Pediatric Cardiology最新文献

Peripheral perfusion index (PPI) represents the ratio of pulsatile to non-pulsatile blood flow in an extremity and may be a valuable supplement to pulse oximetry in identification of critical congenital heart disease (CCHD). To date, reference ranges of PPI of healthy neonates have not been published in the United States. We performed an observational retrospective study evaluating the PPI of healthy neonates between 24 and 48 h of life born in Minnesota, United States based on electronic reporting of CCHD screening to the Minnesota Department of Health. We evaluated preductal and postductal PPI measured at 24-48 h of age in 11,179 healthy infants born between 35- and 41-weeks gestation. We found that preductal PPI is significantly greater than postductal PPI and that PPI increases with gestational age and with birth weight. There was no difference in PPI by sex when adjusting for birth weight. Smoothed percentile curves of preductal and postductal PPI by gestational age were derived using the GAMLSS R Package with a Sinh-Arcsinh distribution. Our study is the first large, population-based study to establish normative data for PPI in healthy neonates in the United States. Our data overall replicate trends observed in large studies from India and China. Further research is needed to determine feasibility and utility of incorporation of PPI into CCHD screening.

Background: Left atrial (LA) volume and function are important for assessing pediatric diastolic dysfunction (DD). Three-dimensional echocardiographic (3DE) LA assessment may overcome limitations and assumptions inherent in two-dimensional echocardiographic (2DE).

Methods: We conducted a cross-sectional study of children under 18 years of age with structurally normal hearts, using transthoracic 3DE apical volume datasets. LA volumetric function and phasic deformation analyses were performed using 3DE (GE, EchoPAC 4DLAQ v206, near fully-automated) and 2DE (TomTec, TTA2.51, CPA, manual) was performed by three operators and processing time was collected. Agreement and reproducibility was assessment for each modality.

Results: Fifty patients (median age 12.8 years) were included. Processing time for 3DE was shorter than for 2DE (0.6 vs. 2.7 min, p < 0.001). 3DE and 2DE showed good agreement for maximum LA volume (LAV_max, ICC = 0.79), with 3DE estimates being lower on average by 5.9 mL than 2DE. Agreement for 3DE LA reservoir strain (LAS_r) was weak (ICC = 0.17), with 3DE values lower by an average of 16.6%. Interobserver agreement for 3DE LAV_max was excellent (ICC = 0.94, 95% CI 0.90-0.96) and comparable to 2DE (ICC = 0.86, 95% CI 0.63-0.94, p = 0.16). Intra-observer reliability for LA volume was similar between 2DE to 3DE (p = 0.11-0.85). No meaningful differences were found in inter-/intra-observer reliability for LA strain between modalities.

Conclusion: 3DE provides excellent interobserver reproducibility for LA volume assessment and faster processing times compared to 2DE. 3DE and 2DE show good agreement for LA volume. Agreement for LA strain is weak. Further studies are needed to determine which better predicts clinical outcomes.

We retrospectively reviewed 895 patients with Kawasaki disease treated at two Japanese pediatric centers between 2015 and 2024 to assess whether N-terminal pro-brain natriuretic peptide (NT-pro BNP) levels at diagnosis can predict the need for infliximab (IFX) therapy. Patients were divided into (1) the IFX group (n = 35), including patients who received IFX as third-line therapy due to resistance to first- and second-line treatments, including intravenous immunoglobulin, and (2) the non-IFX group (n = 860), including patients who responded to initial therapies. Clinical and laboratory variables were compared between the groups, and predictors of IFX use were analyzed using multivariate logistic regression and receiver operating characteristic (ROC) curves. Multivariate analysis comparing the IFX and non-IFX groups identified older age, lower hemoglobin levels, and higher NT-pro BNP Z-score at diagnosis as independent predictors of later IFX requirement. Additionally, the incidence of coronary arterial lesions did not differ significantly between the groups. ROC analysis demonstrated NT-pro BNP Z-score as a significant predictor (AUC 0.66, p < 0.001), with a cutoff of 2.2 yielding 71.4% sensitivity, 63.3% specificity, 7.3% positive predictive value, and 98.2% negative predictive value. Elevated serum NT-pro BNP Z-score at diagnosis was associated with later IFX use, whereas a low Z-score reliably identified patients unlikely to require IFX. Early assessment incorporating NT-pro BNP may optimize Kawasaki disease therapy and guide the appropriate timing of IFX administration.

Post-Fontan patients often face significant hemodynamic challenges, including systolic dysfunction and diminished cardiac output due to reduced preload and venous return. Long-term complications such as hepatic dysfunction and protein-losing enteropathy arise from poor venous return, increasing morbidity and mortality. Enhancing venous return could potentially improve long-term outcomes for these patients. This literature review examines the role of the skeletal muscle pump in Fontan circulation and evaluates non-pharmacological strategies to augment venous return, with discussion of mechanical compression devices as a potential translational application. A comprehensive literature search identified 26 studies focusing on the relationship between lower limb muscle mass, venous return, and cardiac performance in post-Fontan patients, as well as the efficacy of peristaltic leg pumps in enhancing venous return. Across included studies, higher lower-limb skeletal muscle mass and structured exercise interventions were consistently associated with improved functional and cardiopulmonary performance metrics in Fontan patients. Additionally, the use of peristaltic leg pumps in other patient cohorts enhanced venous return across various patient positions, suggesting an avenue for implementation in the Fontan patient. Peristaltic leg pumps may serve as a valuable tool in managing the hemodynamic challenges of post-Fontan patients, potentially leading to better long-term outcomes. Further research, particularly randomized controlled trials, is necessary to establish the efficacy and optimal application of these devices in the paediatric Fontan population.

KONAR-MF™ device is a novel, cone-shaped, medium profile, self-expanding, double-disk, nitinol occlusive device designed for the ventricular septal defect (VSD). Due to its unique design and easy deployment from either side and multiple occlusion layers, it has also been used to occlude defects other than VSD. A retrospective review was done from 2019 to 2024 from three institutions. All patients where the KONAR-MF™ device was used other than for VSD closure were included in this study. Standard post-procedure follow-up was at 1, 6, and 12 months for all patients. 79 off-label implantations of the KONAR-MF™ device were done for conditions that included 59 shunt lesions (patent ductus arteriosus -34, coronary arteriovenous fistula -12, pulmonary arteriovenous fistula -2, systemic arteriovenous fistula -3, aortopulmonary window -3, aortopulmonary collateral -5, Fontan fenestration closures -7, Fontan antegrade pulmonary valve closures -2, Abernethy malformations -3, decompressing vein -2, and paravalvular leak -2, others 4. The median fluoroscopy time was 10 min (IQR 6-18). The median duration of hospital stay was 2 days (IQR 1-4 days). There were no significant complications. Complete occlusion at the end of the procedure was documented in 72 (91.13%) patients. At a median follow-up of 18 months (IQR 12-28 months) in all except one patient who had mild residual flow with no device-/(procedure) related complications. The unique structure and compact profile of KONAR-MF™ enable a wide range of uses in catheter-based management of various CHDs with the potential to simplify the inventory of the catheterization laboratory.