Factors Associated With Gastrostomy Tube Complications in Infants With Congenital Heart Disease.

INTRODUCTION: Children with congenital heart disease (CHD) often experience feeding intolerance due to aspiration, inability to tolerate feed volume, or reflux within the first few months of life, requiring a surgically placed gastrostomy tube (GT) for durable enteral access. However, complications such as GT dislodgement, cellulitis, and leakage related to GT use are common. GT-related complications can lead to unscheduled pediatric surgery clinic or emergency room (ER) visits, which can be time consuming for the family and increase overall healthcare costs. We sought to identify factors associated with GT complications within 2 wk after GT surgery and 1-y after discharge home following GT placement in infants with CHD. METHODS: We performed a retrospective cohort study using the Society of Thoracic Surgeons database and electronic medical records from a tertiary children's hospital. We identified infants <1 y old underwent CHD surgery followed by GT surgery between September 2013-August 2018. Demographics, pre-operative feeding regimen, comorbidities, and GT-related utilization were measured. Postoperative GT complications (e.g., GT cellulitis, leakage, dislodgement, obstruction, and granulation tissue) within 2 wk after the GT surgery and an unplanned pediatric surgery clinic or ER visit within 1-y after discharge home were captured. Bivariate comparisons and multivariable logistic regression evaluated factors associated with GT complications and unplanned clinic or ER visits. A Kaplan-Meier failure curve examined the timing of ER/clinic visits. RESULTS: Of 152 infants who underwent CHD then GT surgeries, 66% (N = 101) had postoperative GT complications. Overall, 83 unscheduled clinic visits were identified after discharge, with 37% (N = 31) due to concerns about granulation tissue. Of 137 ER visits, 48% (N = 66) were due to accidental GT dislodgement. Infants who were hospitalized for ≥2 wk after GT surgery had more complications than those discharged home within 2 wk of the GT surgery (40.6% versus 15.7%, P = 0.002). Infants receiving oral nutrition before CHD surgery (38.6% versus 60%, P=<0.001) or with single ventricle defects (19.8% versus 37.3%, P = 0.02) had fewer GT complications. After adjusting for type of cardiac anomaly, infants receiving oral nutrition prior to CHD surgery had a decreased likelihood of GT complications (odds ratio OR 0.46; 95% confidence intervals CI:0.23-0.93). A Kaplan-Meier failure curve demonstrated that 50% of the cohort experienced a complication leading to an unscheduled ER/clinic visit within 6 mo after discharge. CONCLUSIONS: Unplanned visits to the ER or pediatric surgery clinic occur frequently for infants with CHD requiring a surgically placed GT. Oral feedings before cardiac surgery associated with fewer GT complications. Prolonged hospitalization associated with more GT complications. Optimizing outpatient care and family education regarding GT maintenance may reduce unscheduled visits for this high-risk, device-dependent infant population.

Resident education in congenital heart surgery does not compromise outcomes.

OBJECTIVE: Most of all congenital cardiac surgical programs participate in public outcomes reporting. The primary end point is transparency. In this era, academic programs with surgical residents face the challenge of producing outstanding results while allowing residents to learn by doing. We sought to understand the effect of education on our surgical outcomes. METHODS: We collected data for all American Board of Thoracic Surgery index cases done at our institution over a 10-year period. We identified 3406 cases and categorized them into 2 groups according to primary surgeon: attending (2269) versus resident (1137). In a multivariable logistic regression model we examined the effect of operating surgeon on in-hospital mortality, major morbidity, and length of stay. We used propensity score matching subsequently to balance differences between cohorts, and multivariable logistic regression was repeated. RESULTS: Using the entire cohort, multivariable logistic regression model adjusted for age, sex, weight, lack of preoperative comorbidity, presence of preoperative respiratory failure, The Society of Thoracic Surgeons--European Association for Cardio-Thoracic Surgery category, and need for deep hypothermic circulatory arrest, showed a higher odds of survival in the resident cohort (odds ratio, 1.484; 95% confidence interval, 0.998-2.206; P = .05). Propensity score matching identified 1137 pairs of attending and resident cases with well-balanced preoperative variables. Logistic regression modeling using the matched cohort showed equivalent 30-day mortality, 30-day major morbidity, and length of stay. CONCLUSIONS: There was no difference in mortality, major morbidity, or length of stay when similar cases were compared that were operated on by attendings versus those by a resident. Effectively educating congenital heart surgeons without compromising an operation's quality requires thoughtful approach, including case selection and graded responsibility.

Factors Impacting Long-Term Gastrostomy Tube Dependence in Infants with Congenital Heart Disease.

BACKGROUND: Infants with congenital heart disease (CHD) often experience oral feeding intolerance requiring gastrostomy (GT). Complications related to GT use are common. The study aim was to identify factors associated with continued GT use at one-year. METHODS: A retrospective cohort study was performed at a tertiary children's hospital using the Society of Thoracic Surgeons database and patients' electronic medical record. Infants <1-year with CHD who underwent cardiac and GT surgery between January 2014-October 2019 were identified. Patient demographics, preoperative feeding, clinical variables, and GT use at one-year was evaluated. A separate cohort discharged with a nasogastric tube (NGT) was identified for longitudinal comparisons. RESULTS: Of 137 infants who received a GT, 115 (84%) continued using their GT at one-year. Factors associated with continued GT use included lower median percent of goal oral feeding before GT placement (0% IQR 0-6.5 versus 3.7% IQR 0-31), prolonged hospitalization after GT placement (36% versus 14%, P-value = 0.048), and failure to take oral feeds at discharge (69% versus 27%, P-value <0.001). There was no difference in demographics or clinical comorbidities between groups. Clinic/emergency room visits for GT complications were common (72%). Eight infants discharged with a NGT did not require GT placement. CONCLUSIONS: Patients with CHD tolerating minimal oral nutrition before GT placement, prolonged hospitalization after GT, and difficulty with oral feeds at discharge were more likely to use their GT at 1-year. Outpatient NGT feeding is feasible for select infants with CHD. Efforts to optimize care for this complex, device-dependent population are warranted to minimize risks and facilitate family engagement for long-term care.

Adapting Civility Education in an Academic-Practice Partnership.

BACKGROUND: Incivility results in nurse burnout, decreased job performance, and decreased patient safety. Leaders of an academic-practice partnership developed educational activities promoting organizational civility during the COVID-19 pandemic. The purpose of this article is to describe an educational activity about civility that was transitioned to a virtual platform and participants' comfort engaging in and responding to incivility. METHOD: Face-to-face education was converted to a synchronous online event, supporting 75 nurses, nursing students, and other health care professionals in attendance. Activities consisted of cognitive rehearsal techniques, breakout rooms, simulation videos, group debriefs, and panel discussions delivered via Zoom and Mentimeter software. RESULTS: Workplace Civility Index results were significantly different from pretest to post-test. Seventy-two percent of participants were not comfortable gossiping about others, but only 30% were comfortable responding to incivility. CONCLUSION: Promoting civility awareness through a virtual education platform using cognitive rehearsal techniques and reflection can provide support for current and future nurses. [J Contin Educ Nurs. 2021;52(12):575-580.].

Non-invasive biomarkers of Fontan-associated liver disease.

BACKGROUND & AIMS: Fontan-associated liver disease (FALD) has emerged as an important morbidity following surgical palliation of single ventricle congenital heart disease. In this study, non-invasive biomarkers that may be associated with severity of FALD were explored. METHODS: A retrospective cohort of paediatric patients post-Fontan who underwent liver biopsy at a high volume at a paediatric congenital heart disease centre was reviewed. RESULTS: Among 106 patients, 66% were male and 69% were Hispanic. The mean age was 14.4 ± 3.5 years, and biopsy was performed 10.8 ± 3.6 years post-Fontan. The mean BMI was 20.8 ± 5 kg/m2, with 27.4% meeting obesity criteria. Bridging fibrosis was observed in 35% of patients, and 10.4% of all patients had superimposed steatosis. Bridging fibrosis was associated with lower platelet counts (168.3 ± 58.4 vs. 203.9 ± 65.8 K/µl for congestive hepatic fibrosis score [CHFS] 0-2b, p = 0.009), higher bilirubin (1.7 ± 2.2 vs. 0.9 ± 0.7 mg/dl, p = 0.0090), higher aspartate aminotransferase-to-platelet ratio index [APRI] and fibrosis-4 [FIB-4] scores (APRI: 0.5 ± 0.3 vs. 0.4 ± 0.1, p <0.01 [AUC: 0.69] and FIB-4: 0.6 ± 0.4 vs. 0.4 ± 0.2, p <0.01 [AUC: 0.69]), and worse overall survival (median 2 years follow-up post-biopsy, p = 0.027). Regression modelling of temporal changes in platelet counts before and after biopsy correlated with fibrosis severity (p = 0.005). CONCLUSIONS: In this large, relatively homogeneous adolescent population in terms of age, ethnicity, and Fontan duration, bridging fibrosis was observed in 35% of patients within the first decade post-Fontan. Bridging fibrosis was associated with worse survival. Changes in platelet counts, even years before biopsy, and APRI/FIB-4 scores had modest discriminatory power in identifying patients with advanced fibrosis. Steatosis may represent an additional risk factor for disease progression in obese patients. Further prospective studies are necessary to develop strategies to screen for FALD in the adolescent population. LAY SUMMARY: In this study, the prevalence of Fontan-associated liver disease (FALD) in the young adult population and clinical variables that may be predictive of fibrosis severity or adverse outcomes were explored. Several lab-based, non-invasive markers of bridging fibrosis in FALD were identified, suggesting that these values may be followed as a prognostic biomarker for FALD progression in the adolescent population.

Progression in Fontan conduit stenosis and hemodynamic impact during childhood and adolescence.

OBJECTIVE: To characterize changes in Fontan conduit size over time and determine if cross-sectional area (CSA) affects cardiac output, pulmonary artery growth, and exercise capacity. METHODS: We conducted a retrospective cross-sectional study of patients with Fontan physiology who underwent cardiac magnetic resonance imaging or cardiac catheterization between January 2013 and October 2019. We collected Fontan and pulmonary artery measurements, hemodynamic data, and cardiopulmonary exercise test data. We identified 158 patients with an extracardiac Fontan. We measured minimum and mean Fontan conduit CSA and assessed whether these correlated with Nakata index, cardiac index, or exercise capacity. RESULTS: Minimum Fontan CSA decreased by a median of 33% (24%, 40%) during a mean follow-up of 9.6 years. Median percentage decrease in Fontan CSA did not differ among 16-, 18-, and 20-mm conduits (P = .29). There was a significant decrease in the minimum Fontan CSA (33% [25%, 41%]) starting less than 1-year post-Fontan. Median Nakata index was 177.6 mm2/m2 (149.1, 210.8) and was not associated with Fontan CSA/BSA (ρ = 0.09, P = .29). Fontan CSA/BSA was not associated with cardiac index (ρ = -0.003, P = .97). A larger Fontan CSA/BSA had a modest correlation with % predicted oxygen consumption (ρ = 0.31, P = .013). CONCLUSIONS: Fontan conduit CSA decreases as early as 6 months post-Fontan. The minimum Fontan CSA/BSA was not associated with cardiac index or pulmonary artery size but did correlate with % predicted peak oxygen consumption.

A Novel Restorative Pulmonary Valve Conduit: Early Outcomes of Two Clinical Trials.

Objectives: We report the first use of a biorestorative valved conduit (Xeltis pulmonary valve-XPV) in children. Based on early follow-up data the valve design was modified; we report on the comparative performance of the two designs at 12 months post-implantation. Methods: Twelve children (six male) median age 5 (2 to 12) years and weight 17 (10 to 43) kg, had implantation of the first XPV valve design (XPV-1, group 1; 16 mm (n = 5), and 18 mm (n = 7). All had had previous surgery. Based on XPV performance at 12 months, the leaflet design was modified and an additional six children (five male) with complex malformations, median age 5 (3 to 9) years, and weight 21 (14 to 29) kg underwent implantation of the new XPV (XPV-2, group 2; 18 mm in all). For both subgroups, the 12 month clinical and echocardiographic outcomes were compared. Results: All patients in both groups have completed 12 months of follow-up. All are in NYHA functional class I. Seventeen of the 18 conduits have shown no evidence of progressive stenosis, dilation or aneurysm formation. Residual gradients of >40 mm Hg were observed in three patients in group 1 due to kinking of the conduit (n = 1), and peripheral stenosis of the branch pulmonary arteries (n = 2). In group 2, one patient developed rapidly progressive stenosis of the proximal conduit anastomosis, requiring conduit replacement. Five patients in group 1 developed severe pulmonary valve regurgitation (PI) due to prolapse of valve leaflet. In contrast, only one patient in group 2 developed more than mild PI at 12 months, which was not related to leaflet prolapse. Conclusions: The XPV, a biorestorative valved conduit, demonstrated promising early clinical outcomes in humans with 17 of 18 patients being free of reintervention at 1 year. Early onset PI seen in the XPV-1 version seems to have been corrected in the XPV-2, which has led to the approval of an FDA clinical trial. Clinical Trial Registration: www.ClinicalTrials.gov, identifier: NCT02700100 and NCT03022708.

Postoperative heart failure after stage 1 palliative surgery for single ventricle cardiac disease.

Outcomes for patients with single ventricle congenital heart disease (SV-CHD) continue to improve over time. However, the prognosis for patients who develop heart failure immediately after surgery is poorly understood. We conducted a single-center, retrospective cohort study of patients with SV-CHD, who suffered postoperative heart failure. Of 1038 cardiac surgeries performed on 621 SV-CHD patients between 2004 and 2010, 125 patients met inclusion criteria, including non-septatable anatomy, stage 1 surgery, and verified low cardiac output or heart failure state per STS definition. Overall survival was 73.2% at 2 months, 64.9% at 1 year, 60.5% at 2 years, and 54.6% at 4 years. Inotrope dependence beyond 7 days post-op yielded 45% 2-year survival versus 68% for those who weaned from inotropes within 7 days (p = 0.02). Atrioventricular valve regurgitation (AVVR) influenced survival, and patients who developed renal failure or required ECMO fared poorly, even when they survived their hospitalization. Patients with postoperative heart failure and low cardiac output syndrome constitute a high-risk population beyond the term of the initial hospitalization and have an overall mid-term survival of 55% at 4 years. Wean from inotropic therapy is not completely reassuring in this population, as they have ongoing elevated risk of cardiac failure and death in the medium term. Ventricular dysfunction, AVVR, renal failure, and need for ECMO are all important prognostic factors for mid-term mortality. Inotrope dependence for > 7 days has important implications reaching beyond the hospitalization.

Impact of Nurse Peer Review on a Culture of Safety.

BACKGROUND: Nursing peer review (NPR) is essential in evaluating nursing practice. A common theme throughout the literature is that NPR holds nurses accountable for their practice. PROBLEM: The nursing profession has struggled to conduct peer review consistently due to lack of a standardized framework. APPROACH: A NPR program was developed, implemented, and evaluated in a 355-bed acute care hospital in the Midwestern United States. OUTCOMES: A pre- and postdesign using the Agency for Healthcare Research and Quality Hospital Survey on Patient Safety Culture was used for evaluation. Paired t test results revealed statistically significant differences in group means for 2 questions: mistakes have led to positive changes here (P < .001, d = 1.31), and staff will freely speak up (P = .002, d = 0.67). Clinically significant differences in group means were noted for the remaining survey items. CONCLUSIONS: Implementation of an NPR program is a promising means to improve the culture of safety in health care institutions.

Home Monitoring Program Reduces Mortality in High-Risk Sociodemographic Single-Ventricle Patients.

A clinician-driven home monitoring program can improve interstage outcomes in single-ventricle patients. Sociodemographic factors have been independently associated with mortality in interstage patients. We hypothesized that even in a population with high-risk sociodemographic characteristics, a home monitoring program is effective in reducing interstage mortality. We defined interstage period as the time period between discharge following Norwood palliation and second-stage surgery. We reviewed the charts of patients for the three-year period before (group 1) and after (group 2) implementation of the home monitoring program. Clinical variables around Norwood palliation, during the interstage period, and at the time of second-stage surgery were analyzed. There were 74 patients in group 1 and 52 in group 2. 59 % patients were Hispanic, and 84 % lived in neighborhoods where over 5 % families lived below poverty line. There was no significant difference in pre-Norwood variables, Norwood discharge variables, age at second surgery, or outcomes at second surgery. There were more Sano shunts performed at the Norwood procedure as the source of pulmonary blood flow in group 2 (p value <0.05). There were more unplanned hospital admissions and percutaneous re-interventions in group 2. Patients in group 2 whose admission criteria included desaturation had a 45 % likelihood of having an unplanned re-intervention. Group 2 noted an 80 % relative reduction in interstage mortality (p < 0.01). In a multiple regression analysis, after accounting for ethnicity, socio-economic status, and source of pulmonary blood flow, enrollment in a home monitoring program independently predicted improved interstage survival (p < 0.01). A clinician-driven home monitoring program reduces interstage mortality even when the majority of patients has high-risk sociodemographic characteristics.

Survival and quality of life for children with end-stage heart failure who are not candidates for cardiac transplant.

BACKGROUND: Some pediatric patients referred for heart transplant (HTx) are sub-optimal candidates. Their outcomes without HTx are presumed to be dismal, but have not been well described. Knowledge about their outcomes is critical when weighing the risks between a high-risk transplant and "terminal" palliation. METHODS: We retrospectively reviewed all HTx referrals from January 2005 to July 2013. We excluded those who were listed for HTx, or who were denied HTx due to being "too well," seeking only those who were in need of but not suitable for HTx. End-points included mortality and length of survival. RESULTS: Of 212 referrals, 39 (19%) (age 0 to 19 years, median 3.5 years) were denied HTx for reasons other than being too well. Twenty-eight (72%) had palliated congenital heart disease. Overall mortality during the follow-up period was 38% (n = 15) with a median follow-up time of 195 days (8 to 2,832 days). Ten patients received subsequent cardiac surgery with 1 death (10%) and median follow-up of 2.6 years. Mortality risk was not influenced by age, weight, growth failure, congenital heart disease or single-ventricle physiology. Mechanical ventilation (hazard ratio 6.31, p = 0.001) and inotrope dependence (hazard ratio 4.79, p = 0.006) were associated with the highest risk of mortality. Quality of life was measured with the PedsQL cardiac module and completed by 11 of 16 eligible patients with an overall average score of 70.2 ± 23.9. CONCLUSIONS: An advanced heart failure program can achieve satisfactory results for pediatric patients who are not suitable candidates for HTx. For some children, high-risk palliative surgery can result in better outcome than high-risk HTx. Mortality was related to the degree of heart failure at presentation rather than underlying heart disease.

Synchronous HIV/AIDS-related Epstein-Barr Virus-associated smooth muscle tumors in a 20-year-old female.

We present a case of simultaneous endobronchial and adrenal Epstein-Barr Virus-associated smooth muscle tumors in a 20-year-old female with AIDS. Blood Epstein-Barr Virus polymerase chain reaction was negative at the time of diagnosis.

Rapidly fatal disseminated acanthamoebiasis in a single lung transplant recipient.

BACKGROUND: Lung transplant recipients are at great risk for developing various infectious complications. These infections portend a significant morbidity and mortality throughout their lifetime following transplantation. At times, cutaneous manifestations are the only clues to systemic infection. CASE REPORT: A 62 year-old man with a history of idiopathic pulmonary fibrosis presented 6 months after receiving bilateral sequential cadaveric lung transplantation for anorexia, early satiety, weight loss, exertional dsypnea, arthralgia, and depression. On exam, two rapidly growing non-painful 1.5-3 centimeter erythematous nodules with purulent draining on the anterior chest wall were noted. On Hospital Day 7, the patent was found to be un-responsive, hypotensive, and febrile. Brain imaging revealed diffuse thick nodular enhancement of leptomeningeal surface and multiple areas of hypodenisty associated with mass effect in the bilateral vermis and cerebellar hemispheres with effacement of the fourth ventricle. CSF PCR analysis showed Acanthamoeba sp. confirmed by the Center for Disease Control. Despite multi-modal therapy, his clinical course deteriorated and resulted in brain death. CONCLUSION: Acanthamoeba infection is extremely rare in thoracic organ recipients. We report the fifth case of progressive disseminated acanthamoebiasis in a lung transplant recipient.

Outcomes after lung transplantation and practices of lung transplant programs in the United States regarding hepatitis C seropositive recipients.

BACKGROUND: The estimated prevalence of hepatitis C virus (HCV) infection among lung transplant (LT) recipients is 1.9%. Many thoracic transplant programs are reluctant to transplant HCV-seropositive patients due to concerns of hepatic dysfunction caused by immunosuppression. The aims of this study are to survey current practices of US LT programs regarding HCV-seropositive patients and using the Organ Procurement and Transplantation Network/United Network for Organ Sharing database and to assess the clinical outcomes of HCV-positive compared with HCV-negative LT recipients. METHODS: A survey of US transplant centers that have performed more than 100 LTs was conducted. In addition, 170 HCV-seropositive and 9259 HCV-seronegative recipients who received HCV-seronegative donor organs between January 1, 2000, to December 31, 2007, were identified from the Organ Procurement and Transplantation Network/United Network for Organ Sharing database. Outcome variables including patient survival were compared between the two groups. RESULTS: A total of 64.4% centers responded to the survey. Ten of 29 (34.5%) programs would not consider HCV-seropositive patients for LT. Among the 19 programs that will consider HCV-seropositive patients, only five centers would transplant actively viremic patients. Overall patient survival rates of HCV-seropositive patients were similar to HCV-seronegative patients (84.7% at 1 year, 63.9% at 3 years, 49.4% at 5 years for HCV-seropositive group vs. 82.0% at 1 year, 65.0% at 3 years, 51.4% at 5 years for HCV-seronegative group, P=0.712). Relative risk of recipients for death remained statistically insignificant after adjusting for recipient age, donor age, obesity, sensitization, serum creatinine, and medical condition at time of transplant (relative risk [RR]=1.07 [0.84-1.38], P=0.581). CONCLUSIONS: Since 2000, patient survival rates of HCV-positive patients are identical to those who are HCV-negative. However, most of these HCV-seropositive patients were probably nonviremic.

A randomized, placebo-controlled trial of aprotinin to reduce primary graft dysfunction following lung transplantation.

PURPOSE: Severe primary graft dysfunction (PGD) is the major early problem following lung transplantation. Aprotinin, a serine protease inhibitor, has many anti-inflammatory properties that might reduce or prevent lung injury. Our hypothesis was that the incidence of PGD could be reduced by a combination of donor lung perfusion and systemic administration of aprotinin to recipients. METHODS AND MATERIALS: The study was randomized and placebo controlled. Donor lungs were perfused during procurement with 4 L Perfadex containing aprotinin (280 mg load + 70 mg/hL) or placebo. Aprotinin or placebo was also administered peri-operatively to the recipients. The study was powered to detect a 10% improvement in the primary endpoint of developing ISHLT grade III PGD anytime within 48 hr following the transplant procedure. RESULTS: There were 48 patients randomized. Diagnosis and the use of bypass were different between groups. The study was stopped prematurely at the planned interim analysis point because of published concerns about renal toxicity of aprotinin. There was no difference in the occurrence of the primary endpoint between groups of patients. The median change from the baseline creatinine level at 24, 48, 72 hr; 7 and 30 d following the transplant was not associated with the administration of aprotinin. CONCLUSIONS: There was no statistically significant difference in the incidence of the primary endpoint between groups in the study. Excess renal failure related to aprotinin administration in a patient population at high risk for the event was not observed.

The effectiveness of the "open chest" for the unstable patient after bilateral sequential lung transplantation.

Respiratory or hemodynamic instability after lung transplantation is one of the most challenging experiences a lung transplant surgeon and anesthesiologist face. We used an "open chest" approach as a feasible modality for managing these difficult patients. In this single-institution review of patients managed with this technique, we report the criteria for consideration, technical aspects, and overall effectiveness of this peri-operative strategy.

Obstruction of lateral tunnel fontan by an adjustable occluder device.

Innovative ways of adjusting Fontan fenestrations have been developed over the last two decades. Long-term complications have not been described. We successfully treated one such complication of Fontan conduit obstruction due to an adjustable occluder device.