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Objective We sought to evaluate the fidelity with which the patient’s clinical state is represented by the electronic health record (EHR) flow sheet vital signs data compared to a commercially available automated data aggregation platform in a pediatric cardiac intensive care unit (CICU) Methods This is a retrospective observational study of heart rate (HR), systolic blood pressure (SBP), respiratory rate (RR), and pulse oximetry (SpO2) data archived in a conventional EHR and an automated data platform for 857 pediatric patients admitted postoperatively to a tertiary pediatric CICU. Automated data captured for 72 h after admission were analyzed for significant HR, SBP, RR, and SpO2 deviations from baseline (events). Missed events were identified when the EHR failed to reflect the events reflected in the automated platform Results Analysis of 132 054 622 data entries, including 264 966 (0.2%) EHR entries and 131 789 656 (99.8%) automated entries, identified 15 839 HR events, 5851 SBP events, 9648 RR events, and 2768 SpO2 events lasting 3–60 min; these events were missing in the EHR 48%, 58%, 50%, and 54% of the time, respectively. Subanalysis identified 329 physiologically implausible events (eg, likely operator or device error), of which 104 (32%) were nonetheless documented in the EHR Conclusion In this single-center retrospective study of CICU patients, EHR vital sign documentation was incomplete compared to an automated data aggregation platform. Significant events were underrepresented by the conventional EHR, regardless of event duration. Enrichment of the EHR with automated data aggregation capabilities may improve representation of patient condition

AIM: To evaluate the Inadequate oxygen delivery (IDO(2)) index dose as a predictor of cardiac arrest (CA) in neonates following congenital heart surgery. METHODS: Retrospective cohort study in 3 US pediatric cardiac intensive units (1/2011-8/2016). Calculated IDO(2) index values were blinded to bedside clinicians and generated from data collected up to 30 days postoperatively, or until death or ECMO initiation. Control event data was collected from patients who did not experience CA or require ECMO. IDO(2) dose was computed over a 120-minute window up to 30 minutes prior to the CA and control events. A multivariate logistic regression prediction model including the IDO(2) dose and presence or absence of a single ventricle (SV) was used. Model performance metrics were the odds ratio for each regression coefficient and receiver operating characteristic area under the curve (ROC AUC). RESULTS: Of 897 patients monitored during the study period, 601 met inclusion criteria: 29 patients had CA (33 events) and 572 patients were used for control events. Seventeen (59%) CA and 125 (26%) control events occurred in SV patients. Median age/weight at surgery and level of monitoring were similar in both groups. Median postoperative event time was 0.73 days [0.05-22.39] in CA patients and 0.82 days [0.08 25.11] in control patients. Odds ratio of the IDO(2) dose coefficient was 1.008 (95% CI: 1.006 - 1.012, p=0.0445), and 2.952 (95% CI: 2.952 - 3.258, p=0.0079) in SV. The ROC AUC using both coefficients was 0.74 (95% CI: 0.73 - 0.75). These associations of IDO(2) dose with CA risk remained robust, even when censored periods prior to arrest were 10 and 20 minutes. CONCLUSION: In neonates post-CPB surgery, higher IDO(2) index dose over a 120-minute monitoring period is associated with increased risk of cardiac arrest, even when censoring data 10, 20 or 30 minutes prior to the CA event.

Introduction Oxygenators are well known for their application in extracorporeal circulation. Berlin Heart silicone cannulae were specifically designed for use with ventricular assist devices. We describe a novel combination of these two devices for creation of a pumpless, paracorporeal system for bridging an infant to lung transplantation. Case Description A four-month-old baby, born at 36 weeks of gestation with refractory respiratory failure with components of both parenchymal lung disease and pulmonary hypertension, was referred from an outside institution. He had previously undergone ductal stenting at …

Since 2012, we have supported 18 children with single ventricle (SV) physiology on ventricular assist devices (VADs) as a bridge to decision, transplantation, or recovery. We provide a detailed report of our cumulative surgical experience and lessons learned from these patients.We reviewed all SV-VADs between March 2012 and April 2020. Implanted SV-VADs intended for short-term support were excluded. Demographic and clinical data included palliation stage at the time of VAD implantation, cannulation configuration, device type, duration of support, circuit and device interventions, postoperative support, anticoagulation strategy, complications, mortality, and 1-year survival postdischarge.Five SV newborns without prior surgical palliation, 8 infants post-Norwood/hybrid procedure, 4 infants post-Glenn, and 1 infant post-Fontan were initially supported with either continuous-flow (n = 13 of 18, 72%) or pulsatile-flow (n = 5 of 18, 28%) devices. Three (17%) of 18 transitioned to another device during support. Before VAD conversion, 9 (50%) of 18 were supported by extracorporeal membrane oxygenation. Outcomes included 7 (39%) of 18 who transplanted, 2 (11%) of 18 who recovered, and 9 (50%) of 18 who died before discharge. Of these deaths, 2 occurred after transplantation and 2 after explantation, and 5 had redirection of care while on support secondary to previously undiagnosed pulmonary venoocclusive disease (n = 2) or severe neurologic events (n = 3). Overall, 6 (33%) of 18 experienced neurologic injury. At last follow-up, 9 (50%) of 18 children were alive (median 1.2 [interquartile range, 0.8-4.3] years postexplantation/transplantation).Our experience shows that SV children, including newborns, can be successfully bridged to desired endpoints with proper patient selection and using specific cannulation strategies. Continuing utilization of this strategy is warranted for future children requiring VAD support.

Purpose: We sought to determine the impact of a comprehensive, context-responsive anticoagulation and transfusion guideline on bleeding and thrombotic complication rates and blood product utilization during extracorporeal membrane oxygenation (ECMO).Design: Single-center, observational pre- and post-implementation cohort study.Setting: Academic pediatric hospital.Patients: Patients in the PICU, CICU, and NICU receiving ECMO support.Interventions: Program-wide implementation of a context-responsive anticoagulation and transfusion guideline.Measurements: Pre-implementation subjects consisted of all patients receiving ECMO between January 1 and December 31, 2012, and underwent retrospective chart review. Post-implementation subjects consisted of all ECMO patients between September 1, 2013, and December 31, 2014, and underwent prospective data collection. Data collection included standard demographic and admission data, ECMO technical specifications, non-ECMO therapies, coagulation parameters, and blood product administration. A novel grading scale was used to define hemorrhagic complications (major, intermediate, and minor) and major thromboembolic complications.Main Results: Seventy-six ECMO patients were identified: 31 during the pre-implementation period and 45 in the post-implementation period. The overall observed mortality was 33% with no difference between groups. Compared to pre-implementation, the post-implementation group experienced fewer major hemorrhagic and major thrombotic complications and less severe hemorrhagic complications and received less RBC transfusion volume per kg.Conclusions: Use of a context-responsive anticoagulation and transfusion guideline was associated with a reduction in hemorrhagic and thrombotic complications and reduced RBC transfusion requirements. Further evaluation of guideline content, compliance, performance, and sustainability is needed.

Background: Cardiopulmonary bypass-related platelet dysfunction can increase the risk of intra- and post-operative bleeding in children undergoing cardiac surgery. More accurate laboratory tests that identify acquired platelet abnormalities could allow for rapid identification of patients at risk of bleeding and provide therapies that could reduce bleeding and platelet transfusions. We hypothesized that thromboelastography with platelet mapping (TEG-PM) and multiple electrode impedance aggregometry (MEIA) as functional measures of platelet function would predict who will require platelet transfusion. Our secondary hypothesis was that platelet aggregation at both arachidonic acid (AA) and adenosine diphosphate (ADP) receptors would correlate between TEG-PM and MEIA results.Methods: In this prospective study from August 2013 to December 2015, children from newborn to 5 years of age with congenital heart disease undergoing cardiopulmonary bypass had blood samples collected and analyzed at four time points: pre-bypass, post-bypass, post-operatively on arrival to the Cardiac Intensive Care Unit, and 24 h after arrival.Results: Of the 44 patients analyzed, the 10 patients who received peri-operative platelet transfusion were significantly younger (p = 0.05), had higher STAT (Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery) Mortality Categories (p < 0.002) and longer cardiopulmonary bypass times (p = 0.02). In univariate analysis, four variables were associated with peri-operative platelet transfusion: pre-operative age [OR 0.95 (0.93, 0.98), p = 0.03], cardiopulmonary bypass time [1.5 (1.31, 1.68), p = 0.008], STAT Mortality Category [3.64 (3.40, 3.87), p < 0.001], and TEG-PM ADP [0.79 (0.65, 0.93), p = 0.04]. ROC analysis demonstrated moderate predictive value of TEG-PM ADP with AUC of 0.745 (0.59, 0.91). A TEG-PM ADP value of less than or equal to 21 had 85% sensitivity and 70% specificity for platelet transfusion. In the multivariate analysis, only STAT Mortality Category predicted platelet transfusion. TEG-PM and MEIA results correlated for the AA receptor at all 4 time points, but the same tests at the ADP receptors did not correlate.Conclusions: TEG-PM ADP may provide more clinically relevant information regarding platelet function compared to the MEIA at the ADP receptor in children requiring cardiopulmonary bypass. There was limited correlation between TEG-PM and MEIA results which raises a concern about the accuracy of these tests at the ADP receptor. Lower pre-operative TEG-PM ADP MA may predict intra-operative platelet transfusions; however, larger studies are needed to determine the utility of TEG-PM and MEIA in guiding platelet transfusions in this population.

Supplemental Digital Content is available in the text., OBJECTIVES: Advanced clinical decision support tools, such as real-time risk analytic algorithms, show promise in assisting clinicians in making more efficient and precise decisions. These algorithms, which calculate the likelihood of a given underlying physiology or future event, have predominantly been used to identify the risk of impending clinical decompensation. There may be broader clinical applications of these models. Using the inadequate delivery of oxygen index, a U.S. Food and Drug Administration-approved risk analytic algorithm predicting the likelihood of low cardiac output state, the primary objective was to evaluate the association of inadequate delivery of oxygen index with success or failure of weaning vasoactive support in postoperative cardiac surgery patients. DESIGN: Multicenter retrospective cohort study. SETTING: Three pediatric cardiac ICUs at tertiary academic children’s hospitals. PATIENTS: Infants and children greater than 2 kg and less than 12 years following cardiac surgery, who required vasoactive infusions for greater than 6 hours in the postoperative period. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Postoperative patients were identified who successfully weaned off initial vasoactive infusions (n = 2,645) versus those who failed vasoactive wean (required reinitiation of vasoactive, required mechanical circulatory support, renal replacement therapy, suffered cardiac arrest, or died) (n = 516). Inadequate delivery of oxygen index for final 6 hours of vasoactive wean was captured. Inadequate delivery of oxygen index was significantly elevated in patients with failed versus successful weans (inadequate delivery of oxygen index 11.6 [sd 19.0] vs 6.4 [sd 12.6]; p < 0.001). Mean 6-hour inadequate delivery of oxygen index greater than 50 had strongest association with failed vasoactive wean (adjusted odds ratio, 4.0; 95% CI, 2.5–6.6). In patients who failed wean, reinitiation of vasoactive support was associated with concomitant fall in inadequate delivery of oxygen index (11.1 [sd 18] vs 8.9 [sd 16]; p = 0.007). CONCLUSIONS: During the de-escalation phase of postoperative cardiac ICU management, elevation of the real-time risk analytic model, inadequate delivery of oxygen index, was associated with failure to wean off vasoactive infusions. Future studies should prospectively evaluate utility of risk analytic models as clinical decision support tools in de-escalation practices in critically ill patients.

Background Historically, the options for mechanical circulatory support in infants, particularly those with single-ventricle physiology, have been limited and outcomes have generally been poor. We report a new approach implemented for long-term support in a series of such patients. Methods This study is a single-center case series of 7 patients with single-ventricle physiology after stage 1 palliation supported with mechanical circulatory support using a novel technique, between May 2014 and September 2015. Our technique included modification and implantation of commercially available pediatric cannulae into the common atrium and the ascending aorta or reconstructed neoaorta and utilization of a centrifugal extracorporeal pump. Results Median circulatory support duration was 64 days (range, 35 to 99). One adverse neurologic event was observed in 1 patient, and bleeding requiring reoperation in 2 patients. Support to recovery, decision, or heart transplantation was accomplished in all cases. Of all patients, 43% were successfully discharged home. Conclusions Our experience shows that long-term extracorporeal mechanical circulatory support of patients with underlying single-ventricle physiology after stage 1 palliation is feasible utilizing our technique. This approach overcomes several major challenges encountered in these patients, such as high flow requirement and stability of the cannulae, and allows extubation, rehabilitation, and at times, myocardial recovery.

BACKGROUND PGD is a complication after heart transplantation (OHT) and a significant cause of mortality, particularly in infant recipients. Lack of standardized definition of PGD in the pediatric population makes the prevalence and magnitude of impact unclear. METHODS ISHLT PGD consensus guidelines, which include inotrope scores and need for MCS, were applied retrospectively to 208 pediatric OHT recipients from a single institution from 1/2005-5/2016. PGD was defined as: moderate PGD-inotrope score >10 on postoperative day 1 (24-48 hours), and severe PGD-MCS within 24 hours (in the absence of detectable rejection). RESULTS PGD occurred in 34 patients (16.3%); 14 of which had severe PGD (6.7%). Multivariate risk factors for PGD included CPB time (OR 10.3/10 min, 95% 10.05, 10.2, P = 0.03), Fontan palliation (OR 1.9, 95% 1.2, 3.97), and PCM (OR 5.65, 95% 1.52, 22.4); but not age, weight, ischemic time, or donor characteristics. Upon sub-analysis excluding patients with PCM, increased CPB was a significant multivariate risk factor (OR 10.09, 95% 9.89, 10.12, P = 0.003). Patients with PGD had decreased discharge survival compared to those without PGD (85% vs 96%, P

Pulmonary hypertension with transposition of the great arteries is associated with significant morbidity and mortality. At the worst end of the spectrum are patients who undergo extracorporeal support perioperatively. We describe our experience with three patients who received preoperative extracorporeal support and separated from cardiopulmonary bypass successfully on conventional postoperative care, with no significant deficits on follow-up.

OBJECTIVE To describe the epidemiology of noninvasive ventilation therapy for patients admitted to pediatric cardiac ICUs and to assess practice variation across hospitals. DESIGN Retrospective cohort study using prospectively collected clinical registry data. SETTING Pediatric Cardiac Critical Care Consortium clinical registry. PATIENTS Patients admitted to cardiac ICUs at PC4 hospitals. INTERVENTIONS None. MEASUREMENTS AND MAIN RESULTS We analyzed all cardiac ICU encounters that included any respiratory support from October 2013 to December 2015. Noninvasive ventilation therapy included high flow nasal cannula and positive airway pressure support. We compared patient and, when relevant, perioperative characteristics of those receiving noninvasive ventilation to all others. Subgroup analysis was performed on neonates and infants undergoing major cardiovascular surgery. To examine duration of respiratory support, we created a casemix-adjustment model and calculated adjusted mean durations of total respiratory support (mechanical ventilation + noninvasive ventilation), mechanical ventilation, and noninvasive ventilation. We compared adjusted duration of support across hospitals. The cohort included 8,940 encounters from 15 hospitals: 3,950 (44%) received noninvasive ventilation and 72% were neonates and infants. Medical encounters were more likely to include noninvasive ventilation than surgical. In surgical neonates and infants, 2,032 (55%) received postoperative noninvasive ventilation. Neonates, extracardiac anomalies, single ventricle, procedure complexity, preoperative respiratory support, mechanical ventilation duration, and postoperative disease severity were associated with noninvasive ventilation therapy (p < 0.001 for all). Across hospitals, noninvasive ventilation use ranged from 32% to 65%, and adjusted mean noninvasive ventilation duration ranged from 1 to 4 days (3-d observed mean). Duration of total adjusted respiratory support was more strongly correlated with duration of mechanical ventilation compared with noninvasive ventilation (Pearson r = 0.93 vs 0.71, respectively). CONCLUSIONS Noninvasive ventilation use is common in cardiac ICUs, especially in patients admitted for medical conditions, infants, and those undergoing high complexity surgery. We observed wide variation in noninvasive ventilation use across hospitals, though the primary driver of total respiratory support time seems to be duration of mechanical ventilation.

Our goal was to identify risk factors for acute kidney injury in children surviving cardiac arrest.Retrospective analysis of a public access dataset.Fifteen children's hospitals associated with the Pediatric Emergency Care Applied Research Network.Two hundred ninety-six subjects between 1 day and 18 years old who experienced in-hospital or out-of-hospital cardiac arrest between July 1, 2003, and December 31, 2004.None.Our primary outcome was development of acute kidney injury as defined by the Acute Kidney Injury Network criteria. An ordinal probit model was developed. We found six critical explanatory variables, including total number of epinephrine doses, postcardiac arrest blood pressure, arrest location, presence of a chronic lung condition, pH, and presence of an abnormal baseline creatinine. Total number of epinephrine doses received as well as rate of epinephrine dosing impacted acute kidney injury risk and severity of acute kidney injury.This study is the first to identify risk factors for acute kidney injury in children after cardiac arrest. Our findings regarding the impact of epinephrine dosing are of particular interest and suggest potential for epinephrine toxicity with regard to acute kidney injury. The ability to identify and potentially modify risk factors for acute kidney injury after cardiac arrest may lead to improved morbidity and mortality in this population.

In patients with failed Fontan circulation, end-stage heart failure can develop or Fontan physiology failure requiring transplantation. Experience with ventricular assist device support for these patients as a bridge to heart transplantation has been limited and often not resulted in successful hospital discharge. We report the successful use of the Berlin Heart EXCOR (Berlin Heart, The Woodlands, TX) ventricular assist device in bridging a child with Fontan circulation and systolic dysfunction to heart transplantation and discharge home.

Background Bleeding complications are common and decrease the odds of survival in children supported with extracorporeal membrane oxygenation (ECMO). The role of platelet dysfunction on ECMO-induced coagulopathy and resultant bleeding complications is not well understood. The primary objective of this pilot study was to determine the incidence and magnitude of platelet dysfunction according to thromboelastography (TEG®)–platelet mapping (PM) testing. Methods Retrospective chart review of children

The management of decompensating critically ill children with severe PH is extremely challenging and requires a multidisciplinary approach. Unfortunately, even with optimal care, these children might continue to deteriorate and develop inadequate systemic perfusion and at times cardiac arrest secondary to a pulmonary hypertensive crisis. Tools to support these children are limited, and at times, the team should proceed with offering extracorporeal support, especially in newly diagnosed patients who have not benefitted from medical therapy prior to their acute deterioration, in patients with severe pulmonary venous disease and in patients with alveolar capillary dysplasia. Currently, the only approved mode for extracorporeal support in pediatric patients with PH eligible for lung transplantation is ECMO. To decrease the risks associated with ECMO, and offer potential for increased duration of support, extubation, and rehabilitation, we transitioned four small children with refractory PH from ECMO to a device comprising an oxygenator interposed between the PA and LA. This work describes in great detail our experience with this mode of support with emphasis on exclusion criteria, the implantation procedure, and the post-implantation management.

The majority of children awaiting heart transplantation require inotropic support, mechanical ventilation, and/or extracorporeal membrane oxygenation (ECMO) support. Unfortunately, due to the limited pool of organs, many of these children do not survive to transplant. Mechanical circulatory support of the failing heart in pediatrics is a new and rapidly developing field world-wide. It is utilized in children with acute congestive heart failure associated with congenital heart disease, cardiomyopathy, and myocarditis, both as a bridge to transplantation and as a bridge to myocardial recovery. The current arsenal of mechanical assist devices available for children is limited to ECMO, intra-aortic balloon counterpulsation, centrifugal pump ventricular assist devices, the DeBakey ventricular assist device Child; the Thoratec ventricular assist device; and the Berlin Heart. In the spring of 2004, five contracts were awarded by the National Heart, Lung and Blood Institute to support preclinical development for a range of pediatric ventricular assist devices and similar circulatory support systems. The support of early development efforts provided by this program is expected to yield several devices that will be ready for clinical trials within the next few years. Our work reviews the current international experience with mechanical circulatory support in children and summarizes our own experience since 2005 with the Berlin Heart, comparing the indications for use, length of support, and outcome between these modalities.

Primary cardiac tumors are rare in pediatrics. Their clinical presentation differs among the various age groups (fetus to the young adult). Our discussion focuses on the diagnosis and treatment of rhabdomyomas, fibromas, myxomas, and pericardial teratomas in the fetus and neonatal stages. Fetal diagnosis has been described in association with rhabdomyomas, fibromas, and pericardial teratomas. It is made by echocardiography prompted by fetal dysrhythmias, nonimmune hydrops, intrauterine growth restriction, and familial tuberous sclerosis. Based on our experience, it is of the utmost importance to refer these patients to a tertiary center for detailed evaluation, follow-up, and delivery. Tumor diagnosis in the neonatal age group may be prompted by a murmur, dysrhythmia, conduction block, and hemodynamic compromise or hypoxemia due to right or left inflow or outflow tract obstruction. Prenatal diagnosis of a hemodynamically significant mass mandates the presence of a neonatal intensivist in the delivery room for prompt initiation of advanced life support (intubation, mechanical ventilation, and central venous access), and early initiation of prostaglandin E(1) to establish ductus arteriosus patency. Severe inflow obstruction associated with a restrictive atrial septum may require balloon atrial septostomy. Emergent transvenous pacing is indicated in the face of high-degree atrioventricular block. Early surgical removal of the mass after initial stabilization in the neonatal or cardiac intensive care unit may be indicated.

Neonates who experience respiratory failure despite maximal ventilatory support have only extracorporeal membrane oxygenation as a rescue therapy, but it has very poor outcomes as a bridge to transplantation. A pumpless lung-assist device has been used in adults as a bridge to lung transplantation. An alternative membrane oxygenator, the Quadrox iD, is a suitable size for neonatal blood flow. Here we report the use of the Quadrox iD membrane oxygenator with central cannulation as a paracorporeal respiratory support therapy for a neonate with alveolar capillary dysplasia awaiting lung transplantation.

Stabilizing children with decompensating refractory pulmonary hypertension may require extracorporeal circulatory support. Currently, options in this regard are limited to use of venoarterial extracorporeal membrane oxygenation (ECMO), but this approach is frequently fraught with complications. Alternatively, the interventional pumpless lung-assist membrane ventilator (Novalung GmbH, Heilbronn, Germany) can be used through a femoral artery to femoral vein shunt in patients with end-stage lung disease and hypercapnia or hypoxemia refractory to mechanical ventilation and through a pulmonary artery to left atrium shunt in patients with refractory pulmonary hypertension. The latter configuration has several advantages for children with pulmonary hypertension: (1) It provides a parallel low resistance circuit that drastically decreases right ventricular (RV) afterload and native pulmonary pressure, allowing RV recovery and pulmonary vascular remodeling. (2) It may serve as a long-term pulmonary assist device or as a bridge to lung transplant or a bridge to recovery. (3) It allows physical rehabilitation. Here we report the first successful use of the Novalung surgical lung-assist (sLA) device in a small child with pulmonary hypertension.

* Abbreviations: SIDS — : sudden infant death syndrome SUID — : sudden unexpected infant death TOI — : tissue oxygenation index In this issue of Pediatrics , Fyfe et al present convincing evidence that cerebral tissue oxygenation index (TOI) is lower in habitually supine-sleeping preterm infants placed prone rather than supine, and that the cerebral TOI for preterm infants placed prone is lower than the TOI for term infants placed prone. Increased rates of sudden infant death syndrome (SIDS) among all infants sleeping prone, and even higher rates among preterm infants placed prone, provide the epidemiologic rationale for their physiologic studies.1,2 An established approach in attempting to understand sudden unexpected infant deaths (SUID), including SIDS, is the Triple Risk Model: a susceptible infant at a susceptible developmental stage is exposed to a stressor that cannot be overcome.3 The susceptible infants investigated by Fyfe et al were born before term and were studied at 2 to 4 weeks, 2 to 3 months, and 5 to 6 months post-term age, and compared with a group of infants born at term. Much recent study of SUID has focused on the interaction between sleep position and sleep microenvironment. This group from Australia has carried the torch in trying … Address correspondence to James S. Kemp, MD, St Louis Children’s Hospital, 1 Children’s Place, St Louis, MO 63130. E-mail: kemp_j{at}kids.wustl.edu

The main underlying diagnoses of children with end-stage heart failure are congenital heart disease and cardiomyopathy. These children require close clinical monitoring by a cardiac failure specialist, initially as outpatients treated with oral anti congestive heart failure medication, and eventually as inpatients treated with inotropes. In fact, the majority of patients followed by the Pediatric Heart Transplant Study Group are United Network of Organ Sharing (UNOS) status 1 (heart failure requiring intravenous inotropes). Because of the limited availability of organs, waiting time for heart transplantation is long and as a result, cardiac function continues to deteriorate prior to transplantation, eventually leading to inadequate systemic oxygen delivery and end-organ failure. If the latter develops, the patient is no longer a candidate for heart transplantation. In order to avoid end organ failure, close monitoring of oxygen delivery or surrogate measures of oxygen delivery and step-wise escalation of cardiopulmonary support are required. Other than standard monitoring measures, advanced techniques should be utilized to increase the safety margin and allow timely escalation of support. These advanced monitoring techniques include pulmonary and transpulmonary thermodilution, pulse contour analysis, tissue and regional near infrared spectroscopy, and biochemical markers of perfusion and myocardial strain. Inadequate oxygen delivery in a patient treated with one inotrope warrants admission to the intensive care unit and initiation of a second vasoactive medication. The next step is intubation and mechanical ventilation. Since peritransplantation morbidity and mortality are higher in mechanically ventilated children in comparison to children supported with long-term mechanical assist devices, serious consideration should be given to implantation of a ventricular assist device shortly after intubation.

Objective To evaluate paracorporeal lung assist devices to treat neonates and children with decompensated respiratory failure as a bridge to recovery or lung transplantation. Methods One neonate (23 days old) and 3 young children (aged 2, 9, and 23 months) presented with primary lung disease with pulmonary hypertension, including alveolar capillary dysplasia in 2 and right pulmonary hypoplasia and primary pulmonary hypertension in 1. The patients were listed for lung transplantation but decompensated and required extracorporeal membrane oxygenation (ECMO). The patients were transitioned from ECMO to a pumpless paracorporeal lung assist device (Maquet Quadrox-iD oxygenator in 3, Novalung in 1) with inflow from the pulmonary artery and return to the left atrium. Results The patients were weaned from ECMO and supported by the device for 44 ± 29 days (range, 5-74). Three patients were extubated while supported by the device (after 9, 15, and 72 days). One patient was bridged to lung transplant (9 months old, with alveolar capillary dysplasia, supported 5 days). One patient was bridged to recovery with maximal medical therapy (23 months old, with primary pulmonary hypertension, supported 23 days). Two patients died while awaiting a suitable lung donor after a support time of 54 and 72 days. Conclusions Pediatric patients bridged from ECMO to lung transplantation have poor results. An alternative method for longer term respiratory support was necessary as a bridge for these patients. The use of a paracorporeal lung assist device successfully supported 4 patients to recovery, lung transplantation, or past the average wait time for pediatric donor lungs (27 days). This therapy has the potential to bridge children with decompensated respiratory failure to lung transplantation.

Many infants are exclusively fed unmodified goat's milk as a result of cultural beliefs as well as exposure to false online information. Anecdotal reports have described a host of morbidities associated with that practice, including severe electrolyte abnormalities, metabolic acidosis, megaloblastic anemia, allergic reactions including life-threatening anaphylactic shock, hemolytic uremic syndrome, and infections. We describe here an infant who was fed raw goat's milk and sustained intracranial infarctions in the setting of severe azotemia and hypernatremia, and we provide a comprehensive review of the consequences associated with this dangerous practice.

Prompt initiation of appropriate and intensive treatment in children with decompensated heart failure is crucial to avoid irreversible end-organ dysfunction. Initial management of these children includes transfer to the pediatric cardiac intensive care unit, basic hemodynamic monitoring, and establishment of intravenous access. Inotropic support should be instituted peripherally before obtaining central venous and arterial access. The team should be prepared for emergent intubation and initiation of mechanical circulatory support. Two experienced physicians should work together to obtain vascular access and manage sedation, airway control, and cardiovascular support. Acute heart failure syndrome in children may be related to cardiomyopathy, myocarditis, congenital heart disease, and acute rejection post heart transplantation. Each of these causes requires a different approach. Fulminant myocarditis may lead to severe morbidity and requires intensive support, although its outcome is considered to be good. Acute heart failure related to newly diagnosed dilated cardiomyopathy may represent end-stage heart failure; therefore, long-term mechanical circulatory support and heart transplantation may be considered to avoid other end-organ dysfunction. Hypertrophic cardiomyopathy may lead to acute decompensation due to 1) left ventricular outflow obstruction, 2) restrictive physiology leading to pulmonary hypertension, or 3) myocardial is chemia associated with coronary artery bridging. Decompensated heart failure associated with congenital heart disease usually represents end-stage heart failure and requires thorough evaluation for heart transplantation. Children with single-ventricle physiology who develop decompensated heart failure after a Fontan procedure are not candidates for mechanical circulatory support and therefore may not survive to heart transplantation. Acute heart failure due to posttransplantation acute rejection requires aggressive antirejection treatment, which places these patients at significant risk for overwhelming opportunistic infections. In our opinion, mechanical circulatory support should be initiated early in children who present with end-stage heart failure associated with hemodynamic instability to avoid end-organ damage.

Electrocardiographic criteria for the diagnosis of cardiac ischemia in adults are well defined; however, analogous criteria for the diagnosis of cardiac ischemia in infants and children remain ambiguous. The difficulty in defining electrocardiographic criteria in pediatrics relates to age-dependent differences in the pediatric electrocardiogram, the presence or absence of congenital heart disease, and multiple and diverse etiologies of myocardial injury that lead to an ischemic pattern on the electrocardiogram. In this report, we illustrate 3 pediatric cases in which the electrocardiogram met adult diagnostic criteria for acute transmural myocardial infarction, without coronary artery abnormalities in 2 cases and with a transient coronary abnormality in the third. In conclusion, ST-segment changes diagnostic of transmural myocardial infarction in adults may be seen in pediatric patients in the absence of coronary artery occlusion.

Rhabdomyoma is the most common primary cardiac tumor identified in utero and in infancy. Usually it has a benign course, which has prompted an expectant approach to its management. We report herein the cases of three patients who presented prenatally with cardiac rhabdomyomas. Only one of them had a benign course. The other two patients provided recognizable characteristics of rhabdomyomas with an unfavorable course and demonstrated that fetal rhabdomyomas can have a fatal outcome.

The acute abdomen in the pediatric age group is not infrequently fraught with diagnostic pitfalls. The younger the patient, the more problematic the presentation can be. Among the more unusual manifestations of an acute abdomen is an apparent encephalopathic picture. We present 2 cases which serve to illustrate the diagnostic difficulties encountered.

We evaluated the usefulness of patent ductus arteriosus (PDA) occlusion using a new catheter that combines good angiographic properties, easy manipulation across the duct, documentation of aortic and pulmonary artery pressure, and coil delivery for percutaneous transaortic ductal occlusion. Thirty patients (13 females and 17 males) who met the clinical and echocardiographic criteria for the percutaneous closure of PDA were catheterized. The patients selected for this study had a small to moderate PDA with a conal or tubular shape, adequate aortic ampula, and normal or mildly elevated pulmonary artery pressure. The median age was 4.8 years (range, 10 months–20 years). Patients were catheterized by the transaortic approach using the new angiographic and coil delivery catheter specially designed for this procedure. A PDA was closed in every case. A single DuctOcclud pfm coil was delivered in 28 patients, and 2 patients had NitOcclud pfm coils for ductal occlusion. The screening time was measured and found to be 50% less than the measured screening time using other catheters in our laboratory. Only one catheter and one guidewire were used per procedure, with no need for exchange wires or the establishment of an arteriovenous loop. The procedure time was also reduced by 50%. A minimal residual shunt, demonstrated by color-flow Doppler mapping, was present in 2 patients. The new combined angiographic and coil delivery catheter is most suitable for transaortic PDA coil occlusion, significantly decreasing the procedure and screening time.

Thiamine-responsive megaloblastic anemia (TRMA) syndrome is a rare autosomal recessive disorder defined by the occurrence of megaloblastic anemia, diabetes mellitus, and sensorineural deafness, responding in varying degrees to thiamine treatment. Other features of this syndrome gradually develop. We describe three TRMA patients with heart rhythm abnormalities and structural cardiac anomalies. Eight other reported TRMA patients also had cardiac anomalies. Recently, the TRMA gene, SLC19A2, was identified, encoding a functional thiamine transporter. Characterization of the metabolic defect of TRMA may shed light on the role of thiamine in common cardiac abnormalities.

Mutations of the cardiac transcription factor NKX2‐5 cause hypoplastic development of the AV node and conduction block. How the anatomy of the mutant AV node relates to its function is unknown. We thus studied conduction through the AV nodal region in ex vivo preparations of wild‐type and Nkx2‐5+/− mouse hearts in which the central conduction system was highlighted by a transgenic Cx40‐eGFP reporter. Fluorescence imaging guided electrode placement and pacing of the inferior and superior approaches to the AV node. Nkx2‐5+/− hearts had a prolonged atrio‐His interval compared to the wild type, consistent with previous in vivo observations. The conduction time to the His bundle from the Cx40− AV nodal region that is superior to and immediately adjacent to the Cx40+ lower node is slightly, but not significantly greater in Nkx2‐5+/− than wild‐type hearts. A novel phenotype was also observed. Pacing the Cx40− inferior approach to the AV node with increasing stimulus strength led to progressive shortening of the stimulus‐to‐His conduction interval in wild‐type but not Nkx2‐5+/− hearts. The strength of pacing at the Cx40− superior approach had no effect on the conduction interval in either group. The prolonged AV delay in the Nkx2‐5+/− heart appears to arise before the Cx40+ lower node. Whether the pacing phenotype explains the mutant's conduction defect is uncertain, but the observation adds to a number of unique properties of the inferior approach to the AV node., Mutations of the cardiac transcription factor NKX2‐5 cause hypoplastic development of the AV node and associated conduction defects. Here, we use microelectrodes to measure conduction intervals between locations in the atrial myocardium and subdomains of the AV node and the His bundle, as guided by Cx40‐eGFP fluorescence imaging. We also report a novel property of pacing the inferior approach to the AV node and its related mutant phenotype.