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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.

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.

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.

Objectives To characterize the subgroup of outpatient pediatric patients presenting with chest pain and to determine the effectiveness of published pediatric appropriate use criteria (PAUC) to detect pathology. Study design The Pediatric Appropriate Use of Echocardiography study evaluated the use and yield of transthoracic echocardiography (TTE) before and after PAUC release. Data were reviewed on patients ≤18 years of age who underwent TTE for chest pain. Indications were classified as appropriate (A), may be appropriate (M), and rarely appropriate (R) based on PAUC ratings, and findings were normal, incidental, or abnormal. Results Chest pain was the primary indication in 772 of 4562 outpatient TTE studies (17%) (median age 14 years, IQR 10-16) ordered during the study period: 458 of 772 before (59%) and 314 of 772 after (41 %) the release of PAUC with no change in appropriateness. In A indications (n = 654), 642 (98%) were normal, 5 (1%) had incidental findings, and 7 (1%) were abnormal. A and M detected 100% of all abnormal findings (A: n = 7; M: n = 6; R: n = 0), with an association between ratings and findings ( P .001). There was no association between R rating and any pathology. Conclusions There was no change in ordering patterns with publication of the PAUC. Despite the high rate of TTEs ordered for indications rated A, most studies were normal. Studies that detected pathology were performed for indications rated A or M, but not R. This study supports PAUC as a useful tool in pediatric chest pain evaluation that may subsequently improve the use of TTE.

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.

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 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.

Robert M. Campbell, MD, FACC, FAHA, FAAP, FHRS, Chair [∗][1] Pamela S. Douglas, MD, MACC, FAHA, FASE, Moderator [∗][1] Louis I. Bezold, MD, FACC, FAAP, FASE[†][2] William B. Blanchard, MD, FACC, FAHA, FAAP[∗][1] Jeffrey R. Boris, MD, FACC[∗][1] Bryan Cannon, MD[‡][3] Gregory J.

Background We sought to determine any differences between myocardial velocities in healthy children and pediatric heart transplant recipients without acute allograft rejection and to study the clinical factors that could alter these velocities. Methods Fifty-eight pediatric heart transplant recipients without rejection and 27 healthy controls were prospectively enrolled. Doppler tissue imaging (DTI) was performed at the tricuspid annulus, septum, and mitral annulus. The influence of the following factors on DTI was assessed: time since transplant, extracorporeal membrane oxygenation, systemic hypertension, graft atherosclerosis, pulmonary hypertension, donor heart ischemic time, and previous rejection. Results The mean tricuspid annular and septal tissue velocities were significantly reduced in the transplant group compared with controls. The mitral annular velocities were similar in the two groups. Donor heart ischemic time and previous rejection significantly altered DTI velocities. Conclusion Tricuspid annular and septal Doppler tissue velocities are significantly reduced in pediatric heart transplant recipients without allograft rejection and can be altered by prolonged donor heart ischemic time and previous rejection.

Background: The role of tricuspid annular plane systolic excursion (TAPSE) in evaluating right ventricular (RV) function in hypoplastic left heart syndrome (HLHS) has not been fully explored. We sought to evaluate the relation between TAPSE and RV fractional area change (FAC) and cardiac index (CI)

Background: Though there are numerous single center reports of Fontan outcomes, there is lack of comprehensive survival data. The purpose of this meta-analysis was to evaluate the long-term outcomes following Fontan operation and to assess risk factors for mortality. Methods: PubMed, Embase and Web

Syncope is a common reason for referral to pediatric cardiologists and to perform transthoracic echocardiography (TTE). We studied the applicability of pediatric appropriate use criteria (AUC) on initial outpatient evaluation of children with syncope before and after the release of the AUC document

Outcomes following right ventricle (RV) decompression in infants with pulmonary atresia and intact ventricular septum (PA-IVS) vary widely. Descriptions of these outcomes are limited to small single-center studies. Neonates undergoing catheter (cath) pulmonary valve perforation/valvuloplasty or

Background Left ventricular outflow tract obstruction (LVOTO) is an important problem after interrupted aortic arch (IAA) repair, especially when early reoperation is required during infancy. Several anatomic factors have been identified that increase LVOTO risk; surgical strategies such as concomitant resection of the conal septum or left ventricular outflow tract (LVOT) bypass (single-stage Yasui operation, or staged Norwood procedure, followed by the Rastelli procedure) have been proposed for such patients. Methods From 2002 to 2013, a total of 77 neonates underwent IAA repair. Based on the presence of anatomic substrate for LVOTO, patients underwent standard IAA repair and ventricular septal defect closure (n = 53; 69%), concomitant conal resection (n = 7; 9%), or LVOT bypass (n = 17; 22%, staged in 14). We analyzed anatomic details that influence procedure choice, and explored reoperation risk and survival after repair. Results Neonates who had conal resection or LVOT bypass had significantly smaller aortic valve and subaortic areas, as well as a trend toward a higher prevalence of type B IAA, aberrant right subclavian artery, and bicuspid aortic valve. Freedom from LVOT reoperation was 96% and 80%, at 1 and 8 years, respectively, and was lower after conal resection ( P = .008). Most LVOT reoperations (73%) were for discrete subaortic obstruction. Survival was 86% at 8 years, and was not related to procedure choice, whereas freedom from all-cause reoperation was 65% at 8 years, and was higher after standard repair ( P = .029). Conclusions Compared with published reports, this selective management strategy, which is customized to the degree of aortic valve and subaortic area narrowing, has mitigated and delayed LVOTO risk. With this tailored approach, most LVOT reoperations occur after infancy and are commonly for discrete subaortic obstruction. The effect of aortic valve and LVOT narrowing on increased LVOTO risk is neutralized with LVOT bypass procedures; however, it continues to be the highest after conal resection, suggesting the superiority of LVOT bypass, compared with enlargement in neonates who are at risk of developing LVOTO.

Objective Because infants undergoing a Norwood operation have poor interstage weight gain, we hypothesized that preemptive gastrostomy tube (GT) placement would result in earlier discharge, improved growth, and higher survival to stage 2. Study design Records of 74 neonates who underwent a Norwood operation were reviewed until stage 2 palliation. The patients were divided into conventional (n = 43) and preemptive GT groups (n = 31). Data included demographics, cardiac surgery, feeding strategy, length of hospitalization, and mortality. Results Transplant-free survival to stage 2 was significantly higher in the preemptive group, but there were no significant differences in survival to discharge after stage 1, length of hospitalization, and weight-for-age z-score at discharge and at stage 2 palliation. In the conventional group, 27 of 43 underwent GT placement, all via laparotomy, 23 with Nissen fundoplication. In the preemptive group, all underwent GT placement (21 laparoscopic, 10 laparotomy), 7 with Nissen fundoplication. A second gastric intervention was performed in 11 of 21 with laparoscopic GT (7 conversion to gastrojejunostomy tube, 4 Nissen fundoplication). Conclusion Preemptive GT placement is associated with improved survival to stage 2 after a Norwood operation but not with shorter hospitalization or better growth. A thorough gastrointestinal evaluation must be performed before GT placement to avoid additional surgery.