Hepatic dysfunction is a recognized complication after Fontan palliation of congenital heart disease. We sought to quantitatively measure hepatic stiffness and vascular Doppler indices using ...ultrasound (US) and shear wave elastography (SWE) in a Fontan cohort. Subjects were prospectively recruited for echocardiography and real‐time hepatic duplex US with SWE for hepatic stiffness (kPa). Doppler peak velocities, velocity time integral, resistive, pulsatility, acceleration indices (RI, PI, AI), and flow volume were measured in celiac artery, superior mesenteric artery, and main portal vein (MPV). A subset underwent cardiac catheterizations with liver biopsy. Correlations were explored between SWE, duplex, hemodynamic, and histopathologic data. In all, 106 subjects were studied including 41 patients with Fontan physiology (age 13.8 ± 6 years, weight 45.4 ± 23 kg) and 65 controls (age 15.0 ± 8.4 years, weight 47.9 ± 22 kg). Patients with Fontan physiology had significantly higher hepatic stiffness (15.6 versus 5.5 kPa, P < 0.0001), higher celiac RI (0.78 versus 0.73, P = 0.04) superior mesenteric artery RI (0.89 versus 0.84, P = 0.005), and celiac PI (1.87 versus 1.6, P = 0.034); while MPV flow volume (287 versus 420 mL/min in controls, P = 0.007) and SMA AI (829 versus 1100, P = 0.002) were lower. Significant correlation was seen for stiffness with ventricular end‐diastolic pressure (P = 0.001) and pulmonary artery wedge pressure (P = 0.009). Greater stiffness correlated with greater degrees of histopathologic fibrosis. No significant change was seen in stiffness or other duplex indices with age, gender, time since Fontan, or ventricular morphology. Conclusion: Elevated hepatic afterload in Fontan, manifested by high ventricular end‐diastolic pressures and pulmonary arterial wedge pressures, is associated with remarkably increased hepatic stiffness, abnormal vascular flow patterns, and fibrotic histologic changes. The MPV is dilated and carries decreased flow volume, while the celiac and superior mesenteric arterial RI is increased. SWE is feasible in this population and shows promise as a means for predicting disease severity on liver biopsy. (Hepatology 2014;58:251–260)
Donation after circulatory death is emerging as an alternative pathway to donation after brain death to expand the cardiac organ donor pool. We describe the surgical technique and circuit ...configuration for in-situ organ reperfusion with thoracoabdominal normothermic regional perfusion using portable venoarterial extracorporeal membrane oxygenation.
Background
The demonstration of competency in endoscopy is required prior to obtaining American Board of Surgery Certification. To demonstrate competency, the resident must pass a national ...high-stakes cognitive test and a technical skills exam on a virtual reality simulator. The purpose of this preliminary study was to design a proficiency-based endoscopy simulation curriculum to meet this competency requirement.
Methods
This is a mixed methods prospective cohort study at a single academic medical institution. Prior to taking the national exam, surgery residents were required to participate in a skills lab and demonstrate proficiency on 10 simulation tasks. Proficiency was based on time and percent of objects targeted/mucosa seen. Simulation practice time, number of task repetitions to proficiency, and prior endoscopic experience were recorded. Resident’s self-reported confidence scores in endoscopic skills prior to and following simulation lab training were obtained.
Results
From January 1, 2016 through August 1, 2017, 20 surgical residents (8 PGY2, 8 PGY3, 4 PGY4) completed both a faculty-supervised endoscopy skills lab and independent learning with train-to-proficiency simulation tasks. Median overall simulator time per resident was 306 min (IQR: 247–405 min). Median overall time to proficiency in all tasks was 235 min (IQR: 208–283 min). The median time to proficiency decreased with increasing PGY status (
r
= 0.4,
P
= 0.05). There was no correlation between prior real-time endoscopic experience and time to proficiency. Reported confidence in endoscopic skills increased significantly from mean of 5.75 prior to 7.30 following the faculty-supervised endoscopy skills lab (
P
= 0.0002). All 20 residents passed the national exam.
Conclusions
In this preliminary study, a train-to-proficiency curriculum in endoscopy improved surgical resident’s confidence in their endoscopic skills and 100% of residents passed the FES technical skills test on their first attempt. Our findings also indicate that uniform proficiency was not achieved by real-time experience alone.
Background Neurodevelopmental disability is common after operations for congenital heart defects. We previously showed that patient and preoperative factors, center, and calendar year of birth ...explained less than 30% of the variance for the Psychomotor Development Index (PDI) and the Mental Development Index (MDI) of the Bayley Scales of Infant Development-Second Edition. Here we investigate how much additional variance in PDI and MDI is contributed by operative variables and postoperative events. Methods We analyzed neurodevelopmental outcomes after operations with cardiopulmonary bypass at age 9 months or younger between 1996 and 2009. We used linear regression to investigate the effect of operative factors (age, weight, and cardiopulmonary bypass variables) and postoperative events on neurodevelopmental outcomes, adjusting for center, type of congenital heart defect, year of birth, and preoperative factors. Results We analyzed 1,770 children from 22 institutions with neurodevelopmental testing at age 13.3 months (range, 6 to 30 months). Among operative factors, longer total support time was associated with lower PDI and MDI ( p < 0.05). When postoperative events were added, use of either extracorporeal membrane oxygenation or ventricular assist device support, and longer postoperative length of stay were associated with lower PDI and MDI ( p < 0.05). Longer total support time was not a significant predictor in these models. After adjusting for patient, preoperative, intraoperative, and postoperative factors, measured intraoperative and postoperative factors accounted for 5% of the variances in PDI and MDI. Conclusions Operative factors may be less important than innate patient and preoperative factors and postoperative events in predicting early neurodevelopmental outcomes after cardiac operations in infants. Neurodevelopmental outcomes improved over calendar time when adjusted for patient and medical variables.
Background A clinically driven transition in perfusion technique occurred at Children's Hospital and Medical Center, Omaha, Nebraska, from primarily selective cerebral perfusion bracketed by brief ...periods of deep hypothermic circulatory arrest to a technique of dual arterial perfusion including innominate artery and descending aortic cannulation (DAC), with continuous mildly hypothermic (>30°C) full-flow cardiopulmonary bypass to the entire body. This study retrospectively compared outcomes in a recent cohort of neonates undergoing aortic arch reconstruction with the two techniques. Methods The clinical records of 142 consecutive neonates undergoing operations involving aortic arch reconstruction at a single institution between April 2004 and September 2012 were reviewed. Renal function changes were graded according to the pediatric RIFLE score (based on risk, injury, failure, loss, and end-stage kidney disease). Sixteen patients, 8 supported with selective cerebral perfusion bracketed by brief periods of deep hypothermic circulatory arrest and 8 with DAC, required immediate postoperative extracorporeal membrane oxygenation and were excluded from renal function analysis. Multivariable regression models evaluated predictors of pediatric RIFLE score. Results Patients with DAC had shorter median bypass support (113 versus 172 minutes; p < 0.001) and myocardial ischemic time (43 versus 81 minutes; p < 0.001). Patients with DAC had less median fluid gain at 24 hours (37 versus 69 mL/kg; p < 0.001), and lower incidence of acute kidney injury (5% versus 31%; p < 0.001). Fewer patients with DAC (31% versus 58%; p = 0.001) required open chest. Use of selective cerebral perfusion bracketed by brief periods of deep hypothermic circulatory arrest, single-ventricular physiology, and aortic cross-clamp time were found to be multivariable predictors of serious kidney dysfunction. Conclusions Multisite arterial perfusion, including DAC, and maintenance of continuous mildly hypothermic full-flow cardiopulmonary bypass may offer advantages as a perfusion strategy for neonatal arch reconstruction. Prospective investigation of this technique is warranted.
Abstract Objectives We hypothesized that hepatic injury in single-ventricle CHD has origins that predate the Fontan operation. We aimed to measure hepatic stiffness using ultrasound and shear wave ...elastography (SWE) in a bidirectional cavopulmonary connection (BCPC) cohort. Methods Subjects were prospectively recruited for real-time, hepatic, ultrasound-SWE for hepatic stiffness (kPa) and echocardiography. Doppler velocities, a velocity-time integral, flow volume, and resistive index, pulsatility index, and acceleration index were measured in celiac and superior mesenteric arteries, and in the main portal vein (MPV). Comparisons were made among subjects who had BCPC, subjects who were healthy, and a cohort of patients who had undergone the Fontan procedure. Results Forty subjects (20 patients who had BCPC; 20 age- and gender-matched control subjects) were studied. The hepatic stiffness in BCPC was elevated, compared with that in control subjects (7.2 vs 5.7 kPa; P = .039). Patients who had BCPC had significantly higher celiac artery resistive index (0.9 vs 0.8; P = .002); pulsatility index (2.2 vs 1.7; P = .002); and systolic-diastolic flow ratio (10.1 vs 5.9; P = .002), whereas the superior mesenteric artery acceleration index (796 vs 1419 mL/min in control subjects; P = .04) was lower. An elevated resistive index (0.42 vs 0.29; P = .002) and pulsatility index (0.55 vs 0.35; P = .001) were seen in MPV, whereas MPV flow was reduced (137.3 vs 215.7 mL/min in control subjects; P = .036). A significant correlation was found for hepatic stiffness with right atrial pressure obtained at catheterization ( P = .002). Comparison with patients who underwent the Fontan procedure showed patients who had BCPC had lower hepatic stiffness (7.2 vs 15.6 kPa; P < .001). Conclusions Hepatic stiffness is increased with BCPC physiology, and this finding raises concerns that hepatopathology in palliated, single-ventricle CHD is not exclusively attributable to Fontan physiology. Hepatic stiffness measurements using SWE are feasible in this young population, and the technique shows promise as a means for monitoring disease progression.
Improved survival after congenital heart surgery has led to interest in functional health status. We sought to identify factors associated with self-reported functional health status in adolescents ...and young adults with repaired interrupted aortic arch.
Follow-up of survivors (aged 13-24 years) from a 1987 to 1997 inception cohort of neonates included completion of functional health status questionnaires (Child Health Questionnaire-CF87 age <18 years, n = 51 or the Short Form SF-36 age ≥18 years, n = 66) and another about 22q11 deletion syndrome (22q11DS) features (n = 141). Factors associated with functional health status domains were determined using multivariable linear regression analysis.
Domain scores of respondents were significantly greater than norms in 2 of 9 Child Health Questionnaire-CF87 and 4 of 10 SF-36 domains and only lower in the physical functioning domain of the SF-36. Factors most commonly associated with lower scores included those suggestive of 22q11DS (low calcium levels, recurrent childhood infections, genetic testing/diagnosis, abnormal facial features, hearing deficits), the presence of self-reported behavioral and mental health problems, and a greater number of procedures. Factors explained between 10% and 70% of domain score variability (R2 = 0.10-0.70, adj-R2 = 0.09-0.66). Of note, morphology and repair type had a minor contribution.
Morbidities associated with 22q11DS, psychosocial issues, and recurrent medical issues affect functional health status more than initial morphology and repair in this population. Nonetheless, these patients largely perceive themselves as better than their peers. This demonstrates the chronic nature of interrupted aortic arch and suggests the need for strategies to decrease reinterventions and for evaluation of mental health and genetic issues to manage associated deteriorations.
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Use of animal models in preclinical transplant research is essential to the optimization of human allografts for clinical transplantation. Animal models of organ donation and preservation help to ...advance and improve technical elements of solid organ recovery and facilitate research of ischemia–reperfusion injury, organ preservation strategies, and future donor‐based interventions. Important considerations include cost, public opinion regarding the conduct of animal research, translational value, and relevance of the animal model for clinical practice. We present an overview of two porcine models of organ donation: donation following brain death (DBD) and donation following circulatory death (DCD). The cardiovascular anatomy and physiology of pigs closely resembles those of humans, making this species the most appropriate for pre‐clinical research. Pigs are also considered a potential source of organs for human heart and kidney xenotransplantation. It is imperative to minimize animal loss during procedures that are surgically complex. We present our experience with these models and describe in detail the use cases, procedural approach, challenges, alternatives, and limitations of each model.
Large animal models are essential for experimental progress in organ transplant procedures and donor organ interventions. This review provides a summary of two major organ donation procedures, a detailed methods description and pharmacopeia for these procedures, an overview of existing research progress in these areas, and our experience in potential challenges, procedural considerations, and alternative methods relating to porcine organ donor research.
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