Right Heart Remodeling After Pulmonary Valve Replacement in Patients With Pulmonary Atresia or Critical Stenosis With Intact Ventricular Septum.

Background Patients with pulmonary atresia or critical pulmonary stenosis with intact ventricular septum (PA/IVS) and biventricular circulation may require pulmonary valve replacement (PVR). Right ventricular (RV) remodeling after PVR is well described in tetralogy of Fallot (TOF); we sought to investigate RV changes in PA/IVS using cardiac magnetic resonance imaging. Methods and Results A retrospective cohort of patients with PA/IVS who underwent PVR at Boston Children's Hospital from 1995 to 2021 with cardiac magnetic resonance imaging before and after PVR was matched 1:3 with patients with TOF by age at PVR. Median regression modeling was performed with post-PVR indexed RV end-diastolic volume as the primary outcome. A total of 20 patients with PA/IVS (cases) were matched with 60 patients with TOF (controls), with median age at PVR of 14 years. Pre-PVR indexed RV end-diastolic volume was similar between groups; cases had higher RV ejection fraction (51.4% versus 48.6%; P=0.03). Pre-PVR RV free wall and left ventricular (LV) longitudinal strain were similar, although LV midcavity circumferential strain was decreased in cases (-15.6 versus -17.1; P=0.001). At a median of 2 years after PVR, indexed RV end-diastolic volume was similarly reduced; cases continued to have higher RV ejection fraction (52.3% versus 46.9%; P=0.007) with less reduction in RV mass (Δ4.5 versus 9.6 g/m2; P=0.004). Post-PVR, RV and LV longitudinal strain remained unchanged, and LV circumferential strain was similar, although lower in cases. Conclusions Compared with patients with TOF, patients with PA/IVS demonstrate similar RV remodeling after PVR, with lower reduction in RV mass and comparatively higher RV ejection fraction. Although no differences were detected in peak systolic RV or LV strain values, further investigation of diastolic parameters is needed.

Native Bicuspid Pulmonary Valve in D-Loop Transposition of the Great Arteries: Outcomes of the Neo-Aortic Valve Function and Root Dilation After Arterial Switch Operation.

Background Neo-aortic root dilation and neo-aortic regurgitation (AR) are common after arterial switch operation for D-loop transposition of the great arteries. We sought to evaluate these outcomes in patients with bicuspid native pulmonary valve (BNPV). Methods and Results A retrospective analysis of patients with transposition of the great arteries undergoing arterial switch operation at Boston Children's Hospital from 1989 to 2018 matched BNPV patients 1:3 with patients with tricuspid native pulmonary valve by year of arterial switch operation. Kaplan-Meier analyses with log-rank test compared groups for time to first neo-aortic valve reoperation, occurrence of ≥moderate AR, and neo-aortic root dilation (root z score ≥4). A total of 83 patients with BNPV were matched with 217 patients with tricuspid native pulmonary valve. Patients with BNPV more often had ventricular septal defects (73% versus 43%; P<0.001). Hospital length of stay (11 versus 10 days) and 30-day surgical mortality (3.6% versus 2.8%) were similar. During median 11 years follow-up, neo-aortic valve reoperation occurred in 4 patients with BNPV (6.0%) versus 6 patients with tricuspid native pulmonary valve (2.8%), with no significant difference in time to reoperation. More BNPV had AR at discharge (4.9% versus 0%; P=0.014) and during follow-up (13.4% versus 4.3%; hazard ratio [HR], 3.9; P=0.004), with shorter time to first occurrence of AR; this remained significant after adjusting for ventricular septal defects. Similarly, neo-aortic root dilation was more common in BNPV (45% versus 38%; HR, 1.64; P=0.026) with shorter time to first occurrence. Conclusions While patients with BNPV have similar short-term arterial switch operation outcomes, AR and neo-aortic root dilation occur more frequently and earlier compared with patients with tricuspid native pulmonary valve. Further long-term studies are needed to determine whether this results in greater need for neo-aortic valve reoperation.

Long-Term Fate of the Truncal Valve.

Background Long-term survival in patients with truncus arteriosus is favorable, but there remains significant morbidity associated with ongoing reinterventions. We aimed to study the long-term outcomes of the truncal valve and identify risk factors associated with truncal valve intervention. Methods and Results We retrospectively reviewed patients who underwent initial truncus arteriosus repair at our institution from 1985 to 2016. Analysis was performed on the 148 patients who were discharged from the hospital and survived ≥30 days postoperatively using multivariable competing risks Cox regression modeling. Median follow-up time was 12.6 years (interquartile range, 5.0-22.1 years) after discharge from full repair. Thirty patients (20%) underwent at least one intervention on the truncal valve during follow-up. Survival at 1, 10, and 20 years was 93.1%, 87.0%, and 80.9%, respectively. The cumulative incidence of any truncal valve intervention by 20 years was 25.6%. Independent risk factors for truncal valve intervention included moderate or greater truncal valve regurgitation (hazard ratio [HR], 4.77; P<0.001) or stenosis (HR, 4.12; P<0.001) before full truncus arteriosus repair and moderate or greater truncal valve regurgitation at discharge after full repair (HR, 8.60; P<0.001). During follow-up, 33 of 134 patients (25%) progressed to moderate or greater truncal valve regurgitation. A larger truncal valve root z-score before truncus arteriosus full repair and during follow-up was associated with worsening truncal valve regurgitation. Conclusions Long-term rates of truncal valve intervention are significant. At least moderate initial truncal valve stenosis and initial or residual regurgitation are independent risk factors associated with truncal valve intervention. Larger truncal valve root z-score is associated with significant truncal valve regurgitation and may identify a subset of patients at risk for truncal valve dysfunction over time.

Fears and Stressors of Trainees Starting Fellowship in Pediatric Cardiology.

Post-graduate training for physicians involves a high level of stress. High stress during training has the potential to cause burnout, a well-studied phenomenon in medical trainees. Burnout has previously been shown to increase the risk of mental health problems and medical error in trainees. Little research has been done on the impact of stress on new fellows in general and pediatric cardiology fellows in particular; understanding common sources of stress offers the opportunity to design targeted interventions to support trainee wellness. New trainees in Boston Children's Hospital's Pediatric Cardiology Fellowship program were asked to answer to the following question at the beginning of their training: "What are you afraid of in the coming year?" A qualitative content analysis was done on their anonymous responses. Responses were coded and analyzed for common themes. The overall analysis found that 83% of fellows reported fear of "fellowship/career responsibilities." The second most common theme was "failure/disappointment" (78%) followed by "personal life" (74%), "emotional exhaustion" (61%); least common was "new hospital environment" (37%). The most common individual fear was "increased clinical responsibility" reported by 65% of the new fellows, while 62% reported fears of "imposter syndrome," and 58% about "burnout." We found that fellows commonly report fears about both clinical and personal responsibilities, similar to stressors found in studies on residency. It is important for pediatric cardiology fellowships to develop early and specific interventions designed to assist fellows in managing both their new clinical responsibilities and their other stressors.

Effect of an Electronic Health Record Transition on the Provision of Recommended Well Child Services in Pediatric Primary Care Practices.

We sought to determine the effect of transitioning between electronic health record (EHR) systems on the quality of preventive care in a large pediatric primary care network. To study this, we performed a retrospective chart analysis of 42 primary care practices from the Pediatric Physicians' Organization at Children's who transitioned EHRs. We reviewed 24 random encounters per week distributed evenly across 6 age categories before, during, and after a transition period. We reviewed encounter documentation for age-appropriate well child services, per American Academy of Pediatrics/Bright Futures guidelines. Logistic regression and statistical process control analysis were used. In the pretransition period, 84.5% of all recommended elements were documented versus 86.4% posttransition (P = .04). Documentation of age-appropriate anticipatory guidance showed significant positive change (69.0% to 80.2%, P = .005), but it was the only subdomain with a statistically significant increase. These increases suggest that EHR transitions have the opportunity to affect the delivery of preventive care.

Computerized Physician Order Entry in the Neonatal Intensive Care Unit: A Narrative Review.

BACKGROUND: Computerized physician order entry (CPOE) has grown since the early 1990s. While many systems serve adult patients, systems for pediatric and neonatal populations have lagged. Adapting adult CPOE systems for pediatric use may require significant modifications to address complexities associated with pediatric care such as daily weight changes and small medication doses. OBJECTIVE: This article aims to review the neonatal intensive care unit (NICU) CPOE literature to characterize trends in the introduction of this technology and to identify potential areas for further research. METHODS: Articles pertaining to NICU CPOE were identified in MEDLINE using MeSH terms "medical order entry systems," "drug therapy," "intensive care unit, neonatal," "infant, newborn," etc. Two physician reviewers evaluated each article for inclusion and exclusion criteria. Consensus judgments were used to classify the articles into five categories: medication safety, usability/alerts, clinical practice, clinical decision Support (CDS), and implementation. Articles addressing pediatric (nonneonatal) CPOE were included if they were applicable to the NICU setting. RESULTS: Sixty-nine articles were identified using MeSH search criteria. Twenty-two additional articles were identified by hand-searching bibliographies and 6 articles were added after the review process. Fifty-five articles met exclusion criteria, for a final set of 42 articles. Medication safety was the focus of 22 articles, followed by clinical practice (10), CDS (10), implementation (11), and usability/alerts (4). Several addressed more than one category. No study showed a decrease in medication safety post-CPOE implementation. Within clinical practice articles, CPOE implementation showed no effect on blood glucose levels or time to antibiotic administration but showed conflicting results on mortality rates. Implementation studies were largely descriptive of single-hospital experiences. CONCLUSION: CPOE implementation within the NICU has demonstrated improvement in medication safety, with the most consistent benefit involving a reduction in medication errors and wrong-time administration errors. Additional research is needed to understand the potential limitations of CPOE systems in neonatal intensive care and how CPOE affects mortality.