Paediatric cardiology training: burnout, fulfilment, and fears.

BACKGROUND: Burnout is well characterised in physicians and residents but not in paediatric cardiology fellows, and few studies follow burnout longitudinally. Training-specific fears have been described in paediatric cardiology fellows but also have not been studied at multiple time points. This study aimed to measure burnout, training-specific fears, and professional fulfilment in paediatric cardiology fellows with the attention to time of year and year-of-training. METHODS: This survey-based study included the Professional Fulfillment Index and the Impact of Events Scale as well as an investigator-designed Fellow Fears Questionnaire. Surveys were distributed at three-time points during the academic year to paediatric cardiology fellows at a large Midwestern training programme. Fellow self-reported gender and year-of-training were collected. Descriptive analyses were performed. RESULTS: 10/17 (59%) of fellows completed all surveys; 60% were female, 40% in the first-year class, 40% in the second-year class, and 20% in the third-year class. At least half of the fellows reported burnout at each survey time point, with lower mean professional fulfilment scores. The second-year class, who rotate primarily in the cardiac ICU, had higher proportions of burnout than the other two classes. At least half of fellows reported that they "often" or "always" worried about not having enough clinical knowledge or skills and about work-life balance. CONCLUSIONS: Paediatric cardiology fellows exhibit high proportions of burnout and training-specific fears. Interventions to mitigate burnout should be targeted specifically to training needs, including during high-acuity rotations.

The Michigan Appropriateness Guide for Intravenous Catheters in children with congenital heart disease: miniMAGIC-CHD.

BACKGROUND: The approach to vascular access in children with CHD is a complex decision-making process that may have long-term implications. To date, evidence-based recommendations have not been established to inform this process. METHODS: The RAND/UCLA Appropriateness Method was used to develop miniMAGIC, including sequential phases: definition of scope and key terms; information synthesis and literature review; expert multidisciplinary panel selection and engagement; case scenario development; and appropriateness ratings by expert panel via two rounds. Specific recommendations were made for children with CHD. RESULTS: Recommendations were established for the appropriateness of the selection, characteristics, and insertion technique of intravenous catheters in children with CHD with both univentricular and biventricular physiology. CONCLUSION: miniMAGIC-CHD provides evidence-based criteria for intravenous catheter selection for children with CHD.

Urgent hybrid palliation for interrupted aortic arch in a preterm infant.

In patients with ductal-dependent CHD, ductal restriction can be life-threatening. We present the use of urgent hybrid palliation involving placement of bilateral pulmonary artery bands and ductus arteriosus stent implantation in a 1.5 kg premature infant with interrupted aortic arch and ductal restriction.

A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease.

Children with single-ventricle disease experience high mortality and complex care. In other life-limiting childhood illnesses, paediatric palliative care may mitigate maternal stress. We hypothesised that early palliative care in the single-ventricle population may have the same benefit for mothers. In this pilot randomised trial of early palliative care, mothers of infants with prenatal single-ventricle diagnoses completed surveys measuring depression, anxiety, coping, and quality of life at a prenatal visit and neonatal discharge. Infants were randomised to receive early palliative care - structured evaluation, psychosocial/spiritual, and communication support before surgery - or standard care. Among 56 eligible mothers, 40 enrolled and completed baseline surveys; 38 neonates were randomised, 18 early palliative care and 20 standard care; and 34 postnatal surveys were completed. Baseline Beck Depression Inventory-II and State-Trait Anxiety Index scores exceeded normal pregnant sample scores (mean 13.76±8.46 versus 7.0±5.0 and 46.34±12.59 versus 29.8±6.35, respectively; p=0.0001); there were no significant differences between study groups. The early palliative care group had a decrease in prenatal to postnatal State-Trait Anxiety Index scores (-7.6 versus 0.3 in standard care, p=0.02), higher postnatal Brief Cope Inventory positive reframing scores (p=0.03), and a positive change in PedsQL Family Impact Module communication and family relationships scores (effect size 0.46 and 0.41, respectively). In conclusion, these data show that mothers of infants with single-ventricle disease experience significant depression and anxiety prenatally. Early palliative care resulted in decreased maternal anxiety, improved maternal positive reframing, and improved communication and family relationships.

Diagnostic errors in paediatric cardiac intensive care.

IntroductionDiagnostic errors cause significant patient harm and increase costs. Data characterising such errors in the paediatric cardiac intensive care population are limited. We sought to understand the perceived frequency and types of diagnostic errors in the paediatric cardiac ICU. METHODS: Paediatric cardiac ICU practitioners including attending and trainee physicians, nurse practitioners, physician assistants, and registered nurses at three North American tertiary cardiac centres were surveyed between October 2014 and January 2015. RESULTS: The response rate was 46% (N=200). Most respondents (81%) perceived that diagnostic errors harm patients more than five times per year. More than half (65%) reported that errors permanently harm patients, and up to 18% perceived that diagnostic errors contributed to death or severe permanent harm more than five times per year. Medication side effects and psychiatric conditions were thought to be most commonly misdiagnosed. Physician groups also ranked pulmonary overcirculation and viral illness to be commonly misdiagnosed as bacterial illness. Inadequate care coordination, data assessment, and high clinician workload were cited as contributory factors. Delayed diagnostic studies and interventions related to the severity of the patient's condition were thought to be the most commonly reported process breakdowns. All surveyed groups ranked improving teamwork and feedback pathways as strategies to explore for preventing future diagnostic errors. CONCLUSIONS: Paediatric cardiac intensive care practitioners perceive that diagnostic errors causing permanent harm are common and associated more with systematic and process breakdowns than with cognitive limitations.

Myocardial Oxidative Stress in Infants Undergoing Cardiac Surgery.

Cardiac surgery for congenital heart disease often necessitates a period of myocardial ischemia during cardiopulmonary bypass and cardioplegic arrest, followed by reperfusion after aortic cross-clamp removal. In experimental models, myocardial ischemia-reperfusion is associated with significant oxidative stress and ventricular dysfunction. A prospective observational study was conducted in infants (<1 year) who underwent elective surgical repair of a ventricular septal defect (VSD) or tetralogy of Fallot (TOF). Blood samples were drawn following anesthetic induction (baseline) and directly from the coronary sinus at 1, 3, 5, and 10 min following aortic cross-clamp removal. Samples were analyzed for oxidant stress using assays for thiobarbituric acid-reactive substances, protein carbonyl, 8-isoprostane, and total antioxidant capacity. For each subject, raw assay data were normalized to individual baseline samples and expressed as fold-change from baseline. Results were compared using a one-sample t test with Bonferroni correction for multiple comparisons. Sixteen patients (ten with TOF and six with VSD) were enrolled in the study, and there were no major postoperative complications observed. For the entire cohort, there was an immediate, rapid increase in myocardial oxidative stress that was sustained for 10 min following aortic cross-clamp removal in all biomarker assays (all P < 0.01), except total antioxidant capacity. Infant cardiac surgery is associated with a rapid, robust, and time-dependent increase in myocardial oxidant stress as measured from the coronary sinus in vivo. Future studies with larger enrollment are necessary to assess any association between myocardial oxidative stress and early postoperative outcomes.

Transhepatic Central Venous Catheters in Pediatric Patients With Congenital Heart Disease.

OBJECTIVES: Patients with congenital heart disease may have limited venous access routes as a result of multiple central venous catheters, surgical interventions, and catheterization procedures. Unconventional venous access includes transhepatic central venous catheter. We evaluated transhepatic central venous catheter placed in patients with congenital heart disease and risk factors associated with complications and outcomes. DESIGN: Demographic, procedural, and complication data were retrospectively collected on all patients who underwent transhepatic central venous catheter placement at our center over the past 10 years. SETTING: This study was completed in a tertiary congenital heart center. PATIENTS: A total of 92 transhepatic central venous catheters were placed in 54 patients (63% male patients). The median age and weight of the patient population was 5.7 months and 5.5 kg, respectively. INTERVENTIONS: Placement of a transhepatic central venous catheter. MEASUREMENTS AND MAIN RESULTS: Successful catheter placement occurred in 96% of cases with median procedure time of 54 minutes with a procedural complication rate of 14%. A total of 86 complications occurred in 54 catheters placed during 2,166 catheter-days (39.7 complications per 1,000 catheter-days). Individual complication rates per 1,000 catheter-days included catheter dysfunction (14.8), dislodgement (8.8), systemic infection (5.1), thrombosis (4.2), local infection (3.7), and bleeding (3.2). Two complications contributed to patient deaths. Factors associated with developing complications included polyurethane central venous catheters (p = 0.03) and catheter duration at least 21 days (p = 0.004). The overall mortality in this population was 50% with median length of hospitalization of 49 days (interquartile range, 33-97). CONCLUSIONS: Transhepatic central venous catheters represent a viable option for patients with limited access. Polyurethane catheters and catheter duration at least 21 days are associated with increased transhepatic central venous catheter complications. Although complication rates are higher than more traditional forms of central venous catheters, the long duration of hospitalization and high mortality rates in this patient population attest to their risks for poor outcomes irrespective of venous access.

Left atrial deformation predicts pulmonary capillary wedge pressure in pediatric heart transplant recipients.

BACKGROUND: Pulmonary capillary wedge pressure (PCWP) is an important indicator in pediatric heart transplant patients, but commonly used noninvasive surrogates, such as ratio of early diastolic mitral inflow velocity to annular velocity (E/E'), have limitations in this population. This study aimed to evaluate the relation of left atrial (LA) peak systolic strain and distensibility with PCWP in pediatric heart transplant recipients. METHODS: Consecutive pediatric heart transplant patients were enrolled at time of cardiac catheterization, with echocardiogram immediately afterward. E/E' ratio at the lateral and medial mitral annulus, peak LA systolic longitudinal strain by speckle tracking, and LA distensibility were measured from echocardiograms and compared to invasively measured PCWP. RESULTS: In 38 patients (11.1 ± 5.8 years old), PCWP correlated with peak LA systolic strain (r = -0.44, P = 0.01) and LA distensibility (r= -0.43, P = 0.02), but not with E/E'. On receiver operating characteristics analysis, LA strain had a higher area under the curve than LA distensibility (0.846 vs. 0.606). LA strain <18.9% had sensitivity 62% and specificity 95%, with likelihood ratio 12.3 for PCWP ≥12. However, LA strain had lower intra-observer and inter-observer reproducibility than distensibility (intra-class correlation coefficients 0.89 and 0.75 vs. 0.93 and 0.90). CONCLUSIONS: Peak LA systolic strain and LA distensibility may be more useful surrogates of left ventricular filling pressure than E/E' in the pediatric heart transplant population, with greater reproducibility of LA distensibility. Longitudinal studies are needed to evaluate which parameters track changes in PCWP and clinical outcome.

Intervention for recoarctation in the single ventricle reconstruction trial: incidence, risk, and outcomes.

BACKGROUND: Recoarctation after the Norwood procedure increases risk for mortality. The Single Ventricle Reconstruction (SVR) trial randomized subjects with a single right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt or a right ventricle-pulmonary artery shunt. We sought to determine the incidence of recoarctation, risk factors, and outcomes in the SVR trial. METHODS AND RESULTS: Recoarctation was defined by intervention, either catheter based or surgical. Univariate analysis and multivariable Cox proportional hazard models were performed with adjustment for center. Of the 549 SVR subjects, 97 (18%) underwent 131 interventions (92 balloon aortoplasty, 39 surgical) for recoarctation at a median age of 4.9 months (range, 1.1-10.5 months). Intervention typically occurred at pre-stage II catheterization (n=71, 54%) or at stage II surgery (n=38, 29%). In multivariable analysis, recoarctation was associated with the shunt type in place at the end of the Norwood procedure (hazard ratio, 2.0 for right ventricle-pulmonary artery shunt versus modified Blalock-Taussig shunt; P=0.02), and Norwood discharge peak echo-Doppler arch gradient (hazard ratio, 1.07 per 1 mm Hg; P<0.01). Subjects with recoarctation demonstrated comorbidities at pre-stage II evaluation, including higher pulmonary arterial pressures (15.4±3.0 versus 14.5±3.5 mm Hg; P=0.05), higher pulmonary vascular resistance (2.6±1.6 versus 2.0±1.0 Wood units·m(2); P=0.04), and increased echocardiographic volumes (end-diastolic volume, 126±39 versus 112±33 mL/BSA(1.3), where BSA is body surface area; P=0.02). There was no difference in 12-month postrandomization transplantation-free survival between those with and without recoarctation (P=0.14). CONCLUSIONS: Recoarctation is common after Norwood and contributes to pre-stage II comorbidities. Although with intervention there is no associated increase in 1-year transplantation/mortality, further evaluation is warranted to evaluate the effects of associated morbidities.

Parental perspectives on suffering and quality of life at end-of-life in children with advanced heart disease: an exploratory study*.

OBJECTIVE: To describe parent perspectives regarding the end-of-life experience of children with advanced heart disease. DESIGN: Cross-sectional multicenter survey study of bereaved parents. SETTING: Two tertiary care pediatric hospitals. SUBJECTS: Parents of children younger than 21 years with primary cardiac diagnoses who died in the hospital 9 months to 4 years before the survey date. Parents were excluded if they were non-English speakers or had previously denied permission to contact. INTERVENTION: The Survey for Caring for Children with Advanced Heart Disease was developed, piloted, and then sent to parents of all children who died at two sites. MEASUREMENTS AND MAIN RESULTS: Fifty bereaved parents responded (39% response rate) a mean of 2.7 years after their child's death. Median age at death was 6 months (3.6 d to 20.4 yr). At end-of-life, 86% of children were intubated and 46% were receiving mechanical circulatory support. Seventy-eight percent died during withdrawal of life-sustaining interventions and 16% during resuscitative efforts. Parents realized that their child had no realistic chance of survival a median of 2 days prior to death (0-30 d). According to parents, 47% of children suffered "a great deal," "a lot," or "somewhat" during the end-of-life period. The symptoms parents perceived to be causing the most suffering were breathing and feeding difficulties in children under 2 years and fatigue and sleeping difficulties in older children. Seventy-one percent of parents described the quality of life of their child during the last month of life as "poor" or "fair." Most parents (84%) described the quality of care delivered as "very good" or "excellent." CONCLUSIONS: According to their parents, many children with advanced heart disease experience suffering in the end-of-life care period. For most, realization that their child has no realistic chance of survival does not occur until late, some not until death is imminent. Once this realization occurs, however, parents perceive peacefulness, a "good death," and excellent quality of care. Strategies for improved communication around symptom management, quality of life, prognosis, and advance care planning are needed for families of children with advanced heart disease.

An apparent case of undiagnosed donor Kawasaki disease manifesting as coronary artery aneurysm in a pediatric heart transplant recipient.

We present a case of coronary ectasia and LAD coronary artery aneurysm with angiographic characteristics of Kawasaki disease in a three-yr-old girl two-yr status post-orthotopic heart transplant. Coronary anomalies were noted during initial screening coronary angiography two yr after transplant. Subsequent review of the donor echocardiogram revealed that the LMCA had been mildly dilated prior to transplant. In the absence of any symptoms consistent with Kawasaki disease in the transplant recipient, this appears to be a case of Kawasaki disease in the organ donor manifesting with coronary anomalies in the transplant recipient. The patient has done well clinically, and repeat coronary angiography has revealed partial regression of coronary anomalies. Given multiple reports in the literature of persistent abnormalities of coronary artery morphology and function after Kawasaki disease, close monitoring is warranted, with consideration of potential coronary protective medical therapies.

Central venous catheter-associated complications in infants with single ventricle: comparison of umbilical and femoral venous access routes.

OBJECTIVE: Among infants with single-ventricle heart disease who require surgical palliation, central venous access is routinely obtained via the umbilical or femoral veins. Both routes are associated with potential complications, including thrombosis. We sought to analyze the clinical outcomes of patients with umbilical venous catheter vs. femoral central venous catheter placement at the time of initial central venous access in this high-risk patient population. DESIGN: This was a retrospective study, with data collected including demographics, catheter type, duration, complications, and clinical outcomes. Patients were designated as group 1 (initial umbilical venous catheter placed, n = 70) or group 2 (initial femoral central venous catheter placed, n = 19). SETTING: The study was conducted at a single tertiary care referral institution. PATIENTS: We included all 89 patients who underwent single-ventricle palliation at this institution in 2007 and 2008. MEASUREMENTS AND MAIN RESULTS: The overall rates of survival to hospital discharge, thrombosis, and iliofemoral vein occlusion were 82%, 18%, and 21%, respectively. The proportion of thrombosis was 11% in group 1, compared with 42% in group 2 (p < .01). The proportion of iliofemoral vein occlusion was 16% in group 1, compared with 42% in group 2 (p = .02). The proportions of catheter-associated bloodstream infection, need for transhepatic access, and ultrasound-documented thrombus at the inferior vena caval-right atrial junction did not differ significantly between the groups. Patients with non-tunneled femoral central venous catheters for ≥14 days had a higher prevalence of thrombosis (52%) than those with femoral central venous catheters for <14 days (13%) but no difference in the prevalence of iliofemoral vein occlusion. CONCLUSIONS: In this population, initial placement of an umbilical venous catheter rather than a femoral venous catheter resulted in significantly lower risks of catheter thrombosis and iliofemoral vein occlusion. For femoral venous catheters, the prevalence of thrombosis, but not of iliofemoral vein occlusion, is proportional to the duration of catheterization.

Extensive distortion and dysfunction of an Edwards Valeo Lifestent placed for stenosis of the superior vena cava.

We present a case of multiple interventions to treat symptomatic stenosis of the superior vena cava in a child, with end-stage renal disease and a history of long-term hemodialysis, who had required indwelling central venous catheters for several years. The stenosis was successfully treated with an Edwards Valeo Lifestent, but recurrent symptomatic superior vena caval stenosis developed within 5 months, requiring repeat catheterization and stent placement.

The Michigan Appropriateness Guide for Intravenous Catheters in Pediatrics: miniMAGIC.

OBJECTIVES: Vascular access device decision-making for pediatric patients remains a complex, highly variable process. To date, evidence-based criteria to inform these choices do not exist. The objective of the Michigan Appropriateness Guide for Intravenous Catheters in pediatrics (miniMAGIC) was to provide guidance on device selection, device characteristics, and insertion technique for clinicians, balancing and contextualizing evidence with current practice through a multidisciplinary panel of experts. METHODS: The RAND Corporation and University of California, Los Angeles Appropriateness Method was used to develop miniMAGIC, which included the following sequential phases: definition of scope and key terms, information synthesis and literature review, expert multidisciplinary panel selection and engagement, case scenario development, and appropriateness ratings by an expert panel via 2 rounds. RESULTS: The appropriateness of the selection, characteristics, and insertion technique of intravenous catheters commonly used in pediatric health care across age populations (neonates, infants, children, and adolescents), settings, diagnoses, clinical indications, insertion locations, and vessel visualization devices and techniques was defined. Core concepts including vessel preservation, insertion and postinsertion harm minimization (eg, infection, thrombosis), undisrupted treatment provision, and inclusion of patient preferences were emphasized. CONCLUSIONS: In this study, we provide evidence-based criteria for intravenous catheter selection (from umbilical catheters to totally implanted venous devices) in pediatric patients across a range of clinical indications. miniMAGIC also highlights core vascular access practices in need of collaborative research and innovation.

Assessment of Employee Susceptibility to Phishing Attacks at US Health Care Institutions.

Importance: Cybersecurity is an increasingly important threat to health care delivery, and email phishing is a major attack vector against hospital employees. Objective: To describe the practice of phishing simulation and the extent to which health care employees are vulnerable to phishing simulations. Design, Setting, and Participants: Retrospective, multicenter quality improvement study of a convenience sample of 6 geographically dispersed US health care institutions that ran phishing simulations from August 1, 2011, through April 10, 2018. The specific institutions are anonymized herein for security and privacy concerns. Exposures: Simulated phishing emails received by employees at US health care institutions. Main Outcomes and Measures: Date of phishing campaign, campaign number, number of emails sent, number of emails clicked, and email content. Emails were classified into 3 categories (office related, personal, or information technology related). Results: The final study sample included 6 anonymized US health care institutions, 95 simulated phishing campaigns, and 2 971 945 emails, 422 062 of which were clicked (14.2%). The median institutional click rates for campaigns ranged from 7.4% (interquartile range [IQR], 5.8%-9.6%) to 30.7% (IQR, 25.2%-34.4%), with an overall median click rate of 16.7% (IQR, 8.3%-24.2%) across all campaigns and institutions. In the regression model, repeated phishing campaigns were associated with decreased odds of clicking on a subsequent phishing email (adjusted OR, 0.511; 95% CI, 0.382-0.685 for 6-10 campaigns; adjusted OR, 0.335; 95% CI, 0.282-0.398 for >10 campaigns). Conclusions and Relevance: Among a sample of US health care institutions that sent phishing simulations, almost 1 in 7 simulated emails sent were clicked on by employees. Increasing campaigns were associated with decreased odds of clicking on a phishing email, suggesting a potential benefit of phishing simulation and awareness. With cyberattacks increasing against US health care systems, these click rates represent a major cybersecurity risk for hospitals.

Adherence to recommended electronic health record safety practices across eight health care organizations.

Objective: The Safety Assurance Factors for EHR Resilience (SAFER) guides were released in 2014 to help health systems conduct proactive risk assessment of electronic health record (EHR)- safety related policies, processes, procedures, and configurations. The extent to which SAFER recommendations are followed is unknown. Methods: We conducted risk assessments of 8 organizations of varying size, complexity, EHR, and EHR adoption maturity. Each organization self-assessed adherence to all 140 unique SAFER recommendations contained within 9 guides (range 10-29 recommendations per guide). In each guide, recommendations were organized into 3 broad domains: "safe health IT" (total 45 recommendations); "using health IT safely" (total 80 recommendations); and "monitoring health IT" (total 15 recommendations). Results: The 8 sites fully implemented 25 of 140 (18%) SAFER recommendations. Mean number of "fully implemented" recommendations per guide ranged from 94% (System Interfaces-18 recommendations) to 63% (Clinical Communication-12 recommendations). Adherence was higher for "safe health IT" domain (82.1%) vs "using health IT safely" (72.5%) and "monitoring health IT" (67.3%). Conclusions: Despite availability of recommendations on how to improve use of EHRs, most recommendations were not fully implemented. New national policy initiatives are needed to stimulate implementation of these best practices.

Insulin Bolus Calculator in a Pediatric Hospital. Safety and User Perceptions.

BACKGROUND: Insulin dosing in hospitalized pediatric patients is challenging and requires dosing to be matched with the specific clinical and nutritional circumstances. We implemented a customized subcutaneous insulin bolus dose calculator tool integrated with the electronic health record to improve patient care. Here we describe this tool, its utilization and safety, and assess user satisfaction and perceptions of the tool. METHODS: Blood glucose results for all patients who received insulin with and without the calculator tool were compared to assess safety. To assess user perceptions and satisfaction, a survey was sent to all identified users who interacted with the tool during the period from May 2015 to the end of November 2015. Survey responses were summarized, mean user satisfaction calculated, and correlation of Likert scale items with overall satisfaction assessed. RESULTS: Hypoglycemia rates (2.2% and 2.9%, p = 0.17) and severe hypoglycemia rates (0.04% and 0.1%, p = 0.21) were similar for the groups that received insulin with and without the calculator tool. Overall satisfaction for all survey respondents was high (4.05, SD = 0.83). Physicians indicated a slightly higher satisfaction than nurses (4.33 versus 3.94, p = 0.04). User agreement with improvement of quality of care showed the highest correlation with overall satisfaction (r = 0.80, 95% CI 0.7 - 0.87). CONCLUSION: Implementation of an insulin calculator tool streamlined ordering and administration of insulin in a pediatric academic institution while maintaining patient safety. Users indicated high overall satisfaction with the tool.

Functional Status and Quality of Life in Survivors of Extracorporeal Membrane Oxygenation After the Norwood Operation.

BACKGROUND: Infants who require extracorporeal membrane oxygenation (ECMO) support after a Norwood operation are at increased risk for early and late death compared with patients who do not require ECMO post-Norwood. Little is known about the effect that ECMO post-Norwood has on functional status and quality of life among long-term survivors. METHODS: We prospectively evaluated functional status and health-related quality of life in 12 surviving patients (cases) and 19 corresponding patients (controls) from a previous retrospective case-control assessment of long-term survival in patients requiring ECMO post-Norwood. Functional status was assessed with the Vineland Adaptive Behavior Scale-II, and health-related quality of life was assessed with the Pediatric Quality of Life Inventory (PedsQL) core and cardiac modules. RESULTS: There were no differences in demographics, extracardiac or genetic anomalies, or age at follow-up assessment between ECMO cases and non-ECMO controls. The Vineland Adaptive Behavior Scale-II scores were comparable between groups, with both groups demonstrating function in the normal range in all four domains tested. The only difference in PedsQL scores between cases and controls was perceived physical appearance, which was lower among ECMO survivors by both patient and proxy report. PedsQL scores of both groups were comparable to published scores for patients with single-ventricle congenital heart disease but generally lower than scores for the healthy population. CONCLUSIONS: The requirement for ECMO support after a Norwood operation does not appear to significantly affect functional status or quality of life among the subset of patients who achieve long-term survival.

Usefulness of Diastolic Strain Measurements in Predicting Elevated Left Ventricular Filling Pressure and Risk of Rejection or Coronary Artery Vasculopathy in Pediatric Heart Transplant Recipients.

In pediatric heart transplant recipients, elevated pulmonary capillary wedge pressure (PCWP) is associated with rejection and coronary artery vasculopathy. This study aimed to evaluate which echocardiographic parameters track changes in PCWP and predict adverse outcomes (rejection or coronary artery vasculopathy). This prospective single-center study enrolled 49 patients (median 11.4 years old, interquartile range 7.4 to 16.5) at time of cardiac catheterization and echocardiography. Median follow-up was 2.4 years (range 1.2 to 3.1 years), with serial testing per clinical protocol. Ratio of early mitral inflow to annular velocity (E/E'), left atrial (LA) distensibility, peak LA systolic strain, E/left ventricular (LV) diastolic strain, and E/LV diastolic strain rate were measured from echocardiograms. Increase in PCWP ≥3 mm Hg was associated with changes in LA distensibility, E/E', and E/LV diastolic strain, with highest area under the receiver operating characteristic curve for E/LV diastolic strain (0.76). In 9 patients who subsequently developed rejection or coronary artery vasculopathy, E/LV diastolic strain rate at baseline differed from patients without events (median 57.0 vs 43.6, p = 0.02). On serial studies, only change in LV ejection fraction differed in patients with events (median -10% vs -1%, p = 0.01); decrease in LV ejection fraction of -19% had a specificity of 100% and sensitivity of 44%. In conclusion, LV diastolic strain and strain rate measurements can track changes in PCWP and identify patients at risk for subsequent rejection or coronary artery vasculopathy. Further studies are necessary to confirm these data in a larger cohort.