Pediatric Cardiology最新文献

Transcatheter closure (TCC) of certain ventricular septal defect (VSD) subtypes typically requires arteriovenous loop (AVL) formation or retrograde transarterial deployment. Upfront transvenous cannulation from the right ventricle avoids arterial access and loop-related complications. We retrospectively reviewed data of patients who underwent TCC for perimembranous, intraconal, and post-surgical residual VSDs at our institution (January 2019-December 2023). Patients receiving upfront transvenous VSD cannulation and closure were compared to those undergoing antegrade closure after AVL formation. Upfront retrograde closure cases were excluded. In a cohort of 163 patients, upfront transvenous cannulation was performed in 116 (71%) and AVL formation in 31 (19%). Upfront transvenous cannulation use increased from 25.6% in 2019 to 93% in 2023. For the upfront group, the median patient age was 55 months (IQR, 17.8-120.8), median weight was 15.8 kg (IQR, 9.6-29.8), indexed VSD size was 9.2 mm/m² (IQR, 5.5-14.8), and 68.9% had pulmonary arterial hypertension. Cannulation was successful in 83.6% (97/116) of cases, with a switch to retrograde transarterial cannulation in 16.4% (19/116). No deaths or pacemaker implantations occurred. Compared to AVL group, upfront group had significantly shorter procedural times (p = 0.002) and lower radiation exposure (p < 0.001). Smaller patient weight (OR: 0.97, 95%CI: 0.95-0.99), larger indexed VSD size to patient weight (OR: 36.5, 95%CI: 2.49-533.9) and to BSA (OR: 1.23, 95%CI: 1.06-1.4) were significant independent predictors for successful transvenous cannulation. Upfront transvenous VSD cannulation simplifies transvenous TCC by eliminating the need for arterial access and AVL formation, particularly successful in small patients with large defects.

This study aimed to evaluate the hemodynamic and ventricular performance of neonates with hypoxic-ischemic encephalopathy (HIE) undergoing therapeutic hypothermia using conventional and advanced echocardiographic techniques. This observational, prospective study included 22 neonates with HIE matched with 22 healthy neonates. Echocardiographic studies were performed 24 h after achieving target temperature during hypothermia and 24 h after rewarming. Evaluated echocardiographic parameters included ejection fraction (EF), shortening fraction (SF), right ventricular fractional area change, biventricular Tei index, right ventricular s' wave velocity, tricuspid annular plane systolic excursion, biventricular stroke volume and cardiac output, left ventricular (LV) and right ventricular (RV) global longitudinal strain (GLS), LV circumferential and radial strain, LV twist, and LV torsion. LV EF and SF did not change significantly between the hypothermia and rewarming periods (EF:73 ± 7% vs. 74 ± 5%, p = 0.21; SF:39 ± 6% vs. 41 ± 5%, p = 0.26); however, both were higher after rewarming compared to the control group (EF:70 ± 5%, p = 0.003; SF:36 ± 4%, p = 0.002). There were no significant differences in LV GLS, circumferential and radial strain, twist, and torsion between the HIE and control groups. Pulmonary artery systolic pressure (PASP) and RV GLS were worse in the study group compared to the control group (PASP: hypothermia 45 ± 24 mmHg, p = 0.01; rewarming 53 ± 34 mmHg, p = 0.01; control group 29 ± 11 mmHg; RV GLS: hypothermia 18 ± 5%, p = 0.02; rewarming: 18 ± 4%, p = 0.01; control group 21 ± 2%). Therapeutic hypothermia appears to have no detrimental impact on LV systolic function. RV GLS was the only parameter that demonstrated impaired RV systolic function during therapeutic hypothermia, likely due to elevated PASP.

Prenatal diagnosis of congenital heart disease requiring early cardiac catheterization or surgical intervention enables optimal delivery planning for appropriate postnatal cardiovascular intervention and care. This allows for improved morbidity and mortality. Prior national data reported prenatal diagnosis rates of 32% for congenital heart disease requiring intervention in infants in the first 6 months of life in the New England region. With improved technology, access to care and changes to the obstetrical ultrasound guidelines for mid trimester fetal study, it is expected that diagnostic rates should improve. The New England Congenital Cardiology Association (NECCA) conducted a quality improvement study to determine the rates of prenatal detection in the current era with the hypothesis that there has been improvement in detection rates in this region. Ten of fourteen medical centers delivering pediatric cardiology care in New England contributed prenatal diagnosis data for 286 infants born at the participating centers during a one year period. The overall prenatal detection rate was 68%. Detection rates ranged from 39 to 90%. When fetal echocardiogram was performed at a pediatric cardiology center, the detection rate was 95% with only 7 moderate (7/195; 3.6%) and 3 severe (3/195; 1.5%) diagnostic discrepancies. Prenatal diagnostic rate and accuracy are high among pediatric cardiology centers in the New England region, and much improved over historical data. To improve fetal detection of congenital heart disease further, future work is needed to better determine the etiology of missed prenatal diagnoses and efforts should be focused on increasing appropriate referrals to pediatric cardiology centers for fetal evaluation.

Prolonged pleural drainage and chylothorax are common in postoperative Fontan patients and are associated with increased morbidity and mortality. Multiple medical and interventional treatment strategies exist and vary between centers. This is a retrospective multicenter observational cohort study of pediatric patients who underwent Fontan operation at 8 pediatric cardiac surgical institutions from 1/1/2019 to 12/31/2021. Data were obtained from institutional records and collected from the Pediatric Cardiac Critical Care Consortium (PC⁴). 185 patients underwent Fontan operation with median age of 3.8 years [IQR 3.2-4.5]. Chest tube drainage for > 14 days occurred in 40 patients (22%). Chylothorax occurred in 33 patients (18%, incidence 9.1-26.2% across centers). Compared to non-chylothorax patients, those with chylothorax had lower preoperative ventricular end diastolic pressures (8 vs. 9 mm Hg, p = 0.019), greater chest tube utilization (13 vs. 7 days, p =  < 0.001), ICU LOS (7 vs. 4 days, p = 0.001), hospital LOS (12 vs. 9 days, p < 0.001), and more weight loss (- 2.7% vs. 0.8%, p = 0.019). Using a receiver-operating characteristic curve, chest tube output > 18.8 mL/kg/day on POD 2 predicted chylothorax with an AUC of 0.73. Common chylothorax treatments were diet modification (n = 15, 45%) and sildenafil (n = 14, 52%). Interventional procedures were used in six chylothorax patients (18%). Postoperative chylothorax in Fontan patients was associated with increased chest tube utilization, postoperative interventions, greater weight loss, and longer ICU and hospital LOS. Center level variation suggests outcomes and resource utilization could be improved with further studies and establishment of best practices.

Repair or palliation of pulmonary atresia with intact ventricular septum (PA/IVS) often falls into one of 4 categories: cardiac transplant, 2-ventricular circulation, 1.5 ventricle circulation, or single ventricle circulation. The optimal management strategy has been an area of much debate. We sought to review the management strategy of patients with PA/IVS at our institution to better understand what metrics can be used to guide management and initial interventions. The study aims to examine the outcomes of a single-center approach to managing patients with PA/IVS. Our cohort included 29 patients; one patient underwent a planned transplant at ten days of life (3.4%), 12 underwent repair via a two-ventricle circulation (41.4%), 7 underwent repair with 1.5 ventricle circulation (24.1%), and 7 underwent repair with single ventricle circulation (24.1%). Survival was achieved in 93.1% with two patients (6.9%) expiring. The TV annulus z-score was significantly different between the three groups, with the 1 V group having the smallest median TV annulus z-score at - 4.04 (IQR - 4.60- - 3.60) and the 2 V group having the largest median TV z-score at - 1.4 (IQR - 2.24- - 0.12). Six patients underwent late right ventricular decompression. We present a post-hoc algorithm to help guide treatment strategies for patients with PA/IVS.

Total anomalous pulmonary venous return (TAPVR) is a high risk and rare cardiac malformation with a low prenatal detection rate and predicting obstruction in these cases is difficult. We sought to investigate fetal echocardiographic parameters associated with postnatal pulmonary venous obstruction (PPVO). We performed a retrospective review of 26 patients with TAPVR who had a fetal echocardiogram from 2010 to 2021. Blinded readers analyzed the fetal Doppler features of the pulmonary veins (PV) and vertical veins (VV) at final fetal assessment. Of the 26 patients, 17 (65%) were prenatally diagnosed and overall 10 (38.5%) developed PPVO. All patients had additional associated congenital heart disease. Compared to patients without PPVO, patients with PPVO had a lower VV variability index (VI) (0.51 (0.17-0.79) vs 1.43 (0.54-2.58); p = 0.003), a higher likelihood of having an abnormal VV or PV Doppler pattern (p = 0.042, p = 0.015), a higher VV minimum velocity (0.435 m/s (0.17-1.05) vs 0.14 m/s (- 0.16 to 0.35) p = 0.001) and a higher VV mean velocity (0.641 m/s (0.31-1.15) vs 0.321 m/s (0.19-0.5); p = 0.009). A fetal VV VI of ≤ 0.8 was associated with PPVO (100% sensitivity, 86% specificity). Our data show that some fetal PV and VV Doppler indices are significantly different between those that develop PPVO and those who do not, with the most significant difference seen in the VV variability index and VV minimum velocity. Prenatal predictors of PPVO have the potential to influence prenatal counseling, perinatal care, surgical timing, and outcomes associated with this critical diagnosis.

The EDUCATE study investigated the acute impact of energy drink (ED) consumption on heart rate variability (HRV) in children and adolescents, with a focus on how these stimulant-rich beverages influence cardiac autonomic function. Given the popularity of EDs among young people, this study assessed the immediate cardiovascular response to ED intake. This randomized, double-blind, placebo-controlled crossover trial involved 26 healthy participants aged 10-18 years. Each participant consumed a weight-adjusted ED or placebo in two separate test sessions. HRV was monitored via a 24-h Holter ECG, with analysis centered on time-domain measures, such as the standard deviation of normal RR intervals (SDNN) and root-mean square of successive differences (RMSSD), along with frequency-domain metrics. Statistical analysis included a two-way repeated-measures ANOVA to examine the effects of "beverage" and "time." The study revealed a significant increase in the SDNN in the ED group within the first hour post-consumption, with a mean difference of 17.692 ms compared with that in the placebo group (SDNN: ED = 133.346 ± 50.217 ms vs. placebo = 115.654 ± 47.583 ms, p = 0.023). However, no significant differences in the SDNN were observed in the subsequent time intervals (60-240 min). In addition, frequency-domain parameters, such as total power, RMSSD, LF, HF, and the LF/HF ratio, showed no significant changes across the four-hour observation period, indicating that sympathetic activation was transient. The findings suggest that ED consumption in children and adolescents leads to a temporary increase in autonomic activity, marked by elevated SDNN, without lasting dysregulation. While the cardiovascular effects are brief, acute sympathetic activation underscores the need for regulated ED intake among minors. Further studies are recommended to explore the long-term effects of regular ED consumption on cardiovascular health in youth.

Patent ductus arteriosus (PDA) stenting is a vital intervention for neonates with ductal-dependent blood flow, offering an attractive alternative to surgical shunt placement. Despite its benefits, the procedure poses risks such as ductal spasm, branch pulmonary artery compromise, and pseudoaneurysm formation. This report presents two complex neonatal cases with distinct outcomes. The first patient experienced severe ductal spasm, pseudoaneurysm formation, and ductal dissection. Innovative use of percutaneous venovenous extracorporeal membrane oxygenation (VV ECMO) provided hemodynamic stability, enabling staged interventions and eventual successful ductal stenting. The second patient experienced acute left pulmonary artery flow loss due to ductal spasm. Transcatheter attempts to restore flow failed, necessitating surgical stent removal and Blalock-Taussig-Thomas shunt placement. These cases highlight the challenges of PDA stenting in complex ductal anatomies and the importance of advanced imaging, careful wire and catheter selection, and multidisciplinary collaboration. Notably, the first reported use of percutaneous VV ECMO during PDA stenting demonstrates its potential as a lifesaving adjunct for these cases.

Although the long-term outcomes of the surgical grafts are well defined and reported, the data regarding the mid-and long-term results of the balloon-expandable percutaneous valves in the native right ventricular outflow tract (RVOT) is limited. We retrospectively evaluated 42 patients who underwent PPVI (Sapien® XT valve) to native RVOT due to severe pulmonary regurgitation (PR) and/or moderate to severe pulmonary stenosis (PS) between August 2015 and November 2020. The median patient age at the time of PPVI was 13.4 years (6.1-36.5 years). The median body weight of the patients was 42 kg (15-110 kg). The rate of patients who were followed up without the need for percutaneous or surgical intervention was 97.4% at the end of year 1, 89.3% at the end of year 3, and 85.8% at the end of year 5. At the end of year 6, the proportion of patients requiring no procedure remained constant, with year 5 at 85.8%, but decreased to 70.2% at the end of year 7. Although the early results are very encouraging, it is seen that PPVI in patients with RVOT in the long term brings some problems. The most important of these is tricuspid valve problems, which were not considered before the procedure. Patients requiring reintervention due to pulmonary regurgitation show similar characteristics to surgical valves' long-term results.

The IMmediate Postpartum Access to Cardiac Therapy (IMPACT) procedure is a multidisciplinary, collaborative, highly coordinated clinical service in which a planned delivery and intensive neonatal care are offered for conditions where there is a high likelihood of postnatal instability. This process includes prenatal consultation with the parent(s), involving each service engaged with the delivery, postnatal resuscitation, and procedural care. A Cesarean section delivery is planned in an operating room with immediate access to a multifunctional procedural suite where the neonate can undergo rapid cardiac evaluation and initiation of interventional treatments which can have a positive, life-saving impact. This review describes the details of this unique procedure and multidisciplinary program at one institution.