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Objective: We sought to determine the relationship between relative value units (RVUs) and intended measures of work in catheterization for congenital heart disease., Methods: RVU was determined by matching RVU values to Current Procedural Terminology codes generated for cases performed at a single institution. Differences in median case duration, radiation exposure, adverse events, and RVU values by risk category and cases were assessed. Interventional case types were ranked from lowest to highest median RVU value, and correlations with case duration, radiation dose, and a cases-predicted probability of an adverse event were quantified with the Spearman rank correlation coefficient., Results: Between January 2008 and December 2010, 3557 of 4011 cases were identified with an RVU and risk category designation, of which 2982 were assigned a case type. Median RVU values, radiation dose, and case duration increased with procedure risk category. Although all diagnostic cases had similar RVU values (median 10), adverse event rates ranged from 6% to 21% by age group (P < .001). Median RVU values ranged from 9 to 54 with the lowest in diagnostic and biopsy cases and increasing with isolated and then multiple interventions. Among interventional cases, no correlation existed between ranked RVU value and case duration, radiation dose, or adverse event probability (P = .13, P = .62, and P = .43, respectively)., Conclusions: Time, skill, and stress inherent to performing catheterization procedures for congenital heart disease are not captured by measurement of RVU alone.

Objective: We sought to develop a method to adjust for case mix diversity and allow comparison of adverse outcome rates among practitioners in pediatric and congenital cardiac catheterization., Patients and Methods: A single institutional database that captured demographic and procedural data was used to identify patient and procedural characteristics associated with adverse events (AE) and any high severity event classified as preventable or possibly preventable (P/PP). Diagnostic and procedural risk groups were created and indicators of hemodynamic vulnerability were defined. Expected event rates by the practitioners adjusting for case mix were calculated. Standardized adverse event ratios (SAER), defined as the observed rate divided by the expected rate for each practitioner were calculated with 95% confidence intervals., Results: The database included 1727 hemodynamic (30%) and interventional (70%) cases performed by seven practitioners in 18 months. During 147 cases, at least one P/PP AE occurred; among the seven practitioners observed, event rates ranged from 3.2 to 14.2%. In multivariable analysis, risk factors for all P/PP events included highest procedure risk group (odds ratio [OR] 2.1 for group 2, and 2.8 for group 3, relative to group 1, P = .001 and P < .001, respectively) and weight less than 4 kg (OR 2.8, P < .001). High severity P/PP events occurred in 67 cases with rates ranging from 2.0 to 6.6% by the practitioners. For these events, risk factors included: highest procedure risk group (OR 4.5 for group 2, and 4.9 for group 3, both P < .001) and an indicator of hemodynamic vulnerability (OR 1.8, P = .026). For the seven practitioners, the SAER ranged from 0.41 to 1.32 for any P/PP AE and from 0.69 to 1.44 for P/PP high severity events. In this cohort, we did not identify any statistically significant performance differences., Conclusion: Despite wide variations in case mix complexity in pediatric and congenital cardiac catheterization, this study demonstrates a method for risk adjustment which allows equitable comparisons among practitioners at a single institution.

Background Advancements in the field, including novel procedures and multiple interventions, require an updated approach to accurately assess patient risk. This study aims to modernize patient hemodynamic and procedural risk classification through the creation of risk assessment tools to be used in congenital cardiac catheterization. Methods and Results Data were collected for all cases performed at sites participating in the C3PO (Congenital Cardiac Catheterization Project on Outcomes) multicenter registry. Between January 2014 and December 2017, 23 119 cases were recorded in 13 participating institutions, of which 88% of patients were

BackgroundCoronary artery aneurysms (CAA) are a serious complication of Kawasaki disease. Treatment with intravenous immunoglobulin (IVIg) within 10 days of fever onset reduces the risk of CAA from 25% to

ImportanceAmerican Heart Association guidelines recommend echocardiography in Kawasaki disease at baseline, 1 to 2 weeks, and 4 to 6 weeks after treatment to detect coronary artery abnormalities. However, these examinations are expensive and may require sedation in young children, which is burdensome and carries some risk.ObjectiveTo assess the benefit of additional echocardiographic imaging at 6 weeks in patients with uncomplicated Kawasaki disease who had previously normal coronary arteries.Design, setting, and participantsThis is a retrospective review of patients with Kawasaki disease who were cared for between 1995 and 2014 in 2 academic pediatric referral practices Eligibility criteria included receiving intravenous immunoglobulin treatment for acute Kawasaki disease at a center; the absence of significant congenital heart disease; available echocardiographic measurements of both the right and left anterior descending coronary arteries at 10 days or less after diagnosis (baseline), 2 (±1) weeks, and 6 (±3) weeks of illness; and normal coronary arteries at baseline and 2 weeks, defined as maximum coronary artery z scores less than 2.0 and no distal aneurysms. Data analysis was completed from March 2015 to November 2015.Main outcomes and measuresThe number of patients with right coronary artery or left anterior descending coronary artery z scores of 2.0 or more at 6 weeks.ResultsThe median age of the 464 included patients was 3.3 years (interquartile range, 1.8-5.4 years); 264 (56.9%) were male, 351 of 414 for whom data were available (84.8%) had complete Kawasaki disease, and 66 (14.2%) received additional intravenous immunoglobulin treatment. At 6 weeks of illness, 456 patients (98.3%) who had had normal coronary artery z scores at baseline and 2 weeks continued to have normal z scores. Of the remaining 8 patients (1.7%), the maximum z score within 6 weeks was 2.0 to 2.4 in 5 patients (1.2%), 2.5 to 2.9 in 1 patient (0.2%), and 3.0 or more in 2 patients (0.4% [95% CI, 0.1%-1.5%]). Coronary artery dimensions ultimately normalized in all but 1 patient, who had minimal dilation at 6 weeks (right coronary artery z score, 2.1). Sensitivity analyses using less restrictive cut points (eg, a maximum z score

BackgroundThe National Quality Forum previously approved a quality indicator for mortality after congenital heart surgery developed by the Agency for Healthcare Research and Quality (AHRQ). Several parameters of the validated Risk Adjustment for Congenital Heart Surgery (RACHS-1) method were included, but others differed. As part of the National Quality Forum endorsement maintenance process, developers were asked to harmonize the 2 methodologies.Methods and resultsParameters that were identical between the 2 methods were retained. AHRQ's Healthcare Cost and Utilization Project State Inpatient Databases (SID) 2008 were used to select optimal parameters where differences existed, with a goal to maximize model performance and face validity. Inclusion criteria were not changed and included all discharges for patients

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. [ABSTRACT FROM AUTHOR]

BACKGROUND: Current metrics used to adjust for case mix complexity in congenital cardiac catheterization are becoming outdated due to the introduction of novel procedures, innovative technologies, and expanding patient subgroups. This study aims to develop a risk adjustment methodology introducing a novel, clinically meaningful adverse event outcome and incorporating a modern understanding of risk. METHODS: Data from diagnostic only and interventional cases with defined case types were collected for patients =18 years of age and =2.5 kg at all Congenital Cardiac Catheterization Project on Outcomes participating centers. The derivation data set consisted of cases performed from 2014 to 2017, and the validation data set consisted of cases performed from 2019 to 2020. Severity level 3 adverse events were stratified into 3 tiers by clinical impact (3a/b/c); the study outcome was clinically meaningful adverse events, severity level =3b (3bc/4/5). RESULTS: The derivation data set contained 15 224 cases, and the validation data set included 9462 cases. Clinically meaningful adverse event rates were 4.5% and 4.2% in the derivation and validation cohorts, respectively. The final risk adjustment model included age <30 days, Procedural Risk in Congenital Cardiac Catheterization risk category, and hemodynamic vulnerability score (C statistic, 0.70; Hosmer-Lemeshow P value, 0.83; Brier score, 0.042). CONCLUSIONS: CHARM II (Congenital Heart Disease Adjustment for Risk Method II) risk adjustment methodology allows for equitable comparison of clinically meaningful adverse events among institutions and operators with varying patient populations and case mix complexity performing pediatric cardiac catheterization. [ABSTRACT FROM AUTHOR]

The authors comment on a study by Y. Kuniyoshi and colleagues which presented the results of their meta-analysis assessing the performance of clinical prediction models for intravenous immunoglobulin (IVIG) resistance. Topics discussed include purpose of clinical prediction models, evidence of the advantages of using prediction model to select a high-risk study population, and recommendation to have echocardiography at least twice weekly until coronary dimensions stabilize.

Background: Laboratory testing is frequently used to guide postoperative management and contributes to hospital resource utilization; however, there is little evidence identifying patient or clinical factors to inform the appropriate frequency of laboratory testing in the pediatric cardiac intensive care unit. Objectives: To examine the factors associated with increased laboratory utilization following pediatric congenital heart surgery. Methods: For each patient, the total number of tests and types of laboratory tests were recorded. Patients whose number of tests was greater than the 90th percentile were categorized as increased laboratory use. Results: A sample of 250 unique patients and 909 nursing shifts were obtained for patient- and shift-level analyses. The top 10%of patients identified as the high laboratory utilization group (>128 laboratory tests). High-use group reported significantly younger patients and longer bypass time (P < .001). Patients in the highest Risk Adjustment for Congenital Heart Surgery 1 risk category were 34.7 times more likely to be in high laboratory utilization group (P = .006), independent of age at time of surgery and time on bypass (receiver operating characteristic curve = 0.855). At the shift level, time on bypass (P = .002), age younger than 30 days at surgery (P < .001), 3 to 5 years' registered nurse experience (P < .001), staff precepting (P = .03), and weekday shift status (P = .03) were all independently associated with high laboratory utilization. Conclusions: There are multiple factors associated with increased laboratory utilization. Recognition of specific patient and nursing factors can be used to impact patient management [ABSTRACT FROM AUTHOR]

Objective: To determine the impact of provocative electrophysiology testing in postoperative congenital heart disease (CHD) patients on the management of supraventricular tachycardia (SVT) and clinical outcomes. Methods: This is a retrospective study including patients <18 years of age with surgery for CHD who had postoperative SVT between 2006 and 2017. Postoperative outcomes were compared between patients with and without postoperative electrophysiology testing using the Wilcoxon rank sum test, Fisher's exact test, Kaplan‐Meier method with the log‐rank test, and Cox proportional hazard model. Results: From 341 patients who had SVT after surgery for CHD, 65 (19%) had postoperative electrophysiology testing. There was no significant difference in baseline patient characteristics or surgical complexity between patients with and without electrophysiology testing. Patients with inducible SVT on electrophysiology testing were more likely to have recurrence of SVT prior to hospital discharge with an odds ratio 4.0 (95% confidence interval 1.3, 12.0). Patients who underwent postoperative electrophysiology testing had shorter intensive care unit (12 [6, 20] vs 16 [9, 32] days, HR 2.1 [95% CI 1.6, 2.8], P <.001) and hospital (25 [13, 38] vs 31 [18, 54] days, HR 1.8 [95% CI 1.4, 2.4], P <.001) length of stay. Conclusion: Postoperative electrophysiology testing was associated with improved postoperative outcomes, likely related to the ability to predict recurrence of arrhythmia and tailored antiarrhythmic management. [ABSTRACT FROM AUTHOR]

TDI is considered superior to conventional echocardiography for detecting changes in graft function during rejection in adults but has not demonstrated after pediatric OHT. We retrospectively analyzed echocardiograms performed within 24 hours of biopsy in 122 recipients with median age of 8.7 years. Using biopsy findings as the gold standard, we compared paired rejection and non‐rejection echocardiograms using each patient as their own control. We included pairs of LV dimensions, FS, volumes, mass, mass/volume, sphericity, wall stress, SSI, SVI, and TDI velocities in this comparison. C‐statistic was used to assess discrimination for individual echo variables and combinations of variables. Overall, 647 non‐rejection and 24 rejection biopsy‐echo pairs were identified. There was a significant decline in TDI velocities and their Z‐scores during rejection but not in conventional variables (P ≤.005). The variable that best discriminated rejection from non‐rejection was LV S′, with C‐statistic = 0.93. Conventional echo variables performed less well with C‐statistic range 0.65‐0.67 for LV EF, shortening fraction, and mass. TDI is superior to conventional echocardiography measures for discriminating rejection from non‐rejection. The use of newer non‐invasive parameters to detect myocardial dysfunction and shifting the paradigm of rejection surveillance to detection of non‐rejection together provide a promising approach to reducing the need for biopsy in pediatric heart recipients. [ABSTRACT FROM AUTHOR]

Background Mitral valve replacement (MVR) in very young children is challenging. This study investigates the mechanisms for early bioprosthetic valve failure in very young patients through review of the macroscopic and microscopic findings in explanted bioprosthetic valves. Methods Patients who underwent MVR with a bioprosthetic valve at Boston Children's Hospital between January 2010 to April 2016 at <5 years of age were the subjects of this study. Valve failure was defined as prosthetic mitral valve explantation with mitral valve re-replacement. Results Bioprosthetic valves were used in 31 of 77 MVRs during the study period. Valve failure occurred in 10 patients (32%). Freedom from valve failure was 80% at 1 year and was associated with older age at implantation. On gross and microscopic evaluation, valve failure (predominantly stenosis) was found to be due to pannus deposition and intrinsic leaflet calcification. Conclusions Successful long-term use of bioprosthetic valves in the mitral position in very young children continues to be a challenge. In addition to intrinsic calcification, excessive pannus deposition can lead to early bioprosthetic valve failure in this population. Early exuberant pannus growth appears due to thrombus deposition on the valves themselves and to the host's reaction to foreign material. [ABSTRACT FROM AUTHOR]

The sickest children among those listed for heart transplant (HT) are also at higher risk of post-transplant mortality. We hypothesized that transplant benefit, defined as percentage reduction in risk of 1-year mortality on receiving HT, increases with higher risk of wait-list mortality.We analyzed all children aged <18 years listed for first HT in the United States between July 2004 and December 2010. We developed a model for 90-day wait-list mortality (or removal because of deterioration) and stratified listed children into deciles based on their risk of wait-list mortality. Listed children were followed up for 1 year to assess cumulative 1-year wait-list mortality among the 10 risk groups. We developed a model for 1-year post-transplant mortality to estimate the risk of post-transplant mortality for children in each risk group. Of 2979 listed children, 15% reached the wait-list end point within 90 days and 18% within 1 year. Of 2034 HT recipients, 6.8% died within 90 days and 10.8% within 1 year. The risk of 90-day wait-list mortality increased progressively from 2.4% to 51.6% from the first to the tenth risk decile. Transplant benefit increased progressively across the wait-list risk groups (P<0.001 for trend). However, transplant benefit for children in the top 5% of risk (7.4%) was lower than that for children in the 91st to 95th percentile of risk (10.3%).Sicker children on the wait-list benefit more from HT unless the post-transplant mortality is predicted to be very high. Whether consideration of transplant benefit in allocation policy can improve overall survival among listed children requires further analysis. [ABSTRACT FROM AUTHOR]

Maximum oxygen consumption (peak VO2) <50% predicted on exercise testing is a class I indication for heart transplant (HT) listing in children. This recommendation is based on exercise data in adults. We assessed the association of peak VO2<50% predicted during HT evaluation with freedom from death or deterioration in children.We analyzed all children who underwent exercise testing during HT evaluation at our center between 2002 and 2011. Patients with peak VO2<50% predicted were compared with those with peak VO2≥50% predicted for the composite outcome of death before HT, initiation of mechanical circulatory support, and HT at highest urgency status, using time-to-event analyses. There were 50 children in the study (median age, 15 years; interquartile range, 13-17 years; 24 girls; 18 with palliated single ventricle). Overall, 24 children reached the composite end point. Peak VO2<50% predicted was associated with outcome in children with biventricular circulation (hazard ratio, 4.7; 95% confidence interval, 1.8-12.3; P<0.001) but not in those with a palliated single ventricle (hazard ratio, 1.3; 95% confidence interval, 0.1-12.0; P=0.80). Similarly, VE/VCO2 slope ≥34 was associated with outcome in children with biventricular circulation (hazard ratio, 2.7; 95% confidence interval, 1.1-7.1; P<0.001) but not in children with a palliated single ventricle.Exercise testing during HT evaluation in children with biventricular circulation identified those at higher risk of death or deterioration in this small study. Larger studies are needed to assess the role of exercise testing during HT evaluation in children with a palliated single ventricle. [ABSTRACT FROM AUTHOR]

Coronary artery (CA) dilatation on echocardiography is a criterion for treatment with intravenous immunoglobulin for incomplete Kawasaki disease (KD). However, CA dimensions for febrile children are unknown. We compared CA dimensions in children with febrile illnesses other than KD to those of normal afebrile children and to KD patients.We performed echocardiograms in 43 patients who met the following inclusion criteria: (1) age 3 months to 18 years, (2) daily fever >38°C for ≥96 hours, and (3) a diagnosis other than KD. These subjects had mean CA z scores greater than normative values (left main CA=0.66±0.75, P<0.001; right CA=0.28±0.81, P=0.03; left anterior descending CA=0.35±1.0, P=0.03). Maximum CA z score >2 was found in 2 subjects (osteomyelitis, Mycoplasma pneumonia). Among demographic and laboratory measures, only higher platelet count was associated with greater left anterior descending CA z scores (P=0.004) and maximum CA z score (P=0.03). Non-KD febrile subjects, compared with 144 KD patients, had smaller CA z scores (P=0.04, P<0.001, and P<0.001 for left main CA, right CA, and left anterior descending CA, respectively), and lower white blood cell count, erythrocyte sedimentation rate, and platelet count (all P<0.001). A maximum CA z score cutoff of 2.0 had specificity of 95% (95% confidence interval, 84%-99%) and sensitivity of 32% (95% confidence interval, 25%-41%) in distinguishing non-KD febrile from KD patients; for maximum CA z score of 2.5, specificity was 98% and sensitivity was 20%.This pilot study found that mean CA dimensions in children with non-KD febrile illnesses are larger than those in normative afebrile subjects but smaller than dimensions in patients with KD. Future studies should augment the available data on CA dimensions in children with more severe febrile illnesses. [ABSTRACT FROM AUTHOR]

The article presents a study that assesses the recovery of left (LV) and right venticular (RV) function during the first year of heart transplant (HT) in children and young adults using pulsed-wave tissue Doppler imaging. It shows that RV systolic function remained impaired one-year post transplant. It reveals that pediatric HT recipients have biventricular dysfunction using Doppler imaging early after HT.

Previous studies have reported worse outcomes in children with nondilated cardiomyopathy (CMP) listed for heart transplant compared with children with dilated CMP. We sought to compare wait-list and posttransplant outcomes in these groups in the current era.We analyzed all children <18 years of age with a diagnosis of CMP listed for heart transplant in the United States between July 2004 and December 2010. Multivariable risk factors for death on the wait-list (or becoming too sick to transplant) and posttransplant graft loss (median follow-up 2 years) were assessed using Cox models. Of the 1436 children analyzed, 1197 (83%) had dilated CMP and 239 (17%) had nondilated CMP (167 restrictive CMP, 72 hypertrophic CMP). In adjusted analysis, children with nondilated CMP were at higher risk of wait-list mortality only if they were on a ventilator support at listing (hazard ratio, 2.3; CI, 1.2-4.5). The risk was similar among children not on a ventilator support (hazard ratio, 0.6; CI, 0.3-1.1). Posttransplant 30-day and 1-year survival was 98% and 94%, respectively, in children with dilated CMP versus 95% and 89%, respectively, in children with nondilated CMP (P=0.17, log-rank test). In adjusted analysis, the risk of posttransplant graft loss was higher in nondilated CMP (hazard ratio, 1.8; CI, 1.2-2.7) versus dilated CMP.The increased risk of wait-list mortality in children with nondilated CMP is limited to those on ventilator support at listing. Although the risk of graft loss is modestly higher in children with nondilated forms of CMP, their short-term transplant outcomes are good. [ABSTRACT FROM AUTHOR]

Background Nursing and organizational characteristics influence patients' outcomes. Little is known about nursing structure and process supports in the intensive care units of children's hospitals. Objectives To describe and understand nursing and organizational characteristics of cardiovascular care in children's hospitals. Methods Descriptive analyses were used to identify and describe nursing and unit characteristics of 43 children's hospitals. An electronic survey instrument was designed to elicit data from nurse leaders. Results Nursing and unit parameters varied significantly. Most registered nurses (70%) had baccalaureate degrees and worked full time. Every intensive care unit had nurses with specialty certification. A total of 51% of nurses had 5 or fewer years of clinical experience. Indirect resources also varied. Unit secretaries were employed by 98% of units, and 100% had a nurse manager and/or a director plus an attending physician. A total of 77% of the units had advanced practice nurses. Processes of care included checklists in 100% of the units, standardized communication in 95%, quality metrics in 91%, and nurse-developed algorithms in 44%. Among the units, 56% had Magnet recognition, and 3 were recipients of a Beacon Award. Conclusion Cardiovascular intensive care varies widely across children's hospitals. Opportunities exist for support and retention of an intergenerational nursing workforce. These findings may serve as a contemporary benchmarking source and foundation to standardize models of care, support, and resource utilization in pediatric intensive care units. [ABSTRACT FROM AUTHOR]

This article presents the classifications of procedure-type risk that are associated with cardiac catheterization for pediatric and congenital cases as given in the Congenital Cardiac Catheterization Project on Outcomes (C3PO). It provides how the information was gathered for the study and the subject populations that were used in it, as well as the techniques that were used to check on the data. The article asserts that differing levels of cardiac complication are linked to procedure-type risk categories and that they can be useful in pediatric cases.

The article discusses a study which explores the impact of socioeconomic (SE) position on health-related behavior. The study found the association between SE position and graft failure in patients who undergone pediatric heart transplant. The study also concluded that patients in the low tertile SE position and nonwhite race are more at risk of developing graft failure and had higher incidence rate of graft rejection.

Introduction: Clinical evidence supports the use of cardiac resynchronization therapy (CRT) in adults with heart failure, but experience in pediatrics and congenital heart disease (CHD) is limited in terms of patient numbers and follow-up. We sought to determine the functional assessment and clinical outcomes in pediatric and CHD CRT patients followed uniformly at one institution. Methods: Retrospective review of 60 consecutive patients who underwent CRT between 2002 and 2007. Results: At implantation, median age was 15.0 years (5 months to 47 years). Overall, 46 patients had CHD (77%) and 14 had dilated cardiomyopathy. Prior to CRT, 92% were on heart failure treatment drugs and 55% had pacemakers. Median follow-up time was 0.7 years (1 day–5.3 years). Median QRS width decreased from 149 to 120 ms (P < 0.001). Median ejection fraction (EF) increased from 36% to 42% (P < 0.001) and improvement was particularly evident in the group with CHD. Of note, 8 of 13 patients with single ventricle morphology had a “strong CRT response,” defined as either an improvement of 2–3 ordinal points in NYHA classification and/or increased ventricular function by ≥ 10 EF units. Overall, an improvement in functional status was observed in 39 of 45 patients (87%) with sufficient follow-up data. Conclusions: Children and CHD patients treated with CRT have acute improvement in ventricular function, but implantation may require individualized planning and unconventional approaches. Future important goals include preimplant determination of CRT responders in pediatric and CHD patients, optimizing lead placement and programing, as well as long-term CRT device management issues. [ABSTRACT FROM AUTHOR]

LV E/E′ ratio obtained using Doppler echocardiography is considered a surrogate for LV filling pressure in adults but has performed poorly in children. We hypothesized that LV E/E′ ratio Z‐score, adjusted for age, will relate more strongly to LV filling pressures than LV E/E′ ratio in pediatric HT recipients. We analyzed 751 echocardiograms performed within 24 hours of a heart catheterization in 122 pediatric HT recipients (median age at HT 13 years, median 6 studies per patient). The primary end‐point was PCWP, assessed both as a continuous and a binary variable. Associations with LV E/E′ ratios and z‐scores were assessed using generalized estimating equations models. PCWP, LV E/E′ ratios (using E′ from LV free wall, septum, and their average), and LV E/E′ ratio Z‐scores, all declined over time after HT. LV E/E′ ratios and their Z‐scores were significantly associated with PCWP assessed as a continuous variable (P < 0.001 for all); however, the relationship was weak (R2 range, 0.083 to 0.121). LV E/E′ ratios and their Z‐scores were also significantly associated with PCWP as a binary variable (P < 0.001 for all) but with only modest ability to discriminate PCWP ≥15 mm Hg (c‐statistic range, 0.660 to 0.695). The association between LV E/E′ ratio and PCWP in pediatric HT recipients is modest. Using a LV E/E′ ratio Z‐score did not result in significantly improved association with PCWP. Current Doppler echocardiographic methods are unreliable for estimating LV filling pressures in pediatric HT recipients. [ABSTRACT FROM AUTHOR]

Background: Medication non-adherence (NA) can result in life-threatening illness in children after solid-organ transplantation. Little is known about the incidence, risk factors and outcomes of NA in large numbers of pediatric heart transplant (HT) recipients. Methods: Organ Procurement Transplant Network (OPTN) data were used to identify all children <18 years of age in the USA who underwent HT from October 1999 to January 2007. Cox proportional hazards analysis was used to identify risk factors for NA and the effect on graft survival. Results: Of 2,070 pediatric heart transplants performed the median age at transplant was 6 years (interquartile range [IQR] 0 to 13 years); 40% had congenital heart disease (CHD), 7% were re-transplants, 42% were non-white and 43% had Medicaid insurance. Overall, 186 (9%) children had a report of NA at a median age of 15 years with more than two-thirds of NA episodes occurring after 12 years of age. Factors independently associated with NA were: adolescent age at transplant (hazard ratio [HR] 7.0, 95% confidence interval [CI] 4.1 to 12, compared with infants); black race (HR 2.3, 95% CI 1.7 to 3.3, compared with white); Medicaid insurance (HR 2.0, 95% CI 1.5 to 2.7, compared with non-Medicaid insurance); and ventilator or ventricular assist device (VAD) support at transplant. The risk of mortality conditional upon report of NA was 26% at 1 year and 33% at 2 years. Conclusions: Medication NA is an important problem in pediatric HT recipients and is associated with high mortality. Adolescent age, black race, Medicaid insurance and invasive hemodynamic support at transplant were associated with NA, whereas time on the wait list and gender were not. Targeted interventions among at-risk populations may be warranted. [Copyright &y& Elsevier]

The article presents the study that determines predictors of one minute heart rate (HR) recovery after a maximum effort exercise test in healthy children. The study evaluates 485 children who underwent an exercise test during their clinical evaluation to exclude cardiac disease and who were discharged as normal. It found that one minute HR recovery was higher in boys and correlated inversely with age and with aged-adjusted body mass index (BMI) in both boys and girls. It also indicates that one-minute HR recovery after exercise is weakened with age in children, while children with high BMI, especially those who are overweight and with lower exercise endurance, have lowered one minute HR recovery.

OBJECTIVES: With evidence of benefits of pediatric palliative care (PPC) integration, we sought to characterize subspecialty PPC referral patterns and end of life (EOL) care in pediatric advanced heart disease (AHD). METHODS: In this retrospective cohort study, we compared inpatient pediatric (,21 years) deaths due to AHD in 2 separate 3-year epochs: 2007-2009 (early) and 2015--2018 (late). Demographics, disease burden, medical interventions, mode of death, and hospital charges were evaluated for temporal changes and PPC influence. RESULTS: Of 3409 early-epoch admissions, there were 110 deaths; the late epoch had 99 deaths in 4032 admissions. In the early epoch, 45 patients (1.3% admissions, 17% deaths) were referred for PPC, compared with 146 late-epoch patients (3.6% admissions, 58% deaths). Most deaths (186 [89%]) occurred in the cardiac ICU after discontinuation of life-sustaining therapy (138 [66%]). Medical therapies included ventilation (189 [90%]), inotropes (184 [88%]), cardiopulmonary resuscitation (68 [33%]), or mechanical circulatory support (67 [32%]), with no temporal difference observed. PPC involvement was associated with decreased mechanical circulatory support, ventilation, inotropes, or cardiopulmonary resuscitation at EOL, and children were more likely to be awake and be receiving enteral feeds. PPC involvement increased advance care planning, with lower hospital charges on day of death and 7 days before (respective differences $5058 [P = .02] and $25 634 [P = .02]). CONCLUSIONS: Pediatric AHD deaths are associated with high medical intensity; however, children with PPC consultation experienced substantially less invasive interventions at EOL. Further study is warranted to explore these findings and how palliative care principles can be better integrated into care. [ABSTRACT FROM AUTHOR]

Background—Arterial tortuosity is described as a common and distinctive feature of Loeys-Dietz syndrome (LDS), yet reports on arterial tortuosity are based on qualitative observations and none have investigated an association between tortuosity and cardiovascular outcomes in LDS or other connective tissue disorders. Methods and Results—We performed a retrospective analysis of 90 patients ≤50 years of age with Marfan syndrome, LDS, Ehlers-Danlos syndrome, or nonspecific connective tissue disorder who underwent thoracic contrast-enhanced magnetic resonance angiography. Controls (n = 30) underwent magnetic resonance imaging to exclude arrhythmogenic right ventricular dysplasia. Using a volume-rendered angiogram, vertebral arteries were measured along the curvature of the vessel (actual length) and linearly (straight length), and distance factor was calculated: [(actual/straight length-1)X 100]. Each subject's maximum distance factor was designated the Vertebral Tortuosity Index (VT!). The VII was compared among diagnostic groups and among patients with cardiac surgery, dissection, and death. Median age at magnetic resonance imaging was 19.6 years (range 0.2 to 50.1). VTI interrater reliability was excellent (intraclass correlation coefficient =0.987). The VTI was higher in Marfan syndrome (n=57, median 26; interquartile range 10 to 49) and LDS (n= 13, median 58; interquartile range 18 to 92) compared with controls (median 4.5; interquartile range 3 to 6; P<0.001 for both). Higher VTI was associated with younger age at surgery even when controlling for root size (adjusted P=0.002). Vertebral tortuosity index ≥50 was associated with earlier age at dissection and death compared with VTI <50 (P=0.001 versus P<0.001). We found no difference in age at surgery, dissection, or death in Marfan syndrome compared with LDS. Conclusion—Arterial tortuosity measured by magnetic resonance angiography is a reproducible marker of adverse cardiovascular outcomes in connective tissue disorders. [ABSTRACT FROM AUTHOR]

Objectives. The exercise capacity of children with congenital heart disease (CHD) is often depressed. This depression is thought to be attributable to (1) residual hemodynamic defects and (2) deconditioning secondary to physical inactivity. We hypothesized that this latter component would be ameliorated by a formal cardiac rehabilitation program designed specifically for children. The objective of this study was to characterize the effect of a cardiac rehabilitation program on the exercise performance of children with CHD and to define the physiologic mechanisms that might account for any improvements that are observed. Methods. Nineteen patients with CHD who were referred for exercise testing and found to have a peak oxygen consumption (VO2) and/or peak work rate <80% of predicted were enrolled in the study. Sixteen patients (11 Fontan patients, 5 with other CHD) completed the program and had postrehabilitation exercise tests, results of which were compared with the prerehabilitation studies. Results. Improvements were found in 15 of 16 patients. Peak VO2 rose from 26.4 ± 9.1 to 30.7 ± 9.2 mL/kg per min; peak work rate from 93 ± 32 to 106 ± 34 W, and the ventilatory anaerobic threshold from 14.2 ± 4.8 to 17.4 ± 4.5 mL/kg per min. The peak heart rate and peak respiratory exchange ratio did not change, suggesting that the improvements were not attributable merely to an increased effort. In contrast, the peak oxygen pulse rose significantly, from 7.6 ± 2.8 to 9.7 ± 4.1 mL/beat, an improvement that can be attributed only to an increase in stroke volume and/or oxygen extraction at peak exercise. No patient experienced rehabilitation-related complications. Conclusion. Cardiac rehabilitation can improve the exercise performance of children with CHD. This improvement is mediated by an increase in stroke volume and/or oxygen extraction during exercise. Routine use of formal cardiac rehabilitation may greatly reduce the morbidity of complex CHD. [ABSTRACT FROM AUTHOR]