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Junctional ectopic tachycardia (JET) complicates congenital heart surgery in 2% to 8.3% of cases. JET is associated with postoperative morbidity in single-center studies. We used the Pediatric Cardiac Critical Care Consortium data registry to provide a multicenter epidemiologic description of treated JET. This is a retrospective study (February 2019-August 2022) of patients with treated JET. Inclusion criteria were (1) <12 months old at the index operation, and (2) treated for JET <72 hours after surgery. Diagnosis was defined by receiving treatment (pacing, cooling, and medications). A multilevel logistic regression analysis with hospital random effect identified JET risk factors. Impact of JET on outcomes was estimated by margins/attributable risk analysis using previous risk-adjustment models. Among 24,073 patients from 63 centers, 1436 (6.0%) were treated for JET with significant center variability (0% to 17.9%). Median time to onset was 3.4 hours, with 34% present on admission. Median duration was 2 days (interquartile range, 1-4 days). Tetralogy of Fallot, atrioventricular canal, and ventricular septal defect repair represented >50% of JET. Patient characteristics independently associated with JET included neonatal age, Asian race, cardiopulmonary bypass time, open sternum, and early postoperative inotropic agents. JET was associated with increased risk-adjusted durations of mechanical ventilation (incidence rate ratio, 1.6; 95% CI, 1.5-1.7) and intensive care unit length of stay (incidence rate ratio, 1.3; 95% CI, 1.2-1.3), but not mortality. JET is treated in 6% of patients with substantial center variability. JET contributes to increased use of postoperative resources. High center variability warrants further study to identify potential modifiable factors that could serve as targets for improvement efforts to ameliorate deleterious outcomes. [ABSTRACT FROM AUTHOR]

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.ahj.2007.02.019 Byline: Mark A. Law (a), Ronald G. Grifka (b), Charles E. Mullins (a), Michael R. Nihill (a) Abstract: Atrial septostomy is a palliative treatment for patients with pulmonary hypertension (PHTN) refractory to vasodilator therapy. Limited data exist in the pediatric population and in patients with repaired congenital heart defects. Author Affiliation: (a) Section of Cardiology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, MC 19345-C, Houston, TX (b) Section of Cardiology, Department of Pediatrics, DeVos Children's Hospital, Grand Rapids, MI Article History: Received 10 November 2006; Accepted 16 February 2007

Cor triatriatum dexter is an extremely rare congenital heart defect and cause of hypoxia in adults. We describe a case of cor triatriatum dexter discovered incidentally due to an iatrogenic atrial septal defect. The cor triatriatum dexter resolved with balloon dilation - a novel technique to manage this rare clinical condition. < Learning objective: Management of cor triatriatum dexter, an exceptionally rare diagnosis, has often been thought to be surgical. However, percutaneous intervention may be considered in select clinical situations.> [ABSTRACT FROM AUTHOR]

Percutaneous coronary intervention (PCI) is an uncommon procedure in the pediatric population, and the use of intravascular ultrasound (IVUS) to guide stent optimization in this setting is not well described. We present a case of a 22-month old female who developed left main coronary stenosis following surgical correction of anomalous left coronary artery from the pulmonary artery (ALCAPA). PCI was pursued, and with the use of IVUS, stent placement was determined to be optimal. Given the limited data on the use of IVUS in PCI for children, we discuss the unique applications of IVUS, drug-eluting stents, and future clinical concerns following management of post-surgical stenosis following ALCAPA repair. 〈 Learning objective: Percutaneous coronary intervention is a rare procedure in the pediatric population, and the use of intravascular ultrasound to optimize stent placement is not well described in this setting. Intravascular ultrasound may allow for more precise stent placement and may be useful during acute and long-term management of pediatric patients undergoing percutaneous coronary intervention.〉 [ABSTRACT FROM AUTHOR]

Neonates with tetralogy of Fallot and pulmonary atresia (TOF/PA) but no major aorta-pulmonary collaterals are dependent on the arterial duct for pulmonary blood flow and require early intervention, either by primary (PR) or staged repair (SR) with initial palliation (IP) followed by complete repair (CR). The optimal approach has not been established. Neonates with TOF/PA who underwent PR or SR were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Outcomes were compared between PR and SR (IP + CR) strategies. Propensity scoring was used to adjust for baseline differences. The primary outcome was mortality. Secondary outcomes included complications, length of stay, cardiopulmonary bypass and anesthesia times, reintervention (RI), and pulmonary artery (PA) growth. Of 282 neonates, 106 underwent PR and 176 underwent SR (IP: 144 surgical, 32 transcatheter). Patients who underwent SR were more likely to have DiGeorge syndrome and greater rates of mechanical ventilation before the initial intervention. Mortality was not significantly different. Duration of mechanical ventilation, inotrope use, and complication rates were similar. Cumulative length of stay, cardiopulmonary bypass, and anesthesia times favored PR (P ≤.001). Early RI was more common in patients who underwent SR (rate ratio, 1.42; P =.003) but was similar after CR (P =.837). Conduit size at the time of CR was larger with SR. Right PA growth was greater with PR. In neonates with TOF/PA, SR is more common in greater-risk patients. Accounting for this, SR and PR strategies have similar mortality. Perioperative morbidities, RI, and right PA growth generally favor PR, whereas SR allows for larger initial conduit implantation. [Display omitted] [ABSTRACT FROM AUTHOR]

Background. Superior vena cava oxygen saturation monitoring in the early postoperative period after the Norwood procedure (NP) has been associated with improved survival and decreased adverse events (AE). There is no data describing inferior vena cava saturation (SIVO2) monitoring after NP. We sought to investigate the utility of intermittent SIVO2 monitoring after NP and to assess the correlation of SIVO2 with renal near-infrared spectroscopy (rNIRS). We hypothesized failure to achieve SIVO2 greater than 45% within the first 4 hours after NP is predictive of AE, and that rNIRS correlates with SIVO2. Methods. A retrospective study of 26 consecutive NP patients who received postoperative management with SIVO2 monitoring according to a strict protocol was conducted. Primary outcome was AE, defined as cardiopulmonary resuscitation, extracorporeal membrane oxygenation, death before discharge, or residual surgical defects. Results. Ten (38%) patients had one or more AE; mortality was 23%. On admission to the cardiac intensive care unit, patients with AE had lower SIVO2 (45% ± 9.4% versus 62% ± 12.0%; p < 0.001) and lower rNIRS (56 ± 6.5 versus 77 ± 7.2; p < 0.001). At 4 hours, 90% of AE patients had an SIVO2 less than 45% versus 6% of non- AE patients. Both SIVO2 and rNIRS were highly predictive of AE: the area under the receiver-operating characteristic curve was greater than 0.86 and 0.95, respectively. Two hours after admission, an SIVO2 less than 45% predicted AE with a specificity of 93%, a sensitivity of 70%, and a positive predictive value of 82%. The SIVO2 was strongly correlated with rNIRS (r = 0.81). Conclusions. Intermittent SIVO2 can be used to guide early postoperative NP management; rNIRS is an accurate continuous, noninvasive surrogate for SIVO2. An SIVO2 of less than 45% in the first 4 hours after the NP is predictive of AE. [ABSTRACT FROM AUTHOR]

To compare management strategies for neonates <2.5 kg with tetralogy of Fallot and symptomatic cyanosis who either undergo staged repair (SR) (initial palliation followed by later complete repair) or primary repair (PR). Consecutive neonates with tetralogy of Fallot and symptomatic cyanosis weighing <2.5 kg at initial intervention and between 2005 and 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Primary outcome was mortality and secondary outcomes included component (eg, initial palliation, complete repair, or primary repair) and cumulative (SR: initial palliation followed by later complete repair) hospital and intensive care unit lengths of stay, durations of ventilation, inotrope use, cardiopulmonary bypass time, procedural complications, and reintervention. Outcomes were compared with propensity score adjustments with PR as the reference group. The cohort included 76 SR (initial palliation: 53 surgical and 23 transcatheter) and 44 PR patients. The observed risk of overall mortality was similar between SR and PR groups (15.8% vs 18.2%: P =.735). The adjusted hazard of mortality remained similar between groups overall (hazard ratio, 0.59; 95% confidence interval, 0.26-1.36; P =.214), as well as during short-term (<4 months: hazard ratio, 0.37; 95% confidence interval, 0.13-1.09; P =.071) and midterm (>4 months: hazard ratio, 1.32; 95% confidence interval, 0.30-5.79; P =.717) follow-up. Reintervention in the first 18 months was common in both groups (53.2% vs 48.4%; hazard ratio, 1.69; 95% confidence interval, 0.96-2.28; P =.072). Adjusted procedural complications and neonatal morbidity burden were overall lower in the SR group. Cumulative secondary outcome burdens largely favored the PR group. In this study comparing SR and PR treatment strategies for neonates with tetralogy of Fallot and symptomatic cyanosis and weight <2.5 kg, mortality and reintervention burden was high and independent of treatment strategy. Other potential advantages were observed with each approach. One hundred twenty neonates with symptomatic tetralogy of Fallot weighed <2.5 kg at the time either of primary repair, or initial palliation as part of a staged repair strategy. Forty-four patients underwent primary repair, and 76 patients underwent staged palliation. Overall, approximately 17% of patients died at a median follow-up of more than 5 years. The observed mortality was similar in both groups, at 18% in the primary repair group and 16% in the staged repair group. After complete repair, half of the patients in each group had undergone reintervention. The high mortality in this fragile patient population is sobering. Alternative methods, such as randomized trials and prospective registries are necessary to further compare staged and primary repair strategies for symptomatic neonates with tetralogy of Fallot weighing <2.5 kg. Printed with permission from Texas Children's Hospital. [Display omitted] [ABSTRACT FROM AUTHOR]

Abstract: Deletions of chromosome 1p and 19q arms are frequent genetic abnormalities in primary human gliomas and are especially common in oligodendrogliomas. However, the chromosome 1p and 19q status of many glioma cell lines has not been established. Using homozygosity mapping, fluorescence in situ hybridization (FISH), and comparative genomic hybridization to arrayed BAC (CGHa), we screened 17 glioma cell lines for chromosome 1 and 19 deletions. Sequence tagged site polymorphisms were used to evaluate the cell lines for regions of chromosome 1p and 19q homozygosity. Cell lines A172, U251, TP265, U118, SW1088, U87, SW1783, and D32 contained significant regions of 19q homozygosity. In addition, A172, U87, TP483, D37, U118, MO67, and TP265 contained significant regions of 1p homozygosity. FISH probes localized to 1p36.32 and 19q13.33 as well as CGHa were used to determine which cell lines had deletions of 1p and/or 19q. Cell lines A172, U87, TP483, TP265, H4, U251, and D37 were deleted for portions of 1p. CGHa and homozygosity mapping of these cell lines define a 700-kilobase (Kb) common deletion region that is encompassed by a larger deletion region previously mapped in sporadic gliomas. This common deletion region is localized at 1p36.31 and includes CHD5, a putative tumor suppressor gene. Cell line A172 was observed to have a deletion between 19q13.33 and 19q13.41, while U87 was observed to have a smaller deletion of 19q13.33. Cell lines A172 and U87 contain 1p and 19q deletions similar to those found in sporadic gliomas and will be useful cellular reagents for evaluating the function of putative 1p and 19q glioma tumor suppressor genes. [Copyright &y& Elsevier]

A novel technique of valve-sparing aortic root replacement was applied to 2 children younger than 3 years of age with Marfan syndrome and large aortic root aneurysms. Using elements of both the remodeling and reimplantation techniques, circumferential rings from a 20-mm to 22-mm polyester graft provide stabilization at the subannular and sinotubular levels, and bovine pericardial patches create pseudosinuses. Follow-up at 2 years in 1 patient and 7 months in a second patient revealed satisfactory valve function with stable aortic root size. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve replacement (TPVR) with the self-expanding Harmony valve (Medtronic) is an emerging treatment for patients with native or surgically repaired right ventricular outflow tract (RVOT) pulmonary regurgitation (PR). Limited data are available since U.S. Food and Drug Administration approval in 2021. In this study, the authors sought to evaluate the safety and short-term effectiveness of self-expanding TPVR in a real-world experience. This was a multicenter registry study of consecutive patients with native RVOT PR who underwent TPVR through April 30, 2022, at 11 U.S. centers. The primary outcome was a composite of hemodynamic dysfunction (PR greater than mild and RVOT mean gradient >30 mm Hg) and RVOT reintervention. A total of 243 patients underwent TPVR at a median age of 31 years (Q1-Q3: 19-45 years). Cardiac diagnoses were tetralogy of Fallot (71%), valvular pulmonary stenosis (21%), and other (8%). Acute technical success was achieved in all but 1 case. Procedural serious adverse events occurred in 4% of cases, with no device embolization or death. Hospital length of stay was 1 day in 86% of patients. Ventricular arrhythmia prompting treatment occurred in 19% of cases. At a median follow-up of 13 months (Q1-Q3: 8-19 months), 98% of patients had acceptable hemodynamic function. Estimated freedom from the composite clinical outcome was 99% at 1 year and 96% at 2 years. Freedom from TPVR-related endocarditis was 98% at 1 year. Five patients died from COVID-19 (n = 1), unknown causes (n = 2), and bloodstream infection (n = 2). In this large multicenter real-world experience, short-term clinical and hemodynamic outcomes of self-expanding TPVR therapy were excellent. Ongoing follow-up of this cohort will provide important insights into long-term outcomes. [Display omitted] [ABSTRACT FROM AUTHOR]

Neonates with symptomatic tetralogy of Fallot (sTOF) with hypoplastic pulmonary arteries (hPA) are considered high risk. Data are needed to inform the impact of hPA on outcomes, and the ideal management strategy. The objectives of this study were to quantify the impact of hPA on outcomes in neonates with sTOF and measure the impact of strategy on pulmonary artery (PA) growth in this population. Neonates with sTOF from 2005 to 2017 were reviewed from the Congenital Cardiac Research Collaborative. Criteria for hPA included a unilateral PA z score <−2.0 and contralateral PA z score <0. Primary outcome was mortality. Secondary outcomes included reintervention and PA growth. We included 542 neonates with sTOF, including 188 (35%) with hPA and 354 (65%) with normal PA, with median follow-up of 4.1 years. Median right and left hPA z scores were −2.19 (25th-75th percentile: −2.55 to −1.94) and −2.23 (25th-75th percentile: −2.64 to −1.91), respectively. Staged repair (vs primary TOF repair) was less common in the hPA cohort (36 vs 44%; P = 0.07). Survival was similar between groups (unadjusted P = 0.16; adjusted P = 0.25). Reintervention was more common in the hPA group (HR: 1.28; 95% CI: 1.01-1.63; P = 0.044); there was no difference after definitive repair (HR: 1.21; 95% CI: 0.93-1.58; P = 0.16). PA growth at 1 year was greater in the hPA cohort, particularly for the right PA (P < 0.001). Despite perception, the presence of hPA in neonates with sTOF conferred no increase in overall hazard of mortality or reintervention after definitive repair. PA growth was superior in the hPA cohort. These findings suggest that the presence of hPA does not adversely impact outcomes in sTOF. [Display omitted] [ABSTRACT FROM AUTHOR]

We present a case of a neonate with Shone complex in addition to the underdevelopment of the right ventricle associated with dysplasia and stenosis of the tricuspid valve. Because of lack of effective surgical options, the patient was transplanted at the age of 8 days. A gradient was noted in the postoperative period across the inferior vena cava anastomosis. At reoperation, a prominent myxomatous Eustachian valve was resected. We believe the large Eustachian valve was likely responsible for the right-sided pathology. [ABSTRACT FROM AUTHOR]

Background: Recent data have demonstrated that overall mortality and adverse events are not significantly different for primary repair (PR) and staged repair (SR) approaches to management of neonates with symptomatic tetralogy of Fallot (sTOF). Cost data can be used to compare the relative value (cost for similar outcomes) of these approaches and are a potentially more sensitive measure of morbidity.Objectives: This study sought to compare the economic costs associated with PR and SR in neonates with sTOF.Methods: Data from a multicenter retrospective cohort study of neonates with sTOF were merged with administrative data to compare total costs and cost per day alive over the first 18 months of life in a propensity score-adjusted analysis. A secondary analysis evaluated differences in department-level costs.Results: In total, 324 subjects from 6 centers from January 2011 to November 2017 were studied (40% PR). The 18-month cumulative mortality (P = 0.18), procedural complications (P = 0.10), hospital complications (P = 0.94), and reinterventions (P = 0.22) did not differ between PR and SR. Total 18-month costs for PR (median $179,494 [IQR: $121,760-$310,721]) were less than for SR (median: $222,799 [IQR: $167,581-$327,113]) (P < 0.001). Cost per day alive (P = 0.005) and department-level costs were also all lower for PR. In propensity score-adjusted analyses, PR was associated with lower total cost (cost ratio: 0.73; P < 0.001) and lower department-level costs.Conclusions: In this multicenter study of neonates with sTOF, PR was associated with lower costs. Given similar overall mortality between treatment strategies, this finding suggests that PR provides superior value. [ABSTRACT FROM AUTHOR]

Helper-dependent adenoviral vectors (HDAd) are devoid of all viral coding sequences and are thus an improvement over early generation Ad because they can provide long-term transgene expression in vivo without chronic toxicity. However, high vector doses are required to achieve efficient hepatic transduction by systemic intravenous injection, and this unfortunately results in dose-dependent acute toxicity. To overcome this important obstacle, we have developed a minimally invasive method to preferentially deliver HDAd into the liver of nonhuman primates. Briefly, a balloon occlusion catheter was percutaneously positioned in the inferior vena cava to occlude hepatic venous outflow. HDAd was injected directly into the occluded liver via a percutaneously placed hepatic artery catheter. Compared to systemic vector injection, this approach resulted in substantially higher hepatic transduction efficiency using clinically relevant low vector doses and was accompanied by mild-to-moderate acute but transient toxicities. Transgene expression was sustained for up to 964 days. These results suggest that our minimally invasive method of delivery can significantly improve the vector's therapeutic index and may be a first step toward clinical application of HDAd for liver-directed gene therapy.Molecular Therapy (2009) 17 2, 327–333 doi:10.1038/mt.2008.257 [ABSTRACT FROM AUTHOR]

Molecular beam epitaxial (MBE), unstrained Si1−xGex layers of various Ge concentrations, ranging from 0% to 50%, were grown on top of a 〈1 0 0〉 Si substrate. The wafers were subjected to a 10 keV, 1×1014 cm−2 Si+ non-amorphizing implant. To study the defect morphology, the samples were annealed at 750°C for a total of 180 min. Plan-view transmission electron microscopy (PTEM) was utilized to observe and quantify the formation and dissolution of the defects. The Si1−xGex samples with &les;5% Ge exhibited {3 1 1} defect formation and dissolution; however, samples with Ge fractions &ges;25% showed only dislocation loop formation likely caused by a decrease in bond strength with increasing Ge content. [Copyright &y& Elsevier]

Background: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention.Objectives: This study sought to perform a balanced multicenter comparison of staged repair (SR) (initial palliation [IP] and subsequent complete repair [CR]) versus primary repair (PR) treatment strategies.Methods: Consecutive neonates with sTOF who underwent IP or PR at ≤30 days of age from 2005 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was death. Secondary outcomes included component (IP, CR, PR) and cumulative (SR): hospital and intensive care unit lengths of stay; durations of cardiopulmonary bypass, anesthesia, ventilation, and inotrope use; and complication and reintervention rates. Outcomes were compared using propensity score adjustment.Results: The cohort consisted of 342 patients who underwent SR (IP: surgical, n = 256; transcatheter, n = 86) and 230 patients who underwent PR. Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the SR group (p ≤0.01). The observed risk of death was not different between the groups (10.2% vs 7.4%; p = 0.25) at median 4.3 years. After adjustment, the hazard of death remained similar between groups (hazard ratio: 0.82; 95% confidence interval: 0.49 to 1.38; p = 0.456), but it favored SR during early follow-up (<4 months; p = 0.041). Secondary outcomes favored the SR group in component analysis, whereas they largely favored PR in cumulative analysis. Reintervention risk was higher in the SR group (p = 0.002).Conclusions: In this multicenter comparison of SR or PR for management of neonates with sTOF, adjusted for patient-related factors, early mortality and neonatal morbidity were lower in the SR group, but cumulative morbidity and reinterventions favored the PR group, findings suggesting potential benefits to each strategy. [ABSTRACT FROM AUTHOR]

Transcatheter aortic valve replacement (TAVR) offers well established benefit for adults with severe aortic stenosis, although applications in the pediatric population remain limited. We describe a case of a 15-year-old male with complex congenital heart disease presenting with cardiogenic shock in the setting of mixed severe aortic stenosis (AS) and severe aortic insufficiency (AI). Self-expanding TAVR was performed via suprasternal approach with robust clinical improvement. At one month follow up, he had resolution of clinical heart failure with improvement in ejection fraction and no symptoms of valvulopathy. To our knowledge, there have been no described cases of suprasternal TAVR in a pediatric patient. [ABSTRACT FROM AUTHOR]

TAVR is increasingly becoming a common treatment for severe symptomatic aortic stenosis. Although there has been marked reduction in intra-procedural complications with evolution in the TAVR technology, these complications remain a challenge. We present a unique case of paravalvular leak (PVL) and iatrogenic ventricular septal defect as a result of closing attempts of PVL. We aim to highlight our procedural approach to percutaneously repair the aforementioned complications using our heart team approach for decision making. [ABSTRACT FROM AUTHOR]

Significant research effort has been placed into the use of III–V compound semiconductors, including InGaAs as channel materials in CMOS logic devices due to their superior electron mobilities compared to Si and other more conventional semiconductor materials. One of the major factors preventing industrial adoption of InGaAs as a channel material involves the minimization of source and drain contact resistances. To understand challenges to minimization of contact resistance, this work will outline the effectiveness of several doping approaches that have been attempted for n-type InGaAs including the use of silicon as a dopant and the effectiveness of each approach in achieving the highest level of activation possible. Previous and recently reported dopant diffusion behaviors are also included and discussed. [ABSTRACT FROM AUTHOR]

The myelodysplastic syndromes (MDS) are a clinically heterogeneous group of hematologic disorders. Cytogenetic analysis is crucial as it can provide both diagnostic and prognostic information. Herein, 32 cytogenetically normal patients with primary MDS were analyzed both by multiple FISH probes on interphase nuclei (FISH panel testing) and by M-FISH (metaphase nuclei). One patient had a chromosome 13q-arm deletion, while the remaining 31 patients had normal results. These findings confirm standard cytogenetics as an excellent technique in identifying the common chromosomal abnormalities associated with MDS and suggest limited utility for either a FISH panel test or M-FISH in primary MDS. [Copyright &y& Elsevier]

Distinguishing mantle cell lymphoma (MCL), from low-grade B-cell lymphoma is important because MCL is clinically more aggressive and is treated differently. Though most MCL overexpress cyclinD1 (CCND1) and have a t(11;14)(q13;q32), MCL that are negative for CCND1 exist. Some have translocations involving cyclinD2 (CCND2) and either the immunoglobulin heavy chain or kappa light chain locus. We present a CD5-positive, CCND1-negative B-cell lymphoma with a novel translocation involving CCND2 and the immunoglobulin lambda (IGL) gene. A 64-year-old male underwent resection of a polypoid mass of the ileum. Histology showed atypical, medium-sized lymphoid cells positive for CD20, CD5, CD43, and CCND2 by immunohistochemistry, and negative for CCND1, CCND3, and p27. Fluorescence in situ hybridization was negative for CCND1 abnormalities, but demonstrated a CCND2/IGL fusion. Clinical workup revealed stage IV disease. Current diagnostic criteria are insufficient for subclassifying this case, highlighting the need for additional studies on CCND2-translocated B-cell lymphomas to guide therapy appropriately. [ABSTRACT FROM AUTHOR]