Reprint of: How He Did It - The James S. Tweddell Approach to Single Ventricle Palliation in Patients With Hypoplastic Left Heart Syndrome.

This article will detail Dr. James Tweddell's operative technique and considerations for 26 all three stages of SVP in patients with HLHS. This will include the Norwood Procedure with 27 Right-Ventricle-to-Pulmonary-Artery Conduit (Sano Modification), Bidirectional Glenn 28 (Superior Cavopulmonary Connection), and Extracardiac, Fenestrated Fontan Completion (Total 29 Cavopulmonary Connection). These techniques are the culmination of over 20 years of experience 30 and represent the final techniques he employed at both Children's Hospital of Wisconsin and 31 Cincinnati Children's Hospital Medical Center.

Hand-Constructed CorMatrix Tubular Valve Used for Tricuspid Valve Replacement in Neonate With Congenital Tricuspid Dysplasia: Five-Year Follow-Up.

Tricuspid valve (TV) dysplasia is a rare congenital defect that, in severe cases, can cause heart failure necessitating surgical correction. TV replacement options are limited in neonates since few commercial valves are available and the rates of failure and postoperative mortality are high. The authors report a neonate with TV dysplasia who underwent replacement with a hand-constructed tube valve using small intestinal submucosal extracellular matrix, which functioned well for >5 years.

Improvement in Multidisciplinary Provider Rounding (Surgical Rounds) in the Pediatric Cardiac ICU: An Application of Lean Methodology.

OBJECTIVES: Provider-only, combined surgical, and medical multidisciplinary rounds ("surgical rounds") are essential to achieve optimal outcomes in large pediatric cardiac ICUs. Lean methodology was applied with the aims of identifying areas of waste and nonvalue-added work within the surgical rounds process. Thereby, the goals were to improve rounding efficiency and reduce rounding duration while not sacrificing critical patient care discussion nor delaying bedside rounds or surgical start times. DESIGN: Single-center improvement science study with observational and interventional phases from February 2, 2021, to July 31, 2021. SETTING: Tertiary pediatric cardiac ICU. PARTICIPANTS: Cardiothoracic surgery and cardiac intensive care team members participating in daily "surgical" rounds. INTERVENTIONS: Implementation of technology automation, creation of work instructions, standardization of patient presentation content and order, provider training, and novel role assignment. MEASUREMENTS AND MAIN RESULTS: Sixty-one multidisciplinary rounds were observed (30 pre, 31 postintervention). During the preintervention period, identified inefficiencies included prolonged preparation time, redundant work, presentation variability and extraneous information, and frequent provider transitions. Application of targeted interventions resulted in a 26% decrease in indexed rounds duration (2.42 vs 1.8 min; p = 0.0003), 50% decrease in indexed rounds preparation time (0.53 vs 0.27 min; p < 0.0001), and 66% decrease in transition time between patients (0.09 vs 0.03 min; p < 0.0001). The number of presenting provider changes also decreased (9 vs 4; p < 0.0001). Indexed discussion duration did not change (1 vs 0.98 min; p = 0.08) nor did balancing measures (bedside rounds and surgical start times) change (8.5 vs 9 min; p = 0.89 and 38 vs 22 min; p = 0.09). CONCLUSIONS: Lean methodology can be effectively applied to multidisciplinary rounds in a joint cardiothoracic surgery/cardiac intensive care setting to decrease waste and inefficiency. Interventions resulted in decreased preparation time, transition time, presenting provider changes, total rounds duration indexed to patient census, and anecdotal improvements in provider satisfaction.

Is the practice of using oversized organs for recipients with elevated pulmonary vascular resistance justified?

OBJECTIVE: The purpose of this study was to assess post-transplantation outcomes in recipients with increased pulmonary vascular resistance (PVR) in relation to donor size. METHODS: The United Network for Organ Sharing database was used to identify patients ages 0 to 18 years at time of listing who underwent transplantation from 2010 to 2019 and for whom cardiac catheterization and donor-recipient weight ratio data were available. Patients were divided according to listing PVR into <3, 3 to 6, and >6 Wood units. Donor-recipient weight ratio was categorized as undersized (≤0.80), midsize (0.81-1.2), and oversized (>1.2). Subgroup analysis was done with an additional supersized group (>2.0). RESULTS: Fourteen hundred ninety-one patients met study criteria. Median age was 10 (interquartile range, 3-15) years and 45% were female. Four percent of heart transplantation cases used undersized, 45% used midsize, and 51% used oversized organs. More patients with PVR >6 were received an oversized organ transplant compared with patients with PVR <3; 59% (148/252) versus 48% (430/894); P = .003. There was no difference in survival among organ size groups regardless of PVR; this includes patients with PVR >6 at listing who received an oversized organ transplant versus an undersized (P = .359) or midsized (P = .956) organ. In subgroup analysis, even in patients who received a supersized organ transplant, there was no survival difference noted regardless of PVR. CONCLUSIONS: Despite a persistent practice pattern to transplant oversized organs in high-PVR patients, there remains no difference in post-transplantation survival among these patients and those who received smaller organ transplants. Therefore, transplants in patients with high PVR should not be delayed by waiting for larger donors.

Norwood With Obstructed Total Anomalous Pulmonary Venous Connection and Tracheoesophageal Fistula Repair: Operating Room Delivery.

A baby boy with prenatally diagnosed hypoplastic left heart syndrome variant with obstructed veins was born in the operating room (OR) and underwent emergent Norwood operation and repair of obstructed infra-diaphragmatic total anomalous pulmonary venous connection. Post-operatively, esophageal atresia with tracheoesophageal fistula was identified and repaired on day of life 11. The patient is thriving at 22 months of age. (Level of Difficulty: Advanced.).

Disruption of anterior insula modulation of large-scale brain networks in schizophrenia.

BACKGROUND: Systems level modeling of functional magnetic resonance imaging data has demonstrated dysfunction of several large-scale brain networks in schizophrenia. Anomalies across multiple functional networks associated with schizophrenia could be due to diffuse pathology across multiple networks or, alternatively, dysfunction at converging control(s) common to these networks. The right anterior insula has been shown to modulate activity in the central executive and default mode networks in healthy individuals. We tested the hypothesis that right anterior insula modulation of central executive and default mode networks is disrupted in schizophrenia and associated with cognitive deficits. METHODS: In 44 patients with schizophrenia and 44 healthy control subjects, we used seed-based resting state functional connectivity functional magnetic resonance imaging analysis to examine connectivity between right insular subregions and central executive/default mode network regions. We also performed two directed connectivity analyses of resting state data: Granger analysis and confirmatory structural equation modeling. Between-group differences in path coefficients were used to evaluate anterior insula modulation of central executive and default mode networks. Cognitive performance was assessed with the rapid visual information processing task, a test of sustained attention. RESULTS: With multiple connectivity techniques, we found compelling, corroborative evidence of disruption of right anterior insula modulation of central executive and default mode networks in patients with schizophrenia. The strength of right anterior insula modulation of these networks predicted cognitive performance. CONCLUSIONS: Individuals with schizophrenia have impaired right anterior insula modulation of large-scale brain networks. The right anterior insula might be an emergent pathophysiological gateway in schizophrenia.