Improvement in Palivizumab Administration Prior to Discharge for Hospitalized Infants with Hemodynamically Significant Congenital Heart Disease: A Quality Improvement Initiative.

In this quality improvement initiative, we aimed to increase provider adherence with palivizumab administration guidelines for hospitalized infants with hemodynamically significant congenital heart disease. We included 470 infants over four respiratory syncytial virus (RSV) seasons from 11/2017 to 03/2021 (baseline season: 11/2017-03/2018). Interventions included the following: education, including palivizumab in the sign-out template, identifying a pharmacy expert, and a text alert (seasons 1 and 2: 11/2018-03/2020) that was replaced by an electronic health record (EHR) best practice alert (BPA) in season 3 (11/2020-03/2021). The text alert and BPA prompted providers to add "Need for RSV immunoprophylaxis" to the EHR problem list. The outcome metric was the percentage of eligible patients administered palivizumab prior to discharge. The process metric was the percentage of eligible patients with "Need for RSV immunoprophylaxis" on the EHR problem list. The balancing metric was the percentage of palivizumab doses administered to ineligible patients. A statistical process control P-chart was used to analyze the outcome metric. The mean percentage of eligible patients who received palivizumab prior to hospital discharge increased significantly from 70.1% (82/117) to 90.0% (86/96) in season 1 and to 97.9% (140/143) in season 3. Palivizumab guideline adherence was as high or higher for those with "Need for RSV immunoprophylaxis" on the problem list than for those without it in most time periods. The percentage of inappropriate palivizumab doses decreased from 5.7% (n = 5) at baseline to 4.4% (n = 4) in season 1 and 0.0% (n = 0) in season 3. Through this initiative, we improved adherence with palivizumab administration guidelines for eligible infants prior to hospital discharge.

Initial Experience with Telemedicine for Interstage Monitoring in Infants with Palliated Congenital Heart Disease.

Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality; home monitoring programs have mitigated these risks. In 2019, we instituted telemedicine (TM) in our established Infant Single Ventricle Monitoring Program. All consecutive patients discharged following neonatal operation/intervention were monitored until subsequent stage 2 surgical palliation. We offered TM (synchronous video) visits as part of regularly scheduled follow-up, replacing at least one in-person primary care visit with a TM cardiologist visit. We tracked emergency department (ED) visits, hospitalizations, how TM identified clinical concerns, and whether use of TM prevented unnecessary ED visits or expedited in-person assessment. We assessed caregiver and clinician satisfaction. Between 8/2019 and 5/2020, we conducted 60 TM visits for 29 patients. Of 31 eligible patients, 2 families (6.9%) declined. Median monitoring time was 199 days (range 75-264) and median number of TM visits/patient was 2 (range 1-5). In 6 visits (10%), significant clinical findings were identified which avoided an ED visit. Five TM visits led to expedited outpatient assessments, of which 1 patient required hospitalization. There were no missed events or deaths. Median ED visits/patient/month were significantly lower compared to the same calendar period of the prior year (0.0 (0-2.5) vs. 0.4 (0-3.7), p = 0.0004). Caregivers and clinicians expressed high levels of satisfaction with TM. TM for this high-risk population is feasible and effective in identifying clinical concerns and preventing unnecessary ED visits. TM was particularly effective during the COVID-19 pandemic, allowing for easy adaptation of care to ensure patient safety in this fragile cohort.

Feasibility of Digital Stethoscopes in Telecardiology Visits for Interstage Monitoring in Infants with Palliated Congenital Heart Disease.

Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality. Interstage telecardiology visits (TCV) have been effective in identifying clinical concerns and preventing unnecessary emergency department visits in this high-risk population. We aimed to assess the feasibility of implementing auscultation with digital stethoscopes (DSs) during TCV and the potential impact on interstage care in our Infant Single Ventricle Monitoring & Management Program. In addition to standard home-monitoring practice for TCV, caregivers received training on use of a DS (Eko CORE attachment assembled with Classic II Infant Littman stethoscope). Sound quality of the DS and comparability to in-person auscultation were evaluated based on two providers' subjective assessment. We also evaluated provider and caregiver acceptability of the DS. From 7/2021 to 6/2022, the DS was used during 52 TCVs in 16 patients (median TCVs/patient: 3; range: 1-8), including 7 with hypoplastic left heart syndrome. Quality of heart sounds and murmur auscultation were subjectively equivalent to in-person findings with excellent inter-rater agreement (98%). All providers and caregivers reported ease of use and confidence in evaluation with the DS. In 12% (6/52) of TCVs, the DS provided additional significant information compared to a routine TCV; this expedited life-saving care in two patients. There were no missed events or deaths. Use of a DS during TCV was feasible in this fragile cohort and effective in identifying clinical concerns with no missed events. Longer term use of this technology will further establish its role in telecardiology.

Left Ventricular Measurements and Strain in Pediatric Patients Evaluated for Systemic Hypertension and the Effect of Adequate Anti-hypertensive Treatment.

Pediatric hypertension (HTN) is an epidemic that is associated with HTN in adulthood and adverse cardiovascular outcomes. We hypothesized that children with HTN would have left ventricular (LV) hypertrophy and abnormal LV global longitudinal strain (GLS) on echocardiogram and that these values would differ by weight, race, and HTN treatment. Data were collected from first visits to the HTN Program from 12/2011 to 9/2018, excluding patients with cardiac disease or heart transplantation. LV measurements including LV mass index (LVMI), LV GLS, and diastolic indices were compared between groups. Multivariable logistic regression was used to identify risk factors for an abnormal LVMI. There were 212 patients with an interquartile age range of 13-18 years. On univariate analysis, LVMI was higher in hypertensive, obese, and African American patients. LV strain was less negative in obese and African American patients. Adequately treated patients with HTN had a higher LVMI and a higher E/e' ratio compared to patients with no HTN. On multivariate analysis, only obesity was associated with an LVMI ≥ 95th percentile (OR 2.9, 95% CI 1.4, 5.8). LVMI is higher in hypertensive, obese, and African American patients; however, in the multivariate analysis, obesity was the only independent risk factor for an abnormal LVMI. LVMI was still higher in those adequately treated for HTN compared to patients without HTN, possibly due to concomitant obesity. Future studies should focus on subclinical changes in LV performance seen in obese and hypertensive patients and the impact on long-term health.

Sustained improvement in fellows' echocardiographic completeness through the coronavirus pandemic with a standardised imaging protocol.

First-year cardiology fellows must quickly learn basic competency in echocardiography during fellowship orientation. This educational process was disrupted in 2020 due to the coronavirus pandemic, as our hands-on echocardiography teaching transitioned from practice on paediatric volunteers to simulation-based training. We previously described an improvement in echocardiographic completeness after implementation of a standardised imaging protocol for the performance of acute assessments of ventricular function. Herein, we assessed whether this improvement could be sustained over the two subsequent years, including the fellowship year affected by the pandemic. Echocardiograms performed by first-year paediatric cardiology fellows to assess ventricular function were reviewed for completeness. The frequency with which each requested component was included was measured. A total demographic score (out of 7) and total imaging score (out of 23) were calculated. The pre-protocol years (2015-2017) were compared to the post-protocol years (2018-2020), and the pre-COVID years (2018-2019) were compared to the year affected by COVID (2020). There was a sustained improvement in completeness after protocol implementation with improvement in the demographic score (median increasing from 6 to 7, p < 0.001) and imaging score (median increasing from 13 to 16, p < 0.001). More individual components showed a statistically significant increase in frequency compared to our prior publication. The COVID pandemic resulted in very few differences in completeness. Demographic reporting improved modestly (p = 0.04); the imaging score was unchanged (p = 0.59). The only view obtained less frequently was the apical two-chamber view. A standardised imaging protocol allowed sustained improvements in echocardiographic completeness despite the disruption of fellowship orientation by COVID-19.

Characteristics of Interstage Death After Discharge from Stage I Palliation.

BACKGROUND: Interstage mortality (IM) remains high for patients with single-ventricle congenital heart disease (SVCHD) in the period between Stage 1 Palliation (S1P) and Glenn operation. We sought to characterize IM. METHODS: This was a descriptive analysis of 2184 patients with SVCHD discharged home after S1P from 60 National Pediatric Cardiology Quality Improvement Collaborative sites between 2008 and 2015. Patients underwent S1P with right ventricle-pulmonary artery conduit (RVPAC), modified Blalock-Taussig-Thomas shunt (BTT), or Hybrid; transplants were excluded. RESULTS: IM occurred in 153 (7%) patients (median gestational age 38 weeks, 54% male, 77% white), at 88 (IQR 60,136) days of life, and 39 (IQR 17,84) days after hospital discharge; 13 (8.6%) occurred ≤ 30 days after S1P. The mortality rate for RVPAC was lower (5.2%; 59/1138) than BTT (9.1%; 65/712) and Hybrid (20.1%; 27/134). More than half of deaths occurred at home (20%) or in the emergency department (33%). The remainder occurred while inpatient at center of S1P (cardiac intensive care unit 36%, inpatient ward 5%) or at a different center (5%). Fussiness and breathing problems were most often cited as harbingers of death; distance to surgical center was the biggest barrier cited to seeking care. Cause of death was unknown in 44% of cases overall; in the subset of patients who underwent post-mortem autopsy, the cause of death remained unknown in 30% of patients, with the most common diagnosis being low cardiac output. CONCLUSIONS: Most IM occurred in the outpatient setting, with non-specific preceding symptoms and unknown cause of death. These data indicate the need for research to identify occult causes of death, including arrhythmia.

Large aneurysms and pseudoaneurysms of surgically reconstructed right ventricular outflow tracts.

Aneurysm and pseudoaneurysm development is a known, albeit uncommon, complication after right ventricular outflow tract surgical reconstruction. Large right ventricular outflow tract aneurysms and pseudoaneurysms have not been extensively described in recent literature and we report our experience with this unusual complication in five patients at our institution over the last 8 years. Although uncommon, this complication has potentially important clinical implications. Thus, clinicians should be aware of its potential, particularly in certain anatomic conditions.

Standardization of the Perioperative Management for Neonates Undergoing the Norwood Operation for Hypoplastic Left Heart Syndrome and Related Heart Defects.

OBJECTIVES: In-hospital complications after the Norwood operation for single ventricle heart defects account for the majority of morbidity and mortality. Inpatient care variation occurs within and across centers. This multidisciplinary quality improvement project standardized perioperative management in a large referral center. DESIGN: Quality improvement project. SETTING: High volume cardiac center, tertiary care children's hospital. PATIENTS: Neonates undergoing Norwood operation. INTERVENTIONS: The quality improvement team developed and implemented a clinical guideline (preoperative admission to 48 hr after surgery). The composite process metric, Guideline Adherence Score, contained 13 recommendations in the guideline that reflected consistent care for all patients. MEASUREMENTS AND MAIN RESULTS: One-hundred two consecutive neonates who underwent Norwood operation (January 1, 2013, to July 12, 2016) before guideline implementation were compared with 50 consecutive neonates after guideline implementation (July 13, 2016, to May 4, 2018). No preguideline operations met the goal Guideline Adherence Score. In the first 6 months after guideline implementation, 10 of 12 operations achieved goal Guideline Adherence Score and continued through implementation, reaching 100% for the last 10 operations. Statistical process control analysis demonstrated less variability and decreased hours of postoperative mechanical ventilation and cardiac ICU length of stay during implementation. There were no statistically significant differences in major hospital complications or in 30-day mortality. A higher percentage of patients were extubated by postoperative day 2 after guideline implementation (67% [30/47] vs 41% [41/99], respectively; p = 0.01). Of these patients, reintubation within 72 hours of extubation significantly decreased after guideline implementation (0% [0/30] vs 17% [7/41] patients, respectively; p = 0.02). CONCLUSIONS: This initiative successfully implemented a standardized perioperative care guideline for neonates undergoing the Norwood operation at a large center. Positive statistical process control centerline shifts in Guideline Adherence Score, length of postoperative mechanical ventilation, and cardiac ICU length of stay were demonstrated. A higher percentage were successfully extubated by postoperative day 2. Establishment of standard processes can lead to best practices to decrease major adverse events.

Echocardiographic strain analysis reflects impaired ventricular function in youth with pediatric-onset systemic lupus erythematosus.

BACKGROUND: Strain analysis with speckle-tracking echocardiography shows promise as a screening tool for silent myocardial dysfunction in pediatric-onset systemic lupus erythematosus (pSLE). We compared left ventricular (LV) systolic deformation (measured by strain) in children and adolescents with pSLE to controls, and assessed the relationship between strain, disease activity, and other noninvasive measures of cardiovascular health. METHODS: Twenty pSLE subjects ages 9-21 underwent comprehensive cardiovascular testing, including 2D speckle-tracking echocardiography, ambulatory blood pressure monitoring (ABPM), peripheral endothelial function testing, pulse wave velocity and analysis, and carotid ultrasound. Longitudinal apical-4 chamber (LSA4C ) and midpoint circumferential strain (CSmid ) were compared to that of 70 healthy controls using multivariable linear regression. Among pSLE subjects, Pearson correlation coefficients were calculated to evaluate relationships between global longitudinal or circumferential strain and other measures of cardiovascular health. RESULTS: Average SLE disease duration was 3.2 years (standard deviation [SD] 2.1). 2/20 pSLE subjects had persistent disease activity, and only one met criteria for hypertension by ABPM. LSA4C was significantly reduced in pSLE subjects compared to controls (mean -18.3 [SD 3.2] vs -21.8% [SD 2.2], P-value <.001). There was no significant difference in CSmid (-24.8 [SD 3.7] vs -25.7% [SD 3.4], P = .29). Among pSLE subjects, decreased nocturnal blood pressure dipping on ABPM was associated with reduced global circumferential strain (r -0.59, P = .01). CONCLUSIONS: Longitudinal myocardial deformation is impaired in pSLE patients despite clinical remission and may represent early myocardial damage. Strain analysis should be considered in addition to standard echocardiographic assessment during follow-up of patients with pSLE.

A standardized imaging protocol improves quality and reduces practice variability in echocardiographic assessment of ventricular function by first-year pediatric cardiology fellows.

BACKGROUND: Echocardiography education for pediatric cardiology fellows has been a recent focus leading to the implementation of "boot camps." Less is described about continuing education through fellowship and improving image quality. We noticed practice variation in echocardiograms assessing ventricular function performed on nights and weekends. Thus, we implemented a standardized protocol and assessed its impact on imaging and reporting completeness. METHODS: We created an imaging protocol for the assessment of ventricular function in the acute setting. The protocol included demographic information, a list of images to be obtained, and the methods to quantify ventricular function. The protocol was explained to first-year fellows and distributed on an electronic quick reference card. Echocardiograms independently performed by first-year fellows during their first 4 months of on-call time were assessed pre- and postintervention using a standard rubric. RESULTS: Compliance with demographic reporting was high pre- and postintervention, but significantly improved after the standardized protocol (P < 0.001). Use of the protocol increased the median number of unique images obtained per echocardiogram from 13 to 17 (out of 23 required views, P < 0.001). Particularly improved was the performance of quantitative evaluations of function, including Simpson's method for left ventricular ejection fraction (four chamber: 40% vs 67%, P < 0.001; two chamber: 33% vs 67%, P < 0.001) and tricuspid annular plane systolic excursion (45% vs 80%, P < 0.001). CONCLUSIONS: The introduction of a standardized imaging protocol and its distribution to first-year fellows resulted in improvements in echocardiographic reporting completeness and increased the quality of information obtained by providing more quantitative assessments of ventricular function.

Echocardiographic Screening of Cardiovascular Status in Pediatric Sickle Cell Disease.

Although elevated right ventricular pressure and left ventricular diastolic dysfunction measured by echocardiogram are independent predictors of death in adults with sickle cell disease (SCD), the utility of routine echocardiographic screening in the pediatric population is controversial. We performed a 3-year retrospective review of children ≥ 10 years of age with SCD who underwent an outpatient transthoracic echocardiogram as part of a screening program. Of 172 patients referred for screening, 105 (61%) had a measurable tricuspid regurgitation jet velocity (TRV): median 2.4 m/s (IQR 2.3-2.5). Elevated right ventricular (RV) pressure (TRV ≥ 2.5 m/s, 25 mmHg), documented in 30% (32/105), was significantly associated with chronic transfusion therapy and elevated lactate dehydrogenase. Left ventricle (LV) dilation, documented in 25% (44/172), was significantly associated with lower hemoglobin, and higher reticulocyte count, lactate dehydrogenase level, and bilirubin level. There was no association between elevated right ventricular pressure or left ventricle dilation and indices of biventricular systolic or diastolic function. The one death in the cohort during the study period had normal echocardiographic findings. In conclusion, mild RV pressure elevation and LV dilation in children with SCD is associated with abnormal laboratory markers of disease severity, but not with ventricular dysfunction over the 3-year study period.

Evaluating the utility of routine screening catheterisation before interstage discharge of infants with single-ventricle physiology.

INTRODUCTION: Interstage mortality causes are often unknown in infants with shunt-dependent univentricular defects. For 2 years, screening catheterisation was encouraged before neonatal discharge to determine if routine evaluation improved interstage outcomes. METHODS: Retrospective single-centre review of home monitoring programme from December, 2010 to June, 2012. Composite scores were created for physical examination/echocardiography risk factors; catheterisation risk factors; and interstage adverse events. Composite scores were compared between usual care and screening catheterisation groups. The ability of each risk factor composite to predict interstage adverse events, individually and in combination, was assessed with sensitivity, specificity, and receiver operating characteristic curves. RESULTS: There were 27 usual care and 32 screening catheterisation patients. There were no significant differences between groups except rates of catheterisation before discharge (29.6 versus 100%, p < 0.001). Usual care patients who underwent catheterisation for clinical indications had higher intervention rates (37.5 versus 3.1%, p = 0.004). Physical examination/echocardiography risk factor frequency was similar, but usual care patients with catheterisation had a higher catheterisation risk factor frequency. Interstage adverse event frequency was similar (48.2 versus 53.1%, p = 0.7). For interstage adverse event prediction, sensitivity for the physical examination/echocardiography, catheterisation, and either risk factor composites was 53.3, 72, and 80%, respectively; specificity was 59, 60, and 48%. The area under the receiver operating characteristic curve was 0.56, 0.66, and 0.64. CONCLUSION: Screening catheterisation evaluation offered slightly increased sensitivity and specificity, but no difference in interstage adverse event frequency. Given this small advantage versus known risks, screening catheterisations are no longer encouraged.

Intramural Ventricular Septal Defect Is a Distinct Clinical Entity Associated With Postoperative Morbidity in Children After Repair of Conotruncal Anomalies.

BACKGROUND: Intramural ventricular septal defects (VSDs) are interventricular communications through right ventricular free wall trabeculations that can occur after repair of conotruncal anomalies. We assessed the prevalence of residual intramural VSDs and their effect on postoperative course. METHODS AND RESULTS: Children who underwent biventricular repair of a conotruncal anomaly from January 1, 2006, to June 30, 2013, and had a postoperative transthoracic echocardiogram were included. Images were reviewed for residual intramural or nonintramural VSDs. The primary outcome was a composite of mortality, extracorporeal membrane oxygenation use, and need for subsequent catheter or surgical VSD closure. The secondary outcome was postoperative hospital length of stay. A residual VSD was present in 256 of the 442 subjects (58%), of which 231 (90%) were <2 mm in size. Forty-nine patients (11%) had intramural VSDs, and 207 (47%) had nonintramural VSDs. Patients with intramural VSDs were more likely to reach the primary composite outcome compared with those with nonintramural VSDs or no residual VSD (14 of 49 [29%] versus 15 of 207 [7%] versus 6 of 186 [3%]; P<0.0001). In addition, those with intramural VSDs had longer postoperative hospital length of stay compared with those with nonintramural VSDs or no residual VSD (20 days [interquartile range, 11-42 days] versus 7 days [interquartile range, 5-14 days] versus 6 days [interquartile range, 4-11 days]; P=0.0001). These associations remained significant after adjustment for known risk factors for poor outcomes, including residual VSD size and operative complexity. CONCLUSIONS: Among residual VSDs after repair of conotruncal anomalies, intramural VSDs are uniquely associated with postoperative morbidity, mortality, and longer postoperative hospital length of stay. It is important to recognize intramural VSDs in the postoperative period.

The role of echocardiography in the intraoperative management of the fetus undergoing myelomeningocele repair.

INTRODUCTION: Fetal surgery for myelomeningocele (MMC) results in better outcomes compared to postnatal treatment. However, risks are present. We describe our experience with intraoperative fetal echocardiography during repair of MMC and report on the management of serious cardiovascular events. MATERIAL AND METHODS: The subjects included fetuses with intent to repair MMC from January 2011 to February 2014. The protocol involved continuous echocardiography in a looping, sequential manner of systolic function, heart rate and tricuspid and mitral valve regurgitation. RESULTS: A total of 101 cases intended fetal MMC repair; 100 completed surgery. Intraoperative ventricular dysfunction was present in 60% (20 mild, 25 moderate, 15 severe). Heart rate <100 bpm was noted in 11 cases. Tricuspid valve regurgitation was present in 35% (26 mild, 7 moderate, 2 severe); mitral valve regurgitation was present in 19% (15 mild, 4 moderate). Serious cardiovascular events were experienced in 7 cases, which affected the conduct of surgery and/or outcome. In 4 of these, medications were given via the umbilical vein and external cardiac compressions were performed. Fetal echocardiography was used to gauge the efficacy of compressions and to guide resuscitation. DISCUSSION: Cardiovascular compromise is common during fetal surgery for MMC. Intraoperative fetal echocardiography is recommended as a growing number of centers contemplate offering this form of novel, but potentially risky, therapy.

Consensus-Based Development of a Pediatric Echocardiography Complexity Score: Design, Rationale, and Results of a Quality Improvement Collaborative.

BACKGROUND: The complexity of congenital heart disease has been primarily stratified on the basis of surgical technical difficulty, specific diagnoses, and associated outcomes. We report on the refinement and validation of a pediatric echocardiography complexity (PEC) score. METHODS AND RESULTS: The American College of Cardiology Quality Network assembled a panel from 12 centers to refine a previously published PEC score developed in a single institution. The panel refined complexity categories and included study modifiers to account for complexity related to performance of the echocardiogram. Each center submitted data using the PEC scoring tool on 15 consecutive inpatient and outpatient echocardiograms. Univariate and multivariate analyses were performed to assess for independent predictors of longer study duration. Among the 174 echocardiograms analyzed, 68.9% had underlying congenital heart disease; 44.8% were outpatient; 34.5% were performed in an intensive care setting; 61.5% were follow-up; 46.6% were initial or preoperative; and 9.8% were sedated. All studies had an assigned PEC score. In univariate analysis, longer study duration was associated with several patient and study variables (age <2 years, PEC 4 or 5, initial study, preoperative study, junior or trainee scanner, and need for additional imaging). In multivariable analysis, a higher PEC score of 4 or 5 was independently associated with longer study duration after controlling for study variables and center variation. CONCLUSIONS: The PEC scoring tool is feasible and applicable in a variety of clinical settings and can be used for correlation with diagnostic errors, allocation of resources, and assessment of physician and sonographer effort in performing, interpreting, and training in pediatric echocardiography.

Chest pain with elevated troponin assay in adolescents.

OBJECTIVE: We sought to describe the evaluation, treatment, and follow-up of adolescents who presented to a single institution with chest pain and an elevated troponin I value in the absence of typical symptoms of pericarditis or myocarditis. Materials and methods We performed a retrospective review of patients in the age group of 10-18 years of age with no history of significant heart disease admitted to our institution from 2000 to 2010 after presenting with chest pain and an elevated troponin I value. RESULTS: A total of 16 patients were identified with a median age of 16.5 years (range 11.2-17.8 years). Of these 13 (81%) were male and 10 (63%) showed evidence of localised ST elevations on electrocardiogram. The median peak troponin I level was 17.8 nanograms per millilitre (range 0.89-227, normal less than 0.4). There were eight patients (50%) with a diagnosis of coronary vasospasm, three patients (20%) with atypical myopericarditis, one patient with coronary anomaly, one patient with hypercoagulable disorder, and one patient with prolonged supraventricular tachycardia. In two patients, no definitive diagnosis was made. There was one patient who needed catheter-based intervention, which involved stenting of a coronary artery after a procedure-related complication. CONCLUSIONS: In our cohort of adolescents without history of significant cardiac disease, chest pain and elevated troponin I levels were attributed to a variety of causes. Although coronary vasospasm and atypical myopericarditis were seen most commonly, coronary anomaly was identified in one case. Magnetic resonance imaging proved a useful diagnostic tool to assess coronary artery anatomy and myocardial changes suggestive of myocarditis. On the basis of these results and a review of the literature, a general evaluation algorithm is presented.

Association of Home Monitoring and Unanticipated Interstage Readmissions in Infants With Hypoplastic Left Heart Syndrome.

Background The impact of home monitoring on unanticipated interstage readmissions in infants with hypoplastic left heart syndrome has not been previously studied. We sought to examine the association of our institution's Infant Single Ventricle Management and Monitoring Program (ISVMP) with readmission frequency, cumulative readmission days, and readmission illness severity and to identify patient-level risk factors for readmission. Methods and Results We performed a retrospective single-center cohort study comparing infants with hypoplastic left heart syndrome enrolled in ISVMP (December 2010-December 2019) to historical controls (January 2007-November 2010). The primary outcome was number of readmissions per interstage days. Secondary outcomes were cumulative interstage readmission days and occurrence of severe readmissions. Inverse probability weighted and multivariable generalized linear models were used to examine the association between ISVMP and the outcomes. We compared 198 infants in the ISVMP to 128 historical controls. Infants in the ISVMP had more than double the risk of interstage readmission compared with controls (adjusted incidence rate ratio, 2.38 [95% CI, 1.50-3.78]; P=0.0003). There was no difference in cumulative interstage readmission days (adjusted incidence rate ratio, 1.02 [95% CI, 0.69-1.50]; P=0.90); however, infants in the ISVMP were less likely to have severe readmissions (adjusted odds ratio, 0.28 [95% CI, 0.11-0.68]; P=0.005). Other factors independently associated with number of readmissions included residing closer to our center, younger gestational age, genetic syndrome, and discharge on exclusive enteral feeds. Conclusions Infants in the ISVMP had more frequent readmissions but comparable readmission days and fewer severe unanticipated readmissions. These findings suggest that home monitoring can reduce interstage morbidity without increasing readmission days.

Appropriateness of cardiovascular computed tomography and magnetic resonance imaging in patients with conotruncal defects.

BACKGROUND: To promote the rational use of cardiovascular imaging in patients with congenital heart disease, the American College of Cardiology developed Appropriate Use Criteria (AUC), but its clinical application and pre-release benchmarks have not been evaluated. We aimed to evaluate the appropriateness of indications for cardiovascular magnetic resonance (CMR) and cardiovascular computed tomography (CCT) in patients with conotruncal defects and to identify factors associated with maybe or rarely appropriate (M/R) indications. METHODS: Twelve centers each contributed a median of 147 studies performed prior to AUC publication (01/2020) on patients with conotruncal defects. To incorporate patient characteristics and center-level effects, a hierarchical generalized linear mixed model was used. RESULTS: Of the 1753 studies (80% CMR, and 20% CCT), 16% were rated M/R. Center M/R ranged from 4 to 39%. Infants accounted for 8.4% of studies. In multivariable analyses, patient- and study-level factors associated with M/R rating included: age <1 year (OR 1.90 [1.15-3.13]), truncus arteriosus (vs. tetralogy of Fallot, OR 2.55 [1.5-4.35]), and CCT (vs. CMR, OR 2.67 [1.87-3.83]). None of the provider- or center-level factors reached statistical significance in the multivariable model. CONCLUSIONS: Most CMRs and CCTs ordered for the follow-up care of patients with conotruncal defects were rated appropriate. However, there was significant center-level variation in appropriateness ratings. Younger age, CCT, and truncus arteriosus were independently associated with higher odds of M/R rating. These findings could inform future quality improvement initiatives and further exploration of factors resulting in center-level variation.

Speckle tracking-derived myocardial tissue deformation imaging in twin-twin transfusion syndrome: differences in strain and strain rate between donor and recipient twins.

OBJECTIVES: Twin-twin transfusion syndrome (TTTS) is a complex disorder with altered cardiovascular loading conditions that affects both donors and recipients. Myocardial tissue deformation analysis using vector velocity imaging is an angle-independent, speckle-tracking technique which can assess myocardial mechanics and may provide insight into cardiac dysfunction in TTTS. METHODS: Digital dynamic two-dimensional four-chamber views were interrogated offline. Images were acquired utilizing standard video frame rates (30 frames/s). The global longitudinal strain, systolic strain rate, and diastolic strain rate were measured in the left (LV) and right ventricles (RV) of 25 fetal pairs with TTTS and compared to 25 gestational age-matched normal controls. Pulsatility indices for the umbilical artery and middle cerebral artery were measured. RESULTS: The gestational age at evaluation was 20.5 ± 1.3 weeks. The donor LV systolic strain rate was higher, while the donor RV diastolic strain rate was significantly lower, than control values. The recipient longitudinal strain, systolic strain rate, and diastolic strain rate were significantly lower for both LV and RV in comparison to controls. The donor umbilical artery pulsatility index was higher than control values (1.92 ± 0.45 vs. 1.41 ± 0.25, p < 0.001), while the donor middle cerebral artery pulsatility index was lower (1.46 ± 0.28 vs. 1.87 ± 0.21). Recipient umbilical artery and middle cerebral artery pulsatility indices were no different than control values. CONCLUSIONS: In TTTS, both the donor and the recipient exhibit abnormalities of myocardial tissue deformation with ventricle-specific changes evident based on loading conditions. Donor LV systolic function is hyperdynamic due to hypovolemia and selective ejection into a low-resistance cerebrovascular circuit while the donor RV selectively ejects into a high-resistance placental circuit. Recipient RV and LV are both globally depressed with systolic and diastolic dysfunction. Further prospective validation of our findings using high frame rate analysis is indicated.

Prevalence of Patent Foramen Ovale in a Cohort of Children With Cryptogenic Ischemic Stroke.

BACKGROUND AND OBJECTIVES: To determine the significance of patent foramen ovale (PFO) in childhood stroke, we compared PFO prevalence, PFO features, and stroke recurrence risk in 25 children with cryptogenic arterial ischemic stroke (AIS), 54 children with AIS from a known etiology, and 209 healthy controls. METHODS: We performed a case-control analysis of a 14-year prospectively enrolled single-center cohort of children with AIS who underwent transthoracic echocardiogram (TTE) and compared them to TTEs of otherwise healthy children evaluated for benign cardiac concerns. Stroke patients 29 days to 18 years of age at stroke ictus with confirmed acute AIS on imaging, availability of complete diagnostic studies of stroke risk factors, including TTE images available for central review, and at least 1 follow-up evaluation after index stroke were included. Presence of PFO and high-risk PFO features were assessed by 2 independent, blinded reviewers and compared between groups with the Fisher exact test. Stroke/TIA recurrence risk was determined from Cox proportional hazards models. RESULTS: Of 154 children with first-ever AIS, 79 were eligible; 25 had cryptogenic AIS, and 54 had a known cause. PFO prevalence was higher in the cryptogenic group (7, 28%) compared to both the known stroke etiology group (3, 5.6%, p = 0.009) and controls without stroke (24, 11.5%, p = 0.03). There were no significant differences in presence of right-to-left shunt and atrial septal aneurysm. Median follow-up time for entire stroke cohort was 20.9 months. Stroke-free recurrence at 2-years did not differ between children with and without PFO (HR 2.0, 95% CI 0.4-9.3, p = 0.39). DISCUSSION: PFO prevalence was higher in children with cryptogenic stroke compared to patients with AIS with known etiology and healthy controls. PFO was not associated with increased recurrence risk. Optimal secondary preventive treatment in children with cryptogenic stroke and PFO remains uncertain and requires further study. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that children with cryptogenic ischemic stroke have an increased frequency of PFO compared to children with ischemic stroke of known etiology and healthy controls.