Kenan W.D. Stern

Nutrition algorithms for infants with hypoplastic left heart syndrome; birth through the first interstage period.

Failure to thrive is common in infants with hypoplastic left heart syndrome and its variants and those with poor growth may be at risk for worse surgical and neurodevelopmental outcomes. The etiology of growth failure in this population is multifactorial and complex, but may be impacted by nutritional intervention. There are no consensus guidelines outlining best practices for nutritional monitoring and intervention in this group of infants. The Feeding Work Group of the National Pediatric Cardiology Quality Improvement Collaborative performed a literature review and assessment of best nutrition practices from centers participating in the collaborative in order to provide nutritional recommendations and levels of evidence for those caring for infants with single ventricle physiology.

Pediatric Appropriate Use Criteria Implementation Project: A Multicenter Outpatient Echocardiography Quality Initiative

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 <1 year of age than in those >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 < 0.001). 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.

Echocardiographic Evaluation Before Bidirectional Glenn Operation in Functional Single-Ventricle Heart Disease Comparison to Catheter Angiography

Background— Cardiac catheterization is routinely performed in patients with single ventricle before bidirectional Glenn operation (BDG). There is interest in noninvasive evaluation alone before BDG, but concern for echocardiography successfully imaging the relevant anatomy persists. We evaluated the accuracy of echocardiographic imaging of vascular anatomy. Methods and Results— Diagnostic images of 130 patients who had echocardiography and catheterization before BDG were reviewed; diameters of the pulmonary arteries (PAs) and aortic arch were measured, and stenoses were recorded. Patient and procedural factors associated with echocardiographic imaging were analyzed. Median age at echocardiography was 4 months; the most common diagnosis was hypoplastic left heart syndrome (55%). The left PA was imaged by echocardiography in 83 patients (64%), with 4 of 21 stenoses (19%) diagnosed by catheterization identified; similarly, the right PA was imaged in 81 (62%), and 3 of 17 stenoses (18%) were identified. The distal aortic arch was visualized in 104 (80%), with successful identification of 21 of 27 (78%) of coarctations diagnosed by catheterization. Complete vascular echocardiography (visualization of PAs and aortic arch) occurred in 43% and was not obtained more frequently with sedation. Conclusions— In a large cohort of patients presenting for BDG, evaluation by echocardiography frequently failed to image the PAs and missed the majority of PA stenoses. Sedation did not appear to improve the performance of echocardiography for evaluation of the PAs. Echocardiography cannot be relied on as the sole investigation before BDG.

Educational intervention for improving the appropriateness of transthoracic echocardiograms ordered by pediatric cardiologists: Sachdeva et al.

OBJECTIVE The objective of this study was to evaluate effectiveness of educational intervention (EI) in the Pediatric Appropriate Use of Echocardiography (PAUSE) study to improve appropriateness of transthoracic echocardiograms (TTEs) ordered in pediatric cardiology clinics. DESIGN Data were prospectively collected after the publication of the Appropriate Use Criteria (AUC) document during 2 phases: the pre-EI phase (1/1/15 to 4/30/15) and the post-EI phase (7/1/15 to 10/30/15). Pre-EI, site-investigators (SI) determined AUC indications, by reviewing the clinic records. Post-EI, providers assigned indications prior to obtaining TTE. SETTING Pediatric cardiology clinics at six centers. PATIENTS Those ≤18 years old, receiving initial outpatient TTE. INTERVENTIONS EI included (i) sharing the pre-EI appropriateness ratings with providers, (ii) lecture on AUC, (iii) providers self-assigning indications, and (iv) monthly e-mail feedback by SI to individual providers. OUTCOME The primary outcome measure was a change in the proportion of studies for indications rated R following EI. RESULTS Of the 4542 TTEs (1907 pre-EI, 2635 post-EI) ordered by 90 physicians, overall comparison of appropriateness ratings before and after EI showed an increase in Appropriate (72.5%-76.2%, P = .004), no change in May Be Appropriate, and a decline in Rarely Appropriate (R) from 9.6% to 7.4%, P = .008. Following EI, a significant decline in R was observed only in three centers and EI did not affect the variation in TTEs ordered for R indications among physicians (P = .467). Physicians with the highest proportion of TTEs ordered for R before EI, showed the most significant decline in R. CONCLUSIONS Appropriateness of pediatric outpatient TTE varies substantially by center. A customized EI resulted in modest improvement in the appropriateness of TTEs in the PAUSE study, with an increase in Appropriate and a decrease in R TTEs. Multifaceted EIs are required to improve adherence to national standards such as AUC.

Application of pediatric appropriate use criteria for initial outpatient evaluation of syncope

Syncope is a common reason for outpatient transthoracic echocardiography (TTE). We studied the applicability of pediatric appropriate use criteria (AUC) on initial outpatient evaluation of children (≤18 years) with syncope.

Application of the Pediatric Appropriate Use Criteria for Chest Pain

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.

Factors Influencing Pediatric Outpatient Transthoracic Echocardiography Utilization Before Appropriate Use Criteria Release: A Multicenter Study

Background: 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. Methods: 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 TTEs being performed for rarely appropriate and TTEs not being performed for appropriate indications. Results: Concordance between AUC and TTE utilization was 88%. TTE was performed for rarely appropriate indications in 9% and was associated with patient age < 3 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. Conclusions: 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. HighlightsLittle is known about baseline TTE utilization patterns and how well they align with the pediatric AUC.Concordance between pediatric AUC and TTE ordering was 88%.Among discordant ordering, the highest rates of TTE for rarely appropriate indications were in patients less than 3 months of age. An abnormal prior test result (primary abnormal electrocardiographic findings) was the indication most strongly associated with not ordering TTE for appropriate indications.There was high variability in concordance between AUC and TTE utilization between the different participating centers. Abbreviations: AUC = Appropriate use criteria; TTE = Transthoracic echocardiography.

Utility of a standardized postcardiopulmonary bypass epicardial echocardiography protocol for stage I Norwood palliation

OBJECTIVE Stage 1 Norwood palliation is one of the highest risk procedures in congenital cardiac surgery. Patients with superior technical performance scores have more favorable outcomes. Intraoperative epicardial echocardiography may allow the surgeon to address residual lesions prior to leaving the operating room, resulting in improved technical performance. The ability of intraoperative epicardial echocardiography to visualize the relevant anatomy and its association with outcomes is not known. DESIGN A standardized intraoperative epicardial echocardiography protocol was developed and performed at the conclusion of Stage 1 Norwood palliation. Data pertaining to visualization of relevant anatomy, and comparison of intraoperative echocardiogram findings with other postoperative investigations was performed. Clinical outcomes, including technical performance, were collected. A historical cohort who received either no echocardiogram or a nonstandardized examination was used as a comparison group. RESULTS Thirty on-protocol and 30 preprotocol patients, 22 of whom had a nonstandardized intraoperative epicardial echocardiogram, were studied. Compared with preprotocol, visualization of the relevant anatomy was significantly increased for the Damus-Kaye-Stansel anastomosis (93% vs. 68% P = .03) and branch pulmonary arteries (70% vs. 36%, P = .02). One residual lesion requiring immediate operative reintervention was diagnosed in the preprotocol group. There were 5 patients in each cohort with residual lesions during the postoperative hospitalization that were not appreciated on the intraoperative echocardiogram. Technical performance, rates of reintervention and clinical outcomes were not significantly different between the two groups. CONCLUSIONS Intraoperative epicardial echocardiography is technically feasible and increases visualization of the relevant anatomy. Larger investigations may be warranted to determine if there is clinical benefit to such an approach.

APPLICATION OF PEDIATRIC APPROPRIATE USE CRITERIA FOR OUTPATIENT EVALUATION OF SYNCOPE BY PEDIATRIC CARDIOLOGISTS

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

Echocardiographic Evaluation Prior to Bidirectional Glenn Operation in Functional Single Ventricle Heart Disease: Comparison to Catheter Angiography

Background— Cardiac catheterization is routinely performed in patients with single ventricle before bidirectional Glenn operation (BDG). There is interest in noninvasive evaluation alone before BDG, but concern for echocardiography successfully imaging the relevant anatomy persists. We evaluated the accuracy of echocardiographic imaging of vascular anatomy. Methods and Results— Diagnostic images of 130 patients who had echocardiography and catheterization before BDG were reviewed; diameters of the pulmonary arteries (PAs) and aortic arch were measured, and stenoses were recorded. Patient and procedural factors associated with echocardiographic imaging were analyzed. Median age at echocardiography was 4 months; the most common diagnosis was hypoplastic left heart syndrome (55%). The left PA was imaged by echocardiography in 83 patients (64%), with 4 of 21 stenoses (19%) diagnosed by catheterization identified; similarly, the right PA was imaged in 81 (62%), and 3 of 17 stenoses (18%) were identified. The distal aortic arch was visualized in 104 (80%), with successful identification of 21 of 27 (78%) of coarctations diagnosed by catheterization. Complete vascular echocardiography (visualization of PAs and aortic arch) occurred in 43% and was not obtained more frequently with sedation. Conclusions— In a large cohort of patients presenting for BDG, evaluation by echocardiography frequently failed to image the PAs and missed the majority of PA stenoses. Sedation did not appear to improve the performance of echocardiography for evaluation of the PAs. Echocardiography cannot be relied on as the sole investigation before BDG.