Contemporary Care and Outcomes of Critically-ill Children With Clinically Diagnosed Myocarditis.

PURPOSE: To describe contemporary management and outcomes in children with myocarditis who are admitted to a cardiac intensive care unit (CICU) and to identify the characteristics associated with mortality. METHODS: All patients in the Pediatric Cardiac Critical Care Consortium (PC4) registry between August 2014 and June 2021 who were diagnosed with myocarditis were included. Univariable analyses and multivariable logistic regression evaluated the factors associated with in-hospital mortality. RESULTS: There were 847 CICU admissions for myocarditis in 51 centers. The median age was 12 years (IQR 2.7-16). In-hospital mortality occurred in 53 patients (6.3%), and 60 (7.1%) had cardiac arrest during admission. Mechanical ventilation was required in 339 patients (40%), and mechanical circulatory support (MCS) in 177 (21%); extracorporeal membrane oxygenation (ECMO)-only in 142 (16.7%), ECMO-to-ventricular assist device (VAD) in 20 (2.4%), extracorporeal cardiac resuscitation in 43 (5%), and VAD-only in 15 (1.8%) patients. MCS was associated with in-hospital mortality; 20.3% receiving MCS died compared to 2.5% without MCS (P < 0.001). Mortality rates were similar in ECMO-only, ECMO-to-VAD and VAD-only groups. The median time from CICU admission to ECMO was 2.0 hours (IQR 0-9.4) and to VAD, it was 9.9 days (IQR 6.3-16.8). Time to MCS was not associated with mortality. In multivariable modeling of patients' characteristics, smaller body surface area (BSA) and low eGFR were independently associated with mortality, and after including critical therapies, mechanical ventilation and ECMO were independent predictors of mortality. CONCLUSION: This contemporary cohort of children admitted to CICUs with myocarditis commonly received high-resource therapies; however, most patients survived to hospital discharge and rarely received VAD. Smaller patient size, acute kidney injury and receipt of mechanical ventilation or ECMO were independently associated with mortality.

Contemporary Outcomes of Children With Acute Fulminant Myocarditis Supported With Peripheral Extracorporeal Membrane Oxygenation.

Despite extracorporeal membrane oxygenation (ECMO) utilization in nearly 20% of cases, there are limited data in children with acute fulminant myocarditis (AFM) requiring ECMO. Herein we identify risk factors for death or heart transplant (HT) in children with AFM supported with ECMO, describe our experience with left atrial (LA) decompression, and depict long-term outcomes of survivors. We performed a retrospective cohort of patients <18 years with AFM (≤14 days of symptoms, rapid cardiogenic shock, and normal left ventricular [LV] size on presentation) supported with ECMO admitted to a single intensive care unit from 1997 to 2021. Among 28 patients (median age 9 years), 21 (75%) survived to discharge without HT. Patients were supported on ECMO for a median of 6 days. Three patients were bridged to HT with durable ventricular assist devices (VAD). Four patients died, two of whom were supported with VAD. At presentation, seven (25%) patients had high grade or complete atrioventricular block and eight (29%) had ventricular tachycardia. Before ECMO cannulation, 21 (75%) patients received CPR. The death/HT group had higher peak troponin levels (12.5 vs. 1.0 ng/ml, p = 0.02) and initial mean LA or pulmonary capillary wedge pressure (27 vs. 18 mm Hg, p = 0.03). Left atrial decompression was performed in 22 patients (79%). Twenty-two (79%) had acute myocarditis on endomyocardial biopsy. Among transplant-free survivors, 18 (86%) had normalization in LV function (median 7 days); the remaining three patients had persistent mild LV dysfunction at last follow-up (median 842 days). Transplant-free survival of pediatric patients with AFM supported on ECMO was 75% and associated with lower initial LA pressure and lower peak troponin. Recovery in ventricular function among survivors was rapid and durable.

Higher Survival With the Use of Extracorporeal Cardiopulmonary Resuscitation Compared With Conventional Cardiopulmonary Resuscitation in Children Following Cardiac Surgery: Results of an Analysis of the Get With The Guidelines-Resuscitation Registry.

OBJECTIVES: Extracorporeal membrane oxygenation to support cardiopulmonary resuscitation (CPR) is increasingly used in children suffering cardiac arrest after cardiac surgery. However, its efficacy in promoting survival has not been evaluated. We compared survival of pediatric cardiac surgery patients suffering in-hospital cardiac arrest who were resuscitated with extracorporeal CPR (E-CPR) to those resuscitated with conventional CPR (C-CPR) using propensity matching. DESIGN: Retrospective study using multicenter data from the American Heart Association Get With The Guidelines-Resuscitation registry (2008-2020). SETTING: Multicenter cardiac arrest database containing cardiac arrest and CPR data from U.S. hospitals. PATIENTS: Cardiac surgical patients younger than 18 years old who suffered in-hospital cardiac arrest and received greater than or equal to 10 minutes of CPR. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Among 1223 patients, 741 (60.6%) received C-CPR and 482 (39.4%) received E-CPR. E-CPR utilization increased over the study period (p < 0.001). Duration of CPR was longer in E-CPR compared with C-CPR recipients (42 vs. 26 min; p < 0.001). In a propensity score matched cohort (382 E-CPR recipients, 382 C-CPR recipients), E-CPR recipients had survival to discharge (odds ratio [OR], 2.22; 95% CI, 1.7-2.9; p < 0.001). E-CPR survival was only higher when CPR duration was greater than 18 minutes. Propensity matched analysis using patients from institutions contributing at least one E-CPR case (n = 35 centers; 353 E-CPR recipients, 353 C-CPR recipients) similarly demonstrated improved survival in E-CPR recipients compared with those who received C-CPR alone (OR, 2.08; 95% CI, 1.6-2.8; p < 0.001). CONCLUSIONS: E-CPR compared with C-CPR improved survival in children suffering cardiac arrest after cardiac surgery requiring CPR greater than or equal to 10 minutes.

Complex decision making in an intensive care environment: Perceived practice versus observed reality.

RATIONALE: Advancing our understanding of how decisions are made in cognitively, socially and technologically complex hospital environments may reveal opportunities to improve healthcare delivery, medical education and the experience of patients, families and clinicians. AIMS AND OBJECTIVES: Explore factors impacting clinician decision making in the Boston Children's Hospital Cardiac Intensive Care Unit. METHODS: A convergent mixed methods design was used. Quantitative and qualitative data sources consisted of a faculty survey, direct observations of clinical rounds in a specific patient population identified by a clinical decision support system (CDSS) and semistructured interviews (SSIs). Deductive and inductive coding was used for qualitative data. Qualitative data were translated into images using social network analysis which illustrate the frequency and connectivity of the codes in each data set. RESULTS: A total of 25 observations of eight faculty-led interprofessional teams were performed between 12 February and 31 March 2021. Individual patient characteristics were noted by faculty in SSIs to be the most important factor in their decision making, yet ethnographic observations suggested faculty cognitive traits, team expertise and value-based decisions were more heavily weighted. The development of expertise was impacted by role modeling. Decisions were perceived to be influenced by the system and environment. CONCLUSIONS: Clinician perception of decision making was not congruent with the observed behaviours in a complicated and dynamic system. This study identifies important considerations in clinical curricula as well as the design and implementation of CDSS. Our method of using social network analysis to visualize components of decision making could be adopted to explore other complex environments.

Long-Term Quality of Life in Congenital Heart Disease Surgical Survivors: Multicenter Retrospective Study of Surgical and ICU Explanatory Factors.

OBJECTIVES: Greater congenital heart disease (CHD) complexity is associated with lower health-related quality of life (HRQOL). There are no data on the association between surgical and ICU factors and HRQOL in CHD survivors. This study assess the association between surgical and ICU factors and HRQOL in child and adolescent CHD survivors. DESIGN: This was a corollary study of the Pediatric Cardiac Quality of Life Inventory (PCQLI) Testing Study. SETTING: Eight pediatric hospitals participating in the PCQLI Study. PATIENTS: Patients in the study had the Fontan procedure, surgery for tetralogy of Fallot (TOF), and transposition of the great arteries (TGAs). MEASUREMENTS AND MAIN RESULTS: Surgical/ICU explanatory variables were collected by reviewing the medical records. Primary outcome variables (PCQLI Total patient and parent scores) and covariates were obtained from the Data Registry. General linear modeling was used to create the multivariable models. There were 572 patients included: mean ± sd of age 11.7 ± 2.9 years; CHD Fontan 45%, TOF/TGA 55%; number of cardiac surgeries 2 (1-9); and number of ICU admissions 3 (1-9). In multivariable models, lowest body temperature on cardiopulmonary bypass (CPB) was negatively associated with patient total score (p < 0.05). The total number of CPB runs was negatively associated with parent-reported PCQLI Total score (p < 0.02). Cumulative days on an inotropic/vasoactive drug in the ICU was negatively associated with all patient-/parent-reported PCQLI scores (p < 0.04). Neurological deficit at discharge was negatively associated with parent-reported PCQLI total score (p < 0.02). The variance explained by these factors ranged from 24% to 29%. CONCLUSIONS: Surgical/ICU factors, demographic, and medical care utilization variables explain a low-to-moderate amount of variation in HRQOL. Research is needed to determine whether modification of these surgical and ICU factors improves HRQOL, and to identify other factors that contribute to unexplained variability.

Communication challenges: perioperative risks and care directives.

PURPOSE OF REVIEW: Effective communication and shared decision-making is crucial in caring for children with congenital or acquired heart disease. Despite high mortality, complications related to interventions, and suffering in pediatric advanced heart disease, there is a disproportionate lack of research and advancements in communication and shared decision-making surrounding perioperative risks and advance care planning. We have comprehensively reviewed recent research on this multifaceted topic in the past 1.5 years to highlight major findings and propose priorities for future research. RECENT FINDINGS: Research has focused on parental perception of clinician/team communication with a notable paucity of research investigating the clinician perception of barriers to effective communication. Although there are discrepancies between parental and clinician perception, lack of trust between the patient's family and provider was acknowledged as a barrier by both parents and clinicians. Insight into interprofessional needs and expansion of structured language for discussions around shared decision-making should be prioritized. SUMMARY: Clinician discomfort with communication skills and conveying uncertainty in prognosis are two major areas for future research. On the basis of the literature to date, the focus should be placed on interprofessional needs and structured language for discussions around shared decision-making.

Factors associated with morbidity, mortality, and hemodynamic failure after biventricular conversion in borderline hypoplastic left hearts.

OBJECTIVE: A subset of patients with borderline hypoplastic left heart may be candidates for single to biventricular conversion, but long-term morbidity and mortality persist. Prior studies have shown conflicting results regarding the association of preoperative diastolic dysfunction and outcome, and patient selection remains challenging. METHODS: Patients with borderline hypoplastic left heart undergoing biventricular conversion from 2005 to 2017 were included. Cox regression identified preoperative factors associated with a composite outcome of time to mortality, heart transplant, takedown to single ventricle circulation, or hemodynamic failure (defined as left ventricular end-diastolic pressure >20 mm Hg, mean pulmonary artery pressure >35 mm Hg, or pulmonary vascular resistance >6 international Woods units). RESULTS: Among 43 patients, 20 (46%) met the outcome, with a median time to outcome of 5.2 years. On univariate analysis, endocardial fibroelastosis, lower left ventricular end-diastolic volume/body surface area (when <50 mL/m2), lower left ventricular stroke volume/body surface area (when <32 mL/m2), and lower left:right ventricular stroke volume ratio (when <0.7) were associated with outcome; higher preoperative left ventricular end-diastolic pressure was not. Multivariable analysis demonstrated that endocardial fibroelastosis (hazard ratio, 5.1, 95% confidence interval, 1.5-22.7, P = .033) and left ventricular stroke volume/body surface area 28 mL/m2 or less (hazard ratio, 4.3, 95% confidence interval, 1.5-12.3, P = .006) were independently associated with a higher hazard of the outcome. Approximately all patients (86%) with endocardial fibroelastosis and left ventricular stroke volume/body surface area 28 mL/m2 or less met the outcome compared with 10% of those without endocardial fibroelastosis and with higher stroke volume/body surface area. CONCLUSIONS: History of endocardial fibroelastosis and smaller left ventricular stroke volume/body surface area are independent factors associated with adverse outcomes among patients with borderline hypoplastic left heart undergoing biventricular conversion. Normal preoperative left ventricular end-diastolic pressure is insufficient to reassure against diastolic dysfunction after biventricular conversion.

Cardiovascular intensive care unit variables inform need for feeding tube utilization in infants with hypoplastic left heart syndrome.

OBJECTIVE: Feeding strategies in infants with hypoplastic left heart syndrome (HLHS) following stage 1 palliation (S1P) include feeding tube utilization (FTU). Timely identification of infants who will fail oral feeding could mitigate morbidity in this vulnerable population. We aimed to develop a novel clinical risk prediction score for FTU. METHODS: This was a retrospective study of infants with HLHS admitted to the Boston Children's Hospital cardiovascular intensive care unit for S1P from 2009 to 2019. Infants discharged with feeding tubes were compared with those on full oral feeds. Variables from early (birth to surgery), mid (postsurgery to cardiovascular intensive care unit transfer), and late (inpatient transfer to discharge) hospitalization were analyzed in univariate and multivariable models. RESULTS: Of 180 infants, 66 (36.7%) discharged with a feeding tube. In univariate analyses, presence of a genetic disorder (early variable, odds ratio, 3.25; P = .014) and nearly all mid and late variables were associated with FTU. In the mid multivariable model, abnormal head imaging, ventilation duration, and vocal cord dysfunction were independent predictors of FTU (c-statistic 0.87). Addition of late variables minimally improved the model (c-statistic 0.91). A risk score (the HV2 score) for FTU was developed based on the mid multivariable model with high specificity (93%). CONCLUSIONS: Abnormal head imaging, duration of ventilation, and presence of vocal cord dysfunction were associated with FTU in infants with HLHS following S1P. The predictive HV2 risk score supports routine perioperative head imaging and vocal cord evaluation. Future application of the HV2 score may improve nutritional morbidity and hospital length of stay in this population.

Calcium Administration During Cardiopulmonary Resuscitation for In-Hospital Cardiac Arrest in Children With Heart Disease Is Associated With Worse Survival-A Report From the American Heart Association's Get With The Guidelines-Resuscitation (GWTG-R) Registry.

OBJECTIVES: IV calcium administration during cardiopulmonary resuscitation (CPR) for pediatric in-hospital cardiac arrest (IHCA) is associated with worse survival. We evaluated survival to hospital discharge in children with heart disease (HD), where calcium is more frequently administered during CPR. DESIGN: Retrospective study of a multicenter registry database. SETTING: Data reported to the American Heart Association's (AHA) Get With The Guidelines-Resuscitation registry. PATIENTS: Children younger than 18 years with HD experiencing an index IHCA event requiring CPR between January 2000 and January 2019. Using propensity score matching (PSM), we selected matched cohorts of children receiving and not receiving IV calcium during CPR and compared the primary outcome of survival to hospital discharge. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We included 4,556 children with HD experiencing IHCA. Calcium was administered in 1,986 (44%), more frequently in children younger than 1 year old (65% vs 35%; p < 0.001) and surgical cardiac (SC) compared with medical cardiac patients (51% vs 36%; p < 0.001). Calcium administration during CPR was associated with longer duration CPR (median 27 min [interquartile range (IQR): 10-50 min] vs 5 min [IQR, 2-16 min]; p < 0.001) and more frequent extracorporeal-CPR deployment (25% vs 8%; p < 0.001). In the PSM cohort, those receiving calcium had decreased survival to hospital discharge (39% vs 46%; p = 0.02) compared with those not receiving calcium. In a subgroup analysis, decreased discharge survival was only seen in SC cohorts. CONCLUSIONS: Calcium administration during CPR for children with HD experiencing IHCA is common and is associated with worse survival. Administration of calcium during CPR in children with HD should be restricted to specific indications as recommended by the AHA CPR guidelines.

Standardized Training for Physicians Practicing Pediatric Cardiac Critical Care.

OBJECTIVES: In the vast majority of Children's Hospitals, the critically ill patient can be found in one of three locations: the PICU, the neonatal ICU, and the cardiac ICU. Training, certification, and maintenance of certification for neonatology and critical care medicine are over seen by the Accreditation Council for Graduate Medical Education and American Board of Pediatrics. There is no standardization of training or oversight of certification and maintenance of certification for pediatric cardiac critical care. DATA SOURCES: The curricula from the twenty 4th year pediatric cardiac critical care training programs were collated, along with the learning objectives from the Pediatric Cardiac Intensive Care Society published "Curriculum for Pediatric Cardiac Critical Care Medicine." STUDY SELECTION: This initiative is endorsed by the Pediatric Cardiac Intensive Care Society as a first step toward Accreditation Council for Graduate Medical Education oversight of training and American Board of Pediatrics oversight of maintenance of certification. DATA EXTRACTION: A taskforce was established of cardiac intensivists, including the directors of all 4th year pediatric cardiac critical care training programs. DATA SYNTHESIS: Using modified Delphi methodology, learning objectives, rotational requirements, and institutional requirements for providing training were developed. CONCLUSIONS: In the current era of increasing specialized care in pediatric cardiac critical care, standardized training for pediatric cardiac critical care is paramount to optimizing outcomes.

"New Trach Mom Here ": A qualitative study of internet-based resources by caregivers of children with tracheostomy.

OBJECTIVE: Decision-making around tracheostomy placement and chronic respiratory support in children is complicated. Families often seek support and advice from outside the medical care team, including from social media. We undertook this study to characterize the content and nature of online resources created and managed primarily by caregivers of children living with tracheostomy and chronic mechanical ventilation. DESIGN/SETTING: We used a "grey literature" search methodology to identify internet resources created by caregivers of children with tracheostomy. We included only publicly available, nonindustry associated, English language, North American websites updated at least once in 2019. We then applied inductive content analysis to establish central themes, patterns and associations. MEASUREMENTS/MAIN RESULTS: We identified six blogs/forums that met our search criteria. We identified four main themes: (1) Uncertainty, (2) Lived experience-wants, needs, and emotions, (3) Seeking context and meaning, and (4) Advice/information sharing/support. Two patterns of coping were identified on the basis of the relationships between codes. The "Acceptance pathway" is associated with a sense of self-actualization, mastery, satisfaction, return to normalcy, and ultimately acceptance. The "Resignation pathway" is associated with a sense of lack of control, frustration, burnout and stress, persistent lack of normalcy, and resignation to the tracheostomy as a negative but necessary outcome. CONCLUSION: Caregivers often come to see themselves as experts in the care of children with tracheostomy, though many still express ambivalence about their knowledge and skills. Those early in the experience express a desire for community and can potentially benefit from online resources.

Online education in a hurry: Delivering pediatric graduate medical education during COVID-19.

In the setting of the coronavirus disease 2019 (COVID-19) pandemic, an emergency hospital-wide eWork policy was enacted at Boston Children's Hospital on March 16, 2020. The number of clinicians on campus was restricted to only essential personnel, guidelines limited clinical care delivery to solely non-elective patients, and strict maximums were placed on the numbers of people allowed to congregate in the same physical space. With this abrupt transition to social distancing and electronic communication, the established approach to educating graduate medical trainees became obsolete overnight. Anticipating significant impact on trainee and faculty professional and personal lives, the importance of adaptive teaching strategies was evident. This document details one approach to redesigning the clinical learning system including a description of the learners and environment, the pedagogical principles that guided the approach, and technological tools used in implementation. Additionally, available literature pertinent to this topic is explored, assessment of the work to date is presented, and suggestions are provided regarding future directions related to online graduate medical education.

Extracorporeal Membrane Oxygenation in Infants Undergoing Truncus Arteriosus Repair.

BACKGROUND: Infants undergoing truncus arteriosus (TA) repair suffer one of the highest mortality rates of all congenital heart defects. Extracorporeal membrane oxygenation (ECMO) can support patients undergoing TA repair, but little is known about factors contributing to mortality in this cohort. The objective of this study was to identify risk factors for mortality in infants with TA requiring perioperative ECMO. METHODS: Data from the Extracorporeal Life Support Organization from 2002 to 2017 for infants less than 60 days old undergoing TA repair were analyzed. Demographics, clinical characteristics, and ECMO characteristics and complications were compared between survivors and nonsurvivors. Multivariable logistic regression was used to evaluate independent risk factors for mortality. RESULTS: Of 245 patients analyzed, 92 (37.6%) survived to discharge. Nonsurvivors had a lower weight and a longer ECMO duration. A higher proportion of nonsurvivors suffered complications on ECMO, including mechanical complications, circuit thrombus, bleeding, and need for renal replacement therapy. In multivariable analysis lower weight (odds ratio [OR], 0.56; 95% confidence interval [CI], 0.33-0.95), duration of ECMO (OR, 1.1; 95% CI, 1.02-1.18), need for renal replacement therapy (OR, 3.23; 95% CI, 1.68-6.2), cardiopulmonary resuscitation on ECMO (OR, 11.52; 95% CI, 1.3-102.33), and infection on ECMO (OR, 4.47; 95% CI, 1.2-16.64) were independently associated with mortality. CONCLUSIONS: Many factors associated with mortality for infants requiring perioperative ECMO with TA repair are related to complications suffered on ECMO. Thoughtful patient selection and meticulous ECMO management to prevent complications are essential in improving outcomes for these infants.

The impact of pre-implant illness severity on the outcomes of pediatric patients undergoing durable ventricular assist device.

BACKGROUND: Durable ventricular assist devices (VADs) are increasingly used to treat children with heart failure. Studies demonstrate worse outcomes for those in cardiogenic shock at the time of VAD, but limited data exist on less acutely ill children. We describe the association between illness severity and outcomes in this population. METHODS: Data were analyzed from 373 children (aged <19 years) receiving durable VADs from 46 centers in the Pediatric Interagency Registry for Mechanical Circulatory Support. Outcomes were compared by Interagency Registry for Mechanical Circulatory Support (INTERMACS) Patient Profile (PP) and pre-implant characteristics using competing risks methodology. RESULTS: Analyses identified 97 patients in cardiogenic shock (PP 1), 222 with progressive decline (PP 2), and 42 stable on inotropes (PP 3). There were 39 infants, 124 were aged 1 to 9 years and 210 were aged 10 to 19 years. A majority had cardiomyopathy and 66 had congenital heart disease (CHD). There were 224 (62%) continuous-flow VADs. Before implant, 40% received mechanical ventilation (MV). Within 6 months post-implant, 57% underwent transplant and 14% died. PP 1 mortality was highest (25% vs 10% for PP 2, hazard ratio [HR]: 2.5, 95% CI: 1.4-4.4, p = 0.02). In PP 1, CHD was an independent mortality risk factor (HR: 2.9, 95% CI: 1.1-7.8, p = 0.03). In PP 2, pulsatile VADs were associated with death (HR: 3.9, 95% CI: 1.6-9.5, p = 0.003). Patients on MV had high mortality (20%-30%) across PP 1 to PP 3 (HR: 3.0 vs no MV, p < 0.001). CONCLUSIONS: Children in shock at the time of VAD implant have poor outcomes. MV is associated with increased mortality even in lower acuity INTERMACS profiles. Further study is needed to identify modifiable risk factors in this population.

Renal replacement therapy in the pediatric cardiac intensive care unit.

OBJECTIVE: There is an increased risk of mortality in patients in whom acute kidney injury and fluid accumulation develop after cardiothoracic surgery, and the risk is especially high when renal replacement therapy is needed. However, renal replacement therapy remains an essential intervention in managing these patients. The objective of this study was to identify risk factors for mortality in surgical patients requiring renal replacement therapy in a pediatric cardiac intensive care unit. METHODS: We performed a retrospective review of patients requiring renal replacement therapy for acute kidney injury or fluid accumulation after cardiothoracic surgery between January 2009 and December 2017. Survivors and nonsurvivors were compared with respect to multiple variables, and a multivariable logistic regression analysis was performed to identify independent risk factors associated with mortality. RESULTS: The mortality rate for the cohort was 75%. Nonsurvivors were younger (nonsurvivors: 0.8 years; interquartile range, 0.1-8.2; survivors: 14.6 years; interquartile range, 4.2-19.7; P = .002) and had a lower weight-for-age z-score (nonsurvivors: -1.5; interquartile range, -3.1 to -0.4; survivors: -0.5; interquartile range, -0.9 to 0.3; P = .02) compared with survivors. There was no difference with respect to fluid accumulation. In multivariable analysis, a longer duration of stage 3 acute kidney injury before initiation of renal replacement therapy was independently associated with mortality (adjusted odds ratio, 1.39; 95% confidence interval, 1.05-1.83; P = .021). CONCLUSIONS: Mortality in patients requiring renal replacement therapy after congenital heart disease surgery is high. A longer duration of acute kidney injury before renal replacement therapy initiation is associated with increased mortality.

Heterotaxy Syndrome: Proceedings From the 10th International PCICS Meeting.

A session dedicated to heterotaxy syndrome was included in the program of the Tenth International Conference of the Pediatric Cardiac Intensive Care Society in Miami, Florida in December 2014. An invited panel of experts reviewed the anatomic considerations, surgical considerations, noncardiac issues, and long-term outcomes in this challenging group of patients. The presentations, summarized in this article, reflect the current approach to this complex multiorgan syndrome and highlight future areas of clinical interest and research.

Prediction by clinicians of quality of life for children and adolescents with cardiac disease.

OBJECTIVE: To determine whether clinicians could reliably predict health-related quality of life (HRQOL) for children with cardiac disease, the level of agreement in predicted HRQOL scores between clinician sub-types, and agreement between clinician-predicted HRQOL scores and patient and parent-proxy reported HRQOL scores. STUDY DESIGN: In this multicenter, cross-sectional study, a random sample of clinical summaries of children with cardiac disease and related patient and parent-proxy reported HRQOL scores were extracted from the Pediatric Cardiac Quality of Life Inventory data registry. We asked clinicians to review each clinical summary and predict HRQOL. RESULTS: Experienced pediatric cardiac clinicians (n = 140), including intensive care physicians, outpatient cardiologists, and intensive care, outpatient, and advanced practice nurses, each predicted HRQOL for the same 21 pediatric cardiac patients. Reliability within clinician subspecialty groups for predicting HRQOL was poor (intraclass correlation coefficients, 0.34-0.38). Agreement between clinician groups was low (Pearson correlation coefficients, 0.10-0.29). When comparing the average clinician predicted HRQOL scores to those reported by patients and parent-proxies by Bland Altman plots, little systematic bias was present, but substantial variability existed. Proportional bias was found, in that clinicians tended to overestimate HRQOL for those patients and parent-proxies who reported lower HRQOL, and underestimate HRQOL for those reporting higher HRQOL. CONCLUSIONS: Clinicians perform poorly when asked to predict HRQOL for children with cardiac disease. Clinicians should be cognizant of these data when providing counseling. Incorporating reported HRQOL into clinical assessment may help guide individualized treatment decision-making.

Extracorporeal membrane oxygenation in patients undergoing superior cavopulmonary anastomosis.

OBJECTIVE: Patients who have undergone the superior cavopulmonary anastomosis (Glenn procedure) have unique cardiopulmonary-cerebral physiology that may limit the success of cardiopulmonary resuscitation and extracorporeal membrane oxygenation (ECMO). Limited data published to date suggest grim morbidity and mortality when ECMO is used. We utilized the Extracorporeal Life Support Organization registry database to more thoroughly assess outcomes in these patients. METHODS: Data from the Extracorporeal Life Support Organization registry from 1999 to 2012 for children with Glenn physiology aged 3 months to 1 year were retrospectively analyzed. Demographics and ECMO characteristics were compared between survivors and nonsurvivors. Factors associated with mortality were evaluated using multivariate logistic regression. RESULTS: Of 103 infants, 42 (41%) survived to hospital discharge. Neurologic complications (eg, seizure, hemorrhage, or embolic stroke) were documented in 23% of patients (24 of 103) and 14% of survivors (6 of 42). In univariate analysis, inotropic requirement before ECMO, duration of ECMO, mechanical complications with the ECMO circuit, renal failure, and pulmonary hemorrhage or pneumothorax were predictors of mortality. In multivariate logistic regression, inotrope requirement (odds ratio [OR], 3.6; 95% confidence interval [CI], 1.3-9.8), longer duration of ECMO support (OR, 7.2; 95% CI, 1.8-28), combined cardiopulmonary indication for ECMO (OR, 3.7; 95% CI, 1.4-9.7), and renal failure (OR, 4.2; 95% CI, 1.5-12) were associated with mortality. CONCLUSIONS: Mortality in infants with Glenn physiology supported with ECMO is lower than that previously reported, but the incidence of neurologic injury is high. These data support use of ECMO in patients with Glenn physiology with refractory cardiopulmonary failure.

The association of carotid artery cannulation and neurologic injury in pediatric patients supported with venoarterial extracorporeal membrane oxygenation*.

OBJECTIVES: To describe the prevalence of neurologic injury in a recent cohort of patients 18 years old or younger cannulated for venoarterial extracorporeal membrane oxygenation. To evaluate the association of carotid artery cannulation with neurologic injury when compared with other cannulation sites. To determine if age impacts the association of carotid artery cannulation with neurologic injury. DESIGN: Retrospective analysis of data from the Extracorporeal Life Support Organization registry. SETTING: Neonatal and pediatric medical/surgical and cardiac ICUs of 118 international tertiary care centers worldwide. PATIENTS: Pediatric patients 18 years old or younger cannulated for venoarterial extracorporeal membrane oxygenation and reported to the Extracorporeal Life Support Organization registry during 2007 and 2008. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Two thousand nine hundred seventy-seven patients underwent venoarterial extracorporeal membrane oxygenation during the study period. Indications for extracorporeal membrane oxygenation included pulmonary (n = 1,390, 47%), cardiac (n = 1,168, 39%), extracorporeal membrane oxygenation during cardiopulmonary resuscitation (n = 418, 14%), and unknown (n = 1). Arterial cannulation sites were aorta (n = 938, 32%), femoral artery (n = 118, 4%), and carotid artery (n = 1,921, 64%). Overall, 611 patients (21%) had evidence of neurologic injury defined as seizures, infarction, and/or hemorrhage. The occurrence of neurologic injury varied significantly by cannulation site: femoral artery (n = 18, 15%), aorta (n = 160, 17%), and carotid artery (n = 433, 23%); p equals 0.001. Neonates represented the largest group of patients cannulated for venoarterial extracorporeal membrane oxygenation (n = 1,807, 61%), the majority of patients cannulated via the carotid artery (n = 1,276, 66%), and had the highest burden of neurologic injury (n = 398, 22%). Age, preextracorporeal membrane oxygenation high-frequency oscillatory ventilation use, preextracorporeal membrane oxygenation arterial pH and serum bicarbonate level, and preextracorporeal membrane oxygenation cardiac arrest were independently associated with neurologic injury in a covariate model. Carotid artery cannulation site was added to this adjusted model and found to independently increase odds of neurologic injury (odds ratio, 1.4 [95% CI, 1.01-1.69]). An interaction term containing age and cannulation site was not associated with neurologic injury (odds ratio, 1.06 [95% CI, 0.84-1.34]). CONCLUSIONS: Carotid artery cannulation for venoarterial extracorporeal membrane oxygenation in patients 18 years old or younger is associated with statistically significant increased odds of neurologic injury. These increased odds are present across all age groups.