Pediatric Cardiology最新文献

Vascular rings are rare congenital defects that can cause tracheal and/or esophageal compression. Prenatal detection is increasing due to advances in screening and fetal echocardiography. Postnatal outcomes remain variable. We describe our single-center experience of postnatal outcomes in prenatally detected vascular rings and evaluate factors associated with surgery. We performed a retrospective review of all fetal diagnoses of possible vascular ring evaluated between 2016 and 2023. Patients with significant intracardiac abnormalities, without postnatal data, or without confirmed postnatal diagnosis were excluded from postnatal analysis. Outcome variables included symptoms, tracheal/esophageal compression, and surgical repair. The prenatal cohort included 109 patients with 80% right aortic arch (RAA) and 20% double aortic arch (DAA). Of 72 patients in the postnatal cohort, 85% underwent computed tomography angiography (CTA) at median age of 2 months with 0.84 ± 0.34 mSv of radiation. On CTA, 69% had a diverticulum. Of those with RAA, 95% had an aberrant left subclavian. Most (79%) had airway abnormalities on CTA. Fifteen (21%) developed symptoms at median age of 5 months. Fifty-eight percent  of patients underwent surgery at median age of 10 months, of which 33% were due to symptoms. On univariate analysis, DAA was associated with airway abnormalities on CTA and surgical repair. At 30-month follow up, 99% of patients remained asymptomatic. Prenatal diagnosis of vascular rings is associated with high rates of airway abnormalities, even in those without symptoms. Surgical repair was pursued in asymptomatic patients with DAA and airway abnormalities in our institution. As DAA is associated with airway abnormalities, these patients may require closer monitoring for future symptom development.

In response to the article "Evaluation of Cardiac Function in Children Undergoing Liver Transplantation" by Bansal et al., I admire the authors for their valuable insights in evaluating biventricular heart function in pediatric patients undergoing Liver Transplantation. However, I bring up several key concerns regarding study's methodology. The diversity of the pediatric cirrhosis population is limited by the study's small sample size, which consists of only 35 children patients. Advanced diagnostic techniques including tissue Doppler echocardiography and cardiovascular magnetic resonance (CMR), which are valuable for diagnosis of heart diseases, are not included in the study. The lack of a reference group of patients without cirrhosis makes it more difficult to confirm whether the cardiac dysfunction shown is unique to cirrhosis or indicates other chronic disease. Additionally, the study does not provides information regarding the outcomes following liver transplantation especially reversibility of cardaic abnormalities.

Arrhythmias are common after the Fontan operation and increase risk of morbidity and mortality. Their impact on late outcomes remains poorly understood. Our objective was to assess whether long-term studies after the Fontan incorporated the impact of arrhythmia characteristics on outcome. We reviewed the literature since 2005 for studies evaluating the impact of multiple risk factors on long-term outcomes and mortality after the Fontan. Studies focusing only on one or two risk factors (including arrhythmias) and those with a mean follow-up < 10 years were excluded. Of the 17 studies analyzed, only six mentioned rhythm at follow-up. Nine lumped all arrhythmias (tachyarrhythmia, bradyarrhythmia, paced rhythm, early and late arrhythmia) into one single category. Only eight of the 17 studies reported the incidence of tachyarrhythmias in detail and only two studies described bradyarrhythmias in detail as sinus or atrioventricular (AV) node dysfunction subgroups. Five studies reported on treatment of tachyarrhythmias including Fontan revision surgeries, catheter ablation, pacemaker placement and or the use of antiarrhythmic drugs. However, only one included information on the impact of treatment on outcomes. Out of 15 studies that conducted a multivariate analysis of Fontan outcomes, arrhythmia was included in the univariate model in 14 and in the multivariate model in 9. Overall, we found most studies on late outcomes after the Fontan do not include rhythm and arrhythmia characteristics in data collection and analysis. Incorporating more granular rhythm information in large databases and in multivariate analyses will help better determine and contextualize the impact of arrhythmias on outcomes after the Fontan.

Left ventricular assist device (LVAD) utilization is increasing in pediatrics. These patients are often complex and may require treatment of AV block. Leadless pacing has been associated with a lower risk of device-related adverse events, but there are no reports of a leadless pacemaker implant in a pediatric patient with LVAD support. This report describes the use of a leadless pacemaker implantation in a 17-year-old patient with dilated cardiomyopathy and continuous flow LVAD support which facilitated transition to outpatient care.

To determine if pre-operative high-flow nasal cannula (flow > 2 L per minute) or mechanical ventilation impact post-operative outcomes after the superior cavopulmonary connection. A single-center retrospective review of single-ventricle patients who underwent the superior cavopulmonary connection procedure at a tertiary care center over a 10-year period. Patients who underwent the procedure at greater than 2 years of age were excluded. The groups of interest were those who were on mechanical ventilation or high-flow nasal cannula. Patients in room air or on nasal cannula oxygen served as the control group. A total of 269 consecutive patients were included, of which 44 required high-flow nasal cannula and 17 required mechanical ventilation prior to the superior cavopulmonary connection procedure. Thirty-day post-operative survival was high, with 1 death in both the high-flow nasal cannula and the mechanical ventilation groups. At 1-year post-procedure, the transplant-free survival was lower in the mechanically ventilated group when compared to the high-flow nasal cannula and room air groups (46% vs. 84% vs. 95%, p value < 0.01). The same trend was seen at 5 years (20% vs 78% vs 90%, p value < 0.01). These groups also had worse overall survival, longer post-operative lengths of stay, and longer post-operative intubation durations. Pre-operative respiratory support with high-flow nasal cannula or mechanical ventilation is associated with decreased overall survival and reduced transplant-free survival at 1- and 5-year post-superior cavopulmonary connection.

Recent data demonstrate worse heart transplant (HTx) outcomes in children with shorter VAD durations, but do not account for VAD adverse events (AEs)Es. We compared outcomes of patients bridged to HTx with < 30 vs. ≥ 30 days of VAD support in an earlier era by assessing both VAD and HTx risk factors. We merged data from the PediMACS and Pediatric Heart Transplant Study registries to compare one-year post-HTx mortality in patients with < 30 vs. ≥ 30 days of pre-HTx VAD support between 2012 and 2018. We used inverse probability of treatment weighting using propensity scores (PS) to control for confounders, including age, blood type, allosensitization, cardiac diagnosis (cardiomyopathy, congenital heart disease, or myocarditis), VAD support type (left, right, single, or biventricular VAD), and pre-Tx mechanical ventilation and vasoactive support. Among 271 patients, there were 60 in the < 30-days and 211 in the ≥ 30-days groups. At HTx, the < 30-days group used more ventilation (34% vs. 7%, p < 0.001) and vasoactives (60% vs. 24%, p < 0.001 vs. ≥ 30-days). The weighted AE rate/patient was 0.42 in the < 30-days vs. 0.78 in the ≥ 30-days group (p = 0.02). There were 2 deaths in the < 30-days group and 13 in the ≥ 30-days group (p = 0.38). A PS-weighted Cox model, adjusted for 30-day VAD AE rate, demonstrated a non-significant mortality hazard ratio of 0.43 for < 30-days vs. ≥ 30-days group (95%CI 0.07-2.70, p = 0.37). In this era, there was no difference in survival based on VAD duration prior to HTx. This finding requires additional comparisons to the current era of VAD as a bridge to Tx.

The recently published article, "Evaluation of Cardiac Function in Children Undergoing Liver Transplantation" by Bansal et al. presents a significant contribution to the understanding of pediatric cirrhotic cardiomyopathy and its evaluation using advanced techniques like Speckle Tracking Echocardiography (STE). This letter commends the authors for their efforts to correlate pre-transplant echocardiographic parameters with post-operative outcomes, which holds promise for advancing pediatric hepatology and cardiology. However, this study also raises several concerns regarding methodology and interpretation. Key limitations include potential selection bias from single-center recruitment and exclusion criteria, reliance solely on STE without incorporating advanced imaging modalities such as 3D echocardiography or cardiac MRI, and lack of consideration for socioeconomic and geographic factors that influence outcomes. Additionally, the study's cross-sectional design and limited adjustment for confounding variables, such as nutritional status and infections, restrict its ability to provide dynamic and comprehensive insights. Addressing these limitations through future research-encompassing diverse populations, advanced imaging, longitudinal data, and robust control of variables-will enhance the reliability and applicability of findings in this critical domain.

Non-invasive assessment of myocardial work is a newly described technique to assess myocardial energetics. This has not been previously studied to assess the effects of right ventricular pacing at different sites or at different heart rates in children. We aimed to study the effects of right ventricular apical, septal, and His bundle pacing on myocardial work along with the effects of increasing heart rate. This was a prospective pilot study performed on six patients with structurally normal hearts and function following an electrophysiology study. Global work index and global work efficiency was highest during His pacing and lowest during right ventricular apical pacing. The global constructive work, index, and efficiency were progressively worse with increasing heart rates. In this prospective pilot study, we demonstrated that myocardial work indices differ depending on myocardial activation pattern and at different heart rates in pediatric patients. Myocardial performance as assessed by myocardial work efficiency is worse when pacing is performed at the right ventricular apex when compared to His and right ventricular septal pacing. Myocardial performance can be affected by higher heart rates, following a work-frequency relationship.

Tetralogy of Fallot often requires transannular patch repair, leading to pulmonary insufficiency. Percutaneous pulmonary valve implantation (PPVI) with self-expandable valves offers a promising alternative, especially for enlarged right ventricular Queryoutflow tracts (RVOT). Five RVOT types identified in patients with Tetralogy of Fallot reflect anatomical variations due to disease and prior surgeries. This study assesses the Pulsta THV® valve's in vitro hemodynamic performance across these RVOT morphologies using 3D-printed models. Five RVOT morphologies were recreated as 3D models from patient-specific imaging data. The Pulsta THV® valves, available in 28, 30, and 32 mm sizes, were evaluated using the ViVitro Pulse Duplicator System at three cardiac outputs (2, 3.5, and 5 L/min). Hemodynamic performance was assessed by measuring regurgitation rates and pressure gradients in the left and right pulmonary arteries. The Pulsta THV® performed optimally in RVOT Types 1 and 2, demonstrating lower regurgitation rates and pressure gradients, particularly with larger valve sizes. Conversely, RVOT Types 3 and 5 showed increased pressure gradients and hemodynamic variability, indicating less favorable outcomes. The results highlighted the critical role of precise anatomical compatibility, with larger valve sizes proving more effective in enlarged RVOT geometry. Valve sizes tailored to specific RVOT morphologies can enhance PPVI outcomes. Types 1 and 2 are ideal for PPVI, while Types 3 and 5 present challenges due to hemodynamic variability. This study supports 3D modeling and in vitro testing for pre-procedural planning to reduce complications, with future research exploring dynamic imaging and materials mimicking tissue properties.

This study aimed to assess the impact of a virtual reality (VR) educational curriculum on the knowledge of congenital heart disease (CHD) among medical students during their pediatric clerkship. A multi-center study was conducted involving medical students from two large academic medical centers. Participants engaged in the Stanford Virtual Heart (SVH) educational program during their pediatric clerkship from July 2020 to September 2021. The study employed a stratified approach for group assignment based on timing of enrollment, with Center 1 stratifying by the first or second half of the academic year and Center 2 by month-long rotations. A validated 27-question assessment tool focused on six common CHDs was administered, with the intervention group completing the SVH before the assessment and the control group completing the SVH after the assessment. Additionally, a qualitative survey gauging the participants' experience was conducted. Results were compared between the intervention and control groups. A total of 373 medical students (179 intervention and 194 control) participated in the study. The intervention group exhibited significantly better performance on the assessment compared to the control group across both medical centers (P value < .0001). More than 92% of participants rated the VR experience as 'easy' or 'very easy' to use. Over 99% of participants recommended integrating the program into the pediatric clerkship curriculum. The SVH curriculum was seamlessly integrated into two medical school pediatric clerkships, resulting in a significant improvement in CHD understanding. The overwhelmingly positive feedback supports the incorporation of VR-based educational tools into medical education.