A Prospective Randomized Controlled Trial Using Virtual Reality in Pediatric Pre-intervention Echocardiograms to Decrease Child Anxiety and Fear.

Virtual reality (VR) as a distraction tool decreases anxiety and fear in children undergoing procedures, but its use has not been studied during transthoracic echocardiograms (TTEs). We hypothesized that VR in children undergoing pre-intervention TTEs decreases anxiety and fear and increases TTE study comprehensiveness and diagnostic accuracy when compared with standard distractors (television, mobile devices). Patients (6-18 years old) scheduled for pre-intervention TTEs at Lucile Packard Children's Hospital in 2021 and 2022 were prospectively enrolled and randomized to VR and non-VR groups. Patients completed pre- and post-TTE surveys using the Children's Anxiety Meter-State (CAM-S) and Children's Fear Scale (CFS). Patients, parents, and sonographers completed post-TTE experience surveys. TTEs were reviewed by pediatric cardiologists for study comprehensiveness and compared with electronic medical records for diagnostic accuracy. Among 67 enrolled patients, 6 declined VR, 31 randomized to the VR group, and 30 to the non-VR group. Anxiety (average CAM-S difference 0.78 ± 1.80, p = 0.0012) and fear (average CFS difference 0.36 ± 0.74, p = 0.0005) decreased in both groups. There was no difference between groups in the change in anxiety and fear pre- and post-TTE (p = 0.96-1.00). TTE study comprehensiveness and diagnostic accuracy were high in both groups. Procedure time (time in the echocardiography room) was less for the VR group (48.4 ± 18.1 min) than the non-VR group (58.8 ± 24.4 min), but without a statistically significant difference (p = 0.075). VR is similar to standard distractors and may decrease procedure time. Patients, parents, and sonographers rated the VR experience highly and encouraged its use with future procedures.

Maternal Diabetes and Cardiovascular Health in the Offspring.

Pulse wave velocity (PWV) has been explored to predict cardiovascular health in adults. Less is known about neonatal PWV. We evaluated the association between arterial stiffness of neonates of mothers (NoM) with diabetes and childhood health. Neonatal brachial-femoral PWV (bfPWV) was measured after birth and neonates followed for a median of 5.2 years [1 month-6.6 years]. 36 pregnant women with pregestational diabetes mellitus PGDM (n = 12), gestational diabetes mellitus (GDM) (n = 13), and controls (n = 11) were enrolled. Neonates were similar in weight, gestational age, and delivery mode. 26 neonates had follow-up data including weight, height and blood pressure. More mothers with PGDM had poor glycemic control compared to mothers with GDM (83% vs. 8%; p = 0.0002). PWV was higher in NoM with PGDM than controls (3.4 ± 0.5 vs. 2.6 ± 0.8 m/s; p = 0.04). At follow-up, children of mothers with diabetes (n = 16) had higher weight percentile (78.5 ± 27.9 vs 49.5 ± 34.6%; p = 0.02) and diastolic blood pressure (DBP) (68 ± 13.6 vs 57.3 ± 4.3 mmHg; p = 0.01) than controls (n = 10). No correlation emerged between neonatal PWV and childhood body mass index (BMI) or maternal HbA1c. Results suggest maternal diabetes affect neonatal arterial stiffness and childhood blood pressure; however, the mechanism is unclear. The long-term implications of these findings warrant further investigation.

Tele-Clinic Visits in Pediatric Patients with Marfan Syndrome Using Parentally Acquired Echocardiography.

OBJECTIVE: To test feasibility of tele-clinic visits using parentally acquired vital signs and focused echocardiographic images in patients with Marfan syndrome. STUDY DESIGN: We included patients with Marfan syndrome aged 5-19 years followed in our clinic. We excluded patients with Marfan syndrome and history of previous aortic root (AoR) surgery, cardiomyopathy, arrhythmia, or AoR ≥4.5 cm. We trained parents in-person to acquire focused echocardiographic images on their children using a hand-held device as well as how to use a stadiometer, scale, blood pressure (BP) machine, and a digital stethoscope. Before tele-clinic visits, parents obtained the echocardiographic images and vital signs. We compared tele-clinic and on-site clinic visit data. Parental and clinic echocardiograms were independently analyzed. RESULTS: Fifteen patient/parent pairs completed tele-clinic visits, conducted at a median of 7.0 (IQR 3.0-9.9) months from the in-person training session. Parents took a median of 70 (IQR 60-150) minutes to obtain the height, weight, heart rate, BP, cardiac sounds, and echocardiographic images before tele-clinic visits. Systolic BP was greater on-site than at home (median +13 mm Hg, P = .014). Height, weight, diastolic BP, heart rate, and AoR measurements were similar. CONCLUSIONS: This study provides information for implementing tele-clinic visits using parentally acquired vital signs and echocardiographic images in patients with Marfan syndrome. The results show that tele-clinic visits are feasible and that parents were able to obtain focused echocardiographic images on their children. TRIAL REGISTRATION: ClinicalTrials.gov: NCT03581682.

Peripheral Endothelial Function After Arterial Switch Operation for D-looped Transposition of the Great Arteries.

Coronary artery re-implantation during arterial switch operation in patients with D-looped transposition of the great arteries (D-TGA) can alter coronary arterial flow and increase shear stress, leading to local endothelial dysfunction, although prior studies have conflicting results. Endothelial pulse amplitude testing can predict coronary endothelial dysfunction by peripheral arterial testing. This study tested if, compared to healthy controls, patients with D-TGA after arterial switch operation had peripheral endothelial dysfunction. Patient inclusion criteria were (1) D-TGA after neonatal arterial switch operation; (2) age 9-29 years; (3) absence of known cardiovascular risk factors such as hypertension, diabetes, hypercholesterolemia, vascular disease, recurrent vasovagal syncope, and coronary artery disease; and (4) ability to comply with overnight fasting. Exclusion criteria included (1) body mass index ≥85th percentile, (2) use of medications affecting vascular tone, or (3) acute illness. We assessed endothelial function by endothelial pulse amplitude testing and compared the results to our previously published data in healthy controls (n = 57). We tested 20 D-TGA patients (16.4 ± 4.8 years old) who have undergone arterial switch operation at a median age of 5 days (0-61 days). Endothelial pulse amplitude testing indices were similar between patients with D-TGA and controls (1.78 ± 0.61 vs. 1.73 ± 0.54, p = 0.73).In our study population of children and young adults, there was no evidence of peripheral endothelial dysfunction in patients with D-TGA who have undergone arterial switch operation. Our results support the theory that coronary arterial wall thickening and abnormal vasodilation reported in these patients is a localized phenomenon and not reflective of overall atherosclerotic burden.

Decompressing vein and bilateral superior venae cavae in a patient with hypoplastic left heart syndrome.

The levoatrial cardinal vein (LACV), first described in 1926, acts as a decompressing vessel for pulmonary venous return in cases of severe left-sided obstruction with an intact or significantly restrictive atrial septum. The LACV and the persistent left superior vena cava (LSVC) are thought to share similar embryologic origins. To challenge this notion, we present a unique case of a neonate with hypoplastic left heart syndrome, cor triatriatum, and a decompressing LACV in the presence of bilateral superior venae cavae.

Quality Improvement in a Pediatric Echocardiography Laboratory: A Collaborative Process.

Transthoracic echocardiography (TTE) is an essential tool for diagnosis and management of congenital heart disease. Pediatric echocardiography presents unique challenges including complex anatomy, variable patient cooperation and provider expertise. Diagnostic errors inevitably occur. We designed a collaborative and stepwise quality improvement (QI) process to address diagnostic errors within our laboratory. We retrospectively reviewed medical records to identify diagnostic TTE errors in 100 consecutive cardiac surgery patients ≤ 5 years old (July 2020-January 2021). We identified 18 diagnostic errors. Most errors had minor impact (14/18), and 13 were preventable or possibly preventable. We presented these results to our sonographers and faculty and requested input on preventing and managing diagnostic errors. Our root cause analysis based on their responses yielded 7 areas for improvement (imaging, reporting, systems, time, environment, people, QI processes). Our faculty and sonographers chose QI processes and imaging as initial areas for intervention. We defined our SMART goal as a 10% reduction in diagnostic errors. We implemented interventions focused on QI processes. On initial follow up in May 2022, we identified 7 errors in 70 patients (44% reduction in error rate). Utilizing a stepwise and team-based approach, we successfully developed QI initiatives in our echocardiography laboratory. This approach can serve as a model for a collaborative QI process in other institutions.

Can 10 000 Healthy Steps a Day Slow Aortic Root Dilation in Pediatric Patients With Marfan Syndrome?

Background Stiffer aortas are associated with a faster rate of aortic root (AoR) dilation and higher risk of aortic dissection in patients with Marfan syndrome. We have previously shown that mild aerobic exercise reduces aortic stiffness and rate of AoR dilation in a Marfan mouse model. In this study, we investigated if these results could be translated to pediatric patients with Marfan syndrome. Methods and Results We enrolled 24 patients with Marfan syndrome aged 8 to 19 years to participate in a 6-month physical activity intervention, excluding those with ventricular dysfunction or prior history of aortic surgery. We instructed patients to take 10 000 steps per day, tracked by an activity tracker. At baseline and 6 months, we measured AoR dimension, arterial stiffness, endothelial function, physical activity indices, inflammatory biomarkers, and coping scores. Controls consisted of 15 age-matched patients with Marfan syndrome. Twenty-four patients with Marfan syndrome (median age, 14.4 years [interquartile range {IQR}, 12.2-16.8], 14 male patients) were enrolled. Baseline assessment demonstrated that the majority of these patients were sedentary and had abnormal arterial health. Twenty-two patients completed the intervention and took an average of 7709±2177 steps per day (median, 7627 [IQR, 6344-9671]). Patients wore their Garmin trackers at a median of 92.8% (IQR, 84%-97%) of their intervention days. AoR Z score in the intervention group had a significantly lower rate of change per year compared with the controls (rate of change, -0.24 versus +0.008; P=0.01). Conclusions In this clinical intervention in pediatric patients with Marfan syndrome, we demonstrated that a simple physical activity intervention was feasible in this population and has the potential to decrease the AoR dilation rate. REGISTRATION URL: https://www.clinicaltrials.gov; Unique identifier: NCT03567460.

Diagnosis of Anomalous Aortic Origin of the Left Coronary Artery in a Pediatric Patient.

Anomalous aortic origin of the left coronary artery is rare and confers increased risk of sudden cardiac death. Accurate diagnosis is crucial and often requires many diagnostic modalities. This case report highlights the echocardiographic characteristics and pitfalls in diagnosing the anomaly in addition to the advantages of using computed tomography and magnetic resonance imaging in a pediatric patient.