Background Intensive monitoring has been associated with a lower death rate between the Norwood operation and superior cavopulmonary connection, possibly due to early identification and effective ...treatment of residual anatomic lesions like recoarctation before lasting harm occurs. Methods and Results Neonates undergoing a Norwood operation and receiving interstage care at a single center between January 1, 2005, and September 18, 2020, were studied. In those with recoarctation, we evaluated association of era (1 preinterstage monitoring, 2 a transitional phase, 3 current era) and likelihood of hemodynamic compromise (progression to moderate or greater ventricular dysfunction/atrioventricular valve regurgitation, initiation/escalation of vasoactive/respiratory support, cardiac arrest preceding catheterization, or interstage death with recoarctation on autopsy). We also analyzed whether era was associated with technical success of transcatheter recoarctation interventions, major adverse events, and transplant-free survival. A total of 483 subjects were studied, with 22% (n=106) treated for recoarctation during the interstage period. Number of catheterizations per Norwood increased (
=0.005) over the interstage eras, with no significant change in the proportion of subjects with recoarctation (
=0.36). In parallel, there was a lower likelihood of hemodynamic compromise in subjects with recoarctation that was not statistically significant (
=0.06), with a significant difference in the proportion with ventricular dysfunction at intervention (
=0.002). Rates of technical success, procedural major adverse events, and transplant-free survival did not differ (
>0.05). Conclusions Periods with interstage monitoring were associated with increased referral for catheterization but also reduced likelihood of ventricular dysfunction (and a suggestion of lower likelihood of hemodynamic compromise) in subjects with recoarctation. Further study is needed to guide optimal interstage care of this vulnerable population.
The modified Blalock-Taussig-Thomas shunt is the gold standard palliation for securing pulmonary blood flow in infants with ductal-dependent pulmonary blood flow. Recently, the ductus arteriosus ...stent (DAS) has become a viable alternative.
This was a retrospective multicenter study of neonates ≤30 days undergoing DAS or Blalock-Taussig-Thomas shunt placement between January 1, 2017 and December 31, 2020 at hospitals reporting to the Pediatric Health Information Systems database. We performed generalized linear mixed-effects modeling to evaluate trends in intervention and intercenter variation, propensity score adjustment and inverse probability weighting with linear mixed-effects modeling to analyze length of stay and cost of hospitalization, and generalized linear mixed modeling to analyze differences in 30-day outcomes. There were 1874 subjects (58% male, 61% White) from 45 centers (29% DAS). Odds of DAS increased with time (odds ratio OR 1.23, annually,
<0.01 95% CI, 1.10-1.38) with significant intercenter variation (median OR, 3.81 95% CI, 2.74-5.91). DAS was associated with shorter hospital length of stay (ratio of geometric means, 0.76 95% CI, 0.63-0.91), shorter intensive care unit length of stay (ratio of geometric means, 0.77 95% CI, 0.61-0.97), and less expensive hospitalization (ratio of geometric means, 0.70 95% CI, 0.56-0.87). Intervention was not significantly associated with odds of 30-day transplant-free survival (OR,1.18 95% CI, 0.70-1.99) or freedom from catheter reintervention (OR, 1.02 95% CI, 0.65-1.58), but DAS was associated with 30-day freedom from composite adverse outcome (OR, 1.51 95% CI, 1.11-2.05).
Use of DAS is increasing, but there is variability across centers. Though odds of transplant-free survival and reintervention were not significantly different after DAS, and DAS was associated with shorter length of stay and lower in-hospital costs.
Mucosal-associated invariant T cells (MAIT cells) recognize bacterial metabolites as antigen and are found in blood and tissues, where they are poised to contribute to barrier immunity. Recent data ...demonstrate that MAIT cells located in mucosal barrier tissues are functionally distinct from their blood counterparts, but the relationship and circulation of MAIT cells between blood and different tissue compartments remains poorly understood. Previous studies raised the possibility that MAIT cells do not leave tissue and may either be retained or undergo apoptosis. To directly address if human MAIT cells exit tissues, we collected human donor-matched thoracic duct lymph and blood and analyzed MAIT cell phenotype, transcriptome, and T cell receptor (TCR) diversity by flow cytometry and RNA sequencing. We found that MAIT cells were present in the lymph, despite being largely CCR7- in the blood, thus indicating that MAIT cells in the lymph migrated from tissues and were capable of exiting tissues to recirculate. Importantly, MAIT cells in the lymph and blood had highly overlapping clonotype usage but distinct transcriptome signatures, indicative of differential activation states.
Background Procedural volume has been shown to be associated with outcome in cardiac catheterization and intervention in adults. The impact of center-level factors (such as volume) and their ...interaction with subject- and procedure-level factors on outcome after cardiac catheterization in children is not well described. We hypothesized that higher center catheterization volume would be associated with lower risk of catastrophic adverse events. Methods We studied children and young adults 0 to 21 years of age undergoing one or more cardiac catheterizations at centers participating in the Pediatric Health Information Systems database between 2007 and 2012. Using mixed-effects multivariable regression, we assessed the association between center catheterization volumes and the risk of a composite outcome of death and/or initiation of mechanical circulatory support within 1 day of cardiac catheterization adjusting for patient- and procedure-level factors. Results A total of 63,994 procedures performed on 40,612 individuals from 38 of 43 centers contributing data to the Pediatric Health Information Systems database were included. The adjusted risk of the composite outcome was 0.1%. Increasing annual catheterization laboratory volume was independently associated with reduced risk of the composite outcome (odds ratio per a 100-procedure/y increment 0.78 95% CI 0.65-0.93, P < .006). Younger age at catheterization, previous cardiac operation in the same admission as the catheterization, preprocedural vasoactive medications, and hemodialysis were also independently associated with an increased risk of adverse outcomes. Conclusions Higher cardiac catheterization laboratory volume was associated with reduced risk of catastrophic adverse outcome in the immediate postcatheterization period in children. The observed benefit of catheterization at a larger volume center may be attributable to transmissible best practices or inextricable benefits of larger systems.
The thoracic duct is responsible for the circulatory return of most lymphatic fluid. The return is a well‐timed synergy between the pressure in the thoracic duct, venous pressure at the thoracic duct ...outlet, and intrathoracic pressures during respiration. However, little is known about the forces determining thoracic duct pressure and how these respond to mechanical ventilation. We aimed to assess human thoracic duct pressure and identify elements affecting it during positive pressure ventilation and a brief ventilatory pause. The study examined pressures of 35 patients with severe congenital heart defects undergoing lymphatic interventions. Thoracic duct pressure and central venous pressure were measured in 25 patients during mechanical ventilation and in ten patients during both ventilation and a short pause in ventilation. TD contractions, mechanical ventilation, and arterial pulsations influenced the thoracic duct pressure. The mean pressure of the thoracic duct was 16 ± 5 mmHg. The frequency of the contractions was 5 ± 1 min−1 resulting in an average increase in pressure of 4 ± 4 mmHg. During mechanical ventilation, the thoracic duct pressure correlated closely to the central venous pressure. TD contractions were able to increase thoracic duct pressure by 25%. With thoracic duct pressure correlating closely to the central venous pressure, this intrinsic force may be an important factor in securing a successful return of lymphatic fluid. Future studies are needed to examine the return of lymphatic fluid and the function of the thoracic duct in the absence of both lymphatic complications and mechanical ventilation.
In individuals with congenital heart defects undergoing lymphatic interventions, thoracic duct pressure correlates closely to central venous pressure. The thoracic duct spontaneously contracts with a frequency of 5 min−1 increasing thoracic duct pressure by 25%. Spontaneous contractions of the thoracic duct may be an important factor in securing the successful return of lymphatic fluid when challenged by positive pressure ventilation.
Background Clinical outcomes for transcatheter and operative closures of atrial septal defects (ASDs) are similar. Economic cost for each method has not been well described. Methods A single-center ...retrospective cohort study of children and adults<30 years of age undergoing closure for single secundum ASD from January 1, 2007, to April 1, 2012, was performed to measure differences in inflation-adjusted cost of operative and transcatheter closures of ASD. A propensity score weight-adjusted multivariate regression model was used in an intention-to-treat analysis. Costs for reintervention and crossover admissions were included in primary analysis. Results A total of 244 subjects were included in the study (64% transcatheter and 36% operative), of which 2% (n = 5) were ≥18 years. Crossover rate from transcatheter to operative group was 3%. Risk of reintervention ( P = .66) and 30-day mortality ( P = .37) were not significantly different. In a multivariate model, adjusted cost of operative closure was 2012 US $60,992 versus 2012 US $55,841 for transcatheter closure ( P < .001). Components of total cost favoring transcatheter closure were length of stay, medications, and follow-up radiologic and laboratory testing, overcoming higher costs of procedure and echocardiography. Professional costs did not differ. The rate of 30-day readmission was greater in the operative cohort, further increasing the cost advantage of transcatheter closure. Sensitivity analyses demonstrated that costs of follow-up visits influenced relative cost but that device closure remained favorable over a broad range of crossover and reintervention rates. Conclusion For single secundum ASD, cost comparison analysis favors transcatheter closure over the short term. The cost of follow-up regimens influences the cost advantage of transcatheter closure.
With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl ...with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, post-double switch procedure Rastelli and Glenn) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0.9 g/dL) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.
Background Cardiac catheterization is an important but costly component of health care for young patients with cardiac disease. Measurement of variation in their cost between hospitals and ...identification of the reasons for this variation may help reduce cost without compromising quality. Methods and Results Using data from Pediatric Health Information Systems Database from January 2007 to December 2015, the costs of 9 procedures were measured. Mixed-effects multivariable models were used to generate case-mix-adjusted estimates of each hospital's cost for each procedure and measure interhospital variation. Procedures (n=35 637) from 43 hospitals were studied. Median costs varied from $8249 (diagnostic catheterization after orthotopic heart transplantation) to $38 909 (transcatheter pulmonary valve replacement). There was marked variation in the cost of procedures between hospitals with 3.5- to 8.9-fold differences in the case-mix-adjusted cost between the most and least expensive hospitals. No significant correlation was found between hospitals' procedure-specific mortality rates and costs. Higher procedure volume was not associated with lower cost except for diagnostic procedures in heart transplant patients and pulmonary artery angioplasty. At the hospital level, the proportion of cases that were outliers (>95th percentile) was significantly associated with rank in terms of cost (Spearman's ρ ranging from 0.37 to 0.89, P<0.01). Conclusions Large-magnitude hospital variation in cost was not explained by case-mix or volume. Further research is necessary to determine the degree to which variation in cost is the result of differences in the efficiency of the delivery of healthcare services and the rate of catastrophic adverse outcomes and resultant protracted and expensive hospitalizations.
Background Risk-adjusted adverse event (AE) rates have been used to measure the quality of pediatric and congenital cardiac catheterization laboratories. In other settings, failure to rescue (FTR) ...has demonstrated utility as a quality metric. Methods and Results A multicenter retrospective cohort study was performed using data from the IMPACT (Improving Adult and Congenital Treatment) Registry between January 2010 and December 2016. A modified FTR metric was developed for pediatric and congenital cardiac catheterization laboratories and then compared with pooled AEs. The associations between patient- and hospital-level factors and outcomes were evaluated using hierarchical logistic regression models. Hospital risk standardized ratios were then calculated. Rankings of risk standardized ratios for each outcome were compared to determine whether AEs and FTR identified the same high- and low-performing centers. During the study period, 77 580 catheterizations were performed at 91 hospitals. Higher annual hospital catheterization volume was associated with lower odds of FTR (odds ratio: 0.68 per 300 cases;
=0.0003). No association was seen between catheterization volume and odds of AEs. Odds of AEs were instead associated with patient- and procedure-level factors. There was no correlation between risk standardized ratio ranks for FTR and pooled AEs (
=0.46). Hospital ranks by catheterization volume and FTR were associated (
=-0.28,
=0.01) with the largest volume hospitals having the lowest risk of FTR. Conclusions In contrast to AEs, FTR was not strongly associated with patient- and procedure-level factors and was significantly associated with pediatric and congenital cardiac catheterization laboratory volume. Hospital rankings based on FTR and AEs were not significantly correlated. We conclude that FTR is a complementary measure of catheterization laboratory quality and should be included in future research and quality-improvement projects.
Chylopericardium is a rare complication after cardiac transplantation. We report a case of a 69-year-old woman with persistent chylopericardium after a heart transplantation due to Chagas disease. ...Failure of conservative treatment led to dynamic contrast-enhanced magnetic resonance lymphangiography and percutaneous radiologic intervention of the lymphatic leakage and symptoms resolution.
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