. Higher deprivation was associated with increasing disease severity (odds ratio, 1.25; 95% CI, 1.08-1.46). Conclusions The majority of recent US cases of ARF and RHD are endemic rather than the result of foreign exposure. Children who live in more deprived communities are at risk for more severe disease. This study demonstrates a need to improve guideline-based treatment for ARF/RHD with respect to secondary prophylaxis and to increase research efforts to better understand ARF and RHD in the United States.

Left Atrial Size and Function in Patients With Congenital Aortic Valve Stenosis.

Little information exists on left atrial (LA) function in healthy children and in patients with congenital aortic valve stenosis (AS). We sought to determine whether patients with significant congenital AS have detectable abnormalities in LA size and function compared with matched normal controls. Retrospective chart review and analysis of 2-dimensional echocardiographic images for phasic left atrial volumes (LAVs) and longitudinal LA strain by speckle tracking in children ≥1 years of age with ≥ moderate isolated congenital AS. LAVs and LA strain in AS cases were compared with age, gender, and body surface area-matched controls (healthy children with a normal echocardiogram). Between June 2004 and October 2012, there were 36 eligible AS cases [age 13 (range 3 to 27) years; 30 males] with a median maximal Doppler gradient of 63 (range 43 to 94) mm Hg. No significant interobserver and intraobserver differences were noted in the analyses of normal subjects. Significantly higher phasic body surface area-adjusted LAVs [preatrial contraction: p = 0.01; minimal: p = 0.04] and higher LA contractile function parameters [indexed active LA stroke volume: p = 0.03; peak negative strain p = 0.04] were noted in AS cases compared with matched controls. In conclusion, compared with normal, children with congenital AS have higher preatrial contraction and minimal phasic LA volumes and higher LA contractile function.

Effectiveness of robo-assisted lower limb rehabilitation for spastic patients: A systematic review.

BACKGROUND: Though many rehabilitative treatments are available for treatment of spasticity, thus the effectiveness of different robo-rehabilitative devices needs to be evaluated through a systematic review. OBJECTIVE: The objective of this study is to focuses on the efficacy of Robot assistive rehabilitation device for the removal of spasticity from the lower limb of Spastic patients. DATA SOURCESSOURCES: PubMed, Web of Sciences, EMBASE (Excerpta Medical database), CDSR (Cochrane database of systematic reviews), Scopus, IEEE Xplore, Wiley online library, MEDLINE (OvidSP), Science Direct, Springer Link were from January 1980 to September 2017 DATA EXTRACTIONEXTRACTION: Seventy-one publications from eleven databases published were selected using keywords Ankle foot, spasticity, robotic rehabilitation, efficacy of robotics and Ankle foot rehabilitation. The review is narrowed down to twenty-six articles which were selected for they focused on effects of Robot assistive rehabilitation device quantitatively. RESULT: A quantitative study from analyzing 26 studies comprising of 786 subjects is carried out. The major outcome of the effectiveness of the robot assistive therapy for the movement of ankle and functioning of gait is deduced. As the used protocols and treatment procedures vary, made comparative study complex or impracticable. CONCLUSION: Robo-rehabilitation possesses an ability to provide unified therapy protocols with greater ease in comparison to conventional therapies. They continuously prove to be irreplaceable assistant devices when it comes to providing excellent treatment in terms of improvement from this study. Though many mechatronic devices are available but the devices for treatment of early stage rehabilitation of stroke patients is very limited.

Staged Palliation of Cyanotic Obstructive Lesions With a Modified Right Ventricular Outflow Procedure.

BACKGROUND: Traditional palliation for biventricular cyanotic congenital heart lesions often involves staging with systemic-to-pulmonary arterial shunts to secure pulmonary blood flow (PBF) in the newborn period prior to complete repair. However, shunts may lead to life-threatening events secondary to shunt occlusion or acute coronary steal. They may be associated with morbidity secondary to diastolic runoff, systemic steal and volume loading, and do not provide pulsatile flow which has the potential to promote pulmonary artery (PA) growth. We have alternatively performed modified right ventricular outflow (mRVO) procedures by establishing antegrade right ventricle-to-PA flow. METHODS: Retrospective review of data on all patients who underwent the mRVO procedure from 2013 to 2016, including anatomy, number of interstage catheterizations, reoperations, intensive care unit admissions, hypercyanotic episodes, interval to complete repair, and mortality. RESULTS: Seventeen nonconsecutive patients included tetralogy of Fallot (n = 14), pulmonary valve stenosis (n = 2), and 1 with pulmonary atresia-intact septum; 14 had significant branch PA stenosis. Median age of first mRVO procedure was 14 days (range 5-193), and median duration of follow-up was 15.3 months (range 4-47 months). No patients had post-palliation acute hypercyanotic episodes. Nine were admitted to the ICU for persistent interstage hypoxemia, 7 of whom required reintervention prior to complete repair, which was achieved in 11 patients. Two late deaths unrelated to mRVO occurred. CONCLUSIONS: The mRVO procedure is a potential option with satisfactory results. It avoids potential shunt-related sudden death. The physiology of the mRVO palliation may provide unique benefits by providing antegrade pulsatile PBF, facilitates catheter interventions, and avoids branch PA distortion and stenosis.

Left Atrial Volumes and Strain in Healthy Children Measured by Three-Dimensional Echocardiography: Normal Values and Maturational Changes.

BACKGROUND: Assessment of left atrial (LA) size and function is important in a number of pediatric cardiac conditions including those affecting the diastolic performance of the left ventricle. Measurements of LA volume and strain by two-dimensional echocardiography rely upon inaccurate geometric assumptions and are hampered by out-of-plane motion. The objective of this study was to characterize LA volumes and strain by three-dimensional echocardiography in healthy children. METHODS: LA volumes and strain were retrospectively measured by three-dimensional echocardiography in healthy children with no known structural or functional heart disease using a commercial speckle-tracking package applied to the LA to compute maximum volume (Vmax), minimum volume (Vmin), ejection volume (LAEV), ejection fraction (LAEF), and the following components of global strain: 3D principal (3DS), longitudinal (GLS), and circumferential (GCS). RESULTS: The study population included 196 normal subjects (median age, 12 years; range, 4 days to 20.9 years). Vmax, Vmin, and LAEV increased with age and body surface area. Significant age-related declines were present in all measured functional indices including LAEF, 3DS, GLS, and GCS. Analysis of a subset of 50 subjects showed excellent agreement between Vmax derived by three-dimensional and two-dimensional biplane area-length method. Regression equations with standard deviations were generated to enable calculation of Z scores. CONCLUSIONS: LA volume and functional indices can be reliably calculated using a commercial three-dimensional analysis software. All components of LA strain decline modestly with age. Normative data generated in this study have the potential to greatly enhance the utility of three-dimensional echocardiography-derived measurements in a wide range of cardiac pathologies.

Relation of Left Atrial Size to Atrial Fibrillation in Patients Aged ≤22 Years.

Left atrial (LA) dilation has been shown to be associated with atrial fibrillation (AF) in the adult population, with some studies indicating that larger LAs are more prone to AF recurrence. The relation of LA size to AF in the pediatric and young adult population has not been investigated. In this study, all pediatric patients (aged ≤22 years) who presented to Boston Children's Hospital from January 2002 to December 2012 with AF were reviewed. Patients with significant congenital heart disease, cardiomyopathies, proven channelopathies, previous cardiac surgery, end-stage renal disease, or severe lung disease/cystic fibrosis were excluded. LA measurements were taken using the echocardiogram performed at the initial presentation. In total, 48 patients with AF were identified. The median age at presentation was 17.1 years (range 3.7 to 22.9 years); 38 patients (79%) were men. Eleven patients (23%) had at least 1 recurrence of their AF. There was no difference in body mass index, prevalence of systemic hypertension, alcohol, stimulant, or illicit drug use between those who had an isolated episode of AF and those who had a recurrence. There was no significant difference in LA dimension Z-scores between groups, with only 2 patients (1 isolated AF, 1 recurrent AF) having Z-scores >2. In conclusion, AF in the young without underlying heart disease is not associated with LA dilation.

Isolated Left Subclavian Artery From the Pulmonary Artery Masked by Pulmonary Hypertension.

Isolated subclavian artery arising from the pulmonary artery is exceedingly rare. Most cases are associated with other forms of congenital heart disease and typically present with subclavian or pulmonary steal syndromes. We report the case of a patient with pulmonary hypertension which masked the isolated subclavian artery, allowing it to masquerade as another common congenital heart lesion.

Idiopathic pericarditis and pericardial effusion in children: contemporary epidemiology and management.

BACKGROUND: Multicenter studies on idiopathic or viral pericarditis and pericardial effusion (PPE) have not been reported in children. Colchicine use for PPE in adults is supported. We explored epidemiology and management for inpatient hospitalizations for PPE in US children and risk factors for readmission. METHODS AND RESULTS: We analyzed patients in the Pediatric Health Information System database for (1) a code for PPE; (2) absence of codes for underlying systemic disease (eg, neoplastic, cardiac, rheumatologic, renal); (3) age ≥30 days and <21 years; and (4) discharge between January 1, 2007, and December 31, 2012, from 38 hospitals contributing complete data for each year of the study period. Among 11 364 hospitalizations with PPE codes during the study period, 543 (4.8%) met entry criteria for idiopathic or viral PPE. Significantly more boys were noted, especially among adolescents. No temporal trends were noted. Median age was 14.5 years (interquartile range 7.3 to 16.6 years); 78 patients (14.4%) underwent pericardiocentesis, 13 (2.4%) underwent pericardiotomy, and 11 (2.0%) underwent pericardiectomy; 157 (28.9%) had an intensive care unit stay, including 2.0% with tamponade. Median hospitalization was 3 days (interquartile range 2 to 4 days). Medications used at initial admission were nonsteroidal anti-inflammatory drugs (71.3%), corticosteroids (22.7%), aspirin (7.0%), and colchicine (3.9%). Readmissions within 1 year of initial admission occurred in 46 of 447 patients (10.3%), mostly in the first 3 months. No independent predictors of readmission were noted, but our statistical power was limited. Practice variation was noted in medical management and pericardiocentesis. CONCLUSIONS: Our report provides the first large multicenter description of idiopathic or viral PPE in children. Idiopathic or viral PPE is most common in male adolescents and is treated infrequently with colchicine.

Pulmonary deadspace and postoperative outcomes in neonates undergoing stage 1 palliation operation for single ventricle heart disease.

OBJECTIVES: Increased pulmonary dead space fraction (VD/VT) has been associated with prolonged mechanical ventilation after surgery for congenital heart disease. The association of VD/VT with clinical outcomes in neonates undergoing stage 1 palliation for single ventricle congenital heart disease has not been reported. We describe changes in VD/VT, differences in VD/VT based on shunt type (right ventricle to pulmonary artery conduit vs modified Blalock-Taussing shunt) and association of VD/VT with postoperative outcomes in patients undergoing stage 1 palliation. DESIGN: Retrospective chart review for demographic, hemodynamics, outcome information, and VD/VT values were collected at 6-hour intervals during the first 48 postoperative hours in neonates undergoing stage 1 palliation. VD/VT was calculated using mixed expired CO2 (PeCO2) obtained from capnography and paired arterial blood gas CO2 values. SETTING: Cardiac ICU in a tertiary care pediatric hospital. PATIENTS: Newborns with single ventricle congenital heart disease undergoing stage 1 palliation during 2003-2004. MEASUREMENTS AND MAIN RESULTS: Of the 51 patients, 31 had right ventricle to pulmonary artery and 20 had Blalock-Taussing shunt. Although VD/VT was lower in the Blalock-Taussing shunt group over all time points (p = 0.02), maximal VD/VT on day 1 (0.49 ± 0.07) and on day 2 (0.46 ± 0.08) were not different between the shunt groups. VD/VT decreased significantly over time in both shunt groups (p = 0.001 for right ventricle to pulmonary artery; p < 0.001 for Blalock-Taussing shunt). Higher maximal VD/VT during first 48 postoperative hours was independently associated with fewer ventilator (ß = -26.6; p = 0.035) and hospital-free days in the first month after stage 1 palliation (ß = -40.4; p = 0.002) after adjusting for potential confounders in a multivariable linear regression model. CONCLUSIONS: Increased pulmonary dead space exists early after stage 1 palliation operation for single ventricle congenital heart disease. Higher VD/VT during the first 48 postoperative hours was associated with longer duration of ventilation and hospital LOS and may be a useful marker of postoperative outcomes in this population.

Novel phenotypic markers and screening score for the metabolic syndrome in adult Asian Indians.

AIMS: The metabolic syndrome (MS) and type 2 diabetes mellitus (T2DM) are common in Asian Indians. Simple yet reliable phenotypic markers are needed for early detection of the MS in Asian Indians. METHODS: In this cross-sectional study, 126 subjects either with of without the MS were compared. Anthropometric measurements (including assessment of novel phenotypic markers) and metabolic profile were done. RESULTS: On univariate analysis, age more than 35 year, female gender, presence of buffalo hump and double chin and family history of T2DM were significantly associated with the MS. A metabolic syndrome screening (MSS) score (1.0 x double chin+1.2 x buffalo hump+1.3 x gender+1.4 x age category) was generated with a cutoff value of 3.5 out of total points of 4.9, with sensitivity and specificity 82% and 71% [area under the curve=0.83; 95% confidence Interval (0.76-0.90)], respectively in detecting individuals with the MS. CONCLUSION: We describe buffalo hump and double chin as novel phenotypic markers for detection of MS for the first time. The MSS score generated by us may be a useful tool for detection of MS in adult Asian Indians; however, it needs further validation in a larger sample of Asian Indians and in other ethnic groups.