Altered white matter connectivity in children with congenital heart disease with single ventricle physiology.

Children born with congenital heart disease (CHD) have seen a dramatic decrease in mortality thanks to surgical innovations. However, there are numerous risk factors associated with CHD that can disrupt neurodevelopment. Recent studies have found that psychological deficits and structural brain abnormalities persist into adulthood. The goal of the current study was to investigate white matter connectivity in early school-age children (6-11 years), born with complex cyanotic CHD (single ventricle physiology), who have undergone Fontan palliation, compared to a group of heart-healthy, typically developing controls (TPC). Additionally, we investigated associations between white matter tract connectivity and measures on a comprehensive neuropsychological battery within each group. Our results suggest CHD patients exhibit widespread decreases in white matter connectivity, and the extent of these decreases is related to performance in several cognitive domains. Analysis of network topology showed that hub distribution was more extensive and bilateral in the TPC group. Our results are consistent with previous studies suggesting perinatal ischemia leads to white matter lesions and delayed maturation.

A Narrative Review of Multiinstitutional Data Registries of Pediatric Congenital Heart Disease in Pediatric Cardiac Anesthesia and Critical Care Medicine.

Congenital heart disease (CHD) is one of the most common birth anomalies. While the care of children with CHD has improved over recent decades, children with CHD who undergo general anesthesia remain at increased risk for morbidity and mortality. Electronic health record systems have enabled institutions to combine data on the management and outcomes of children with CHD in multicenter registries. The application of descriptive analytics methods to these data can improve clinicians' understanding and care of children with CHD. This narrative review covers efforts to leverage multicenter data registries relevant to pediatric cardiac anesthesia and critical care to improve the care of children with CHD.

Putting a face and context on pediatric surgery cancelations: The development of parent personas to guide equitable surgical care.

Last-minute cancelation of planned surgery can have substantial psychological, social, and economic effects for patients/families and also leads to wastage of expensive health-care resources. In order to have a deeper understanding of the contextual, psychological, practical, and behavioral factors that potentially impact pediatric surgery cancelation, we conducted a qualitative study to create 'personas' or fictional portraits of parents who are likely to cancel surgery. We conducted in-depth qualitative interviews with 21 parents of children who were considered 'at risk' for surgical cancelation and whose scheduled surgery was canceled at late notice. From the themes, patterns, and associated descriptive phrases in the data, we developed and validated five different personas of typical scenarios reflecting parent experiences with surgery and surgery cancelations. The personas are being employed to guide contextualized development of interventions tailored to prototypical families as they prepare and attend for surgery.

Exercise-Induced Systemic Venous Hypertension in the Fontan Circulation.

Increasingly end-organ injury is being demonstrated late after institution of the Fontan circulation, particularly liver fibrosis and cirrhosis. The exact mechanisms for these late phenomena remain largely elusive. Hypothesizing that exercise induces precipitous systemic venous hypertension and insufficient cardiac output for the exercise demand, that is, a possible mechanism for end-organ injury, we sought to demonstrate the dynamic exercise responses in systemic venous perfusion (SVP) and concurrent end-organ perfusion. Ten stable Fontan patients and 9 control subjects underwent incremental cycle ergometry-based cardiopulmonary exercise testing. SVP was monitored in the right upper limb, and regional tissue oxygen saturation was monitored in the brain and kidney using near-infrared spectroscopy. SVP rose profoundly in concert with workload in the Fontan group, described by the regression equation 15.97 + 0.073 watts per mm Hg. In contrast, SVP did not change in healthy controls. Regional renal (p <0.01) and cerebral tissue saturations (p <0.001) were significantly lower and decrease more rapidly in Fontan patients. We conclude that in a stable group of adult patients with Fontan circulation, high-intensity exercise was associated with systemic venous hypertension and reduced systemic oxygen delivery. This physiological substrate has the potential to contribute to end-organ injury.

Management of a child with pulmonary arterial hypertension presenting with systemic hypertension.

We describe the course and management of a 12-year-old girl with severe pulmonary arterial hypertension who initially presented with severe systemic hypertension. Successful therapy included pulmonary vasodilators and an atrial septostomy, while ensuring adequate maintenance of her systemic vascular resistance to maintain cardiac output. Clear understanding of the physiology and judicious medical management in patients with severe pulmonary arterial hypertension using extreme compensatory mechanisms is vitally important.

Reducing Cancelations on the Day of Scheduled Surgery at a Children's Hospital.

BACKGROUND AND OBJECTIVES: Cancelation on the day of surgery (DoSC) represents a costly wastage of operating room (OR) time and causes inconvenience, emotional distress, and financial cost to families. A quality improvement project sought to reduce lost OR time due to cancelation. METHODS: Key drivers of the process included effective 2-way communication with families, compliance with fasting rules, and decision-making on patient illness before the day of surgery. A multidisciplinary team conducted serial tests of change addressing the various key drivers. Interventions were simplified, colorful, personalized preoperative instruction sheets and text-message reminders to caregivers' cellphones, as well as a defined institutional decision-making pathway to permit rescheduling before the day of surgery in case of patient illness concerns. After initial smaller-scale testing, the interventions were implemented across all patients and sites. Data were collected from the hospital information technology system and analyzed by using control charts and statistical process control methods. RESULTS: Mean OR time lost due to DoSC was decreased from a baseline of 5.7 to 3.6 hours/day in testing with a subset of surgical services at the hospital's base campus, and then from 6.6 hours to 5.5 hours/day when implemented across all services at both surgical sites. CONCLUSIONS: By applying quality improvement methods, significant reductions were made in time lost due to DoSC. The impact can be significant by improving institutional resource utilization.

Anesthetic management of children with in situ Berlin Heart EXCOR.

Modern mechanical devices can support children with severely impaired cardiac function until a donor heart is found for transplantation or native function recovers. Pediatric heart transplantation offers a good chance of survival with a high quality of life to individuals with limited life expectancy and/or severe limitation to daily activities, but many die on the transplant list or are not listed because of a shortage of donor organs. In recent cohorts, there is better outcome when ventricular assist devices (VADs) rather than extracorporeal membrane oxygenation are used as a 'bridge' to transplantation. Anesthesiologists working in centers where VADs are available may increasingly be asked to provide anesthesia to children with such devices in situ, including procedures outside the cardiac surgical operating room. The Berlin Heart EXCOR device is a VAD system with increasing popularity in pediatric practice and has system components available in sizes suitable even for neonates. Postimplantation considerations include hemodynamics, thromboembolic complications and their prevention by anticoagulation, antimicrobial therapy, and the rehabilitation and mobilization of recipients. VAD-specific emergencies must be recognized and managed appropriately by anesthesiologists looking after Berlin Heart recipients. These include malignant dysrhythmias, sudden loss of VAD output, air or clot embolism, and sudden cyanosis. Provision of anesthesia for patients with an in situ Berlin Heart requires attention to particular considerations in preoperative assessment, induction, maintenance, and postoperative care.

Co-injection of clonidine prolongs the anesthetic effect of lidocaine skin infiltration by a peripheral action.

BACKGROUND: The addition of clonidine to local anesthesia prolongs the local anesthetic action, but in humans, the contribution of a peripheral mechanism remains unclear. METHODS: We investigated clonidine's peripheral effect in 20 healthy volunteers undergoing double-blind, subcutaneous infiltration of 0.5% lidocaine with normal saline to one forearm and then, immediately, of lidocaine with 10 mug clonidine to the contralateral arm. Pinprick sensation was tested every 15 min for 6 h. RESULTS: Median time to return of normal sensation was 3.5 h for lidocaine alone, but at least 6 h if combined with clonidine (P < 0.001). CONCLUSIONS: Clonidine has a significant peripheral action in enhancing duration of local anesthesia on superficial co-infiltration with lidocaine.