Pediatric Cardiology最新文献

Cardiopulmonary exercise testing (CPET) is a cornerstone of functional assessment in congenital heart disease (CHD), yet there are additional important contributors to exercise limitation that are not directly measured by CPET. This narrative review highlights the complementary utility of spirometry, body composition analysis, and evaluation of handgrip strength in augmenting our understanding of functional capacity in CHD. Spirometry provides insights into restrictive lung disease patterns prevalent in post-surgical CHD populations; body composition analysis, especially lean mass assessment, can reveal sarcopenia not apparent using BMI alone; and handgrip strength serves as a validated surrogate for overall muscular function and frailty. We propose routine integration of all three testing techniques as complements to CPET protocols to enhance clinical assessment, guide rehabilitation, and support prognostication in pediatric CHD patients. This review advocates for broader adoption and standardization of these measures in clinical settings to improve risk stratification and long-term outcomes.

A new era of multimodal opioid-sparing pain management strategies has begun, aiming to control pain while limiting the use of opioids and their associated adverse effects. Peripheral nerve blockade, either single-shot or continuous catheter techniques, constitute an important cornerstone of these strategies. This systematic review and network meta-analysis aimed to compare and evaluate the effects of different single-shot ultrasound-guided regional anesthetic techniques on pain control following open cardiac surgical procedures in pediatric patients. A systematic review and comprehensive search were conducted to retrieve available randomized clinical trials focusing on the use of ultrasound-guided regional anesthesia nerve blockade as part of a multimodal analgesia approach in pediatric patients undergoing open cardiac surgery through median sternotomy. Our search was conducted in major databases, including MEDLINE, EMBASE, Scopus, Web of Science, and Cochrane Central Register of Controlled Trials. The main outcomes of interest were perioperative opioid consumption and postoperative pain scores. A total of 1,511 articles were retrieved for the initial screening of titles, with 16 RCTs deemed eligible and included after full-text screening. Eleven different regional nerve blocks were assessed in the studies, including erector spinae plane block (ESPB), thoracic paravertebral block (PVB), deep parasternal block (DPB), superficial parasternal block (SPB), thoracic retrolaminar paravertebral block (TRLB), pectoral nerve block type II (PECS II), and multiple injection costotransverse block (MICB). Compared to no intervention, parasternal blocks particularly DPB and SPB were associated with a significant reduction in both intraoperative and 24-h postoperative fentanyl consumption. Postoperatively, fentanyl consumption was reduced by -4.92 µg/kg (95% CI: [-6.58 to -3.26]) with DPB, -2.54 µg/kg (95% CI: [-4.16 to -0.91]) with SPB, and -5.6 µg/kg (95% CI: [-8.31 to -2.89]) with TRLB. Intraoperatively, SPB reduced fentanyl use by -5.12 µg/kg (95% CI: [-6.8 to -3.43]) and DPB by -4.31 µg/kg (95% CI: [-5.9 to -2.71]). No significant postoperative opioid-sparing effect was observed with other blocks, such as ESPB, PVB, MICB, or PECS II. The pooled estimates showed considerable heterogeneity, with I2 values of 90.9% for intraoperative and 94.7% for postoperative fentanyl consumption. Ultrasound-guided regional anesthesia-particularly parasternal blocks-may offer superior opioid-sparing effects and enhanced recovery in children undergoing open-heart surgery, making them promising adjuncts to multimodal analgesia. In contrast, blocks such as ESPB, PVB, and MICB showed limited benefit for median sternotomy pain. Given the substantial heterogeneity in the current evidence, the routine use of these techniques should be guided by institutional experience and individualized patient factors.

In pediatric cardiac critical care, patients often require specific anesthesia and sedation considerations due to unique physiological vulnerabilities. This study assessed the incidence of propofol-related infusion syndrome (PRIS) in Pediatric Cardiac Intensive Care Unit (PCICU) patients receiving continuous propofol infusion for non-procedural sedation. We conducted a retrospective review of post-operative congenital heart disease patients < 18 years admitted to the PCICU from 1/1/2000-9/30/2024, who received continuous propofol infusions ≥ 12 hours and/or ≥ 2.4 mg/kg/hour. The primary outcome was the incidence of PRIS. Secondary outcomes included intensive care unit (ICU) length of stay and survival to hospital discharge. Statistical analyses included chi-square tests and the Kruskal-Wallis test, stratified by physiological complexity (single vs. double ventricle) and surgical mortality risk scores (The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery [STAT] score). A total of 641 patients were included. No statistically significant differences in propofol characteristics were found across physiologic or surgical risk groups. The median infusion rate was 1.2 mg/kg/hour, with a median duration of 22.7 hours. No cases of PRIS occurred. Survival to hospital discharge was 97%. The median ICU length of stay was 7.1 days. In this single center, retrospective study of post-operative congenital heart disease patients, continuous propofol infusion was not associated with any cases of PRIS. These findings support the cautious use of propofol in pediatric cardiac critical care. Further prospective studies are needed to evaluate safety across the heterogenous congenital heart disease population, including those with single ventricle physiology.

Fontan palliation is associated with an increased risk for arrhythmias and sinus node dysfunction. Although annual Holter monitoring is recommended for routine surveillance, there is no normative Holter data for this population, with only one study describing such data in healthy children. To describe Holter data for patients with Fontan physiology, compare this data to healthy children, and establish a normative data set for those undergoing routine ambulatory ECG screening. This retrospective, single-center study analyzed 642 Holter monitors from 133 Fontan patients aged 5-21 years, collected between 1970 and 2023. Patients with pacemakers, significant atrioventricular valve regurgitation, or severely depressed ventricular function were excluded. Demographic and clinical data were collected, and HR values were compared to a healthy control cohort. The Fontan cohort was predominantly male (69%), with right ventricular dominance (59%) and extracardiac Fontan palliation (80%). Compared to 502 healthy controls, Fontan patients had significantly higher minimum HRs in adolescents (14-16 years), lower average HRs in younger age groups (4-12 years), and universally lower maximum HRs. Within the Fontan group, males and those on digoxin had lower minimum HRs, while heterotaxy was associated with higher minimum and average HRs. This is the first study to establish normative Holter HR data in pediatric Fontan patients and compare it to healthy counterparts. These findings suggest Fontan patients do not have significantly lower minimum heart rates and provide a valuable reference for clinical evaluation, supporting the need for larger, multicenter studies.

This case report details a neonate with functional pulmonary atresia (FPA), initially misdiagnosed prenatally as anatomical pulmonary atresia. Fetal findings included cardiomegaly, severe tricuspid regurgitation, and right ventricular dysfunction. Postnatally, the absence of antegrade pulmonary flow raised concern for anatomical atresia. However, saline contrast echocardiography definitively confirmed patent pulmonary valves, establishing the diagnosis of transient FPA secondary to severe functional right heart impairment without anatomical atresia of the pulmonary valves. This case highlights the potential value of the saline contrast test in distinguishing FPA from anatomical pulmonary atresia, which can help avoid unnecessary interventions. It also provides additional insight into the natural history of prenatally suspected isolated FPA.

Histoplasmosis in children remains understudied, drawing most clinical understanding from adult populations. Located in a histoplasmosis-hyperendemic region, our institution aimed to characterize the cardiovascular manifestations and interventional treatments in children diagnosed with histoplasmosis. We conducted a retrospective cohort study of 160 patients under 18 years of age diagnosed with histoplasmosis between January 2009 and March 2023 at a tertiary-care children's hospital. Data reviewed included clinical presentation, imaging, electrocardiograms, echocardiograms, and catheter-based or surgical interventions. Of the 160 patients, 65 (41%) demonstrated cardiovascular involvement from mediastinal manifestations of histoplasmosis (mediastinal lymphadenopathy, mediastinal granuloma, and fibrosing mediastinitis) including all 8 patients with fibrosing mediastinitis. Among these 65 children, pericarditis with pericardial effusion was the most common (58%), 30 patients requiring urgent intervention. Other manifestations from mass effect included pulmonary artery stenosis (34%), superior vena cava (SVC) stenosis (15%), pulmonary vein stenosis (8%), inferior vena cava (IVC) stenosis (3%), and aortic arch obstruction (2%). Fourteen patients underwent catheter-based stenting procedures with a total of 22 stents placed (17 in pulmonary artery branches, 4 in pulmonary veins, and 1 in the SVC), typically not performed during the initial presentation. Five patients underwent mass resection for cardiac indications. Cardiovascular complications from histoplasmosis-related mediastinal lymphadenopathy in children are significant, presenting with pericarditis or compression of central vascular structures. Fibrosing mediastinitis was consistently associated with cardiac involvement, frequently necessitating intervention. Surgical repair is challenging due to extensive fibrosis. However, percutaneous vascular interventions are feasible and effective in managing vascular obstructions. Early recognition and timely intervention are critical to prevent irreversible vascular atresia.