Pediatric Cardiology最新文献

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.

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.

Pulmonary arteriovenous fistulas (PAVFs) are rare congenital anomalies characterized by abnormal vascular connections that bypass the capillary bed, causing hypoxemia, cyanosis, and systemic embolic risks. Early diagnosis and intervention are critical to prevent complications, particularly in neonates with severe right-to-left shunts. This report describes the first documented case of neonatal transcatheter closure of a large PAVF using a KONAR-MF™ VSD Occluder (MFO). A 1-day-old neonate with a prenatally diagnosed PAVF presented with oxygen saturation of 82%. Angiographic and echocardiographic assessments confirmed the presence of a large PAVF between the right pulmonary artery and right inferior pulmonary vein. The fistula was successfully closed using an 8 × 6 mm MFO device, with no complications. Post-procedure, oxygen saturation improved to 100%, and follow-up at 5 months demonstrated sustained closure. This report highlights the safety and versatility of the MFO occluder in complex cardiac cases, offering a minimally invasive alternative to surgical intervention.

Premature and small-for-gestational-age neonates with congenital heart disease increasingly require congenital cardiac catheterization (CCC). These patients present unique procedural and patient-specific risks that standard risk models do not fully capture. This study aims to assess risk in infants < 2.5 kg undergoing CCC, further stratifying by procedural type to better understand predictors of clinically meaningful adverse events (CMAE). Patient and Procedural data were collected on diagnostic and interventional catheterization procedures for infants < 2.5 kg from the Congenital Cardiac Catheterization Project on Outcomes (C3PO) registry between 2014 and 2022. Cases were stratified into historical (2014-2018) and modern (2019-2022) eras and further categorized into PDA closure and 'All Other Cases'. Multivariable logistic regression assessed associations between covariates and the risk of CMAE. Analysis included 1,345 cases. In the modern era, PDA closures (n = 898, 66.8%) had a lower CMAE rate at 3.6% versus 8.1% for 'All Other Cases' (P < 0.001). Among 'All Other Cases' (n = 447) 23% were diagnostic and 77% interventional, with CMAE rates of 7.7% and 6.4%, respectively. CMAE types varied, with PDA cases mainly experiencing respiratory events (22%) and 'All Other Cases' showing higher rates of access complications (27%) and arrhythmias (29%). Risk in infants < 2.5 kg undergoing CCC is heavily dependent on procedural type and specific patient factors, highlighting the need for tailored risk assessment tools. This study, the largest to date in this population, emphasizes the importance of individualized care plans to improve outcomes.

We sought to determine the impact of the COVID-19 on prenatal diagnosis (PND) of clinically significant congenital heart disease (CHD) and the role of socioeconomic status (SES), complexity of diagnosis, and proximity to advance testing. This single-center retrospective study evaluated 2 eras of infants (COVID (born July 1, 2020-July 31, 2023) and pre-COVID (born June 1, 2017-July 1, 2020) who had cardiac surgery in the first year of life. 512 infants, 292 in pre-COVID era and 220 in COVID era with no significant difference in the rate of prenatal care (PNC) or PND in the COVID era (88%/42%) versus pre-COVID era (93%/48%) (χ² = 3.22, p = 0.07, χ² = 1.9, p = 0.17). Distance from advanced testing had no influence on PND in the COVID era [55% close versus 53% further away (χ² = 2, p = 0.65)]. When evaluating SES with income per zip code, the higher SES group had increased PND during the pandemic compared to both pre-COVID era and low SES group. However, social deprivation index (SDI) based on zip code showed the higher SES group had a decrease in PND rates. Both metrics showed no change in PND in the lower SES group during COVID. COVID-19 had no significant change in the PND of clinically significant CHD during the pandemic. The differing SES results using income versus SDI of patient zip codes suggest that barriers to PND is multifactorial. The discrepancy in PND reflects poor referral rates to advanced testing, highlighting the importance of educating frontline healthcare professionals to improve outcomes.