Left Heart Decompression on Veno-Arterial Extracorporeal Membrane Oxygenation in Children With Dilated Cardiomyopathy and Myocarditis: An Extracorporeal Life Support Organization Registry Review.

OBJECTIVES: To describe the association between left heart decompression on veno-arterial extracorporeal membrane oxygenation and survival in patients with myocarditis and dilated cardiomyopathy. The secondary outcome is to study association of left heart decompression with survival in children with myocarditis compared with those with dilated cardiomyopathy. DESIGN: Retrospective study of a multicenter registry database. SETTING: Data reported to Extracorporeal Life Support Organization from international extracorporeal membrane oxygenation centers. PATIENTS: Patients less than or equal to 18 years old with a diagnosis of myocarditis or dilated cardiomyopathy receiving extracorporeal membrane oxygenation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 1,438 pediatric extracorporeal membrane oxygenation runs were identified. Thirty-seven percent of the patients had myocarditis (n = 532), whereas the rest had dilated cardiomyopathy. Survival to hospital discharge was 63%. Median extracorporeal membrane oxygenation duration was 148 hours with interquartile range (84-248 hr). Nineteen percent of patients (n = 274) had left heart decompression. Multivariable analysis revealed using left heart decompression (adjusted odds ratio, 1.42; 95% CI, 1.06-1.89; p = 0.02), e-cardiopulmonary resuscitation (adjusted odds ratio, 0.63; 95% CI, 0.51-0.79; p < 0.001), higher pH (adjusted odds ratio, 3.69; 95% CI, 1.80-7.53; p < 0.001), and diagnosis of myocarditis (adjusted odds ratio, 1.69; 95% CI, 1.35-2.08; p < 0.001) were associated with greater odds of survival. In the multivariable analysis for patients with dilated cardiomyopathy, left heart decompression failed to reveal a significant association with survival (20% among survivors vs 17% among nonsurvivors, 95% CI, -2.2% to 8.0%). Meanwhile in patients with myocarditis, the multivariable analysis failed to exclude the possibility that left heart decompression was associated with up to a three-fold greater odds of survival (adjusted odds ratio, 1.77; 95% CI, 0.99-.15). CONCLUSIONS: Retrospective review of the Extracorporeal Life Support Organization registry revealed an association between left heart decompression and greater odds of survival in children with myocarditis and dilated cardiomyopathy on extracorporeal membrane oxygenation. When comparing patients with dilated cardiomyopathy against those with myocarditis, we could not exclude a three-fold greater odds of survival associated with the use of left heart decompression. This finding warrants further prospective evaluation.

ECMO support in SARS-CoV2 multisystem inflammatory syndrome in children in a child.

In this report, we describe the case of a 5-year-old male with SARS-CoV-2 associated MIS-C with progressive respiratory failure and vasoplegic shock requiring extracorporeal support. At presentation, reverse transcription-polymerase chain reaction (RT-PCR) testing for SARS-CoV-2 was negative, however, SARS-CoV2 antibody testing was positive. Multiple inflammatory markers and cardiac biomarkers were elevated. Echocardiogram demonstrated mildly depressed left ventricular function and no coronary anomalies noted. The patient required mechanical ventilation, vasopressors, and eventually extracorporeal membrane oxygenation (ECMO) for profound circulatory shock and progressive respiratory failure. During his clinical course, recovery of cardiac function was demonstrated however, a middle cerebral artery infarct and left frontal subarachnoid hemorrhage was suffered which ultimately the patient succumbed to. To the best of our knowledge, this is the youngest previously healthy child who had multi-system hyperinflammatory syndrome requiring ECMO support and the first case of SARS-CoV-2 related pediatric stroke.Clinical Trial Registration: None.

Safety of Enalapril in Infants: Data from the Pediatric Heart Network Infant Single Ventricle Trial.

OBJECTIVE: To assess the safety profile of angiotensin-converting enzyme inhibitor therapy in infants with single ventricle. STUDY DESIGN: The Pediatric Heart Network conducted a double-blind trial involving infants with single ventricle physiology randomized to receive enalapril or placebo and followed to 14 months of age. Data including demographics, drug administration, hemodynamic monitoring, laboratory measurements, adverse events, and survival were extracted from the public use data set and compared between the placebo and enalapril-treated groups. RESULTS: The Infant Single Ventricle trial randomized 230 patients, with 115 patients in each group. Initial enalapril dose was 0.10 mg/kg/d and median maximal dose was 0.38 mg/kg/d. There was no significant difference in change in blood pressure at study drug initiation or when resuming study drug after Glenn surgery. The incidence of hyperkalemia and neutropenia did not differ between groups. Renal dysfunction occurred in 3% of the enalapril group and none of the placebo patients, which was not statistically significant. There was a high frequency of serious adverse events in both groups. There was no difference in the frequency of heart transplant or death between groups. CONCLUSIONS: Enalapril did not have sustained hemodynamic effects at initiation or up-titration of drug. Creatinine and potassium were not different between groups, although renal dysfunction occurred more often in the patients on enalapril. Although efficacy of enalapril in neonates with single ventricle has not been demonstrated, the safety profile of angiotensin-converting enzyme inhibitors appears to be low risk in infants and children with significant heart disease.

Multisystem Inflammatory Syndrome in Children Associated with Severe Acute Respiratory Syndrome Coronavirus 2 Infection (MIS-C): A Multi-institutional Study from New York City.

OBJECTIVE: To assess clinical characteristics and outcomes of severe acute respiratory syndrome coronavirus 2-associated multisystem inflammatory syndrome in children (MIS-C). STUDY DESIGN: Children with MIS-C admitted to pediatric intensive care units in New York City between April 23 and May 23, 2020, were included. Demographic and clinical data were collected. RESULTS: Of 33 children with MIS-C, the median age was 10 years; 61% were male; 45% were Hispanic/Latino; and 39% were black. Comorbidities were present in 45%. Fever (93%) and vomiting (69%) were the most common presenting symptoms. Depressed left ventricular ejection fraction was found in 63% of patients with median ejection fraction of 46.6% (IQR, 39.5-52.8). C-reactive protein, procalcitonin, d-dimer, and pro-B-type natriuretic peptide levels were elevated in all patients. For treatment, intravenous immunoglobulin was used in 18 (54%), corticosteroids in 17 (51%), tocilizumab in 12 (36%), remdesivir in 7 (21%), vasopressors in 17 (51%), mechanical ventilation in 5 (15%), extracorporeal membrane oxygenation in 1 (3%), and intra-aortic balloon pump in 1 (3%). The left ventricular ejection fraction normalized in 95% of those with a depressed ejection fraction. All patients were discharged home with median duration of pediatric intensive care unit stay of 4.7 days (IQR, 4-8 days) and a hospital stay of 7.8 days (IQR, 6.0-10.1 days). One patient (3%) died after withdrawal of care secondary to stroke while on extracorporeal membrane oxygenation. CONCLUSIONS: Critically ill children with coronavirus disease-2019-associated MIS-C have a spectrum of severity broader than described previously but still require careful supportive intensive care. Rapid, complete clinical and myocardial recovery was almost universal.

Prevalence, predictors, and outcomes of cardiorenal syndrome in children with dilated cardiomyopathy: a report from the Pediatric Cardiomyopathy Registry.

BACKGROUND: The association of cardiorenal syndrome (CRS) with mortality in children with dilated cardiomyopathy (DCM) is unknown. METHODS: With a modified Schwartz formula, we estimated glomerular filtration rates (eGFR) for children ≥1 year of age with DCM enrolled in the Pediatric Cardiomyopathy Registry at the time of DCM diagnosis and annually thereafter. CRS was defined as an eGFR of <90 mL/min/1.73 m(2). Children with and without CRS were compared on survival and serum creatinine concentrations (SCr). The association between eGFR and echocardiographic measures was assessed with linear mixed-effects regression models. RESULTS: Of 285 eligible children with DCM diagnosed at ≥1 year of age, 93 were evaluable. CRS was identified in 57 of these 93 children (61.3%). Mean (standard deviation) eGFR was 62.0 (22.6) mL/min/1.73 m(2) for children with CRS and 108.0 (14.0) for those without (P < 0.001); median SCr concentrations were 0.9 and 0.5 mg/dL, respectively (P < 0.001). The mortality hazard ratio of children with CRS versus those with no CRS was 2.4 (95% confidence interval 0.8-7.4). eGFR was positively correlated with measures of left ventricular function and negatively correlated with age. CONCLUSIONS: CRS in children newly diagnosed with DCM may be associated with higher 5-year mortality. Children with DCM, especially those with impaired left ventricular function, should be monitored for renal disease.

Acute Kidney Injury in Pediatric Acute Decompensated Heart Failure.

OBJECTIVE: Acute kidney injury in adult patients with acute decompensated heart failure is associated with increased mortality. There is limited literature in pediatric patients with acute decompensated heart failure and acute kidney injury. We aim to study acute kidney injury in the pediatric acute decompensated heart failure population and its association with specific outcomes. DESIGN: Retrospective, case-control study. SETTING: Cardiac ICU in a children's tertiary care hospital. PATIENTS: Index admissions of patients younger than 21 years with acute decompensated heart failure between January 2008 and December 2012. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Index admissions of patients younger than 21 years with acute decompensated heart failure between January 2008 and December 2012 were reviewed, and the presence or absence of acute kidney injury at admission was determined based on the Pediatric Risk, Injury, Failure, Loss, End-Stage criteria. Descriptive statistics and multivariate analyses were performed to determine the association between acute kidney injury and a composite outcome of cardiac transplantation and/or mortality. Fifty-seven patients, with median age 12 years (interquartile range, 1.1, 16), were included for study. The median left ventricular ejection fraction was 27% (interquartile range, 18, 48). Twenty-one patients (36%) underwent cardiac transplantation and five patients (8.7%) died. Of the 57 patients, 44 (77%) had evidence of acute kidney injury (41% Risk; 39% Injury; 20% Failure). Of the 44 patients with acute kidney injury, 25 (57%) met the composite outcome, compared with 1 (7%) without acute kidney injury. Multivariate analyses demonstrated that a left ventricular ejection fraction up to 25% was significantly associated with the presence of acute kidney injury (adjusted odds ratio, 12.3; 95% CI, 1.4-109; p = 0.03), and acute kidney injury was significantly associated with the composite outcome (adjusted odds ratio, 19.1; 95% CI, 2.3-160; p < 0.001). CONCLUSIONS: Acute kidney injury is common during the initial presentation of pediatric patients with acute decompensated heart failure. A left ventricular ejection fraction up to 25% is associated with acute kidney injury. The presence of acute kidney injury in this population is significantly associated with cardiac transplantation and/or death.

Arrhythmias in the paediatric intensive care unit: a prospective study of the rates and predictors of arrhythmias in children without underlying cardiac disease.

OBJECTIVE: Arrhythmias are common in patients admitted to the paediatric intensive care unit. We sought to identify the rates of occurrence and types of arrhythmias, and determine whether an arrhythmia was associated with illness severity and paediatric intensive care unit length of stay. DESIGN: This is a prospective, observational study of all patients admitted to the paediatric intensive care unit at the Children's Hospital at Montefiore from March to June 2012. Patients with cardiac disease or admitted for the treatment of primary arrhythmias were excluded. Clinical and laboratory data were collected and telemetry was reviewed daily. Tachyarrhythmias were identified as supraventricular tachycardia, ventricular tachycardia, and arrhythmias causing haemodynamic compromise or for which an intervention was performed. RESULTS: A total of 278 patients met the inclusion criteria and were analysed. There were 97 incidences of arrhythmia in 53 patients (19%) and six tachyarrhythmias (2%). The most common types of arrhythmias were junctional rhythm (38%), premature atrial contractions (24%), and premature ventricular contractions (22%). Tachyarrhythmias included three supraventricular tachycardia (50%) and three ventricular tachycardia (50%). Of the six tachyarrhythmias, four were related to placement or migration of central venous lines and two occurred during aminophylline infusion. Patients with an arrhythmia had longer duration of mechanical ventilation and paediatric intensive care unit stay (p<0.001). In multivariate analysis, central venous lines (odds ratio 3.1; 95% confidence interval 1.3-7.2, p=0.009) and aminophylline use (odds ratio 5.1; 95% confidence interval 1.7-14.9, p=0.003) were independent predictors for arrhythmias. CONCLUSIONS: Arrhythmias were common in paediatric intensive care unit patients (19%), although tachyarrhythmias occurred rarely (2%). Central venous lines and use of aminophylline were identified as two clinical factors that may be associated with development of an arrhythmia.

Volumetric Capnography in Pediatric Extracorporeal Membrane Oxygenation: A Case Series.

The kinetics of carbon dioxide elimination (VCO 2 ) may be used as a surrogate for pulmonary blood flow. As such, we can apply a novel use of volumetric capnography to assess hemodynamic stability in patients requiring extracorporeal membrane oxygenation (ECMO). We report our experience of pediatric patients requiring ECMO support who were monitored using volumetric capnography. We describe the use of VCO 2 and its association with successful decannulation. This is a prospective observational study of pediatric patients requiring ECMO support at The Children's Hospital at Montefiore from 2017 to 2019. A Respironics NM3 monitor was applied to each patient. Demographics, hemodynamic data, blood gases, and VCO 2 (mL/min) data were collected. Data were collected immediately prior to and after decannulation. Over the course of the study period, seven patients were included. Predecannulation VCO 2 was higher among patients who were successfully decannulated than nonsurvivors (109 [35, 230] vs. 12.4 [7.6, 17.2] mL/min), though not statistically significant. Four patients (57%) survived without further mechanical support; two (29%) died, and one (14%) was decannulated to Berlin. Predecannulation VCO 2 appears to correlate with hemodynamic stability following decannulation. This case series adds to the growing literature describing the use of volumetric capnography in critical care medicine, particularly pediatric patients requiring ECMO. Prospective studies are needed to further elucidate the use of volumetric capnography and optimal timing for ECMO decannulation.

Newborn With Severely Depressed Left Ventricular Function: Acute Myocardial Infarction in a Newborn.

We describe a rare case of spontaneous coronary artery thrombosis in a newborn leading to rapid severe ventricular dysfunction. Early diagnosis is critical and management strategies are varied including hemodynamic support with extracorporeal membrane oxygenation, systemic/local thrombolytic therapy with tissue plasminogen activator, or surgical thrombectomy. (Level of Difficulty: Advanced.).

Acute Kidney Injury in Children With Acute Respiratory Failure.

BACKGROUND: Acute kidney injury (AKI) is common in critically ill children and develops in association with organ system dysfunction, with acute respiratory failure (ARF) one of the most common. We aim to study AKI in the pediatric ARF population. METHODS: Data were retrospectively collected on children aged 1 day to 18 years admitted to the pediatric intensive care unit (PICU) with ARF between 2010 and 2013. Descriptive statistics and multivariate analyses utilizing Mann-Whitney U, Wilcoxon signed rank, χ2, or Fisher's exact tests were performed to identify risk factors associated with AKI. RESULTS: A total of 186 patients, with median age of 36 months (interquartile range 4-120 months) met the inclusion criteria. ARF was related to pulmonary disease in 49%. AKI was noted in 53% of patients. Patients with AKI had significantly higher serum creatinine ( P < .001) and lower estimated creatinine clearance ( P < .001) compared with those without AKI. Among patients with moderate and severe acute respiratory distress syndrome (ARDS), 64% had AKI versus 46% with mild or no ARDS ( P = .02). Patients with AKI had significantly lower PaO2/FiO2 ratio ( P = .03), longer PICU ( P = .03), and longer hospital length of stay ( P = .01). ARDS patients were less likely to be AKI free on day 7 of hospitalization, as compared with those without ARDS. Multivariate analysis revealed positive end expiratory pressure (odds ratio [OR] = 1.2, confidence interval [CI] = 1.0-1.4; P = .03) and admission serum creatinine (OR = 27.9, CI = 5.2-148.5; P < .001) to be independently associated with AKI. CONCLUSIONS: AKI is common in children with ARF. In patients with ARF and AKI, AKI is associated with ARDS and longer PICU and hospital length of stay. Positive end expiratory pressure and serum creatinine are independently associated with AKI.

Venovenous extracorporeal membrane oxygenation for patients with single-ventricle anatomy: A registry report.

OBJECTIVE: Support with extracorporeal membrane oxygenation for cardiopulmonary failure is done so with venoarterial cannulation in the majority of children with single-ventricle anatomy. However, there is a growing experience for patients with pure oxygenation/ventilation impairment supported with venovenous extracorporeal membrane oxygenation. We describe that experience. METHODS: Data were collected from the Extracorporeal Life Support Organization registry for patients with single-ventricle anatomy supported with venovenous extracorporeal membrane oxygenation from 1990 to 2012. Descriptive statistics and multivariate analyses for associations with mortality were conducted. RESULTS: A total of 89 patients with single-ventricle anatomy had venovenous extracorporeal membrane oxygenation performed at a median age of 66 days (8-221). Survival to discharge was 48%. Fifty-four patients (61%) had shunt physiology, 22 patients (25%) had cavopulmonary connections, and 13 patients (14%) had single-ventricle anatomy but with no previous cardiac surgery. Indication for extracorporeal membrane oxygenation was respiratory failure in 59 patients (63%) and cardiac failure in 30 patients (32%). Double-lumen cannulas were used in 62 patients (70%). Bivariate analysis demonstrated that the duration of intubation before extracorporeal membrane oxygenation, mean airway pressure before cannulation, partial pressure carbon dioxide before cannulation, peak inspiratory pressure before cannulation, pump flow at 24 hours, extracorporeal membrane oxygenation run duration, and presence of renal injury were associated with mortality. Multivariate logistic analysis demonstrated that the duration of intubation (adjusted odds ratio, 1.01; 95% confidence interval, 1.003-1.016; P = .003), partial pressure carbon dioxide (adjusted odds ratio, 1.04; 95% confidence interval, 1.01-1.068; P = .007), mean airway pressure (adjusted odds ratio, 1.16; 95% confidence interval, 1.0-1.342; P = .05), and renal injury (adjusted odds ratio, 6.6; 95% confidence interval, 1.879-23.2; P = .003) were associated with mortality. CONCLUSIONS: Patients with single-ventricle anatomy in respiratory failure may be treated successfully with venovenous extracorporeal membrane oxygenation, with survival comparable to those treated with venoarterial extracorporeal membrane oxygenation for cardiac failure. Future research on indications for venovenous extracorporeal membrane oxygenation may aid clinicians in deciding the optimal approach for this challenging cohort.

Acute kidney injury after surgery for congenital heart disease.

BACKGROUND: The RIFLE criteria (risk, injury, failure, loss, and end-stage kidney disease) have been used to assess acute kidney injury (AKI) in various populations of critically ill children. There are limited reports of AKI using RIFLE criteria in large pediatric populations undergoing congenital heart disease surgery. METHODS: Records of patients 18 years and younger who underwent surgery for congenital heart disease between January 2006 and November 2009 were reviewed. The RIFLE score was determined for each patient postoperatively. Multivariate logistic regression analyses were performed to determine risk factors for AKI and the association with clinical outcomes, with subanalyses of patients 1 month of age or younger. RESULTS: Data for 458 patients (median age, 7.6 months) were collected and analyzed. Evidence of AKI was demonstrated in 234 patients (51%), the vast majority of whom recovered within 48 hours. Younger age, higher RACHS-1 (risk-adjusted classification for congenital heart surgery) category, and longer cardiopulmonary bypass time were associated with development of AKI. Acute kidney injury was associated with longer duration of ventilation and lengths of intensive care unit and hospital stay. Incidence of AKI in patients 1 month of age or younger was 60.9%, of which more than half required greater than 72 hours to recover. In patients 1 month of age or younger, use of cardiopulmonary bypass, lower preoperative serum creatinine, and higher preoperative blood urea nitrogen were associated with AKI, and AKI was the only factor associated with longer intensive care unit and hospital lengths of stay. CONCLUSIONS: Incidence of AKI based on RIFLE criteria in patients undergoing congenital heart disease surgery is higher than previously reported. Risk factors include age 1 month or younger and use of cardiopulmonary bypass. Acute kidney injury is associated with longer lengths of stay.