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OBJECTIVE While the surgical stages of single ventricle (SV) palliation serve to separate pulmonary venous and systemic venous return, and to volume-unload the SV, staged palliation also results in transition from parallel to series circulation, increasing total vascular resistance. How this transition affects pressure loading of the SV is as yet unreported. METHODS We performed a retrospective chart review of Stage I, II, and III cardiac catheterization (CC) and echocardiographic data from 2001-2017 in all SV pts, with focus on systemic, pulmonary, and total vascular resistance (SVR, PVR, TVR respectively). Longitudinal analyses were performed with log-transformed variables. Effects of SVR-lowering medications were analyzed using Wilcoxon rank-sum testing. RESULTS There were 372 total patients who underwent CC at a Stage I (median age of 4.4 months, n=310), Stage II (median age 2.7 years, n = 244), and Stage III (median age 7.3 years, n = 113). Total volume loading decreases with progression to Stage III (P< 0.001). While PVR gradually increases from Stage II to Stage III, and SVR increases from Stage I to Stage III, TVR dramatically increases with progress towards series circulation. TVR was not affected by use of systemic vasodilator therapy. TVR, PVR, SVR, and CI did not correlate with indices of SV function at Stage III. CONCLUSIONS TVR steadily increases with an increasing contribution from SVR over progressive stages. TVR was not affected by systemic vasodilator agents. TVR did not correlate with echo-based indices of SV function. Further studies are needed to see if modulating TVR can improve exercise tolerance and outcomes.

Normalizing cardiovascular measurements for body size allows for comparison among children of different ages and for distinguishing pathologic changes from normal physiologic growth. Because of growing interest to use height for normalization, the aim of this study was to develop height-based normalization models and compare them to body surface area (BSA)-based normalization for aortic and left ventricular (LV) measurements. The study population consisted of healthy, non-obese children between 2 and 18 years of age enrolled in the Pediatric Heart Network Echo Z-Score Project. The echocardiographic study parameters included proximal aortic diameters at 3 locations, LV end-diastolic volume, and LV mass. Using the statistical methodology described in the original project, Z-scores based on height and BSA were determined for the study parameters and tested for any clinically significant relationships with age, sex, race, ethnicity, and body mass index (BMI). Normalization models based on height versus BSA were compared among underweight, normal weight, and overweight (but not obese) children in the study population. Z-scores based on height and BSA were calculated for the 5 study parameters and revealed no clinically significant relationships with age, sex, race, and ethnicity. Normalization based on height resulted in lower Z-scores in the underweight group compared to the overweight group, whereas normalization based on BSA resulted in higher Z-scores in the underweight group compared to the overweight group. In other words, increasing BMI had an opposite effect on height-based Z-scores compared to BSA-based Z-scores. Allometric normalization based on height and BSA for aortic and LV sizes is feasible. However, height-based normalization results in higher cardiovascular Z-scores in heavier children, and BSA-based normalization results in higher cardiovascular Z-scores in lighter children. Further studies are needed to assess the performance of these approaches in obese children with or without cardiac disease.

Background:Although cardiac catheterisation (cath) is the diagnostic test for pulmonary hypertension, it is an invasive procedure. Echocardiography (echo) is commonly used for the non-invasive diagnosis of pulmonary hypertension but maybe limited by lack of adequate signals. Therefore, emphasis has been placed on biomarkers as a potential diagnostic tool. No prior paediatric studies have simultaneously compared N-terminal pro-B-type-natriuretic peptide (NTproBNP) with cath/echo as a potential diagnostic tool. The aim of this study was to determine if NTproBNP was a reliable diagnostic tool for pulmonary hypertension in this population.Methods:Patients were divided into Study (echo evidence/established diagnosis of pulmonary hypertension undergoing cath) and Control (cath for small atrial septal defect/patent ductus arteriosus and endomyocardial biopsy post cardiac transplant) groups. NTproBNP, cath/echo data were obtained.Results:Thirty-one patients met inclusion criteria (10 Study, 21 Control). Median NTproBNP was significantly higher in the Study group. Echo parameters including transannular plane systolic excursion z scores, pulmonary artery acceleration time and right ventricular fractional area change were lower in the Study group and correlated negatively with NTproBNP. Receiver operation characteristic curve analysis demonstrated NTproBNP > 389 pg/ml was 87% specific for the diagnosis of pulmonary hypertension with the addition of pulmonary artery acceleration time improving the specificity.Conclusions:NTproBNP may be a valuable adjunctive diagnostic tool for pulmonary hypertension in the paediatric population. Echo measures of transannular plane systolic excursion z score, pulmonary artery acceleration time and right ventricular fractional area change had negative correlations with NTproBNP. The utility of NTproBNP as a screening tool for pulmonary hypertension requires validation in a population with unknown pulmonary hypertension status.

BACKGROUND: Retrospective multicenter research using echocardiograms obtained for routine clinical care can be hampered by issues of individual center quality, thus limiting the application of post-hoc research analytics. We sought to evaluate imaging and patient characteristics associated with poorer quality of archived echocardiograms from a cohort of childhood cancer survivors. METHODS: A single blinded reviewer at a central core lab graded quality of clinical echocardiograms from 5 contributing centers focusing on images to derive 2D and M-mode fractional shortening (FS), biplane Simpson ejection fraction (EF), myocardial performance index (MPI), tissue Doppler imaging (TDI) derived velocities and global longitudinal strain (GLS). RESULTS: Of 535 studies analyzed in 102 subjects from 2004 to 2017, all measures of cardiac function could be assessed in only 7%. While FS by 2D or M-mode, MPI and septal E/E’ could be measured in >80% studies, mitral E/E’ was less consistent (69%), but better than EF (52%) and GLS (10%). At least one quality issue was identified in 66% studies, with technical issues (ex. lung artifact, poor endocardial definition) being the most common (33%). Lack of 2- and 3-chamber views was associated with the performing center. Patients < 5 years had a higher chance of apex cut-off in 4-chamber views compared to 16–35 year old (RR 1.99 (1.07–3.72), p = 0.03). Overall, for any quality issue, earlier era of echo and center were the only significant risk factors. CONCLUSION: Assessment of cardiac function using pooled multicenter archived echocardiograms was significantly limited. Efforts to standardize clinical echocardiographic protocols to include apical 2- and 3-chamber views and TDI will improve the ability to quantitate LV function.

Objective:Operators are mindful of the balloon-to-aortic annulus ratio when performing balloon aortic valvuloplasty. The method of measurement of the aortic valve annulus has not been standardised.Methods and results:Patients who underwent aortic valvuloplasty at two paediatric centres between 2007 and 2014 were included. The valve annulus measured by echocardiography and angiography was used to calculate the balloon-to-aortic annulus ratio and measurements were compared. The primary endpoint was an increase in aortic insufficiency by ≥2 degrees. Ninety-eight patients with a median age at valvuloplasty of 2.1 months (Interquartile range (IQR): 0.2–105.5) were included. The angiographic-based annulus was 8.2 mm (IQR: 6.8–16.0), which was greater than echocardiogram-based annulus of 7.5 mm (IQR: 6.1–14.8) (p < 0.001). This corresponded to a significantly lower angiographic balloon-to-aortic annulus ratio of 0.9 (IQR: 0.9–1.0), compared to an echocardiographic ratio of 1.1 (IQR: 1.0–1.1) (p < 0.001). The degree of discrepancy in measured diameter increased with smaller valve diameters (p = 0.041) and in neonates (p = 0.044). There was significant disagreement between angiographic and echocardiographic balloon-to-aortic annulus ratio measures regarding “High” ratio of >1.2, with angiographic ratio flagging only 2/12 (16.7%) of patients flagged by echocardiographic ratio as “High” (p = 0.012). Patients who had an increase in the degree of aortic insufficiency post valvuloplasty, only 3 (5.5%) had angiographic ratio > 1.1, while 21 (38%) had echocardiographic ratio >1.1 (p < 0.001). Patients with resultant ≥ moderate insufficiency more often had an echocardiographic ratio of >1.1 than angiographic ratio of >1.1 There was no association between increase in balloon-to-aortic annulus ratio and gradient reduction.Conclusions:Angiographic measurement is associated with a greater measured aortic valve annulus and the development of aortic insufficiency. Operators should use caution when relying solely on angiographic measurement when performing balloon aortic valvuloplasty.

Background The pediatric Appropriate Use Criteria (AUC) for outpatient transthoracic echocardiography (TTE) aim to reduce practice variation. Little is known on variation in TTE use between physicians. Understanding this variation will help identify areas for improvement in standardization of TTE use. Methods and results This is a retrospective review of initial outpatient visits at 6 pediatric cardiology centers in the United States prior to AUC release. Variation in TTE use was examined using multilevel generalized mixed effects models. Forward selection identified combinations of variables that explained the most variance in TTE use between physicians. Due to collinearity, physician compensation model and center were analyzed separately. Of 2883 encounters, the most common indication was murmur (36%), followed by chest pain (15.2%). Overall TTE use was 41.9%, and varied widely between centers (22.9%-52.6%), and between physicians within centers. Center alone explained 29% of this physician variance. Adding physician characteristics increased the variance explained to 57%, which only minimally improved by adding patient characteristics. The variance explained was driven by subspecialty. The center-based multivariable model explained more variance over compensation model. Conclusions Center was the single largest determinant of physician variance in TTE use, followed by physician subspecialty. Efforts to reduce practice variation, such as the AUC, should be employed across centers and all pediatric cardiac providers. Center appears to have a stronger impact on variance than compensation model, though in this dataset the effect of center and compensation are hard to separate from each other and deserve further evaluation.

Background Longitudinal clinical surveillance by transthoracic echocardiography (TTE) is an established practice in children with repaired tetralogy of Fallot (TOF). Non-Invasive Imaging Guidelines recommends a list of reporting elements that should be addressed during routine TTE in this population. In this study, we assessed the adherence to these recommendations. Methods This was a multi-center (n = 8) retrospective review of TTE reports in children ≤11 years of age who have had complete TOF repair. We included 10 patients from each participating center (n = 80) and scored 2 outpatient follow-up TTE reports on each patient. The adherence rate was based on completeness of TTE reporting elements derived from the guidelines. Results We reviewed 160 TTE reports on 80 patients. Median age was 4.4 months (IQR 1.5-6.6) and 3.6 years (IQR 1.3-6.4) at the time of complete surgical repair and first TTE report, respectively. The median adherence rate to recommended reporting elements was 61% (IQR 53–70). Of the 160 reports, 9 (7%) were ≥80% adherent and 40 (25%) were ≥70% adherent. Quantitative measurements of right ventricular outflow tract (RVOT), right ventricular (RV) size and function, and branch pulmonary arteries were least likely to be reported. Conclusions Overall adherence to the most recent published imaging guidelines for surveillance of children with repaired TOF patients was suboptimal, especially for reporting of RVOT, RV size and function, and branch pulmonary arteries. Further studies are needed to explore the barriers to adherence to guidelines and most importantly, whether adherence is associated with clinical outcomes.

Objectives: The purpose of this study was to assess the timing of changes in serial echocardiographic parameters in pediatric cancer survivors and to evaluate their associations with cardiomyopathy development. Background: Pediatric cancer survivors undergo serial echocardiograms to screen for cardiotoxicity. It is not clear whether small longitudinal changes in functional or structural parameters over time have clinical significance. Methods: This is a multicenter, retrospective, case-control study of ≥1-year survivors following the end of cancer therapy. Cardiomyopathy cases (fractional shortening [FS] ≤28% or ejection fraction [EF] ≤50% on ≥2 occasions) were matched to control subjects (FS ≥30%, EF ≥55%, not on cardiac medications) by cumulative anthracycline and chest radiation dose, follow-up duration, and age at diagnosis. Digitally archived clinical surveillance echocardiograms were quantified in a central core laboratory, blinded to patient characteristics. Using mixed models with interaction terms between time and case status, we estimated the least square mean differences of 2-dimensional, M-mode, pulsed wave Doppler, and tissue Doppler imaging–derived parameters over time between cases and control subjects. Results: We identified 50 matched case-control pairs from 5 centers. Analysis of 412 echocardiograms (cases, n = 181; control subjects, n = 231) determined that indices of left ventricular systolic function (FS, biplane EF), diastolic function (mitral E/A ratio), and left ventricular size (end-diastolic dimension z-scores) were significantly different between cases and control subjects, even 4 years prior to the development of cardiomyopathy. Conclusions: Longitudinal changes in cardiac functional parameters can occur relatively early in pediatric cancer survivors and are associated with the development of cardiomyopathy.

Introduction: To promote the rational use of cardiovascular imaging in the delivery of high-quality and cost-effective care to patients with congenital heart disease (CHD), the American College of Cardiology developed Appropriate Use Criteria (AUC). We aimed to evaluate the appropriateness of indications for cardiac CT and MRI in patients with conotruncal defects and to identify factors associated with Maybe or Rarely Appropriate (M/R) indications. Methods: Twelve US centers contributed a median of 147 studies performed prior to AUC publication (01/2020) on patients with conotruncal defects. Studies were excluded if the patient did not receive primary cardiac care at the center or were not seen > 5 years prior to study date. To account for the nesting of physicians within center, a hierarchical generalized linear mixed model was used to account for center-level variation with physicians nested within center. Results: Of the 1,753 studies (20% CT, 80% MRI) from 12 centers, 16% (range 4-39%) were rated M/R and 2.4% were unclassifiable (Figure 1). Patients age Conclusions: Most cardiac CTs and MRIs ordered for the follow-up care of patients with conotruncal defects were rated Appropriate and nearly all were able to be classified. However, there was significant center-level variation and higher annual MRI volume was independently associated with lower odds of M/R rating. These findings inform future quality improvement initiatives.

The COVID pandemic necessitated an altered approach to transthoracic echocardiography, especially in COVID cases. Whether this has effected echocardiography lab quality is unknown.We sought to determine whether echocardiography lab quality measures during the COVID pandemic were different from those prior to the pandemic and whether quality and comprehensiveness of echocardiograms performed during the pandemic was different between COVID and non-COVID patients.The four quality measures (diagnostic errors, appropriateness of echocardiogram, American College of Cardiology Image Quality metric and Comprehensive Exam metric in structurally normal hearts) reported quarterly in our lab were compared between two quarters during COVID (2020) and pre-COVID (2019). Each component of these metrics was also assessed in randomly selected echocardiograms in COVID patients and compared to non-COVID echocardiograms.For non-COVID echocardiograms, the image quality metric did not change between 2019 and 2020 and the comprehensive exam metric improved. Diagnostic error rate did not change, and appropriateness of echocardiogram indications improved. When COVID and non-COVID echocardiograms were compared, the image quality metric and comprehensiveness exam metric were lower for COVID cases (image quality mean 21.3/23 for non-COVID, 18.6/23 for COVID, p 0.001 and comprehensive exam mean 29.5/30 for non-COVID, 27.7/39 for COVID, p 0.001). In particular, systemic and pulmonary veins, pulmonary arteries and aortic arch were not adequately imaged in COVID patients. For studies in which a follow-up echocardiogram was available, no new pathology was found.At our center, though diagnostic error rate did not change during the pandemic and the proportion of echocardiograms ordered for appropriate indications increased, imaging quality in COVID patients was compromised, especially for systemic and pulmonary veins, pulmonary arteries and arch. Though no new pathology was noted on the small number of patients who had follow-up studies, we are paying careful attention to these structures to avoid diagnostic errors going forward.

Introduction Right ventricular pacing is associated with pacemaker induced cardiomyopathy and lesser degrees of pacing-induced LV dysfunction (PIVD) manifested by a reduction in left ventricular ejection fraction (LVEF). Our objective was to determine whether apical 4 chamber strain (A4C) by echocardiography can identify patients at risk of PIVD before LVEF declines. Methods and results A retrospective chart review of patients (0-21 years) who had a pacemaker with a ventricular lead placed between 2011 and 2017 was performed. Patients were divided into group A (LVEF 10% decline in LVEF within 12 months of pacemaker placement) and group B. Data have collected before and 1 and 12 months postpacemaker implantation. There were 30 patients in the group A and 60 in group B. At 1 and 12 months postpacemaker implantation, the LVEF was significantly lower while the A4C and QRS duration on electrocardiogram were significantly higher in the group A. While the LVEF and A4C became markedly abnormal in group A as early as 1 month, the A4C did not seem to demonstrate such marked abnormalities in group B. However, a sub-analysis of patients in the group A with preserved LVEF at 1 month demonstrated significant worsening in their A4C at that time. Conclusion Myocardial deformation imaging may be a clinically useful tool for the prediction of a decline in LV systolic function following pacemaker implantation. Abnormalities in A4C seem to appear before LVEF decline and as soon as 1-month postpacemaker implantation.

Introduction: Adverse outcomes in hypertrophic cardiomyopathy (HCM) includes arrhythmias, myocardial fibrosis, heart failure, and sudden cardiac arrest (SCA). These are well documented in children. Exercise stress echocardiography (ESE) can identify the severity and mechanism of left ventricular outflow tract (LVOT) obstruction that may not be present at rest. Our aim was to compare clinical outcomes in pediatric patients with varying severity of LVOT obstruction. Methods: We reviewed records of patients < 22 years with HCM who underwent staged ESE Jan 2009 - Dec 2019 at our center. Patients were divided into 3 groups: no resting or exercise LVOT gradient (Group 1), no resting gradient with LVOT gradient > 30 mmHg at peak exercise (Group 2), and resting LVOT gradient > 30 mmHg (Group 3). We assessed echocardiographic indices, advanced imaging, and clinical data. Statistical analysis performed with p-value Results: 93 patients met inclusion criteria. 6 SCA events occurred, though not during ESE.. Patients in Group 3 were classified less often as NYHA Class 1 compared to Group 1 or Group 2, (p < 0.01). Group 3 had the highest rate of myectomy (p=0.005) and ICD placement (p=0.003). There was no significant difference in exercise symptoms, peak oxygen consumption, or ischemic changes during ESE between the groups. Peak heart rate was also higher in Group 2 with no difference in blood pressure response. Groups were not associated with family history of disease, positive gene status, SCA, or the presence of fibrosis (Table). LVOT gradients differed as early as Stage 1 in Groups 1 & 2. Inducible LVOT obstruction was most commonly midcavitary in Group 2. Conclusion: The presence of resting versus inducible LVOT obstruction in pediatric HCM does not appear associated with adverse outcomes. This may be hampered by the rarity of events in pediatrics. The predominant mechanism of inducible LVOT obstruction was midcavitary, which has a link to adverse long-term outcomes in adult HCM.

Background Different methods have resulted in variable Z scores for echocardiographic measurements. Using the measurements from 3,215 healthy North American children in the Pediatric Heart Network (PHN) echocardiographic Z score database, the authors compared the PHN model with previously published Z score models. Methods Z scores were derived for cardiovascular measurements using four models (PHN, Boston, Italy, and Detroit). Model comparisons were performed by evaluating (1) overlaid graphs of measurement versus body surface area with curves at Z = −2, 0, and +2; (2) scatterplots of PHN versus other Z scores with correlation coefficients; (3) Bland-Altman plots of PHN versus other Z scores; and (4) comparison of median Z scores for each model. Results For most measurements, PHN Z score curves were similar to Boston and Italian curves but diverged from Detroit curves at high body surface areas. Correlation coefficients were high when comparing the PHN model with the others, highest with Boston (mean, 0.99) and lowest with Detroit (mean, 0.90). Scatterplots suggested systematic differences despite high correlations. Bland-Altman plots also revealed poor agreement at both extremes of size and a systematic bias for most when comparing PHN against Italian and Detroit Z scores. There were statistically significant differences when comparing median Z scores between the PHN and other models. Conclusions Z scores from the multicenter PHN model correlated well with previous single-center models, especially the Boston model, which also had a large sample size and similar methodology. The Detroit Z scores diverged from the PHN Z scores at high body surface area, possibly because there were more subjects in this category in the PHN database. Despite excellent correlation, significant differences in Z scores between the PHN model and others were seen for many measurements. This is important when comparing publications using different models and for clinical care, particularly when Z score thresholds are used to guide diagnosis and management.

Background Physicians are exposed to workplace factors that may result in acute or chronic stress resulting in burnout. This may impact the productivity and result in suboptimal patient care practices. Methods We surveyed pediatric cardiology attending physicians at our institution to assess their perception of burnout and work-life balance using the Maslach Burnout Inventory and the Areas of Work-Life Survey. Results Forty-five out of the 50 pediatric cardiology attendings responded to the survey. They were divided into 4 groups: Interventional/Electrophysiology [n = 3], Cardiac Intensive Care/Inpatient [n = 8], Non-Invasive Imaging [n = 6], and Outpatient [n = 28]. The Maslach Burnout Inventory demonstrated group-specific scores in the areas of emotional exhaustion, depersonalization, and personal accomplishment that were all significantly better than the general population. However, group-specific Areas of Work-Life Survey results demonstrated concerning findings with respect to the perception of work-life balance. Conclusions Although the Maslach Burnout Inventory did not demonstrate significant burnout among the attending physicians, the Areas of Work-Life Survey results demonstrated reduced work engagement, which can impact patient care and lead to burnout in the future. Based on these results, we plan to implement strategies to help increase work engagement and improve overall organizational effectiveness.

In the pediatric Appropriate Use Criteria (AUC), abnormal electrocardiogram (ECG) in an asymptomatic patient has been rated as an "Appropriate" indication for transthoracic echocardiogram (TTE). We hypothesized that the yield of abnormal findings on TTE for this indication will be low.All asymptomatic patients (≤ 18 years) from January 1, 2015 to December 31, 2017 who underwent initial outpatient evaluation at our center and had a TTE ordered for an abnormal ECG, were included. Clinic records were reviewed to obtain ECG and TTE findings.Of the 199 study patients, 13 (6.5%) had abnormal findings. Incomplete right bundle branch block (IRBBB) had the highest yield of abnormal TTE findings (7/28), with secundum atrial septal defect being the most common (5/7); (Odds ratio (OR) compared to other ECG findings 9.2, 95% CI (2.8-29.9), P .001). OR further increased to 14.6, 95% CI (3.1-68.0), P .001 when either IRBBB, right axis deviation, or right ventricular hypertrophy were present. Left ventricular hypertrophy on ECG had only one incidental abnormality on TTE, while ST segment changes, left axis deviation, right/left atrial enlargement, premature atrial/ventricular contractions, ectopic atrial rhythm, sinus bradycardia/pause, preexcitation, low-grade atrioventricular block, and junctional rhythm did not yield abnormal TTEs.The yield of abnormal findings on TTE when performed for the AUC indication for an abnormal ECG in asymptomatic pediatric patients is low except when performed for ECG abnormalities suggestive of right heart disease such as IRBBB, right axis deviation, or right ventricular hypertrophy. Future revisions of the AUC document could consider further stratification of this indication and corresponding appropriateness ratings based on ECG findings rather than combining into one broad category.

OBJECTIVE The educational intervention (EI) through the Pediatric Appropriate Use of Echocardiography (PAUSE) multicenter study resulted in improved appropriateness of transthoracic echocardiogram (TTE) orders at our center. The current study evaluated if this pattern persisted after cessation of EI and the potential physician characteristics influencing appropriateness. DESIGN Outpatients (≤18 years old) seen for initial evaluation during the EI (July to October, 2015) and 6-month post-EI (May to August, 2016) phases were included. Comparison was made between TTE rates and appropriateness ratings during EI and post-EI phase. Association between TTE rate and appropriateness with physician characteristics (age, experience, patient volume, and area of practice) was determined using odds ratio. RESULTS The study included 7781 patients (EI: N = 4016; post-EI: N = 3765) seen by 31 physicians. Comparison of appropriateness ratings in a randomized sample (EI: N = 1270; post-EI: N = 1325 patients) showed no significant differences between the two phases (appropriate: 75.2% vs 74.9%, P = .960; rarely appropriate 4.1% vs 6.5%, P = .065). Though there was significant variability among physicians for TTE order appropriateness (P = .044) and ordering rate (P

BACKGROUND In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echocardiographic parameters associated with 2V circulation, (2) identify echocardiographic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions. METHODS We performed a retrospective analysis of patients who underwent RV decompression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1-year or prior to Glenn circulation). RESULTS Eighty-one patients were included. At last follow-up, 70 (86%) patients had 2V circulations, 7 (9%) had 1.5 ventricle circulations, and 4 (5%) had single ventricle circulations. Follow-up echocardiograms were available in 43 (53%) patients. The majority of patients had improved RV systolic function, less tricuspid regurgitation (TR), and more left-to-right atrial shunting at a median of 350 days after decompression. Multivariable analysis demonstrated that larger baseline tricuspid valve (TV) z-score (P = .017), ≥ moderate baseline TR (P = .045) and smaller baseline RV area (P

ObjectiveMore patients with Fontan physiology are reaching adulthood. The purpose of this meta-analysis was to evaluate the late outcomes of patients palliated with Fontan procedure and to assess the risk factors for mortality.MethodsPubMed, Embase and Web of Science were queried to retrieve observational studies of survival in patients following the Fontan procedure with ≥5 years of follow-up. A random-effects model was used to determine pooled survival estimates at 5, 10 and 15 years. Meta-regression was used to assess potential moderators for death.ResultsNineteen articles with a total of 5859 patients were included. The weighted mean follow-up time was 8.94±2.64 years with overall 8.3% deaths and 1.5% transplants. Pooled survival estimates at 5, 10 and 15 years were 90.7%, 87.2% and 87.5%, respectively; and 88.4%, 85.7% and 84.1%, respectively, for studies that included all three time intervals (n=4). Earliest surgical year included in the study, proportion of atriopulmonary connections versus extracardiac conduit or lateral tunnel, and older age at Fontan were associated with higher rates of death, but ventricular morphology was not. Protein-losing enteropathy, reoperation and pacemaker insertion were reported in 2.1%, 5.6% and 6.8% patients, respectively.ConclusionsSurvival following the Fontan procedure has improved with time and is influenced by Fontan type and age at the time of Fontan. At a mean follow-up of 8.9 years, there was no significant association between survival and ventricular morphology, not taking into account the mortality prior to Fontan.

Objective It is unclear whether neonates with interrupted aortic arch (IAA) and a smaller left ventricular outflow tract may have improved outcomes with a Yasui operation (ventricular outflow bypass procedure) over a primary complete repair. This study sought to identify preoperative echocardiographic parameters to differentiate which neonates may have improved outcomes with a primary vs Yasui operation. Design Patient demographics, cardiac surgery type, complications, need for reoperation and/or interventional catheterization, and date of last follow-up were collected on neonates who underwent a biventricular repair for IAA from 2003 to 2014. Preoperative echocardiograms were analyzed for: IAA type, valve annulus size, aortic valve morphology, ventricular size and aortic arch anatomy. Results Seventy-seven neonates underwent IAA repair between 2003 and 2013. 60 neonates had a primary repair and 17 a Yasui operation. Neonates that underwent a Yasui operation had significantly smaller mitral and aortic valves with aortic arch hypoplasia. Within the primary repair group, a decreasing aortic root z-score on univariate analysis increased the odds of reoperation by twofold [OR = 1.98, 95% CI: (1.15-3.42), P = .014]. A significant interaction between repair type and aortic root z-score was identified on multivariable analysis (P = .039), for neonates with aortic root z-scores less than −2.5, the probability of reoperation during the follow up time period [mean 4.5 years (3.3 months-10 year)] was significantly higher in the primary repair group compared to the Yasui group (64.3% vs 37.5%). Conclusions Neonates with IAA and an aortic root z-score less than −2.5 have lower odds of subsequent reoperations with a Yasui operation compared to a primary repair over the follow up period. These findings suggest a Yasui operation should be considered if the preoperative aortic root z-score is less than −2.5. Careful evaluation of these morphologic predictors on preoperative echocardiograms can be helpful in surgical planning in neonates with IAA.

Although pediatric appropriate use criteria (AUC) for outpatient transthoracic echocardiography (TTE) are available, little is known about TTE utilization patterns before their release. The aims of this study were to determine the relation between AUC and TTE utilization and to identify patient and physician factors associated with discordance between the AUC and clinical practice.A retrospective review of 3,000 initial outpatient pediatric cardiology encounters at six centers was performed. Investigator-determined indications were classified using AUC definitions. Concordance between AUC and TTE utilization was determined. Multivariate analysis was performed to identify patient and physician factors associated with TTE's being performed for rarely appropriate and TTE's not being performed for appropriate indications.Concordance between AUC and TTE utilization was 88%. TTE was performed for rarely appropriate indications in 9% and was associated with patient age3 months, indications of murmur, noninvasive imaging physician subspecialty, and physician volume. No TTE was ordered for appropriate indications in 3% and was associated with indications including prior test result (primarily abnormal electrocardiographic findings), older patients, and physician subspecialty other than generalist or imaging. There was high variability in TTE utilization among centers.There was a reasonable degree of concordance between AUC and clinical practice before AUC publication. Several patient and physician factors were associated with discordance with the AUC. These findings should be considered in efforts to disseminate the AUC and in the development of future iterations. The causes for variation among centers deserve further exploration.

The aim of this study was to describe serial changes in echocardiographic Doppler pulmonary vein flow (PVF) patterns in infants with single right ventricle (RV) anomalies enrolled in the Single Ventricle Reconstruction trial. Measurement of PVF peak systolic (S) and diastolic (D) velocities, velocity time integrals (VTI), S/D peak velocity and VTI ratios, and frequency of atrial reversal (Ar) waves were made at three postoperative time points in 261 infants: early post-Norwood, pre-stage II surgery, and 14 months. Indices were compared over time, between initial shunt type [modified Blalock–Taussig shunt (MBTS) and right ventricle-to-pulmonary artery shunt (RVPAS)] and in relation to clinical outcomes. S velocities and VTI increased over time while D wave was stable, resulting in increasing S/D peak velocity and VTI ratios, with a median post-Norwood S/D VTI ratio of 1.14 versus 1.38 at pre-stage II and 1.89 at 14 months (P

Background Concern exists over exponential growth in cardiac imaging in adults, but there is paucity of such data for cardiac imaging trends in pediatric patients. The aims of this study were to determine temporal trends in the use of noninvasive cardiac imaging and compare these with trends in the use of noncardiac imaging and to identify factors influencing those trends using the Pediatric Health Information Service database. Methods Pediatric inpatient encounter data from January 2004 to December 2017 at 35 pediatric hospitals were extracted from the Pediatric Health Information Service database. Temporal imaging utilization trends in cardiac and noncardiac ultrasound or echocardiography, magnetic resonance imaging (MRI), and computed tomography (CT) were assessed using linear mixed-effects models. Models were adjusted for case-mix index, complex chronic conditions, patient age, length of stay, payer source, and cardiac surgical volume. Results A total of 5,869,335 encounters over 14 years were analyzed (median encounters per center per year, 11,411; median patient age, 4 years; median length of stay, 3 days). From 2004 to 2017, the rates of pediatric inpatient cardiac and noncardiac ultrasound and MRI increased, whereas the rate of noncardiac CT decreased. Cardiac CT use increased beginning in 2014 (+0.264 cardiac CT encounters per 1,000 encounters per year), surpassing the rate of rise of cardiac MRI. Case-mix index, cardiac surgical volume, and payer source affected the largest number of imaging trends. Conclusions Among pediatric inpatients, utilization of cardiac and noncardiac ultrasound and MRI has steadily increased. Noncardiac CT use declined and cardiac CT use increased after 2014. Factors influencing imaging trends include case-mix index, cardiac surgical volume, and payer source. This study lays a foundation for investigations of imaging-related resource utilization and outcomes among pediatric inpatients.

Background:Cardiovascular disease is a leading cause of morbidity and mortality in childhood cancer survivors. Cardiologists must be aware of risk factors and long-term follow-up guidelines, which have historically been the purview of oncologists. Little is known about paediatric cardiologists’ knowledge regarding the cardiotoxicity of cancer treatment and how to improve this knowledge.Methods:A total of 58 paediatric cardiologists anonymously completed a 21-question, web-based survey focused on four cardio-oncology themes: cancer treatment-related risk factors (n = 6), patient-related risk factors (n = 6), recommended surveillance (n = 3), and cardiac-specific considerations (n = 6). Following the baseline survey, a multi-disciplinary team of paediatric cardiologists and cancer survivor providers developed an in-person and web-based educational intervention. A post-intervention survey was conducted 5 months later.Results:The response rate was 41/58 (70.7%) pre-intervention and 30/58 (51.7%) post-intervention. On the baseline survey, the percentage of correct answers was 68.8 ± 10.3%, which improved to 79.2 ± 16.2% after the intervention (p = 0.009). The theme with the most profound knowledge deficit was surveillance; however, it also had the greatest improvement after the intervention (49.6 ± 26.7 versus 66.7 ± 27.7% correct, p = 0.025). Individual questions with the largest per cent improvement pertained to risk of cardiac dysfunction with time since treatment (52.4 versus 93.1%, p = 0.002) and the role of dexrazoxane (48.8 versus 82.8%, p = 0.020).Conclusion:Specific knowledge deficits about the care of paediatric cancer survivors were identified amongst cardiologists using a web-based survey. Knowledge of surveillance was initially lowest but improved the most after an educational intervention. This highlights the need for cardio-oncology-based educational initiatives among paediatric cardiologists.

BACKGROUND: The reliability of LV systolic functional indices calculated from blinded echocardiographic measurements of LV size has not been tested in a large cohort of healthy children. The objective of this study was to estimate interobserver variability in standard measurements of left ventricular (LV) size and systolic function in children with normal cardiac anatomy and qualitatively normal function. METHODS: The Pediatric Heart Network Normal Echocardiogram Database collected normal echocardiograms from healthy children ≤18 years old distributed equally by age, gender, and race. A core lab used 2-dimensional echocardiograms to measure LV dimensions from which a separate data coordinating center calculated LV volumes and systolic functional indices. To evaluate interobserver variability, two independent expert pediatric echocardiographic observers re-measured LV dimensions on a subset of studies, while blinded to calculated volumes and functional indices. RESULTS: Of 3215 subjects with measurable images, 552 (17%) had a calculated LV shortening fraction (SF)

Pediatric appropriate use criteria (AUC) were recently published for initial outpatient transthoracic echocardiography (TTE). The purpose of this study was to determine the effect of AUC publication on TTE ordering patterns of pediatric cardiologists. Data were prospectively collected on patients who had initial outpatient TTE ordered before (phase I, April to September 2014) and 3 months after (phase II, January to April 2015) AUC document publication at 6 centers. Site investigators assessed each study's indication and assigned AUC appropriateness as "appropriate" (A), "may be appropriate" (M), "rarely appropriate" (R), or "unclassifiable." One hundred three physicians ordered 4,562 TTEs (2,655 phase I and 1,907 phase II). Overall, there was no statistically significant change in the proportion of A, M, or unclassifiable, but R decreased (12.0% to 9.6%, p = 0.01). There was significant variability among the centers in the percentage of studies for indications rated R (4.9% to 34.8%). There was no significant change in any of the appropriateness ratings at 4 centers, a decrease in R and an increase in A at 1 and a decrease in R and increase in unclassifiable at another. The first pediatric AUC document had only a small impact on physician ordering behavior for initial TTEs, including a small decrease in R. There was a significant variability in appropriateness of studies among centers. These data suggest that active educational interventions are required to substantially improve the appropriate use of pediatric TTE in the outpatient setting.

Background Aortopulmonary window (APW) is a rare congenital defect that is often associated with other cardiac lesions. We analyzed our operative strategy to determine whether this had any relationship with outcomes. Methods Early and late outcomes of 40 children who underwent APW repair at our institution during a 20-year period (1994 to 2013) were analyzed. Results Median age at time of the operation was 22 days (interquartile range, 9 to 63 days), and median weight was 3.2 kg (interquartile range, 2.5 to 3.8 kg). Eleven patients (28%) were born prematurely at or before 36 weeks' gestation, and 10 (25%) had genetic/extracardiac malformations. Mean APW size was 0.84 ± 0.28 cm, and by the Mori classification was type I in 17 patients (43%), type II in 18 (45%), and type III in 5 (13%). Twenty-five patients (63%) had simple APW, with no associated cardiac lesions other than atrial septal defect or patent ductus arteriosus, whereas 15 (38%) had complex APW with one or more associated lesions, including interrupted aortic arch (n = 6), ventricular septal defect (n = 6), or other (n = 5). There were no hospital or late deaths. Four patients required cardiac reoperations, 3 of whom had interrupted aortic arch. The 10-year freedom from cardiac reoperation was 100% for simple APW vs 73% for complex APW ( p = 0.008), with 75% of reoperations related to aortic obstruction. Age, weight, prematurity, extracardiac anomalies, APW size and type, and APW repair technique were not associated with reoperation risk. Conclusions Current outcomes of early repair of APW are excellent, including infants with complex associated cardiac lesions. Compared with historic results, contemporary outcomes are favorable, supporting early and complete repair of APW and associated lesions. Cardiac reoperation can be required in complex APW, mainly with concomitant arch repair, and is usually related to aortic obstruction.

Objective To determine the appropriateness and yield of transthoracic echocardiograms (TTE) for murmur evaluation based on the pediatric Appropriate Use Criteria (AUC) and study the influence of patient age and physician experience on TTE appropriateness, yield, and ordering frequency. Design Retrospective review of medical records of patients referred to our practice for murmur evaluation from April to September 2014. Data collected included indication for TTE, patient age, physician experience since fellowship, TTE findings and exit diagnosis. Appropriateness was assigned based on the AUC document. Setting Pediatric cardiology clinics affiliated with a large pediatric cardiology practice. Patients. One thousand seven hundred one consecutive patients (≤ 18 years) referred to our practice for murmur evaluation. Interventions Not applicable Outcome Measures The primary outcome was appropriateness of TTE orders. The secondary outcomes were the yield of abnormal TTE findings and the influence of patient age and physician experience on appropriateness, yield, and frequency of ordering TTEs. Results Of the 1701 patients referred for a murmur, 526 (30.9%) had a TTE [441/526 (83.8%) Appropriate; 85/526 (16.2%) Rarely Appropriate]. Abnormal findings were present in 130/441 rated Appropriate and none rated Rarely Appropriate. Infants 20 years of experience not only had the lowest TTE ordering rate but also the lowest appropriateness rate with no difference in the yield of abnormal findings. Conclusions Most TTEs ordered for murmur were for indications rated Appropriate. Abnormal findings were present in one-fourth and only those rated Appropriate. Patient age and physician experience can significantly influence TTE utilization. This information is helpful in designing quality initiatives to optimize TTE utilization for murmur evaluation.

BACKGROUND Left ventricular outflow tract obstruction (LVOTO) is a common complication following surgical repair of atrioventricular septal defect (AVSD). OBJECTIVE We sought to determine predictors of LVOTO based on echocardiograms performed prior to initial repair of AVSD. METHODS Of the 415 children that had repair of AVSD from 2003 to 2012, 17 children were identified with LVOTO that required surgical intervention. Thirty-four patients with repaired AVSD and no LVOTO served as controls. Patient demographics, cardiac surgery type, and echocardiogram results at last follow-up were collected. Off-line analysis of the echocardiogram prior to AVSD repair was done to obtain: left ventricular outflow tract (LVOT) and interventricular septal diameter, chordae across LVOT, aortoseptal angle, left ventricular (LV) inflow/outflow length ratio, inferior displacement of the atrioventricular (AV) valve, atrioventricular valve index (AVVI) and presence of coarctation of the aorta. RESULTS The LVOTO group had significantly smaller indexed LVOT diameters (P

Polyvalvar myxomatous valve degeneration is a clinical pathology rarely encountered during cardiac anesthesia, but, when present, most commonly occurs in the context of a connective tissue disorder. Filamin A mutations have begun to be recognized as a source of progressive myxomatous mitral and tricuspid valve degeneration. These lesions can be diagnosed by echo, but their clinical presentation can be equivocal. We present a patient with significant echocardiographic findings of mitral and tricuspid valvar regurgitation, aortic dilatation, and intraoperative findings of aortic ectasia. In our case, a detailed family history led to a preoperative echocardiogram revealing myxomatous mitral and tricuspid valve degeneration with significant regurgitation and aortic dilatation. Genetic evaluation led to the diagnosis of a Filamin A mutation. Pre- and postrepair echocardiographic assessments of valvar function played a key role in the management of this patient. Continued surveillance of his aortic dilation and evaluation of postrepair valve function warrants close follow-up with a high likelihood for further surgical intervention.

BACKGROUND The role of exercise stress echocardiography (ESE) in the pediatric population is less well defined as compared to adults. We aimed to determine the utility and impact of ESE on clinical decision-making in pediatric patients. METHODS We identified patients who underwent an ESE at our center from 2011 to 2015. Test indications were categorized into symptoms with exercise; sports/activity clearance; hypertrophic cardiomyopathy (HCM) or suspected HCM; coronary anomalies; or abnormal electrocardiogram (EKG). Change in clinical management was assessed by comparing pre- and post-test activity restrictions, which were categorized into unrestricted from exercise or activity; restricted from exercise or activity; and surgical referral. RESULTS During the study period, 353 ESEs met inclusion criteria. Of all ESEs performed, 263 (75%) were normal. Clinical management changed as a result of ESE in 144 (40%). Of the abnormal ESEs, 44 were restricted from activity, including 25 (56.8%) restricted from competitive or varsity athletics, 14 (31.8%) restricted from recreational sports, and 5 (11.4%) restricted from all activity. Surgical referrals included valve repair/replacement in 7 (50%), ICD placement in 5 (35.8%), coronary re-implantation in 1 (7.1%), and atrial septal defect repair in 1 (7.1%). CONCLUSION Exercise stress echocardiography provides the pediatric cardiologist with useful information that impacts management in a wide variety of cardiac disorders. Clinical management changed in nearly half the patients that were subjected to an ESE at our center. This supports the value of ESE for informing clinical decision-making. Future studies should aim to refine patient selection and examine its impact on patient outcomes.

Background Tricuspid annular plane systolic excursion (TAPSE) has emerged as a reliable marker of right ventricular (RV) systolic function. Recently, TAPSE derived using 2D images (2D-TAPSE) was shown to correlate with M-mode TAPSE (MM-TAPSE). We have developed a novel technique for semiautomatic evaluation of TAPSE (SA-TAPSE). The purpose of this study was to determine the accuracy of this novel technique and validate it on normal hearts and pulmonary hypertension (PH). Methods A total of 110 patients (56 with normal heart and 54 with PH) were retrospectively identified for analysis. The semiautomatic algorithm tracked the lateral tricuspid valve hinge point (TVHP) and the apex in the apical 4-chamber view. SA-TAPSE was calculated as displacement of the TVHP in end-diastole (ED) and end-systole (ES). The same points were manually identified to derive 2D-TAPSE. Results The system was able to accurately identify ED and ES in 304/330 heartbeats within three cardiac frames. The automatically identified TVHP points were within 1.2 ± 0.7 mm from the manually identified points. Intra-class correlation between SA-TAPSE and 2D-TAPSE was 0.96 (95% CI 0.93–0.98) for normal hearts and 0.92 (95% CI 0.87–0.96) for those with PH. Bland–Altman analysis showed a strong agreement between SA-TAPSE and 2D-TAPSE for normal hearts and those with PH. Conclusion Using the novel custom-made software, SA-TAPSE could be measured in majority of our patients and was accurate when applied to normal hearts and those with PH. Future work will focus on fully automating the system for a rapid retrospective analysis of TAPSE.

Objective To determine whether implementation of a standardized clinical practice guideline (CPG) for the evaluation of syncope would decrease practice variability and resource utilization. Design A retrospective review of medical records of patients presenting to our practice for outpatient evaluation of syncope before and after implementation of the CPG. The guideline included elements of history, physical exam, electrocardiogram, and “red flags” for further testing. Setting Outpatient pediatric cardiology offices of a large pediatric cardiology practice. Patients. All new patients between 3 and 21 years old, who presented to cardiology clinic with a chief complaint of syncope. Interventions The CPG for the evaluation of pediatric syncope was presented to the providers. Outcome Measures Resource utilization was determined by the tests ordered by individual physicians before and after initiation of the CPG. Patient final diagnoses were recorded and the medical records were subsequently reviewed to determine if any patients, who presented again to the system, were ultimately diagnosed with cardiac disease. Results Of the 1496 patients with an initial visit for syncope, there was no significant difference in the diagnosis of cardiac disease before or after initiation of the CPG: (0.6% vs. 0.4%, P = .55). Electrocardiography provides the highest yield in the evaluation of pediatric syncope. Despite high compliance (86.9%), there were no overall changes in costs ($346.31 vs. $348.53, P = .85) or in resource utilization. There was, however, a decrease in the variability of ordering of echocardiograms among physicians, particularly among those at the extremes of utilization. Conclusions Although the CPG did not decrease already low costs, it did decrease the wide variability in echo utilization. Evaluation beyond detailed history, physical exam, and electrocardiography provides no additional benefit in the evaluations of pediatric patients presenting with syncope.

Newborns with hypoplastic left heart syndrome and other single right ventricular variants require substantial health care resources. Weekend acute care has been associated with worse outcomes and increased resource use in other populations but has not been studied in patients with single ventricle. Subjects of the Single Ventricle Reconstruction trial were classified by whether they had a weekend admission and by day of the week of Norwood procedure. The primary outcome was hospital length of stay (LOS); secondary outcomes included transplant-free survival, intensive care unit (ICU) LOS, and days of mechanical ventilation. The Student's t test with log transformation and the Wilcoxon rank-sum test were used to analyze associations. Admission day was categorized for 533 of 549 subjects (13% weekend). The day of the Norwood was Thursday/Friday in 39%. There was no difference in median hospital LOS, transplant-free survival, ICU LOS, or days ventilated for weekend versus non-weekend admissions. Day of the Norwood procedure was not associated with a difference in hospital LOS, transplant-free survival, ICU LOS, or days ventilated. Prenatally diagnosed infants born on the weekend had lower mean birth weight, younger gestational age, and were more likely to be intubated but did not have a difference in measured outcomes. In conclusion, in this cohort of patients with single right ventricle, neither weekend admission nor end-of-the-week Norwood procedure was associated with increased use of hospital resources or poorer outcomes. We speculate that the complex postoperative course following the Norwood procedure outweighs any impact that day of admission or operation may have on these outcomes.

Background Qualitative assessment of ventricular septal flattening is commonly used in pediatric patients with pulmonary hypertension (PH) who lack adequate tricuspid regurgitation (TR) Doppler signal. We sought to determine the relation between quantitative measures of septal flattening including the eccentricity index (EIs) and a novel marker, the septal flattening angle (SFA) with right ventricular systolic pressure (RVSP). Methods Subjects (≤18 years) with an anatomically normal heart, an adequate TR signal to obtain a peak velocity, and a simultaneous systemic systolic blood pressure (SBP) was included. RVSP was derived using TR gradient. Eccentricity index (EIs) and the SFA in systole were measured offline and correlated with RVSP/SBP. Results Of the 108 subjects, RVSP/SBP was < 50% in 77 and ≥ 50% in 31. In those with RVSP/SBP ≥50%, the median SFA was significantly lower (7.4° vs. 22°, p < 0.0001), and the median EIs was higher (1.61 vs. 1.07, p < 0.0001). SFA and EIs had a significant correlation with RVSP/SBP (rs = −0.70 and 0.61, respectively). Area under the curve was higher for SFA compared to EIs (0.92 and 0.85, respectively). The sensitivity and specificity of SFA for predicting an RVSP/SBP ≥ 50% using a cut point of 16° was 84% and 95% and for an EIs cut point of 1.35 was 74.2% and 96.1%, respectively. Conclusion Septal flattening angle and EIs are quantitative measures of ventricular septal flattening that correlate well with RVSP/SBP and should be considered more routinely in clinical practice, especially in patients with inadequate TR Doppler signal.

Background Recently published appropriate use criteria (AUC) for initial pediatric outpatient transthoracic echocardiography (TTE) have not yet been evaluated for clinical applicability. Objectives This study sought to determine the appropriateness of TTE as currently performed in pediatric cardiology clinics, diagnostic yield of TTE for various AUC indications, and any gaps in the AUC document. Methods Data were prospectively collected from patients undergoing initial outpatient TTE in 6 centers. TTE indications (appropriate [A], may be appropriate [M], or rarely appropriate [R]) and findings (normal, incidental, or abnormal) were recorded. Results Of the 2,655 studies ordered by 102 physicians, indications rated A, M, and R were found in 1,876 (71%), 316 (12%), and 319 studies (12%), respectively, and 144 studies (5%) were unclassifiable. Twenty-four of 113 indications (21%) were not used. Innocent murmur and syncope or palpitations with no other indications of cardiovascular disease, a benign family history, and normal electrocardiogram accounted for 75% of indications rated R. Pathologic murmur had the highest yield of abnormal findings (40%). Odds of an abnormal finding in an A or M TTE were 6 times that of R (95% confidence interval [CI]: [2.8 to 12.8]). Abnormal findings were more common in patients 10 years of age (odds ratio: 6.4; 95% CI: 4.7 to 8.7). Age was a significant predictor of an abnormal finding after adjusting for indication and site (p Conclusions Most TTEs ordered in pediatric cardiology clinics were for indications rated A. AUC ratings successfully stratified indications based on the yield of abnormal findings. This study identified differences in the yield of TTE based on patient age and most common indications rated R. These findings should inform quality improvement efforts and future revisions of the AUC document.

We sought to review current-era experience with multistage palliation of neonates with hypoplastic left heart syndrome (HLHS) and to examine the patients' anatomic, and surgical risk factors influencing outcomes.A retrospective review of 219 HLHS patients who underwent the Norwood operation from 2002 to 2012 was performed. Competing risks analyses modeled events after the Norwood operation (death/transplantation, progression to Glenn operation) and after the Glenn operation (death/transplantation progression to Fontan operation), and examined the risk factors affecting outcomes.Competing risks analysis showed that 1 year after the Norwood operation, 25% of patients had died and 75% had undergone the Glenn operation. Three years after the Glenn operation, 11% of patients had died or received transplantation, 48% had undergone the Fontan procedure, and 41% were alive awaiting the Fontan. The overall 8-year survival after the Norwood operation was 66%. The risk factors for mortality on multivariable models were postoperative requirement for extracorporeal membrane oxygenation (hazard ratio [HR]: 3.1 [1.8-5.4], p0.001), genetic/major extracardiac anomalies (HR: 2.7 [1.4-5.2], p = 0.002), unplanned cardiac reoperation (HR: 2.5 [1.3-5.0], p = 0.007), modified Blalock-Taussig shunt use (HR: 2.4 [1.4-3.8], p0.001), and prematurity (HR: 1.9 [1.1-3.5], p = 0.030). Of note, anatomic factors such as HLHS subtype, ascending aorta diameter, restrictive atrial septum, and associated cardiac lesions did not affect outcomes (p0.10 each).This current single-institution experience demonstrates that patients' factors (ie, prematurity, low weight, genetic/extracardiac anomalies) continue to adversely affect survival. Conversely, surgical and perioperative management advances, along with the use of the right ventricle to pulmonary artery shunt, might have neutralized the effects of anatomic factors on survival. Technical imperfections requiring reoperations are associated with failure to progress through palliation stages and diminished survival.

The initial experience with the miniaturized multiplane micro-transesophageal echocardiographic probe (MTEE) reported high-quality diagnostic imaging in small infants. The aim of this study was to compare the diagnostic accuracy and image quality of the intraoperative MTEE with the pediatric multiplane transesophageal echocardiographic probe (PTEE).Infants weighing5 kg who underwent intraoperative transesophageal echocardiography were identified. Studies using the MTEE were matched 1:1 with those using the PTEE by cardiac diagnosis. The postoperative transesophageal echocardiograms, obtained using either probe, were reviewed for the presence of 11 cardiac abnormalities. Postoperative transesophageal echocardiograms were compared with predischarge transthoracic echocardiograms to assess accuracy. Using receiver operating characteristic curves, the areas under the curve for the MTEE and PTEE were compared. Two pediatric cardiologists scored six image quality metrics on equal numbers of studies obtained with the MTEE and the PTEE. Composite scores from both reviewers were used to compare image quality.The study included 110 transesophageal echocardiograms per probe type. The mean weight for the MTEE was lower than for the PTEE (3.15 ± 0.58 vs 3.70 ± 0.52 kg, P .001). There was no significant difference in the diagnostic accuracy of the MTEE and PTEE using receiver operating characteristic curves. The numbers of residual anatomic lesions missed by the MTEE and PTEE were similar (19 vs 22, respectively). The composite image quality score was worse for the MTEE compared with the PTEE (81% vs 92%, respectively, P .0001).Although the image quality of the MTEE is inferior compared with the PTEE, its diagnostic accuracy in infants weighing5 kg is comparable.