The effect of antipsychotic medications on QTc and delirium in paediatric cardiac patients with ICU delirium.

OBJECTIVE: Children with prolonged hospital admissions for CHD often develop delirium. Antipsychotic medications (APMs) have been used to treat delirium but are known to prolong the QTc duration. There is concern for prolongation of the QTc interval in cardiac patients who may be more vulnerable to electrocardiogram (ECG) changes and may have postoperative QTc prolongation already. The goal of this study was to determine the effect of APM on QTc duration in postoperative paediatric cardiac patients and determine the effect of quetiapine and risperidone in treating delirium and QTc prolongation. DESIGN: Retrospective study, July 1, 2017-May 31, 2022. SETTING: Tertiary children's hospital. PATIENTS: Included were patients admitted to the paediatric cardiac ICU at Children's Healthcare of Atlanta. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: ECGs, delirium scores, and drug information were collected. Delirium was defined as Cornell Assessment of Pediatric Delirium (CAPD) score >9. Mixed effect models were performed to evaluate the effect of surgery on QTc change and the effect of antipsychotics on QTc and CAPD changes. There were 139 children, 55% male and 67% surgical admissions. Median age was 5.9 months. Mean QTc increased after cardiac surgery by 18 ms (p = 0.014, 95% CI 3.65-32.4). There was no significant change in QTc after antipsychotic administration (p = 0.064). The mean CAPD score decreased (12.5-7.2; p < 0.001). Quetiapine had the most improvement in delirium, and risperidone had the least improvement (77.8%, n = 14; 37.8%, n = 34, respectively; p = 0.002). CONCLUSIONS: The QTc interval did not have a statistically significant change after the administration of antipsychotics, while there was improvement in the CAPD score. APMs may be administered safely without significant prolongation of the QTc and are an effective treatment for delirium.

Antimicrobial Stewardship and Improved Antibiotic Utilization in the Pediatric Cardiac Intensive Care Unit.

Background: We developed a multidisciplinary antimicrobial stewardship team to optimize antimicrobial use within the Pediatric Cardiac Intensive Care Unit. A quality improvement initiative was conducted to decrease unnecessary broad-spectrum antibiotic use by 20%, with sustained change over 12 months. Methods: We conducted this quality improvement initiative within a quaternary care center. PDSA cycles focused on antibiotic overuse, provider education, and practice standardization. The primary outcome measure was days of therapy (DOT)/1000 patient days. Process measures included electronic medical record order-set use. Balancing measures focused on alternative antibiotic use, overall mortality, and sepsis-related mortality. Data were analyzed using statistical process control charts. Results: A significant and sustained decrease in DOT was observed for vancomycin and meropenem. Vancomycin use decreased from a baseline of 198 DOT to 137 DOT, a 31% reduction. Meropenem use decreased from 103 DOT to 34 DOT, a 67% reduction. These changes were sustained over 24 months. The collective use of gram-negative antibiotics, including meropenem, cefepime, and piperacillin-tazobactam, decreased from a baseline of 323 DOT to 239 DOT, a reduction of 26%. There was no reciprocal increase in cefepime or piperacillin-tazobactam use. Key interventions involved electronic medical record changes, including automatic stop times and empiric antibiotic standardization. All-cause mortality remained unchanged. Conclusions: The initiation of a dedicated antimicrobial stewardship initiative resulted in a sustained reduction in meropenem and vancomycin usage. Interventions did not lead to increased utilization of alternative broad-spectrum antimicrobials or increased mortality. Future interventions will target additional broad-spectrum antimicrobials.

Opioid Utilization after Cardiac Surgery in the Pediatric Medicaid-Insured Population.

OBJECTIVE: To determine the variation of outpatient opioid prescribing across the US in postoperative pediatric cardiac patients. STUDY DESIGN: Retrospective, cross-sectional study using a concatenated database of Medicaid claims between from 2016 through 2018 of children 0-17 years, discharged after cardiac surgery and receiving an opioid prescription within 30 days. Filled prescriptions were identified and converted to morphine milligram equivalents (MME). Use, duration, and dose were analyzed by sex, race, ethnicity, residence urbanicity, and region. RESULTS: Among 17 186 Medicaid-enrolled children after cardiac surgery, 2129 received opioids within 30 days of discharge. Females received lower doses than males (coefficient -0.17, P = .022). Hispanic individuals were less likely to receive opioids (coefficient 0.53, P < .05, 95% CI: 0.38-0.71) and for shorter periods (coefficient 0.83, P < .001). Midwest (MW) (OR 0.61, 95% P-values < 0.05, 95% CI: 0.46-0.80) and Northeast (NE) (OR 0.43, 95% P-values < 0.05, 95% CI: 0.30-0.61) regions were less likely to receive opioids but used higher doses compared with the Southeast (SE) (MW coefficient 0.41, Southwest (SW) coefficient 0.18, NE coefficient 0.32, West (W) coefficient 0.19, P < .05). CONCLUSIONS: There were significant variations in opioid prescribing after cardiac surgery by race, ethnicity, sex, and region. National guidelines for outpatient use of opioids in children after cardiac surgery may help limit practice variation and reduce potential harms in outpatient opioid usage.

Bullying among children with heart conditions, National Survey of Children's Health, 2018-2020.

Children with chronic illnesses report being bullied by peers, yet little is known about bullying among children with heart conditions. Using 2018-2020 National Survey of Children's Health data, the prevalence and frequency of being bullied in the past year (never; annually or monthly; weekly or daily) were compared between children aged 6-17 years with and without heart conditions. Among children with heart conditions, associations between demographic and health characteristics and being bullied, and prevalence of diagnosed anxiety or depression by bullying status were examined. Differences were assessed with chi-square tests and multivariable logistic regression using predicted marginals to produce adjusted prevalence ratios and 95% confidence intervals. Weights yielded national estimates. Of 69,428 children, 2.2% had heart conditions. Children with heart conditions, compared to those without, were more likely to be bullied (56.3% and 43.3% respectively; adjusted prevalence ratio [95% confidence interval] = 1.3 [1.2, 1.4]) and bullied more frequently (weekly or daily = 11.2% and 5.3%; p < 0.001). Among children with heart conditions, characteristics associated with greater odds of weekly or daily bullying included ages 9-11 years compared to 15-17 years (3.4 [2.0, 5.7]), other genetic or inherited condition (1.7 [1.0, 3.0]), ever overweight (1.7 [1.0, 2.8]), and a functional limitation (4.8 [2.7, 8.5]). Children with heart conditions who were bullied, compared to never, more commonly had anxiety (40.1%, 25.9%, and 12.8%, respectively) and depression (18.0%, 9.3%, and 4.7%; p < 0.01 for both). Findings highlight the social and psychological needs of children with heart conditions.

COVID Vaccination as a Strategy for Cardiovascular Disease Prevention.

PURPOSE OF REVIEW: Cardiovascular (CV) disease is a known complication of SARS-CoV-2 infection. A clear benefit of COVID-19 vaccination is a reduction mortality; however, COVID-19 vaccination may also prevent cardiovascular disease (CVD). We aim to describe CV pathology associated with SARS-CoV-2 infection and describe how COVID-19 vaccination is a strategy for CVD prevention. RECENT FINDINGS: The risks and benefits of COVID-19 vaccination have been widely studied. Analysis of individuals with and without pre-existing CVD has shown that COVID-19 vaccination can prevent morbidity associated with SARS-CoV-2 infection and reduce mortality. COVID-19 vaccination is effective in preventing myocardial infarction, cerebrovascular events, myopericarditis, and long COVID, all associated with CVD risk factors. Vaccination reduces mortality in patients with pre-existing CVD. Further study investigating ideal vaccination schedules for individuals with CVD should be undertaken to protect this vulnerable group and address new risks from variants of concern.

Cryoextraction via flexible bronchoscopy in children with tracheobronchial obstruction.

BACKGROUND: Cryoextraction via flexible bronchoscopy (FB) can be used to alleviate airway obstruction due to blood clots, casts, mucus, and foreign bodies. There is limited literature regarding the utility of cryoextraction to restore airway patency in critically ill children, especially on extracorporeal membrane oxygenation (ECMO). The aims of this study were to describe the clinical course and outcomes of children who underwent cryoextraction via FB. METHODS: A singlecenter retrospective review of children who underwent cryoextraction via FB between 2017 and 2021 was conducted. The analyzed data included diagnoses, indications for cryoextraction, respiratory support modalities, FB and chest imaging results, and outcomes. RESULTS: Eleven patients aged 3-17 years underwent a total of 33 cryoextraction sessions via FB. Patients required ECMO (n = 9) or conventional mechanical ventilation (CMV) for pneumonia, pulmonary hemorrhage, pulmonary embolism, asthma exacerbation, and cardiorespiratory failure following cardiac surgery. One patient underwent elective FB and cryoextraction for plastic bronchitis. Indications for cryoextraction included airway obstruction due to tracheobronchial thrombi (n = 8), mucus plugs (n = 1), or plastic bronchitis (n = 2). Cryoextraction via FB was performed on patients on ECMO (n = 9) and CMV (n = 2) with 6 patients requiring ≥3 cryoextraction sessions for airway obstruction. There were no complications related to cryoextraction. Patient outcomes included partial (n = 5) or complete (n = 6) restoration of airway patency with ECMO decannulation (n = 5) and death (n = 4) due to critical illness. CONCLUSIONS: Cryoextraction via FB is an effective intervention that can be utilized in critically ill children with refractory tracheobronchial obstruction to restore airway patency and to facilitate liberation from ECMO.

Mitral Valve Replacement in Infants and Children: Experience Using a 15-mm Mechanical Valve.

BACKGROUND: Reports using a 15-mm mechanical valve for mitral valve replacement (MMVR) in children are limited. We review our center's operative and postoperative experience with this valve. METHODS: We performed a single-center retrospective chart review identifying patients having undergone MMVRs between 2009 and 2022. We analyzed short- and long-term outcomes using descriptive statistics. RESULTS: Fifteen patients underwent 16 MMVRs with no operative deaths. The median age and weight at the time of operation was 6.2 months (interquartile range [IQR] 4.4-13.7), and 5.16 kg (IQR 4.5-6.9), respectively. Ten implants (66%) were placed in the supraannular position. Median postoperative duration of intubation was 1.5 days (IQR 1.0-3.75), cardiac intensive care unit length of stay was 6 days (IQR 3-13.5), and overall hospital length of stay was 17.0 days (IQR 12-48.5). Three patients (20%) experienced major adverse events postoperatively. Four of 13 patients discharged home (31%) required readmission within 30 days for subtherapeutic/supratherapeutic international normalized ratio values. There were no surgical mortalities and 4 late mortalities (27%). Six patients underwent subsequent MMVR at a median time to second MMVR of 6.8 (IQR 3.6-8.9) years. There are 6 patients with the original 15-mm MVR at a median time of 4.7 years since placement. CONCLUSIONS: We present the largest single-center cohort of patients having undergone 15-mm MMVR. Our experience is distinguished by a lower rate of major adverse events than previously reported, durability of the device, and a rapid postoperative recovery time. Appropriate and consistent anticoagulation is a notable challenge in this age group.

A comparison of high-flow nasal cannula versus non-invasive positive pressure ventilation for respiratory support in infants following cardiac surgery.

BACKGROUND: Following cardiac surgery, infants often remain endotracheally intubated upon arrival to the cardiac ICU. High-flow nasal cannula and non-invasive positive pressure ventilation are used to support patients following extubation. There are limited data on the superiority of either mode to prevent extubation failure. METHODS: We conducted a single-centre retrospective study for infants (<1 year) and/or <10 kg who underwent cardiac surgery between 3/2019-3/2020. Data included patient and clinical characteristics and operative variables. The study aimed to compare high-flow nasal cannula versus non-invasive positive pressure ventilation following extubation and their association with extubation failure. Secondarily, we examined risk factors associated with extubation failure. RESULTS: There were 424 patients who met inclusion criteria, 320 (75%) were extubated to high-flow nasal cannula, 104 (25%) to non-invasive positive pressure ventilation, and 64 patients (15%) failed extubation. The high-flow nasal cannula group had lower rates of extubation failure (11%, versus 29%, p = 0.001). Infants failing extubation were younger and had higher STAT score (p < 0.05). Compared to high-flow nasal cannula, non-invasive positive pressure ventilation patients were at 3.30 times higher odds of failing extubation after adjusting for patient factors (p < 0.0001). CONCLUSIONS: Extubation failure after cardiac surgery occurs in smaller, younger infants, and those with higher risk surgical procedures. Patients extubated to non-invasive positive pressure ventilation had 3.30 higher odds to fail extubation than patients extubated to high-flow nasal cannula. The optimal mode of respiratory support in this patient population is unknown.

Outcomes for unplanned reinterventions following paediatric cardiac surgery for tetralogy of Fallot.

BACKGROUND: Advances in surgical techniques and post-operative management of children with CHD have significantly lowered mortality rates. Unplanned cardiac interventions are a significant complication with implications on morbidity and mortality. METHODS: We conducted a single-centre retrospective case-control study for patients (<18 years) undergoing cardiac surgery for repair of Tetralogy of Fallot between January 2009 and December 2019. Data included patient characteristics, operative variables and outcomes. This study aimed to assess the incidence and risk factors for reintervention of Tetralogy of Fallot after cardiac surgery. The secondary outcome was to examine the incidence of long-term morbidity and mortality in those who underwent unplanned reinterventions. RESULTS: During the study period 29 patients (6.8%) underwent unplanned reintervention, and were matched to 58 patients by age, weight and sex. Median age was 146 days, and median weight was 5.8 kg. Operative mortality was 7%, and 1-year survival was 86% for the entire cohort (cases and controls). Hispanic patients were more likely to have reinterventions (p = 0.04) in the unadjusted analysis, while Asian, Pacific Islander and Native American (p = 0.01) in the multi-variate analysis. Patients that underwent reintervention were more likely to have post-op arrhythmia, genetic syndromes and higher operative and 1-year mortality (p < 0.05). CONCLUSION: Unplanned cardiac interventions following Tetralogy of Fallot repair are common, and associated with increased operative, and 1-year mortality. Race, genetic syndromes and post-operative arrhythmia are associated with increased odds of unplanned reinterventions. Future studies are needed to identify modifiable risk factors to minimise unplanned reinterventions.

The Association of Workload and Outcomes in the Pediatric Cardiac ICU.

OBJECTIVES: Healthcare workload has emerged as an important metric associated with poor outcomes. To measure workload, studies have used bed occupancy as a surrogate. However, few studies have examined frontline provider (fellows, nurse practitioners, physician assistants) workload and outcomes. We hypothesize frontline provider workload, measured by bed occupancy and staffing, is associated with poor outcomes and unnecessary testing. DESIGN: A retrospective single-center, time-stamped orders, ordering provider identifiers, and patient data were collected. Regression was performed to study the influence of occupancy on orders, length of stay, and mortality, controlling for age, weight, admission type, Society of Thoracic Surgery-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Mortality score, diagnosis, number of surgeries, orders, provider staffing, attending experience, and time fixed effects. SETTING: Twenty-seven bed tertiary cardiac ICU in a free-standing children's hospital. PATIENTS: Patients (0-18 yr) admitted to the pediatric cardiac ICU, January 2018 to December 2019. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 16,500 imaging and 73,113 laboratory orders among 1,468 patient admissions. Median age 6 months (12 d to 5 yr), weight 6.2 kg (3.7-16.2 kg); 840 (57.2%) surgical and 628 (42.8%) medical patients. ICU teams consisted of 16 attendings and 31 frontline providers. Mortality 4.4%, median stay 5 days (2-11 d), and median bed occupancy 89% (78-93%). Every 10% increase in bed occupancy had 7.2% increase in imaging orders per patient (p < 0.01), 3% longer laboratory turn-around time (p = 0.015), and 3 additional days (p < 0.01). Higher staffing (> 3 providers) was associated with 6% less imaging (p = 0.03) and 3% less laboratory orders (p = 0.04). The number of "busy days" (bed occupancy > 89%) was associated with longer stays (p < 0.01), and increased mortality (p < 0.01). CONCLUSIONS: Increased bed occupancy and lower staffing were associated with increased mortality, length of stay, imaging orders, and laboratory turn-around time. The data demonstrate performance of the cardiac ICU system is exacerbated during high occupancy and low staffing.

Comparison of Invasive and Oscillometric Blood Pressure Measurement in Obese and Nonobese Children.

BACKGROUND: Obesity and hypertension are public health priorities, with obesity considered to be a potential cause of hypertension. Accurate blood pressure (BP) determination is required and often obtained by automated oscillometric cuff devices. We sought to determine the correlation of oscillometric measurement in children, and if obesity was associated with worse correlation between methods than nonobese children. METHODS: Retrospective matched case-controlled study of 100 obese (97-99th percentile) and 100 nonobese (25-70th percentile) children after cardiac surgery with simultaneous systolic, diastolic, and mean invasive and oscillometric measurements. Matching was 1:1 for age, sex, race, and Risk Adjustment for Congenital Heart Surgery-1 score. Intraclass correlation coefficients and Bland-Altman plots were used to determine agreement with 0.75 as threshold. RESULTS: Median age was 13 years (10-15). Agreement was low for systolic (0.65 and 0.61), diastolic (0.68 and 0.61), and mean measurements (0.73 and 0.69) (obese/nonobese). Bland-Altman plots demonstrated oscillometric BP measurements underestimated systolic hypertension (oscillometric readings lower than intra-arterial). Oscillometric measurements underestimated hypotension (systolic oscillometric measurements were higher than intra-arterial). This occurred in obese and nonobese patients. Correlation of oscillometric measurements was similar for nonobese and obese patients. CONCLUSIONS: In this first ever study of simultaneous BP measurement by oscillometric vs. intra-arterial in obese and nonobese children, correlation is below accepted norms. The correlation of oscillometric cuff measurements is not affected by habitus in children. There is less correlation between oscillometric measurements and intra-arterial measurements during hypertension or hypotension. Healthcare providers should be aware of the limitations of oscillometric measurements.

Futility in the paediatric cardiac ICU.

INTRODUCTION: Studies have suggested 5-20% of paediatric ICU patients may receive care felt to be futile. No data exists on the prevalence and impact of futile care in the Paediatric Cardiac ICU. The aim is to determine the prevalence and economic impact of futile care. MATERIALS AND METHOD: Retrospective cohort of patients with congenital cardiac disease 0-21 years old, with length of stay >30 days and died (2015-2018). Documentation of futility by the medical team was retrospectively and independently reviewed. RESULTS: Of the 127 deaths during the study period, 51 (40%) had hospitalisation >30 days, 13 (25%) had received futile care and 26 (51%) withdrew life-sustaining treatment. Futile care comprised 0.69% of total patient days with no difference in charges from patients not receiving futile care. There was no difference in insurance, single motherhood, education, income, poverty, or unemployment in families continuing futile care or electing withdrawal of life-sustaining treatment. Black families were less likely than White families to elect for withdrawal (p = 0.01), and Hispanic families were more likely to continue futile care than non-Hispanics (p = 0.044). CONCLUSIONS: This is the first study to examine the impact of futile care and characteristics in the paediatric cardiac ICU. Black families were less likely to elect for withdrawal, while Hispanic families more likely to continue futile care. Futile care comprised 0.69% of bed days and little burden on resources. Cultural factors should be investigated to better support families through end-of-life decisions.

Application of the Pediatric Appropriate Use Criteria for Chest Pain.

OBJECTIVES: To characterize the subgroup of outpatient pediatric patients presenting with chest pain and to determine the effectiveness of published pediatric appropriate use criteria (PAUC) to detect pathology. STUDY DESIGN: The Pediatric Appropriate Use of Echocardiography study evaluated the use and yield of transthoracic echocardiography (TTE) before and after PAUC release. Data were reviewed on patients ?18 years of age who underwent TTE for chest pain. Indications were classified as appropriate (A), may be appropriate (M), and rarely appropriate (R) based on PAUC ratings, and findings were normal, incidental, or abnormal. RESULTS: Chest pain was the primary indication in 772 of 4562 outpatient TTE studies (17%) (median age 14 years, IQR 10-16) ordered during the study period: 458 of 772 before (59%) and 314 of 772 after (41 %) the release of PAUC with no change in appropriateness. In A indications (n?=?654), 642 (98%) were normal, 5 (1%) had incidental findings, and 7 (1%) were abnormal. A and M detected 100% of all abnormal findings (A: n?=?7; M: n?=?6; R: n?=?0), with an association between ratings and findings (P?<.001). There was no association between R rating and any pathology. CONCLUSIONS: There was no change in ordering patterns with publication of the PAUC. Despite the high rate of TTEs ordered for indications rated A, most studies were normal. Studies that detected pathology were performed for indications rated A or M, but not R. This study supports PAUC as a useful tool in pediatric chest pain evaluation that may subsequently improve the use of TTE.

Evaluation of Residual Coarctation in Infants with a Single Right Ventricle after Stage I Palliation.

In infants with a single right ventricle (RV), stage I palliation involves aortic reconstruction, systemic-to-pulmonary shunt placement, and atrial septectomy. Many require re-intervention for residual coarctation of the aorta (CoA). Doppler echocardiography can detect residual CoA in most infants, but its ability to predict severity has not been studied. This study compares gradients from Doppler interrogation to those from cardiac catheterization in infants with residual CoA. We performed a retrospective study of infants after stage I palliation from 2000 to 2014. Infants with an echocardiogram and catheterization before the second-stage palliative surgery were included. Infants with an echocardiogram >30 days before catheterization were excluded. Doppler-derived gradients were compared to catheterization-derived gradients. Echocardiographic assessment of tricuspid valve (TV) and RV function were recorded. The cohort included 95 infants, and thirty-three (35%) had CoA. Doppler-derived and catheterization-derived gradients correlated weakly in infants with CoA (r = 0.37, p = 0.036) and without CoA (r = 0.35, p = 0.005). Among infants with CoA, 17/33 had none or trivial tricuspid regurgitation (TR) and normal RV function, and Doppler-derived gradients correlated with catheterization gradients in this group (r = 0.71, p = 0.001). In 16/33 infants with ≥moderate TR or RV dysfunction, gradients did not correlate (r = -0.003, p = 0.992). After a stage I palliation in infants with single RV and CoA, Doppler-derived gradients poorly predicted the severity of CoA. Infants with normal TV or RV function had Doppler-derived gradients more predictive of catheterization-derived gradients. Doppler-derived gradients have limited utility in determining the severity of CoA after a stage I palliation.

Echocardiographic and Surgical Correlation of Coronary Artery Patterns in Transposition of the Great Arteries.

OBJECTIVE: Determine the accuracy of echocardiography to diagnose coronary anatomy in transposition of the great arteries and to evaluate the effect of accuracy on surgical outcomes and changes in accuracy over time. DESIGN: Retrospective chart review of neonates admitted February 1999 to March 2013 with transposition. Coronary pattern from the preoperative echocardiogram and operative reports were collected and compared with determine diagnostic accuracy. Coronary patterns were further confirmed by intraoperative images taken during surgery. SETTING: Tertiary care children's hospital. PATIENTS: Neonates with transposition of the great arteries and planned arterial switch operation with an echo and operative report or image describing the coronaries. INTERVENTIONS: Not applicable. OUTCOME MEASURES: Accuracy of echocardiography to diagnose coronary anatomy in transposition, and to identify factors related to correct diagnosis. RESULTS: One hundred forty-two patients met inclusion criteria with 122 correctly diagnosed, 16 incorrect, and 4 inconclusive. Accuracy was 86%, with 95% accuracy in patients with typical coronary patterns, 85% with the most common variant (left coronary from the leftward sinus and right and circumflex from the rightward sinus), and 61% with less common patterns. Typical and common variants were more likely to be correct than atypical patterns (P < .001). Cases with ventricular septal defect were more likely to have correctly diagnosed coronaries than with an intact ventricular septum (94% vs. 79%, P = .01). There was no change in accuracy over time (P > .05). There was no difference in duration of cardiopulmonary bypass, cross-clamp times, length of stay, or postoperative stay between the correct and incorrectly diagnosed groups (P > .05). CONCLUSIONS: In our center, accuracy of echocardiographic imaging of the coronary arteries in transposition was 86% without improvement over time, and perioperative outcomes were not affected by diagnostic accuracy. Further invasive imaging may not be necessary to determine the coronary pattern in this lesion.

Spontaneous Upper Extremity Venous Thrombosis in a Collegiate Soccer Player: A Case Report.

Spontaneous effort-induced thrombosis is a rare but reported phenomena that was originally described over 100 years ago. The pathogenesis of this thrombosis arises from an abnormality of the thoracic outlet usually combined with a history of physical activity that includes repetitive arm motions, usually of the dominant hand. We present the case of an adolescent patient who presented to a pediatric emergency department with progressive pain, discoloration, and swelling of the shoulder of his nondominant hand. The pain became acutely worse with graying appearance of his arm. The patient was diagnosed with spontaneous thrombosis of the upper extremity extending from the left subclavian vein extending to the axillary vein. Treatment of this patient included aggressive anticoagulation, thrombolysis, and costectomy.