Pediatric Cardiology最新文献

Improved survival following Fontan surgery is tempered by late Fontan failure. Heterogeneous multi-organ complications contribute to eventual Fontan failure varying over time. The prevalence of Fontan-related complications and their association with adverse outcomes were evaluated. A Fontan cohort of 668 pediatric (n = 490) and adult (n = 178) patients from two healthcare systems were linked to 2010-2019 healthcare encounters and death certificates. Bivariate analyses examined covariates by the primary composite outcome of heart transplant, hepatocellular carcinoma (HCC) or death. Multivariate logistic regression explored associations of risk factors with the primary composite outcome and a secondary outcome of hospitalization. Kaplan-Meier curves compared freedom from the primary composite outcome stratified by number of concurrent risk factors at initial encounter. Of 668 Fontan patients, 12.3% developed the primary composite outcome (21.3% adults, 9.0% children). Clinical risk factors increased with age and time, and were significantly associated with HCC, a component of the primary outcome. Patients with 3 + risk factors had significantly worse survival (log-rank p < 0.01). Heart failure (HF) was the strongest independent predictor of outcomes (aOR 5.90, p < 0.01). Dysrhythmia, cyanosis, and HF were associated with more hospitalizations. The primary composite outcome was 4-fold higher with 3 + risk factors (aOR 3.71, 95% CI 2.26-6.16), significant in children (aOR 3.01, 95% CI 1.17-7.0), and adults (aOR 2.58, 95% CI 1.04-7.48). Complications increase as Fontan patients age and are associated with transplant, HCC, death, and hospitalizations in a pediatric and adult cohort. Early identification and intervention for Fontan-related complications may improve late outcomes.

Transcatheter pulmonary valve replacement (TPVR) is effective for treating significant pulmonary regurgitation (PR), but mid-term data in patients with pyramidal right ventricular outflow tract (RVOT) anatomy are limited. This multi-center retrospective study included patients who underwent TPVR between May 2014 and September 2023. Baseline and echocardiographic data were compared between patients with pyramidal and non-pyramidal RVOT. The primary endpoint was peri-procedural device success, defined as technical success, optimal valve function on discharge echocardiography (RVOT gradient < 30 mmHg, less than moderate PR, no significant paravalvular leak), and absence of in-hospital mortality or re-intervention. The secondary endpoint was 1-year freedom from all-cause mortality, cardiac hospitalization, endocarditis, significant stent fracture, valve dysfunction, and device-related re-intervention. Sixty-eight patients were enrolled. Device success was achieved in 86.7% of pyramidal RVOT patients and 100% of non-pyramidal patients (P = 0.067). At 1 year, no significant difference was observed in the composite secondary endpoint (91.7% vs. 86.0%, P = 0.605). Both groups showed similar improvements in echocardiographic and clinical outcomes. Additionally, no statistically significant differences were observed between the two groups regarding post-TPVR regurgitation and paravalvular leakage. TPVR using self-expanding valves in pyramidal RVOT showed acceptable device success rates and favorable mid-term outcomes in both clinical and hemodynamic parameters. Morphology-based pre-procedural planning is essential for optimizing results.

The American College of Cardiology's Adult Congenital and Pediatric Cardiology Quality Network has developed a metric for assessing comprehensive transthoracic echocardiographic (TTE) exam. This study evaluates the relationship between the study comprehensiveness score (CS) and diagnostic errors in patients undergoing congenital heart surgery. The echocardiography lab quality database at a single-center identified preoperative TTEs with diagnostic errors when compared with transesophageal echocardiograms or direct surgical inspection from 1/1/2018 to 8/1/2023. TTEs without diagnostic errors served as controls (similar age, gender, and surgical risk score). TTEs were assigned a CS by a single blinded reviewer. Diagnostic errors were characterized by types of errors, primary contributors to errors (procedural/conditional, cognitive, technical, patient-and-disease-related factors), severity, and preventability. Of the 2448 TTEs, 159 (6.5%) had diagnostic errors. There was no significant difference in CS between those with diagnostic errors compared to controls (95.4% vs. 93.1%, p = 0.06). CS was significantly associated with contributors to errors (p = 0.004). Cognitive factors were the most frequent contributors to errors (51.6%), followed by technical factors (33.4%), procedural/conditional factors (7.5%), and patient-and-disease-related factors (7.5%). CS > 90% was associated with cognitive errors (p = 0.042), which contributed the most to errors. CS ≤ 90% was associated with procedural/conditional errors (p = 0.001). Application of the CS did not help discriminate studies with diagnostic errors. Cognitive errors accounted for half of the errors and had a high CS. While the CS may prevent some procedural errors, these constitute a minority. Future quality interventions should target cognitive errors.

The use of intracardiac echocardiography (ICE) has been well established for use in catheter ablation of arrhythmias and offers direct visualization of atrial septum and left-sided structures in adults. There, however, is a dearth of data in the pediatric population. The primary objective of this study was to determine the outcomes of left-sided accessory pathway catheter ablation in children and young adults where ICE was used in addition to 3-dimensional (3D)/traditional mapping and to compare outcomes in catheter ablations where only traditional fluoroscopy was used in addition to 3D/traditional mapping. The results of this study showed that the use of ICE showed no significant differences in major adverse events when compared to traditional fluoroscopy. In conclusion, ICE is an effective and safe modality to use in left-sided accessory pathway ablation in pediatric populations, but further large-scale studies and cost-benefit analysis are needed to determine its practical usage compared to traditional fluoroscopy.

TAVR is an alternative to surgical aortic valve [AoV] replacement. In adults, rates of atrioventricular block [AVB] requiring permanent pacemaker [PPM] placement in the modern era are 4-24%. Post-TAVR conduction abnormality incidence and risk factors are unknown in children and young adults. Describe post-TAVR conduction abnormalities in children and young adults. Retrospective single-center review of patients undergoing TAVR (9/2014 to 6/2021). Patients with pre-existing complete AVB or PPM were excluded (N = 1). The relationship between described adult risk factors for AVB and primary outcome of new conduction abnormality was assessed. Of 28 cases (Ages 3.5-22 y), 50% were male and 43% had isolated AoV disease, and the remainder with multilevel obstructive left-sided heart disease (29%) or complex congenital heart disease (29%). Baseline conduction abnormality was present in 57% (16/28), with right bundle branch block the most prevalent (9, 56%). Post-TAVR, acute- and late-onset conduction abnormalities occurred in 9 and 2 patients, respectively, and resolved in 8/11 patients during follow-up. One patient required PPM for complete heart block. There was no association between new conduction abnormality and previously reported adult risk factors-baseline RBBB, membranous septum length, valve implantation depth, or degree of valve oversizing. There was no relationship between outcome and baseline conduction abnormality nor history of multiple AoV interventions. In our pediatric series, AVB requiring PPM was rare following TAVR, with an incidence 3.6%-lower than average rates reported in adult literature. We identified no association of conduction abnormality with described adult risk factors.

The Accreditation Council for Graduate Medical Education and the American Board of Pediatrics currently require a minimum of 12 months dedicated to research during pediatric cardiology fellowship training. Current data regarding the implementation of this requirement in pediatric cardiology fellowship programs are not available. We aimed to characterize current perceptions and practices of program directors regarding research supports, practices, and training in pediatric cardiology fellowship training programs. A web-based survey facilitated by the Society of Pediatric Cardiology Training Program Directors was sent to the 64 fellowship program directors in the USA. Data from responses were aggregated and reported. A total of 46 program directors responded to the survey for a response rate of 72%. Most programs (n = 39, 84.8%) included 12 months of research in their current curricula. Most programs included overnight call (n = 39, 84.8%), mandatory academic requirements (n = 42, 91.2%), and additional mandatory clinical responsibilities (n = 37, 80.4%) during research time. Many fellows pursued additional non-mandatory and elective clinical time during their research months. Most program directors (n = 29, 63.0%) thought the requirement for 12 months of research during the 36-month fellowship training period should be shortened. Most pediatric cardiology program directors believe the current 12-month research requirement should be shortened, with a goal of providing greater flexibility in training and a more individualized curriculum. Fellows wishing to perform additional research could utilize the additional elective rotations created by this change.

The NatA complex is composed of the NAA10, NAA15, and HYPK sub-units. It is primarily responsible for N-terminal acetylation, a critical post-translational modification in eukaryotes. Pathogenic variants within NAA10 cause Ogden Syndrome (OS), which is characterized by varying degrees of intellectual disability, hypotonia, developmental delay, and cardiac abnormalities. Although the cardiac manifestations of the disease have been described extensively in case reports, there has not been a study focusing on the cardiac manifestations and their recommended clinical cardiac management. In this study, we describe the cardiac manifestations of OS in a cohort of 85 probands. We found increased incidence of structural and electrophysiologic abnormalities, with particularly high prevalence of QT interval prolongation. Sub-analysis showed that male probands and those with variants within the NAA15-binding domain had more severe phenotypes than females or those with variants outside of the NAA15-binding domain. Our results suggest that an OS diagnosis should be accompanied by full cardiac workup with emphasis on echocardiogram for structural defects and EKG/Holter monitoring for electrophysiologic abnormalities. Additionally, we strongly recommend that the use of QT-prolonging drugs be followed up with routine electrophysiological monitoring or consultation with a pediatric cardiologist. We hope this study guides clinicians and caregivers treating patients with OS and moves the field toward a standardized diagnostic workup for patients with this condition.

The "nutmeg lung pattern" on fetal magnetic resonance imaging (MRI) indicates pulmonary lymphangiectasia. This is associated with adverse outcomes, particularly in fetuses with congenital heart defects and impaired pulmonary venous return. Whereas lymphedema is common in fetuses with Turner syndrome, pulmonary lymphangiectasia is not. A 26-year-old woman presented with a fetus with hypoplastic left heart syndrome (HLHS) without restrictive atrial septum (RAS). The family declined amniocentesis, yet non-invasive prenatal testing showed an increased risk for Turner syndrome. The patient underwent a fetal MRI as part of a blinded research protocol. Postnatal echocardiogram confirmed the fetal echocardiographic findings without evidence of RAS. Norwood stage I palliation was performed at two days of age. Significant neonatal respiratory morbidity including pneumonia, diaphragmatic and vocal cord pareses and chylothorax occurred. Subsequent review of fetal MRI revealed a prominent thoracic duct and mild pulmonary lymphangiectasia. Turner syndrome was confirmed by genetic testing. After one month, the patient was discharged to her home hospital with respiratory support, which was discontinued a few weeks later. Respiratory problems continued, but the vocal cord paresis resolved over time. This is a unique case of a fetus with HLHS/non-RAS with mild fetal pulmonary lymphangiectasia, and significant neonatal respiratory morbidity, probably in part due to Turner syndrome. The infant survived the neonatal period and underwent uneventful Glenn surgery. The patient's tolerance to the total cavopulmonary connection (TCPC) procedure is yet to be seen. An MRI lymphography should precede it to assess residual lymphatic abnormalities and serve as baseline for post-TCPC changes.

To investigate the correlation between prognostic nutritional index (PNI) and vasovagal syncope (VVS) in children, as well as its predictive value. 151 children (68 males, aged 4-18 years) diagnosed with VVS due to unexplained syncope and presyncope in our hospital from January 2022 to December 2023 were the study group, 152 healthy children (72 males, aged 7-14 years) who underwent physical examination in the same hospital during the same period of time were matched as the control group. Serum albumin (SA), serum globulin (SG), albumin/globulin (AGR), and peripheral blood lymphocyte absolute count (Lc) were measured, and PNI was calculated. ①PNI (51.35 vs. 55.28), SA (40.90 g/L vs. 43.05 g/L), AGR (1.65 vs. 1.75), and Lc (2.08 × 10⁹/L vs. 2.49 × 10⁹/L) were decreased in VVS group compared with control group (P < 0.05). ②Predictive analysis of VVS by PNI: The area under receiver operator characteristic curve of PNI prediction of VVS was 0.814, which indicated that PNI had moderate predictive value for VVS diagnosis. When PNI cutoff value was 55.00, the sensitivity, specificity, and Youden index of predicting VVS were 90.73%, 54.61%, and 0.45, respectively. ③PNI (OR = 0.65, 95%CI: 0.58-0.74, P < 0.001) is also as an independent protective factor for VVS. PNI is correlated with VVS in children, holds predictive value for the diagnosis of pediatric VVS, and serves as an independent protective factor for VVS occurrence in children.

Cardiac tumors in children, although rare, can lead to obstruction of valves or cardiac conduction problems. Large multi-site or national data on pediatric cardiac tumors is lacking. The objective of this study was to determine the prevalence of primary cardiac tumors in hospitalized children from 1997 to 2019 in the US and analyze the demographic and clinical characteristics of children with cardiac tumors. We conducted a cross-sectional analysis using the Healthcare Cost and Utilization Project's Kid's Inpatient Database from 1997 to 2019. Children with cardiac tumors were identified using ICD-9 and ICD-10 diagnosis codes. The prevalence is presented per 100,000 hospital discharges and 1,000,000 age-specific US population. The Chi-square test was used to compare the discharges with and without cardiac tumors for demographic and clinical variables. The Chi-square for linear trend (Extended Mantel-Haenszel) test was used for trend analyses. There were 3,166 discharges with primary cardiac tumors out of a total of 55.1 million discharges with a hospitalization rate of 5.8 per 100,000 discharges and 4.82 per million US children. Out of all children with cardiac tumors, 29.9% were neonates, 20.7% were infants, and 71% were children less than 5 years of age. Males comprised 52.9% of all cases. An increase in the prevalence of cardiac tumors from 1997 to 2019 was observed (p < 0.001). Of all admissions with cardiac tumors, 88.9% were reported benign, and 37.3% were admitted to Children's Hospitals. Cardiac arrhythmias were reported in 19.2%, seizures in 28%, and stroke in 1.4%. 51.9% of cases were associated with tuberous sclerosis. Cardiac surgical procedures were performed in 13.9% of patients. The overall hospital mortality rate was 1.6%. Primary cardiac tumor is a rare condition in children and is most common in neonates and young children. Most pediatric cardiac tumors are benign, and many are associated with tuberous sclerosis.