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Background: The Physician Payments Sunshine Act was enacted to understand financial relationships with industry that might influence provider decisions. We investigated how industry payments within the congenital heart community relate to experience and reputation., Methods: Congenital cardiothoracic surgeons and pediatric cardiologists were identified from the Open Payments Database. All payments from 2013 through 2017 were matched to affiliated hospitals' U.S. News & World Report (USNWR) rankings, The Society of Thoracic Surgeons-Congenital Heart Surgery Public Reporting Star Ratings, and Optum Center of Excellence (COE) designation. Surgeon payments were linked to years since terminal training. Univariable analyses were conducted., Results: The median payment amount per surgeon ($71; interquartile range [IQR], $41-$99) was nearly double the median payment amount per cardiologist ($41; IQR, $18-$84; P < .05). For surgeons, median individual payment was 56% higher to payees at USNWR top 10 children's hospitals ($100; IQR, $28-$203) vs all others ($64; IQR, $23-$140; P < .001). For cardiologists, median individual payment was 26% higher to payees at USNWR top 10 children's hospitals ($73; IQR, $28-$197) vs all others ($58; IQR, $19-$140; P < .001). Findings were similar across The Society of Thoracic Surgeons-Congenital Heart Surgery star rankings and Optum Center of Excellence groups. By surgeon experience, surgeons 0 to 6 years posttraining (first quartile) received the highest number of median payments per surgeon (17 payments; IQR, 6.5-28 payments; P < .001). Surgeons 21 to 44 years posttraining (fourth quartile) received the lowest median individual payment ($51; IQR, $20-132; P < .001)., Conclusions: Industry payments vary by hospital reputation and provider experience. Such biases must be understood for self-governance and the delineation of conflict of interest policies that balance industry relationships with clinical innovation., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Mortality in cohorts with a single ventricle remains high with multiple associated factors. The effect of heart block during stage I palliation remains unclear., Objective: The purpose of this study was to study patient and surgical risks of heart block and its effect on 12-month transplant-free survival in patients with a single ventricle., Methods: Patient, surgical, outcome data and heart block status (transient and permanent) were obtained from the National Pediatric Cardiology Quality Improvement Collaborative single ventricle database. Bivariate analysis was performed comparing patients with and without heart block, and multivariate modeling was used to identify variables associated with block. One-year outcomes were analyzed to identify variables associated with lower 12-month transplant-free survival., Results: In total, 1423 patients were identified, of whom 28 (2%) developed heart block (second degree or complete) during their surgical admission. Associated risk factors for block included heterotaxy syndrome (odds ratio [OR] 6.4) and atrial flutter/fibrillation (OR 3.8). Patients with heart block had lower 12-month survival, though only in patients with complete heart block as opposed to second degree block. At 12 months of age, 43% (12/28) of patients with heart block died and were more likely to experience mortality at 12 months than patients without block (OR 4.9; 95% confidence interval 1.4-17.5; P = .01)., Conclusion: Although rare, complete heart block after stage I palliation represents an additional risk of poor outcomes in this high-risk patient population. Heterotaxy syndrome was the most significant risk factor for the development of heart block after stage I palliation. The role of transient block in outcomes and potential rescue with long-term pacing remains unknown and requires additional study., (Copyright © 2021 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Objectives: The aim of this study was to evaluate practice patterns and outcomes of a contemporary group of patients undergoing balloon aortic valvuloplasty (BAV) for congenital aortic stenosis (AS)., Background: BAV is the most common treatment for isolated congenital AS., Methods: Within the IMPACT (Improving Pediatric and Adult Congenital Treatments) Registry, all BAV procedures performed between January 2011 and March 2015 were identified. Procedures were separated into those performed for critical versus noncritical AS. Outcomes were stratified into optimal, adequate, and inadequate, with optimal and adequate outcomes defining "successful" procedures. Multivariate logistic regression was used to identify patient and procedural characteristics associated with unsuccessful BAV. Mortality and adverse events rates were compared across patient cohorts., Results: Of the 1,026 isolated BAV procedures captured in IMPACT, 718 (70%) were "successful." Success rates were 70.9% for noncritical AS (n = 916) and 62.7% for critical AS (n = 110). Multivariate analysis revealed that prior cardiac catheterization, mixed valve disease, baseline aortic valve gradient >60 mm Hg, baseline aortic insufficiency greater than mild, presence of a trainee, and multiple balloon inflations were associated with unsuccessful BAV in the noncritical AS cohort. There were no factors associated with unsuccessful procedures in the critical AS group. No procedural deaths occurred, but 2.4% of patients did not survive to hospital discharge. Adverse events occurred in 15.8% of all cases and were more frequent in procedures performed for critical AS (30.0% vs. 14.1%; p < 0.001)., Conclusions: BAV is an effective treatment for congenital AS with low rates of mortality and adverse events. Patients with critical AS have a higher risk for procedure-related adverse events., (Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

COMPARE computer program suggested that benzamide riboside, BR, 3-(1-deoxy-beta-D-ribofuranosyl)benzamide, should have a similar mechanism of action as that of tiazofurin, an inhibitor of IMP dehydrogenase (IMPDH). This hypothesis was tested in K562 cells in culture. BR was cytotoxic to K562 cells with an IC50 of 2 microM. Incubation of K562 cells with BR resulted in a significant decrease in GMP and GTP levels with a concurrent increase in IMP pools, and with a significant inhibition of IMPDH activity. However, 290-fold higher BR concentration was needed to demonstrate in vitro inhibition of IMPDH activity, suggesting that the agent may require metabolism to exert its action. These results provide evidence that BR is a new inhibitor of IMPDH. This investigation should be helpful to design new analogues having activity against IMPDH.

A 3-month-old infant with acute airway obstruction due to the combination of left aortic arch and right descending aorta with persistence of a right ligamentum arteriosum is presented. This is a rare form of a vascular ring and carries special diagnostic and therapeutic implications. Guidelines for diagnosis and surgical treatment are outlined.