Outcomes of Surgical Repair of Vascular Rings and Slings in Children: A Word for the Asymptomatic.

Vascular rings (VRs) are rare aortic arch anomalies that may present with a wide variety of symptoms related to esophageal and/or airway compression. We reviewed our surgical experience in both symptomatic and asymptomatic children. All children (n = 58) who underwent surgical repair of VRs or slings (mean age 27.4 ± 45.60 months; 36 males [62%]) between March 2000 and April 2020 were included. The most common anatomic variant was a right aortic arch (RAA) with aberrant left subclavian artery (ALSCA) (n = 29; 50%). Kommerell's diverticulum was present in 23 of these patients (79%). The second most common variant was a double aortic arch (n = 22; 38%), followed by pulmonary artery sling (n = 4; 6%), RAA with mirror image branching and left ligamentum arteriosum (n = 3; 5.2%), and left aortic arch (LAA) with aberrant right subclavian artery (n = 1; 1.7%). One patient had a double ring with pulmonary artery sling and RAA with ALSCA. Symptoms were present in 42 patients (72%). Left lateral thoracotomy was the approach in 50 patients (86%), while sternotomy was used in 8 (14%). Symptomatic improvement occurred in the majority of symptomatic patients (93%). There was one perioperative mortality (1.7%) in the symptomatic group which was non-VR related. Morbidities included recurrent laryngeal nerve injury in three patients (5.2%) and transient chylothorax in two (3.4%). Persistence/recurrence of symptoms resulted in one early and one late reoperation. The mean follow-up was 3 ± 5 years. In the current era, VR repair in children including asymptomatic ones can be performed with excellent results. We recommend complete repair of RAA with aberrant LSCA by resection of Kommerell's diverticulum and translocation of the ALSCA to avoid recurrence.

Off-Pump "Clamp and Sew" Extracardiac Fontan With Inverted Bifurcated Graft for Dextrocardia With Heterotaxy Syndrome.

Avoiding cardiopulmonary bypass during palliation of single ventricle has the advantages of minimizing transfusions, pulmonary vascular resistance, and avoiding the inflammatory response from cardiopulmonary bypass. It is however not always straightforward, and the technique may be faced with challenges.

Quality Improvement Project in Congenital Cardiothoracic Surgery Patients: Reducing Surgical Site Infections.

BACKGROUND: Healthcare-associated infections are a major focus for quality improvement in hospitals today. Surgical site infections (SSIs), a postoperative complication in cardiac surgery, are associated with increased morbidity, mortality, hospital length of stay, and financial burden. METHODS: A recent increase in cardiothoracic surgery SSIs (CT-SSIs) at our institution instigated a multidisciplinary team to explore infection prevention, bundle element compliance, and to identify interventions to reduce the CT-SSI rate. Key interventions included preoperative screening and decolonization of methicillin-sensitive Staphylococcus aureus and methicillin-resistant S. aureus with repeated intranasal applications of mupirocin, universal skin prep with chlorhexidine for all patients, and additional antibiotic dosing upon initiating cardiopulmonary bypass. RESULTS: In 2014, the CT-SSI rate at our institution was 1.9/100 cases, which increased during the "intervention period" to 3.6 infections/100 cases in 2015 (16 total infections). Postinterventions, the CT-SSI rate decreased to 0.3 infections/100 cases (2 total infections), which was significantly lower than our baseline before the spike in infection rate. CONCLUSIONS: A comprehensive interdisciplinary approach with multiple interventions was successful in significantly reducing the CT-SSI rate in cardiothoracic surgery at a tertiary care pediatric hospital.

There's an App for That; Improving Communication during Pediatric Cardiothoracic Surgery.

INTRODUCTION: Waiting while a loved one is in surgery can be a very stressful time. Current processes for updating families vary from institution to institution. Providing timely and relevant updates, while important to the family, may strain a surgical team's operational system. In our initial experience with the Electronic Access for Surgical Events (EASE) application (app), we tested the extent to which its implementation improved communication with patient families. METHODS: We compared compliance data collected pre-EASE (December 2013 through September 2014) and post-EASE implementation (October 2014 until December 2015). RESULTS: Although the pre-EASE compliance rate for bi-hourly updates was 46% (118/255) of cases, post-EASE implementation achieved a compliance rate of 97% (171/176). A 2-sample test of proportions confirmed a significant improvement in compliance after the introduction of EASE technology (P < 0.001). Analysis of the 177 noncompliant cases in the pre-EASE period indicated that noncompliance occurred most frequently at the end of the case (97/177, 55%) when the patient remained in the operating room > 2 hours after the last update to the family. We also observed noncompliance at the beginning of the case (46/177, 26%), when the patient arrived in the operating room > 2 hours before the time of the first update. Family satisfaction scores that rated their experience during surgery as "Very Good" improved from 80% pre-EASE implementation to 97% postimplementation. We sustained this improvement for 1 year. CONCLUSIONS: A mobile technology app (EASE) improved both frequency and compliance with surgical updates to families, which resulted in a statistically significant increase in family satisfaction scores.

Interstage Survival for Patients with Hypoplastic Left Heart Syndrome After ECMO.

There is a reported 5-20 % incidence of extracorporeal membrane oxygenation (ECMO) following stage I palliation for hypoplastic left heart syndrome (HLHS). This study compares the interstage mortality of HLHS patients supported with ECMO (HLHS-ECMO) to those who were not supported with ECMO (HLHS-nECMO) using the National Pediatric Cardiology Quality Improvement Initiative database. Patients with HLHS who survived to hospital discharge after stage I palliation were analyzed. HLHS-ECMO patients were compared to HLHS-non-ECMO patients with respect to demographics, surgical variables, and interstage survival. A total of 931 patients were identified in the database. Sixty-six (7.1 %) patients were supported with ECMO during their stage I palliation admission. There were no statistically significant differences between the groups with respect to demographics or anatomic subtype. HLHS-ECMO patients were more likely to have a preoperative risk factor identified (62 vs. 48 %, p = 0.03) or require ECMO prior to stage I palliation (3 vs. 0.5 %, p = 0.03). HLHS-ECMO patients had a significantly higher incidence of death or transplant versus the HLHS-nECMO group (18 vs. 9 %, p = 0.03). Despite survival to discharge, patients with HLHS requiring ECMO after their palliation continue to have an increased risk of death/cardiac transplant versus patients that do not require ECMO. ECMO use is likely a marker for a high-risk patient group. These patients may benefit from closer follow-up during the interstage period.

Reducing variation in feeding newborns with congenital heart disease.

OBJECTIVE: Enteral feeding is associated with decreased infection rates, decreased mechanical ventilation, decreased hospital length of stay, and improved wound healing. Enteral feeding difficulties are common in congenital heart disease. Our objective was to develop experience-based newborn feeding guidelines for the initiation and advancement of enteral feeding in the cardiothoracic intensive care unit. DESIGN: This is a retrospective analysis of a quality improvement project. SETTING: This quality improvement project was performed in a cardiothoracic intensive care unit. PATIENTS: Newborns admitted to the cardiothoracic intensive care unit for cardiac surgery from January 2011 to May 2015 were retrospectively reviewed. INTERVENTION: Newborn feeding guidelines for the initiation and advancement of enteral feeding were implemented in January 2012. OUTCOME MEASURES: Guideline compliance and clinical variables before and after guideline implementation were reviewed. RESULTS: Compliance with the guidelines increased from 83% in 2012 to 100% in the first two quarters of 2015. Preguidelines (January 2011-December 2011): 45 newborns underwent cardiac surgery; 8 deaths prior to discharge; 1 patient discharged from NICU, therefore, N = 36. Postguidelines (January 2012-May 2015): 131 newborns with 12 deaths, 12 admitted from home, 8 in the NICU, 3 on the floor preop, and 3 back transferred, therefore, N = 93. No difference in feeding preop (post 75% vs pre 69%; P = .5) or full po feeds at discharge (post 78% vs pre 89%; P = .2). Mesenteric ischemia was not statistically different postguidelines (post 6% vs pre 14%; P = .14). Length of hospital stay decreased postguidelines (post 27 + 17 d vs pre 34 + 42 d; P < .001). CONCLUSIONS: Implementation of experience-based newborn feeding guidelines for initiation and advancement of enteral feeding in the cardiothoracic intensive care unit was successful in reducing practice variation supported by increasing guideline compliance. Percentage of patient's full oral feeding at discharge did not change. Length of hospital stay was reduced although cannot be fully attributed to feeding guideline implementation.

ECMO: Incidence and Outcomes of Patients Undergoing the Hybrid Procedure.

OBJECTIVE: To document the extracorporeal membrane oxygenation (ECMO) incidence and outcome in patients undergoing the hybrid procedure at an institution that routinely performs this procedure. DESIGN: A retrospective chart review on all patients with single ventricle physiology that underwent the hybrid procedure between 7/2002 and 12/2014. Patients were excluded if they underwent the hybrid procedure after 60 days of birth or subsequently underwent a biventricular repair. SETTING: A single center, tertiary pediatric hospital. PATIENTS: One hundred eighty-one patients with single ventricle physiology that underwent the hybrid procedure between 7/2002 and 12/2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: We studied a total of 181 patients (105 males: 76 females). Gestational age was 37.8 ± 2.0 weeks and birth weight was 3.0 ± 0.7 kg. Underlying diagnosis was hypoplastic left heart syndrome in 149 patients and other in 32 patients. Age at surgery was 7.8 ± 6.8 days and weight at surgery was 3.1 ± 0.6 kg. Two patients underwent ECMO support after the hybrid procedure. One patient had aortic atresia/mitral atresia and weighed 2.3 kg and the other patient had aortic atresia/mitral stenosis and weighed 2.1 kg at time of surgery. Both patients died. Incidence of ECMO support after hybrid procedure was 1.3% (2/149) for the hypoplastic left heart syndrome patients and 1.1% (2/181) for the entire cohort. CONCLUSION: Mortality in patients who underwent ECMO after the hybrid procedure was higher than reported for the Norwood procedure, however, the incidence of ECMO after hybrid procedure was also significantly lower than reported for the Norwood procedure. Future studies are needed to determine how to improve outcomes in this complex patient population.

Improving Surveillance and Prevention of Surgical Site Infection in Pediatric Cardiac Surgery.

BACKGROUND: Postoperative cardiovascular surgical site infections are preventable events that may lead to increased morbidity, mortality, and health care costs. OBJECTIVE: To improve surgical wound surveillance and reduce the incidence of surgical site infections. METHODS: An institutional review of surgical site infections led to implementation of 8 surveillance and process measures: appropriate preparation the night before surgery and the day of surgery, use of appropriate preparation solution in the operating room, appropriate timing of preoperative antibiotic administration, placement of a photograph of the surgical site in the patient's chart at discharge, sending a photograph of the surgical site to the patient's primary care physician, 30-day follow-up of the surgical site by an advanced nurse practitioner, and placing a photograph of the surgical site obtained on postoperative day 30 in the patient's chart. RESULTS: Mean overall compliance with the 8 measures from March 2013 through February 2014 was 88%. Infections occurred in 10 of 417 total operative cases (2%) in 2012, in 8 of 437 total operative cases (2%) in 2013, and in 7 of 452 total operative cases (1.5%) in 2014. CONCLUSIONS: Institution of the surveillance process has resulted in improved identification of suspected surgical site infections via direct rather than indirect measures, accurate identification of all surgical site infections based on definitions of the National Healthcare Safety Network, collaboration with all persons involved, and enhanced communication with patients' family members and referring physicians.

Risk Factors for Unanticipated Readmissions During the Interstage: A Report From the National Pediatric Cardiology Quality Improvement Collaborative.

This study describes unanticipated interstage readmissions in patients with hypoplastic left heart syndrome, identifies independent risk factors for unanticipated interstage readmissions, and evaluates variation in unanticipated readmission rates among collaborative centers. Retrospective data of patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from July 2008 to July 2013 were analyzed. Risk factors present at the beginning of the interstage were captured. Competing risks time to event analyses determined the association between these factors and unanticipated interstage readmission. Readmission center variation was examined using funnel plots. Unanticipated interstage readmissions occurred in 66% of 815 patients at 50 centers. The median readmission length of stay was 2 days (interquartile range: 0-6) and median time to first readmission was 29 days (interquartile range: 9-63). Most readmissions were prompted by minor changes in clinical status (64%), whereas only 6% were major adverse event readmissions. Independent readmission risk factors included genetic syndrome (HR = 1.40, 95% CI: 1.05-1.88), center volume (small vs large HR = 1.32, CI: 1.04-1.66, medium vs large HR = 1.35, CI: 1.09-1.68), preoperative ventricular dysfunction (HR = 2.02, CI: 1.31-3.10), tricuspid regurgitation (HR = 1.36, CI: 1.08-1.72), duration of circulatory arrest (HR = 0.99, CI: 0.989-0.998), and undergoing Hybrid procedure relative to Norwood/right ventricle to pulmonary artery conduit (HR = 1.40, CI: 1.02-1.93). There was significant center variation in the number of readmissions and duration of readmissions. Unanticipated readmissions are common during the interstage period with notable center variation. However, these readmissions are short and are rarely in response to major adverse events.

Eosinophilic esophagitis in children following cardiac transplantation: association with post-transplant lymphoproliferative disorder and other transplant outcomes.

Although cardiac transplantation is life-saving, morbidities from immunosuppression are significant. EoE is a complication of calcineurin inhibitors following liver transplant causing feeding intolerance, weight loss, vomiting, and dysphagia. There are limited reports of EoE following heart transplantation. We performed a retrospective single-center review of pediatric cardiac transplant patients from 2000 to 2010. A case-control analysis of patients with and without EoE was performed evaluating heart transplantation outcomes such as rates of rejection, CAV, PTLD, and graft loss. Eighty-six transplants were performed in 84 patients; 34 (40%) underwent diagnostic endoscopy, and 10 (12%) had EoE. Median time to diagnosis of EoE was 3.7 yr (IQR: 2.0-5.2). There were no differences in demographics or use of induction medications between patients with or without EoE. Patients with EoE had fewer episodes of treated rejection (1.0 vs. 2.5; p = 0.04). Four of 10 (40%) EoE patients had PTLD compared with only 2/24 (8%) of those without EoE (p = 0.048; OR 7.33 [95% CI: 1.1-50.2]). There were no differences in CAV or graft loss between groups. EoE should be considered as a cause of GI symptoms in children after cardiac transplantation and may be associated with fewer rejection episodes and increased rates of PTLD, thus representing a marker of over-immunosuppression.

Standardized multidisciplinary protocol improves handover of cardiac surgery patients to the intensive care unit.

OBJECTIVES: To determine whether the implementation of a standardized handover protocol could reduce the number of errors occurring during patient transitions from the operating room to the intensive care unit. DESIGN: Prospective, interventional study. SETTING: Pediatric cardiac intensive care unit. SUBJECTS: Seventy-nine patient handovers in patients transitioning from the operating room to the cardiac intensive care unit after congenital cardiac surgery. INTERVENTIONS: A preintervention assessment of patient handovers was obtained by direct observation using a standardized checklist. A teamwork-driven handover process and protocol was developed using traditional and novel quality-improvement techniques. The postimplementation observational assessment of handovers was performed using the same preintervention assessment tool. Preintervention and postintervention data metrics were analyzed and compared. MEASUREMENTS AND MAIN RESULTS: Forty-one and 38 observations were performed in the preintervention and postintervention periods, respectively. Protocol implementation improved key areas of the handover process. Technical errors per handover were reduced from 6.24 to 1.52 (p < .0001), and critical verbal handoff information omissions were reduced from 6.33 to 2.38 (p < .0001) per handover. There was no change in duration of either the verbal handoff briefing or the overall handover process. Caregivers noted improvement in teamwork and handoff content received after the intervention. CONCLUSIONS: A formal, structured handover process for pediatric patients transitioning to the intensive care unit after cardiac surgery can reduce medical errors that occur during the admission process and improve teamwork among caregivers.