Pre- and Peri-Operative Factors Associated with Chronic Critical Illness in Liver Transplant Recipients.

Background: Chronic critical illness (CCI) is a new and increasing entity that accounts for substantial cost despite its low incidence. We hypothesized that patients with end-stage liver failure undergoing liver transplant would be at high risk for developing CCI. With limited liver donors it is essential to understand pre- and peritransplant predictors of CCI. Methods: To accomplish this we performed a retrospective cohort study at a large academic transplant center of all adult liver transplant patients from 2011 to 2017. We defined CCI as the need for mechanical ventilation for seven days or more post-transplant. Recipients who had re-transplantation during their index admission, acute rejection, or who died during transplant surgery were excluded. Logistic regression was performed using the Akaike information criterion (AIC) and the likelihood ratio test. Results: We identified 382 transplant recipients. Forty-five (11.8%) developed CCI. Univariable analysis identified 16 pre-transplant factors associated with post-transplant CCI. Subsequent multivariable logistic regression identified eight independent factors associated with CCI in liver transplant recipients including previous liver transplant, acute renal failure, frailty, lower albumin level, higher international normalized ratio, need for mechanical ventilation, and higher systolic pulmonary artery pressure. Pre-transplant factors associated with protection against CCI included higher Model for End-Stage Liver Disease (MELD) score. Conclusion: The incidence of CCI post-liver transplant is similar to the general population admitted to the intensive care unit. Pre-transplant factors associated with CCI can help identify at-risk patients, and furthermore, promote further research and interventions with the goal to decrease the incidence of CCI in the liver transplant recipients.

Polyp Sizing Poster Improves Polyp Measurement but not Adenoma Detection Rates by Endoscopists in a Large Community Practice.

BACKGROUND & AIMS: Accurate sizing of polyps and improving adenoma detection rates (ADR) are important goals for high-quality colonoscopy. Surveillance intervals are based on accurate sizing of polyps. There are no clinical tools or interventions that have demonstrated improvement in both these metrics. We investigated the efficacy of a simple, low-cost intervention, based on use of polyp sizing posters to improve measurements of polyps and increase ADRs during colonoscopy at a large gastroenterology community practice. METHODS: We collected data on polyp measurements and ADRs by 62 gastrointestinal endoscopists at a large multi-site community practice, from January to November 2015 (baseline). In a prospective study, endoscopy units were given a polyp sizing poster to be hung above the endoscopy video monitor (intervention group, for 33 endoscopists) or for usual care (control group, for 29 endoscopists) in December 2015, and we collected data on polyp measurements and ADRs over the following 6 months (January-June 2016). We compared the endoscopists' assessment of polyp size and their ADRs before and after the intervention using a mixed effects proportional odds model, controlling for provider age and sex and patient and indication for colonoscopy. Our primary aim was to assess the effect of the snare and forcep-based polyp sizing poster on change in polyp size. The secondary aim was to study the effect of the polyp sizing poster on ADR. RESULTS: Our final analysis included 85,657 polyps from 38,307 colonoscopies. The characteristics of patients who underwent colonoscopy were similar between the control and intervention group (median age, 61 years; 48.1% female; 53.9% undergoing screening; 31.4% undergoing surveillance; 14.7% receiving a diagnostic colonoscopy). The endoscopists' median age was 51 years (range, 33-76) years, and 15 were women (24.2%). During the baseline period, male endoscopists were more likely to size polyps larger than measurements made by female endoscopists (odds ratio [OR], 1.78; 95% CI, 1.24-2.55; P = .002). For the intervention group, 78.6% of polyps were assigned to the 1-5 mm category during the baseline period compared to 76.0% after the intervention, whereas the proportions of polyps assigned to the 6-10 mm category increased from 16.9% during the baseline period to 18.3% after the intervention. In the control group, 78.9% of polyps were assigned to the 1-5 mm category during the baseline period and 78.3% were assigned to this group in the prospective study; 16.5% of polyps were assigned to the 6-10 mm during the baseline period and 17.5% were assigned to this group in the prospective study. The interaction between intervention group and timing (baseline vs after the intervention) was statistically significant, with an increase in the odds of larger polyp sizing after the intervention (OR, 1.15; 95% CI, 1.08-1.23; P < .001). The odds of larger polyp measurement during the intervention period, compared to the baseline period, increased for male endoscopists (OR, 1.17; 95% CI, 1.09-1.27; P < .001) and female endoscopists (OR, 1.18; 95% CI, 1.01-1.36; P = .04), as well as for younger physicians (<50 years; OR, 1.32; 95% CI, 1.20-1.46; P < .001) but not for older physicians (>50 years; OR, 0.96; 95% CI, 0.88-1.06; P = .44). The average ADR for male and female endoscopists combined during the baseline period was 42%. The change in ADR from the baseline vs the post-intervention was an increase of 2.6% in the control group compared to 5.7% in the intervention group (P = .39) CONCLUSIONS: Placement of a polyp sizing poster above the endoscopy video monitor increases the odds of polyps being assigned a larger size but does not affect ADRs.

Mortality Following Pediatric Congenital Heart Surgery: An Analysis of the Causes of Death Derived From the National Death Index.

Background Prior research has focused on early outcomes after congenital heart surgery, but less is known about later risks. We aimed to determine the late causes of death among children (<21 years of age) surviving their initial congenital heart surgery. Methods and Results This is a retrospective cohort study from the Pediatric Cardiac Care Consortium, a US-based registry of interventions for congenital heart defects (CHD). Excluding patients with chromosomal anomalies or inadequate identifiers, we matched those surviving their first congenital heart surgery (1982-2003) against the National Death Index through 2014. Causes of death were obtained from the National Death Index to calculate cause-specific standardized mortality ratios (SMRs). Among 31 132 patients, 2527 deaths (8.1%) occurred over a median follow-up period of 18 years. Causes of death varied by time after surgery and severity of CHD but, overall, 69.9% of deaths were attributed to the CHD or another cardiovascular disorder, with a SMR for CHD/cardiovascular disorder of 67.7 (95% confidence interval: 64.5-70.8). Adjusted odds ratios revealed increased risk of death from CHD/cardiovascular disorder in females [odds ratio=1.28; 95% confidence interval (1.04-1.58); P=0.018] with leading cardiovascular disorder contributing to death being cardiac arrest (16.8%), heart failure (14.8%), and arrhythmias (9.1%). Other major causes of death included coexisting congenital malformations (4.7%, SMR: 7.0), respiratory diseases (3.6%, SMR: 8.2), infections (3.4%, SMR: 8.2), and neoplasms (2.1%, SMR: 1.9). Conclusions Survivors of congenital heart surgery face long-term risks of premature mortality mostly related to residual CHD pathology, heart failure, and arrhythmias, but also to other noncardiac conditions. Ongoing monitoring is warranted to identify target factors to address residual morbidities and improve long-term outcomes.

Decreased Pseudomonas aeruginosa detection in children after separation of pediatric from adult cystic fibrosis clinics: A single center experience.

BACKGROUND: The impact of separating the adult from pediatric patients on Pseudomonas aeruginosa (P. aeriginosa) detection in the respiratory cultures of patients was examined at the University of Minnesota CF Center. METHODS: This study was a retrospective review using data recorded in the University of Minnesota CF Database between 1995 and 2010. Respiratory culture results obtained during routine University of Minnesota Cystic Fibrosis (CF) Center. CF clinic encounters of two cohorts of pediatric and adult CF patients (pre- and post-separation) were analyzed for presence of P. aeruginosa. RESULTS: The odds of a pediatric patient having P. aeruginosa were significantly less if the first culture was obtained after separation of pediatric and adult clinics. Being diagnosed by newborn screening or introduction of inhaled tobramycin did not affect this outcome. This reduction in P. aeruginosa was not detected in the adult cohort. CONCLUSIONS: Separation of pediatric and adult CF clinics has contributed to decrease in P. aeruginosa detection in pediatric patients.

Barriers in Hepatitis C Treatment in Somali Patients in the Direct Acting Antiviral Therapy Era.

BACKGROUND AND AIMS: Hepatitis C virus (HCV) treatment has changed dramatically in the last few years. Our observations suggest that a minority of HCV infected Somalis are treated. In this study, we aimed to evaluate for treatment and health outcome disparities between Somali and non-Somali patients during the direct acting antiviral (DAA) era. METHODS: Patients with HCV seen in the gastroenterology clinic in 2015 were included in the study. Patients were identified using ICD9 and 10 codes. Electronic medical records were analyzed to evaluate for treatment candidacy, acceptance and reasons for refusal of treatment. RESULTS: Genotype 4 followed by 3 were the most common genotypes in the Somalis while genotype 1 was the most common in the non-Somalis. Majority of patients were offered treatment, active alcohol and substance abuse was a common reason for not offering treatment in non-Somalis while the presence of hepatocellular carcinoma was the most common reason in Somalis. Somalis had higher rates of declining treatment given the asymptomatic nature of their disease and the feeling that treatment is not needed. Sustained virologic response rates were comparable in both groups. CONCLUSIONS: Disparities in acceptance of HCV treatment persist in the DAA era. The asymptomatic nature of the infection and potential cultural mistrust makes patients hesitant to undergo treatment. Healthcare providers must find interventions aimed at reducing barriers to treatment and increasing acceptance of HCV treatment.

Randomized controlled trial of a self-administered five-day antiseptic bundle versus usual disinfectant soap showers for preoperative eradication of Staphylococcus aureus colonization.

OBJECTIVE: To determine the efficacy in eradicating Staphylococcus aureus (SA) carriage of a 5-day preoperative decolonization bundle compared to 2 disinfectant soap showers, with both regimens self-administered at home. DESIGN: Open label, single-center, randomized clinical trial. SETTING: Ambulatory orthopedic, urologic, neurologic, colorectal, cardiovascular, and general surgery clinics at a tertiary-care referral center in the United States.ParticipantsPatients at the University of Minnesota Medical Center planning to have elective surgery and not on antibiotics. METHODS: Consenting participants were screened for SA colonization using nasal, throat, axillary, and perianal swab cultures. Carriers of SA were randomized, stratified by methicillin resistance status, to a decolonization bundle group (5 days of nasal mupirocin, chlorhexidine gluconate [CHG] bathing, and CHG mouthwash) or control group (2 preoperative showers with antiseptic soap). Colonization status was reassessed preoperatively. The primary endpoint was absence of SA at all 4 screened body sites. RESULTS: Of 427 participants screened between August 31, 2011, and August 9, 2016, 127 participants (29.7%) were SA carriers. Of these, 121 were randomized and 110 were eligible for efficacy analysis (57 decolonization bundle group, 53 control group). Overall, 90% of evaluable participants had methicillin-susceptible SA strains. Eradication of SA at all body sites was achieved for 41 of 57 participants (71.9%) in the decolonization bundle group and for 13 of 53 participants (24.5%) in the control group, a difference of 47.4% (95% confidence interval [CI], 29.1%-65.7%; P<.0001). CONCLUSION: An outpatient preoperative antiseptic decolonization bundle aimed at 4 body sites was significantly more effective in eradicating SA than the usual disinfectant showers (ie, the control).Trial RegistrationClinicalTrials.gov identifier: NCT02182115.

Trends in Long-Term Mortality After Congenital Heart Surgery.

BACKGROUND: Congenital heart surgery has improved the survival of patients with even the most complex defects, but the long-term survival after these procedures has not been fully described. OBJECTIVES: The purpose of this study was to evaluate the long-term survival of patients (age <21 years) who were operated on for congenital heart defects (CHDs). METHODS: This study used the Pediatric Cardiac Care Consortium data, a U.S.-based, multicenter registry of pediatric cardiac surgery. Survival analysis included 35,998 patients who survived their first congenital heart surgery at <21 years of age and had adequate identifiers for linkage with the National Death Index through 2014. Survival was compared to that in the general population using standardized mortality ratios (SMRs). RESULTS: After a median follow-up of 18 years (645,806 person-years), 3,191 deaths occurred with an overall SMR of 8.3 (95% confidence interval [CI]: 8.0 to 8.7). The 15-year SMR decreased from 12.7 (95% CI: 11.9 to 13.6) in the early era (1982 to 1992) to 10.0 (95% CI: 9.3 to 10.8) in the late era (1998 to 2003). The SMR remained elevated even for mild forms of CHD such as patent ductus arteriosus (SMR 4.5) and atrial septal defects (SMR 4.9). The largest decreases in SMR occurred for patients with transposition of great arteries (early: 11.0 vs. late: 3.8; p < 0.05), complete atrioventricular canal (31.3 vs. 15.3; p < 0.05), and single ventricle (53.7 vs. 31.3; p < 0.05). CONCLUSIONS: In this large U.S. cohort, long-term mortality after congenital heart surgery was elevated across all forms of CHD. Survival has improved over time, particularly for severe defects with significant changes in their management strategy, but still lags behind the general population.

Outcomes after surgical coronary artery revascularisation in children with congenital heart disease.

OBJECTIVE: Surgical coronary revascularisation in children with congenital heart disease (CHD) is a rare event for which limited information is available. In this study, we review the indications and outcomes of surgical coronary revascularisation from the Pediatric Cardiac Care Consortium, a large US-based multicentre registry of interventions for CHD. METHODS: This is a retrospective cohort study of children (<18 years old) with CHD who underwent surgical coronary revascularisation between 1982 and 2011. In-hospital mortality and graft patency data were obtained from the registry. Long-term transplant-free survival through 2014 was achieved for patients with adequate identifiers via linkage with the US National Death Index and the Organ Procurement and Transplantation Network. RESULTS: Coronary revascularisation was accomplished by bypass grafting (n=72, median age 6.8 years, range 3 days-17.4 years) or other operations (n=65, median age 2.6 years, range 5 days-16.7 years) in 137 patients. Most revascularisations were related to the aortic root (61.3%) or coronary anomalies (27.7%), but 10.9% of them were unrelated to either of them. Twenty in-hospital deaths occurred, 70% of them after urgent 'rescue' revascularisation in association with another operation. Long-term outcomes were available by external linkage for 54 patients surviving to hospital discharge (median follow-up time 15.0 years, max follow-up 29.8 years) with a 15-year transplant-free survival of 91% (95% CI 83% to 99%). CONCLUSIONS: Surgical coronary revascularisation can be performed in children with CHD with acceptable immediate and long-term survival. Outcomes are dependent on indication, with the highest mortality in rescue procedures.

Long-Term Transplant-Free Survival After Repair of Total Anomalous Pulmonary Venous Connection.

BACKGROUND: Long-term survival, risk of transplantation, and causes of death after repair of total anomalous pulmonary venous connection (TAPVC) remain unknown. By linking the Pediatric Cardiac Care Consortium with the National Death Index and the United Network for Organ Sharing, we evaluated long-term transplant-free survival in children undergoing repair of TAPVC. METHODS: We identified 777 infants within the Pediatric Cardiac Care Consortium who underwent TAPVC repair (median 21 days; interquartile range, 5 to 80) and had sufficient personal identifiers for linkage with the National Death Index and United Network for Organ Sharing. Sixty-six deaths, ten cardiac transplantations, and one bilateral lung transplantation had occurred by the end of 2014. Data collected included age and weight at time of procedure, TAPVC type, associated cardiac lesions, and postoperative length of stay. The study cohort was divided into simple and complex TAPVC based on the presence of an associated cardiac lesion. Parametric survival plots were constructed, and risk factor analyses were performed to identify demographic and clinical characteristics associated with long-term outcomes. RESULTS: Mortality or need for transplantation was 9.7% with a median follow-up of 18.4 years and a median age of death or transplant of 0.74 years. The risk of mortality and transplant after TAPVC repair was highest during the first 18 months after hospital discharge. Cardiac causes accounted for the majority of deaths. Multivariate regression models for transplant-free survival demonstrated that complex TAPVC, mixed TAPVC, and postoperative length of stay were associated with increased risk of death/transplant. CONCLUSIONS: Transplant-free survival after TAPVC repair is excellent, with most deaths or transplant events occurring early. Factors associated with the worst long-term outcomes included complex TAPVC, mixed TAPVC, and prolonged postoperative length of stay.

BMI Is a Risk Factor for Colorectal Cancer Mortality.

BACKGROUND: The relationship between dietary and lifestyle risk factors and long-term mortality from colorectal cancer is poorly understood. Several factors, such as obesity, intakes of red meat, and use of aspirin, have been reported to be associated with risk of colorectal cancer mortality, though these findings have not been replicated in all studies to date. METHODS: In the Minnesota Colon Cancer Control Study, 46,551 participants 50-80 years old were randomly assigned to usual care (control) or annual or biennial screening by fecal occult blood testing. Colon cancer mortality was assessed after 30 years of follow-up. Dietary intake and lifestyle risk factors were assessed by questionnaire at baseline. RESULTS: Age [hazard ratio (HR) 1.09; 95% CI 1.07, -1.11], male sex (HR 1.25; 95% CI 1.01, 1.57), and higher body mass index (BMI) (HR 1.03; 95% CI 1.00-1.05) increased the risk of CRC mortality, while undergoing screening for CRC was associated with a reduced risk of colorectal cancer mortality (HR 0.76; 95% CI 0.61-0.94 and 0.67; 95% CI 0.53-0.83 for biennial and annual screening, respectively). Intakes of grains, meats, proteins, coffee, alcohol, aspirin, fiber, fruits, and vegetables were not associated with colorectal cancer mortality. CONCLUSIONS: Our study confirms the relationship between BMI and long-term colorectal cancer mortality. Modulation of BMI may reduce risk of CRC mortality.

Management of Risks From Water and Ice From Ice Machines for the Very Immunocompromised Host: A Process Improvement Project Prompted by an Outbreak of Rapidly Growing Mycobacteria on a Pediatric Hematopoietic Stem Cell Transplant (Hsct) Unit.

BACKGROUND In 2011, pediatric hematopoietic stem cell transplant (HSCT) patients were moved from an older hospital to a new children's hospital. To minimize bacterial growth in the new hospital's water during construction, the plumbing system was flushed and disinfected before occupancy. However, 6 months after occupancy, an increased incidence of rapidly growing mycobacteria (RGM) was detected in clinical cultures. Over 10 months, 15 pediatric HSCT patients were infected, while no pediatric HSCT patients had been infected in the preceding 12 months. OBJECTIVE To determine the cause of the outbreak and to interrupt patient acquisition of RGM. METHODS Water samples were collected from water entering the hospital and from drinking water and ice machines (DWIMs) from the old and new hospitals. Total heterotrophic plate counts (HPCs, CFU/mL) of water were undertaken, and select isolates were identified as RGM. RESULTS The cause of the outbreak was increased bacterial levels in the water (including RGM) in the DWIMs in the new (2011) hospital. Tests revealed higher HPCs in drinking water and ice from the DWIMs in the new hospital than in the DWIMs in the old hospital. Ultimately, HPCs were reduced by several different interventions. CONCLUSION In response to an RGM outbreak, HSCT patients were banned from ingesting DWIM ice and water and bottled water was provided. Since this interverntion 4 years ago, no additional RGM isolates have been identified in HSCT patient cultures. Our measures to reduce HPCs to goal levels in drinking water from DWIMs were successful, but the HPCs for ice have not consistently reached the goal of <500 CFU/mL. Infect Control Hosp Epidemiol 2017;38:792-800.

Objective Measurement of Adherence to Out-Patient Airway Clearance Therapy by High-Frequency Chest Wall Compression in Cystic Fibrosis.

BACKGROUND: Objective measures of adherence to high-frequency chest wall compression (HFCWC), a form of airway clearance therapy for patients with cystic fibrosis, are lacking. We used a novel electronic monitoring device integrated into an HFCWC vest to measure adherence compared with self-reported adherence. We determined factors that influenced adherence and how adherence correlated with baseline pulmonary function and pulmonary exacerbations. METHODS: Data were collected by direct measurement of date, time of day, and duration of HFCWC use to determine the number of daily treatments and daily duration of treatments. Chart review provided prescribed airway clearance therapy treatment and demographic and clinical information. Subject and caregiver report of the daily number of airway clearance therapy treatments was obtained by telephone interviews. Analysis used 2-sample and paired t test, analysis of variance, and linear regression. RESULTS: Average adherence was 69%. Adherence was highest in children (82%, P = .02) and those receiving assistance with treatment (82%, P < .001). Subjects overestimated therapy duration from a mean ± SD of 127 ± 169% by adults to 19.2 ± 26.3% by parents or guardians of children. Average adherence decreased with increasing prescribed therapy time (P = .02). Average daily therapy time and adherence had significant positive associations with baseline FEV1 percent of predicted (P = .02 and P = .02, respectively) and negative associations with pulmonary exacerbations during the pre-study period and at baseline (P = .044 and P = .02, respectively). CONCLUSIONS: Greater adherence to HFCWC measured directly by a novel recorder was associated with better baseline pulmonary function and fewer exacerbations in the pre-study and baseline period. Adherence decreased with age and prescribed therapy time and increased with therapy assistance. Self-report overestimation is large and thus not an accurate measure of adherence.

Characterization of Mast Cell Activation Syndrome.

BACKGROUND: Mast cell activation syndrome (MCAS), a recently recognized nonneoplastic mast cell disease driving chronic multisystem inflammation and allergy, appears prevalent and thus important. We report the first systematic characterization of a large MCAS population. METHOD: Demographics, comorbidities, symptoms, family histories, physical examination and laboratory findings were reviewed in 298 retrospective and 115 prospective patients with MCAS. Blood samples from prospective subjects were examined by flow cytometry for clonal mast cell disease and tested for cytokines potentially driving the monocytosis frequent in MCAS. RESULTS: Demographically, white females dominated. Median ages at symptom onset and diagnosis were 9 and 49 years, respectively (range: 0-88 and 16-92, respectively) and median time from symptom onset to diagnosis was 30 years (range: 1-85). Median numbers of comorbidities, symptoms, and family medical issues were 11, 20, and 4, respectively (range: 1-66, 2-84, and 0-33, respectively). Gastroesophageal reflux, fatigue and dermatographism were the most common comorbidity, symptom and examination finding. Abnormalities in routine laboratories were common and diverse but typically modest. The most useful diagnostic markers were heparin, prostaglandin D2, histamine and chromogranin A. Flow cytometric and cytokine assessments were unhelpful. CONCLUSIONS: Our study highlights MCAS׳s morbidity burden and challenging heterogeneity. Recognition is important given good survival and treatment prospects.

Factors Affecting Length of Postoperative Hospitalization for Pediatric Cardiac Operations in a Large North American Registry (1982-2007).

Surgical treatment of congenital heart disease represents a major cause of pediatric hospitalization and healthcare resource use. Larger centers may provide more efficient care with resulting shorter length of postoperative hospitalization (LOH). Data from 46 centers over 25 years were used to evaluate whether surgical volume was an important determinant of LOH using a competing risk regression strategy that concurrently accounted for deaths, transfers, and discharges with some time interactions. Earlier discharge was more likely for infants and older children compared to neonates [subhazard ratios at postoperative day 6 of 1.64 (99 % confidence interval (CI) 1.57, 1.72) and 2.67 (99 % CI 2.53, 2.80), respectively], but less likely for patients undergoing operations in Risk Adjustment for Congenital Heart Surgery categories 2, 3, 4, and 5/6 compared to category 1 [subhazard ratios at postoperative day 6 of 0.66 (99 % CI 0.64, 0.68), 0.34 (95 % CI 0.33, 0.35), 0.28 (99 % CI 0.27, 0.30), and 0.10 (99 % CI 0.09, 0.11), respectively]. There was no difference by sex [non-time-dependent subhazard ratio 1.019 (99 % CI 0.995, 1.040)]. For every 100-operation increase in center annual surgical volume, the non-time-dependent subhazard for discharge was 1.035 (99 % CI 1.006, 1.064) times greater, and center-specific exponentiated random effects ranged from 0.70 to 1.42 with a variance of 0.023. The conditional discharge rate increased with increasing age and later era. No sex-specific difference was found. Centers performing more operations discharged patients sooner than lower volume centers, but this difference appears to be too small to be of clinical significance. Interestingly, unmeasured institutional characteristics estimated by the center random effects were variable, suggesting that these played an important role in LOH and merit further investigation.

Mortality and Operative Management for Patients Undergoing Repair of Coarctation of the Aorta: A Retrospective Review of the Pediatric Cardiac Care Consortium.

BACKGROUND: The surgical mortality associated with repair of coarctation of the aorta (CoA) over a 25-year period was examined. Risk factors for discharge mortality were evaluated as well as the surgical techniques and its evolution over the period studied. METHODS: Utilizing the pediatric cardiac care consortium, we conducted a retrospective review of patients less than 18 years of age submitted between 1982 and 2007. Variables reviewed included weight at birth, age and weight at the operation, type of coarctation repair, associated cardiac anomalies, year of repair, center-specific volume, postoperative length of stay, and in-hospital mortality. RESULTS: There were 7,860 patients submitted with the procedural code for repair of CoA. Forty-five percent underwent repair within 30 days of life (n = 3,549), including 1,444 patients who were operated upon within the first 7 days of life (18% of all patients). Seventy percent (n = 5,528) of patients had an isolated CoA (iCoA). The overall mortality for the entire group was 4.2% (n = 331), decreasing to 2.0% (n = 114) for iCoA (P < .0001). A hypoplastic aortic arch occurred in 4.6%, with a mortality of 10.6%. Coarctectomy with an end-to-end reconstruction was the most common procedure performed. Multivariable modeling for discharge mortality was significant for diagnosis of ventricular septal defect, operative weight, operative year, and diagnosis of aortic arch hypoplasia. CONCLUSIONS: Operative repair of CoA is accomplished with a low mortality, although certain subgroups have persistently inferior outcomes. The techniques utilized for aortic reconstruction have evolved, with coarctectomy and an end-to-end anastomosis becoming the dominant surgical procedures.

Global left ventricular relaxation: A novel tissue Doppler index of acute rejection in pediatric heart transplantation.

BACKGROUND: Serial invasive cardiac catheterization with endomyocardial biopsies (EMBs) is the current standard of reference for evaluation after orthotopic heart transplant (OHTx). We developed a novel, non-invasive echocardiographic index of global left ventricular relaxation (LVRI) and assessed its sensitivity, specificity and predictive value for detecting rejection compared with EMB results in a prospective study conducted from September 2012 through May 2014. METHODS: LVRI was calculated as the sum of diastolic tissue Doppler imaging (TDI) velocities (E') of the left ventricular lateral, septal and posterior walls divided by the percentage of left ventricular posterior wall (LVPW) thinning by M-mode. LVRI was measured in 47 OHTx patients and 50 patients with normal hearts. Of the 33 patients who underwent clinically indicated EMB, 22 patients had Grade 0R EMB, 6 had Grade 1R and 5 had Grade 2R to 3R biopsy results. Sensitivity, specificity and predictive value of LVRI for discriminating Grade 1R to 3R EMB were calculated. The LVRI was compared before and after OHTx rejection treatment and during the early and late post-transplant period. To characterize LVRI, 1-way analysis of variance was used to compare all groups, including non-OHTx patients. RESULTS: LVRI was lower in patients with Grade 0R EMBs compared with non-OHTx patients. Patients with Grade 1R to 3R EMBs had lower LVRI than those with Grade 0R EMBs. LVRI recovered after treatment for rejection. LVRI appeared to normalize between 40 and 90 days post-transplantation. After 90 days, sensitivity was 100% and specificity was 90.9% for detecting patients with Grade 1R to 3R EMBs using an LVRI of 0.8. CONCLUSION: LVRI, a novel, non-invasive TDI index of global left ventricular diastolic dysfunction, appears to be useful for detecting rejection in children beyond 3 months post-OHTx.

Management of an associated ventricular septal defect at the time of coarctation repair.

BACKGROUND: Management of a ventricular septal defect (VSD) at time of coarctation of the aorta (CoA) repair remains controversial, with recent studies advocating concomitant repair of both defects. We evaluated the surgical management and mortality for patients undergoing CoA repair associated with a VSD. METHODS: We retrospectively reviewed data submitted to the Pediatric Cardiac Care Consortium of patients undergoing repair of CoA from 1982 to 2007. The cohort was divided into three groups: CoA repair plus VSD closure (group 1); CoA repair plus pulmonary artery band (group 2); and CoA repair without repair of VSD (group 3). Variables reviewed included era, age, and weight at repair, and in-hospital mortality. RESULTS: There were 7,860 patients who underwent repair of CoA, of whom 2,022 had an associated VSD (25.7%). Mortality after CoA repair with and without an associated diagnosis of VSD was 8.3% versus 2.1% (p < 0.001). Mean age at repair for group 1 (n = 286) and group 2 (n = 472) was 87.4 days and 21.6 days, respectively (p = 0.004), and median weight was 3.31 kg and 3.30 kg, respectively (p = 0.130). Discharge mortality for group 1 and group 2 was similar, at 8.7% and 9.1%, respectively (p = 0.852). Patients with CoA/VSD who had neither VSD closure nor pulmonary artery banding (group 3) had a hospital mortality of 7.9%. CONCLUSIONS: The association of CoA and VSD is common. A strategy of concomitant VSD closure at CoA repair does not result in worse discharge mortality when compared with pulmonary banding with anticipated staged repair of the VSD. These outcomes support continued evaluation of a one-stage approach.

Age-dependent sex effects on outcomes after pediatric cardiac surgery.

BACKGROUND: Sex has been linked to differential outcomes for cardiovascular disease in adults. We examined potential sex differences in outcomes after pediatric cardiac surgery. METHODS AND RESULTS: We retrospectively analyzed data from the Pediatric Cardiac Care Consortium (1982-2007) by using logistic regression to evaluate the effects of sex on 30-day within-hospital mortality after pediatric (<18 years old) cardiac operations and its interaction with age, risk category, z-score for weight, and surgical year for the whole cohort. Of 76 312 operations, 55% were in boys. Unadjusted mortality was similar for boys and girls (5.2% versus 5.0%, P=0.313), but boys were more likely to have cardiac surgery as a neonate and to have more complex operations. After adjustment, the overall test of any association between postsurgical mortality and sex was significant (P=0.002), but the overall test of any interaction was not (P=0.503). However, a potential age-dependent sex effect on postsurgical mortality was observed among infants subjected to high-risk operations, with girls doing worse during the first 6 months of life. CONCLUSIONS: Patient sex has a significant effect on mortality after pediatric cardiac operations, with an increased risk of death in early infancy for girls after high-risk cardiac operations. This age-dependent relationship supports a sex-related biological effect on postoperative cardiovascular stress.

Primed low-frequency repetitive transcranial magnetic stimulation and constraint-induced movement therapy in pediatric hemiparesis: a randomized controlled trial.

AIM: The aim of this study was to determine the feasibility and efficacy of five treatments of 6 Hz primed, low-frequency, repetitive transcranial magnetic stimulation (rTMS) combined with constraint-induced movement therapy (CIMT) to promote recovery of the paretic hand in children with congenital hemiparesis. METHOD: Nineteen children with congenital hemiparesis aged between 8 and 17 years (10 males, nine females; mean age 10 years 10 months, SD 2 years 10 months; Manual Ability Classification Scale levels I-III) underwent five sessions of either real rTMS (n=10) or sham rTMS (n=9) alternated daily with CIMT. CIMT consisted of 13 days of continuous long-arm casting with five skin-check sessions. Each child received a total of 10 hours of one-to-one therapy. The primary outcome measure was the Assisting Hand Assessment (AHA) and the secondary outcome variables were the Canadian Occupational Performance Measure (COPM) and stereognosis. A Wilcoxon signed-rank sum test was used to analyze differences between pre- and post-test scores within the groups. Analysis of covariance was used to compute mean differences between groups adjusting for baseline. Fisher's exact test was used to compare individual change in AHA raw scores with the smallest detectable difference (SDD) of 4 points. RESULTS: All participants receiving treatment finished the study. Improvement in AHA differed significantly between groups (p=0.007). No significant differences in the secondary outcome measures were found. Eight out of 10 participants in the rTMS/CIMT group showed improvement greater than the SDD, but only two out of nine in the sham rTMS/CIMT group showed such improvement (p=0.023). No serious adverse events occurred. INTERPRETATION: Primed, low-frequency rTMS combined with CIMT appears to be safe, feasible, and efficacious in pediatric hemiparesis. Larger clinical trials are now indicated.

Frequency of superior vena cava obstruction in pediatric heart transplant recipients and its relation to previous superior cavopulmonary anastomosis.

The risk factors for superior vena cava (SVC) obstruction after pediatric orthotopic heart transplantation (OHT) have not been identified. This study tested the hypothesis that pretransplant superior cavopulmonary anastomosis (CPA) predisposes patients to SVC obstruction. A retrospective review of the Pediatric Cardiac Care Consortium registry from 1982 through 2007 was performed. Previous CPA, other cardiac surgeries, gender, age at transplantation, and weight at transplantation were assessed for the risk of developing SVC obstruction. Death, subsequent OHT, or reoperation involving the SVC were treated as competing risks. Of the 894 pediatric OHT patients identified, 3.1% (n = 28) developed SVC obstruction during median follow-up of 1.0 year (range: 0 to 19.5 years). Among patients who developed SVC obstruction, 32% (n = 9) had pretransplant CPA. SVC surgery before OHT was associated with posttransplant development of SVC obstruction (p <0.001) after adjustment for gender, age, and weight at OHT and year of OHT. Patients with previous CPA had increased risk for SVC obstruction compared with patients with no history of previous cardiac surgery (hazard ratio 10.6, 95% confidence interval: 3.5 to 31.7) and to patients with history of non-CPA cardiac surgery (hazard ratio 4.7, 95% confidence interval: 1.8 to 12.5). In conclusion, previous CPA is a significant risk factor for the development of post-heart transplant SVC obstruction.