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Objective: The objective was to evaluate and improve accuracy of anticipatory counseling regarding neonatal intervention for prenatally diagnosed tetralogy of Fallot (TOF) by assessing new and previously published predictors of neonatal intervention., Methods: This is a multi-center, retrospective study from three centers of 112 fetal TOF patients undergoing third trimester fetal echocardiograms from 2004 to 2017. Additional cardiac defects requiring neonatal intervention were excluded. Fetal echocardiographic, clinical, and consultation data were compared between neonatal and late intervention. Optimal echocardiographic values were determined., Results: Twenty-six infants (23%) required neonatal intervention. Those infants had significantly different pulmonary valve (PV) z-scores, PV:aortic valve (AoV) ratios, PV:AoV z-score differences (absolute difference between z-scores), and increased likelihood of abnormal ductal flow. Counseling during fetal echocardiogram regarding interventional timing was accurate for 50% needing neonatal intervention and 86% undergoing late intervention (P = .002). The best neonatal intervention predictors were PV:AoV ratio of <0.6 and counseling for neonatal intervention. PV:AoV z-score difference ≥5 provided 89% negative predictive value for excluding patients from neonatal repair., Conclusions: Third trimester fetal echocardiograms can predict interventional timing. The best predictors of neonatal intervention are PV:AoV ratio <0.6, PV:AoV z-score difference ≥5, and cardiologist counseling that neonatal intervention was likely., (© 2020 John Wiley & Sons, Ltd.)

Objectives: To examine changes in transition readiness (knowledge, self-efficacy, self-management) over time and explore factors associated with transition readiness, including psychosocial quality of life (QOL) and health service utilization in teens/young adults with congenital heart disease., Study Design: In a multicenter prospective cohort study, 356 patients, age 14-27 years, completed transition readiness and QOL assessments at routine cardiology visits at baseline and 1-year follow-up., Results: Median patient age was 19.8 years at 1.03 years (IQR 0.98-1.24) following baseline transition readiness assessment. Average knowledge deficit scores decreased at follow-up (P < .0001) and self-efficacy scores increased (P < .0001). Self-management scores increased (P < .0001), but remained low (mean 57.7, 100-point scale). Information was requested by 73% of patients at baseline and was associated with greater increase in knowledge at follow-up (P = .005). Increased knowledge (P = .003) and perceived self-efficacy (P = .01) were associated with improved psychosocial QOL, but not health service utilization at follow-up. Patients who preferred face-to-face information from healthcare providers (47%) vs other information sources were more likely to request information (P < .0001). In patients <18 years old, greater agreement between teen and parental perception of teen's knowledge was associated with greater increase in patient knowledge (P = .02) and self-efficacy (P = .003)., Conclusion: Transition readiness assessment demonstrated improved knowledge, self-efficacy, and self-management at 1-year follow-up in teens/young adults with congenital heart disease. Improved knowledge and self-efficacy were associated with improved psychosocial QOL. Self-management remained low. Supplemental media for conveying information and greater involvement of parents may be needed to optimize transition readiness., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Both cardiac magnetic resonance (CMR) and cardiac catheterization (cath) may assess patients with single ventricle physiology prior to stage II or Fontan palliation. However, development of significant aortopulmonary collaterals may invalidate assumptions of the Fick method. We compared CMR and cath flow measurements and evaluated the relation to collateral flow. This single-center study included all pre-stage II and pre-Fontan patients between 2010 and 2017 with CMR and cath within 1 month. Pulmonary (Qp) and systemic flow (Qs) by cath were calculated by Fick method. CMR Qp was calculated by total pulmonary venous flow, and Qs by total vena caval flow. Collateral flow by CMR was the difference of pulmonary vein and pulmonary artery flow. In 26 studies (16 pre-stage II and 10 pre-Fontan) in 21 patients, collateral flow was higher in pre-Fontan patients (1.8 ± 0.6 vs 0.9 ± 0.8 L/min/m 2 , p = 0.01). Overall, CMR and cath had good agreement for Qs and Qp:Qs, with moderate correlation (r = 0.44, p = 0.02 for Qs, r = 0.48, p = 0.02 for Qp:Qs). In pre-Fontan but not in pre-stage II patients, CMR had higher Qp (mean difference - 1.71 L/min/m 2 ) and Qp:Qs (mean difference - 0.36). The underestimation of cath Qp correlated with amount of collateral flow (r = - 0.47, p = 0.02). Neither cath nor CMR flow measurements correlated with outcomes in this small cohort. In conclusion, collaterals lead to systematically higher Qp and Qp:Qs measurements by CMR vs cath in single ventricle patients. Measurements may not be used interchangeably, with potential clinical significance in estimating pulmonary vascular resistance. Further study is necessary to evaluate possible relation to clinical outcomes.

In repaired tetralogy of Fallot (rTOF), pulmonary insufficiency results in varying degrees of right ventricle (RV) dilation. A subset of patients is diagnosed at initial cardiac magnetic resonance imaging (CMR) with a massively dilated RV, far beyond pulmonary valve replacement (PVR) criteria, which is unlikely to return to normal size after PVR. This study aimed to identify risk factors for massive RV dilation at initial CMR. This nested case-control study included all patients at our institution with rTOF and massive RV dilation (indexed RV end-diastolic volume [RVEDVi] ≥200 ml/m 2 ) on initial CMR. Patients were matched by age at first CMR, gender, and type of repair with rTOF controls with RVEDVi<200 ml/m 2 . In 39 cases (median RVEDVi 227 ml/m 2 , interquartile range [IQR] 213 to 250) and 73 controls (median RVEDVi 155 ml/m 2 , IQR 130 to 169), repair at >6 months of age, longer QRS duration, and non-Caucasian race were significantly associated with massive RV dilation on univariate analysis. In multivariate analysis, repair at >6 months of age (adjusted odds ratio [AOR] 2.90, 95% confidence interval [CI] 1.12 to 7.55, p = 0.03), longer QRS duration (AOR = 1.03, 95% CI 1.01 to 1.05, p = 0.005), and non-Caucasian race (AOR = 7.84, 95% CI 1.76 to 34.8, p = 0.01) remained independently associated with massive RV dilation. Era of repair, history of systemic to pulmonary shunt palliation, genetic anomaly, and additional cardiac lesions did not differ between groups. In conclusion, these risk factors identify a subset of patients who may benefit from earlier CMR evaluation to avoid massive irreversible RV dilation., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Adolescents and young adults undergoing heart transplantation experience risks of morbidity and mortality both pre- and post-transplant. To improve end-of-life care for this population, it is necessary to understand their medical and end-of-life decision-making preferences., Aim: (1) To examine adolescent/young adult decision-making involvement specific to heart transplant listing, and (2) to characterize their preferences specific to medical and end-of-life decision making., Design: This cross-sectional research study utilized survey methods. Data were collected from October 2016 to March 2018., Setting/participants: Twelve adolescent and young adult patients listed for heart transplant (ages = 12-19 years) and one parent for each were enrolled at a single-center, US children's hospital., Results: Consistent with their preferences, the majority of adolescent/young adult participants (82%) perceived a high level of involvement in the decision to be listed for transplant. Patient involvement in this decision was primarily by way of seeking advice or information from their parents and being asked to express their opinion from parents. Despite a preference among patients to discuss their prognosis and be involved in end-of-life decision making if seriously ill, only 42% of patients had discussed their end-of-life wishes with anyone. Few parents recounted having such discussions. Preferences regarding the timing and nature of end-of-life decision-making discussions varied., Conclusions: Although young people are involved in the decision to pursue heart transplantation, little attention is paid to involving them in discussions regarding end-of-life decision making in a manner that is consistent with individual preferences.

Production of adenosine in the extracellular tumor microenvironment elicits strong immunosuppression and is associated with tumor progression. Thus, targeting adenosine-generating ectonucleotidases is a potential strategy to stimulate and prolong antitumor immunity. Because the reaction products of ectonucleotidases differ by a single phosphate group, selective detection in an assay format that is compatible with high-throughput screening (HTS) has been elusive. We report the development of biochemical assays capable of measuring the activity of ectonucleoside triphosphate diphosphohydrolase-1 (ENTPD1; also known as CD39) and ecto-5'-nucleotidase (CD73). Both assays leverage the Transcreener HTS Assay platform, which facilitates selective immunodetection of nucleotides with homogenous fluorescent readouts, fluorescence polarization or time-resolved fluorescence energy transfer. The Transcreener AMP2 Assay was used to measure CD39 activity, allowing detection of adenosine monophosphate (AMP) production (Z' > 0.6) with subnanomolar amounts of CD39, allowing IC 50 determination for tool compounds, consistent with previously reported values. To detect the production of adenosine by CD73, the Transcreener ADP2 Assay was coupled with adenosine kinase (AK); conversion of adenosine to AMP and adenosine diphosphate (ADP) by AK allows detection with ADP2 antibody. The Transcreener AMP2 Assay was used to screen a 1280 Library of Pharmacologically Active Compounds (LOPAC) library and a 1600-compound subset of a ChemBridge diversity library for CD39 inhibitors, allowing the identification of nine and eight candidate compounds from each library, respectively. The Transcreener ADP2 Assay was used to screen 1600 compounds from the ChemBridge diversity library for CD73 inhibitors and identified 14 potential candidates. HTS-compatible assays for ectonucleotidase activity may allow identification of purinergic signaling pathway inhibitors important for tumor-specific immune responses during tumor pathogenesis.

Mental health concerns are associated with worse outcomes after adult heart transplant. Illness-specific anxiety is associated with worsened psychological well-being after other solid organ transplants but has never been characterized after pediatric heart transplant. This single-center cross-sectional study aimed to evaluate illness-specific and generalized anxiety after heart transplantation in adolescents. A novel 12-item PHTF, GAD-7, and the PedsQL were administered. Univariate associations of demographics, clinical features, and medication adherence as measured by immunosuppression standard deviation with the PHTF and GAD-7 scores were evaluated. Internal consistency and validity of the PHTF were examined. In total, 30 patients participated. The most common illness-specific fears were retransplantation, rejection, and more generally post-transplant complications. The PHTF had good internal consistency (Cronbach α = .88). Construct validity was demonstrated between PHTF and GAD-7 (r = .62) and PedsQL (r = -.54 to -.62). 23% endorsed moderate to severe generalized anxiety symptoms. More severe symptoms were associated with older age at survey (P = .03), older age at listing (P = .01) and having post-transplant complications (P = .004). Patients with moderate or severe symptoms were more likely to report late immunosuppression doses (P = .004). Illness-specific and generalized anxiety may be prevalent after pediatric heart transplant. Screening for anxiety in adolescents post-transplant may identify those at risk for adverse outcomes including non-adherence. The PHTF is a brief, valid, and reliable instrument identifying illness-specific anxiety in this population., (© 2019 Wiley Periodicals, Inc.)

Background: Although overall outcomes have improved for single-ventricle patients, substantial morbidity and mortality remain for certain high-risk groups. The hybrid stage I procedure is an alternative to the Norwood operation for stage I palliation, but it remains unclear whether it is associated with improved outcomes in high-risk patients., Methods: This single-center, nested, case-control study included high-risk patients with a systemic right ventricle who underwent hybrid stage I or Norwood palliation from January 2000 to December 2016. High-risk features included prematurity < 34 weeks, birth weight < 2.5 kg, restrictive/intact atrial septum, at least moderate atrioventricular valve regurgitation or right ventricular dysfunction, genetic or extracardiac anomalies, or left ventricular sinusoids. Patients were matched by presence of genetic anomaly, restrictive/intact atrial septum, and prematurity/weight < 2 kg. Early and midterm outcomes were compared in the matched hybrid vs Norwood groups., Results: The study included 96 patients (35 hybrid, 61 Norwood). Despite improved 30-day survival in hybrid patients (91% vs 66%, P < .01), 1-year survival was similar between the hybrid and Norwood groups (46% vs 48%, P = .9). No hybrid patients required dialysis or extracorporeal membrane oxygenation after stage I palliation as compared with 19% and 22% of Norwood patients, respectively (both P < .01). Hybrid patients, however, required more unplanned reinterventions (43% vs 21%, P = .02)., Conclusions: There remains significant morbidity and mortality among high-risk single-ventricle infants. Despite an early survival benefit, hybrid stage I palliation has not been associated with improved midterm outcomes at our center., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

We sought to assess acute hemodynamic changes after implementation of negative extrathoracic pressure (NEP) in spontaneously breathing ambulatory Fontan patients with symptomatic heart failure. We hypothesized that application of NEP would result in an acute decrease in pulmonary artery pressure. Ten patients with clinical evidence of Fontan failure underwent baseline hemodynamic catheterization while breathing spontaneously. Hemodynamic measurements were then repeated after 30 min of continuous NEP. After 30 min of continuous NEP, 4/10 patients had a decrease in their Fontan pressure by 2 mmHg and one patient had a decrease by 1 mmHg. There were three patients that had an increase in Fontan pressure by 2 mmHg. In 7/10 patients, indexed pulmonary vascular resistance decreased by an average of 31%. In symptomatic Fontan patients with a favorable hemodynamic response to NEP during catheterization, potential benefit of longer-term NEP to improve clinical status should be explored.

In patients with D-looped transposition of the great arteries (D-TGA) status post atrial switch operation, the systemic right ventricle (RV) shifts to predominantly circumferential (CS) rather than longitudinal strain (LS), which may represent adaptation or dysfunction. We aimed to evaluate myocardial mechanics in pressure loaded, volume-loaded, and normal RVs by cardiac magnetic resonance (CMR). Patients with D-TGA post atrial switch operation with CMR from 2008 to 2015 were matched 1:1 for age and RV ejection fraction (EF) with repaired tetralogy of Fallot (TOF) patients (volume-loaded RVs), and 1:1 for age with control patients. RV free wall LS and CS were measured using feature tracking software (TomTec, Unterscleissheim, Germany). A total of 32 D-TGA (median age 32 years, 56% male), 32 TOF, and 32 control patients were included. D-TGA patients had less dilatation than TOF patients (125 ± 35 ml/m 2 vs. 149 ± 44 ml/m 2 , p = 0.02) and lower RVEF than controls (42.9 ± 7.7% vs. 56.3 ± 5.6%, p < 0.0001). RV LS was similar in D-TGA and TOF ( - 13.2 ± 4.5% vs. - 14.5 ± 5.9%, p = 0.32), both decreased compared to controls. However, CS in D-TGA was higher than controls ( - 14.1 ± 4.1% vs. - 11.4 ± 4.4%, p = 0.01), with a higher CS:LS ratio (1.2 ± 0.7 vs. 0.6 ± 0.3, p < 0.0001), while CS in TOF and controls did not differ. RVEF in D-TGA correlated closely with CS (r =  - 0.85, p < 0.0001) but not LS (r = 0.10, p = 0.58). I n conclusion, CMR can differentiate strain patterns in pressure- and volume-loaded RVs, with decreased LS in both conditions, while systemic RVs compensate with supra-normal CS. CS may be a more clinically relevant measure of RV function in this population.

Objectives: We previously reported common knowledge deficits and lack of transition readiness in 13- 25-year-olds with congenital or acquired heart disease. The aims of this study were to re-evaluate transition readiness in this cohort at follow-up and to examine the relationship between changes in transition readiness and quality of life (QOL)., Study Design: In this prospective cohort study, patients completed the Transition Readiness Assessment and the Pediatric Quality of Life Inventory using an e-tablet, web-based format at a routine follow-up visit. Changes from initial to follow-up scores were evaluated., Results: Sixty-five percent of patients (106 of 164) completed follow-up assessments at a median age of 18.7 years (IQR, 16.5-21.2 years) at a median follow-up of 1 year. The average perceived knowledge deficit score (percent of items with no knowledge) at follow-up was 18.0 ± 15.2%, which decreased from 24.7 ± 16.5% (P < .0001). On a 100-point scale, the mean score for self-efficacy increased from 71.4 ± 17.0 to 76.7 ± 18.2 (P = .0004) and for self-management increased from 47.9 ± 18.4 to 52.0 ± 20.7 (P = .004). Although physical QOL did not change, the mean psychosocial QOL score increased significantly (P = .02). A decrease in the knowledge deficit score at follow-up was significantly associated with an increased psychosocial QOL score (P = .03). An increase in the self-efficacy score was associated with an increase in psychosocial QOL score (P = .04), especially social QOL (P = .02)., Conclusions: Although deficits in knowledge and self-management skills persist, transition readiness assessment and recognition of deficits can improve transition readiness with improved psychosocial QOL., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objectives: To assess the variation in timing of left atrial decompression and its association with clinical outcomes in pediatric patients supported with venoarterial extracorporeal membrane oxygenation across a multicenter cohort., Design: Multicenter retrospective study., Setting: Eleven pediatric hospitals within the United States., Patients: Patients less than 18 years on venoarterial extracorporeal membrane oxygenation who underwent left atrial decompression from 2004 to 2016., Interventions: None., Measurements and Main Results: A total of 137 patients (median age, 4.7 yr) were included. Cardiomyopathy was the most common diagnosis (47%). Cardiac arrest (39%) and low cardiac output (50%) were the most common extracorporeal membrane oxygenation indications. Median time to left atrial decompression was 6.2 hours (interquartile range, 3.8-17.2 hr) with the optimal cut-point of greater than or equal to 18 hours for late decompression determined by receiver operating characteristic curve. In univariate analysis, late decompression was associated with longer extracorporeal membrane oxygenation duration (median 8.5 vs 5 d; p = 0.02). In multivariable analysis taking into account clinical confounder and center effects, late decompression remained significantly associated with prolonged extracorporeal membrane oxygenation duration (adjusted odds ratio, 4.4; p = 0.002). Late decompression was also associated with longer duration of mechanical ventilation (adjusted odds ratio, 4.8; p = 0.002). Timing of decompression was not associated with in-hospital survival (p = 0.36) or overall survival (p = 0.42) with median follow-up of 3.2 years., Conclusions: In this multicenter study of pediatric patients receiving venoarterial extracorporeal membrane oxygenation, late left atrial decompression (≥ 18 hr) was associated with longer duration of extracorporeal membrane oxygenation support and mechanical ventilation. Although no survival benefit was demonstrated, the known morbidities associated with prolonged extracorporeal membrane oxygenation use may justify a recommendation for early left atrial decompression.

Background: The influence of Fontan-associated protein-losing enteropathy's (PLE) severity, duration, and treatment on heart transplant (HTx) outcomes is unknown. We hypothesized that long-standing PLE and PLE requiring more intensive therapy are associated with increased post-HTx mortality., Methods: This 12-center, retrospective cohort study of post-Fontan patients with PLE referred for HTx from 2003 to 2015 involved collection of demographic, medical, surgical, and catheterization data, as well as PLE-specific data, including duration of disease, intensity/details of treatment, hospitalizations, and complications. Factors associated with waitlist and post-HTx outcomes and PLE resolution were sought., Results: Eighty patients (median of 5 per center) were referred for HTx evaluation. Of 68 patients listed for HTx, 8 were removed due to deterioration, 4 died waiting, and 4 remain listed. In 52 patients undergoing HTx, post-HTx 1-month survival was 92% and 1-year survival was 83%. PLE-specific factors, including duration of PLE pre-HTx, pre-HTx hospitalizations, need for/frequency of albumin replacement, PLE therapies, and growth parameters had no association with post-HTx mortality. Immunosuppressant regimen was associated with mortality; standard mycophenolate mofetil immunotherapy was used in 95% of survivors compared with only 44% of non-survivors (p = 0.03). Rejection (53%) and infection (42%) post-HTx were common, but not associated with PLE-specific factors. PLE resolved completely in all but 1 HTx survivor at a median of 1 month (interquartile range 1 to 3 months); resolution was not affected by PLE-specific factors., Conclusions: PLE severity, duration, and treatment do not influence post-HTx outcome, but immunosuppressive regimen may have an impact on survival. PLE resolves in nearly all survivors., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Recurrence of subaortic stenosis (SubAS) is up to ~ 19% following resection. Historically, treatment has consisted of membrane resection alone. This study investigated the effect of routine septal myectomy in addition to membrane resection. A single-center retrospective review was performed in all patients < 18 years of age undergoing membrane resection with septal myectomy for SubAS from 2003 to 2013. Demographic, perioperative, and follow-up data were collected. Freedom from reoperation and risk factors for reoperation were determined. 107 patients (median age 4.8 years) were included. There was one in-hospital death, five patients (5%) requiring pacemaker, and no iatrogenic ventricular septal defects. Follow-up was 80% complete and median follow-up was 4.9 years (range 0.5-12 years). Fourteen (16%) subjects required reoperation. Freedom from reoperation was 98% at 1 year, 86% at 5 years, and 69% at 10 years (Fig. 1). There was no difference in decrease of peak gradient between subjects who did and did not require reoperation (- 47 vs. - 40 mmHg; p = 0.59). In univariate analysis, chromosomal anomaly (hazard ratio [HR] 5.0, p = 0.02), smaller body surface area (HR 0.1, p = 0.03), and younger age at surgery (HR 0.7, p = 0.01) were significantly associated with reoperation. The routine use of myectomy with membrane excision did not result in a lower rate of reoperation or higher rates of complications compared to historical controls. Younger age, smaller size, and chromosomal anomaly were associated with increased risk for reoperation. Patients with these risk factors may benefit from more intensive long-term follow-up.

We report the case of a 6-year-old girl with a penetrating ocular injury caused by a Burmese python. She received intravenous cefazolin before presenting and was treated thereafter with daily topical antibiotics and atropine. Six weeks after injury, she underwent cataract extraction and sulcus implantation of an intraocular lens and iris synechiolysis, with postoperative patching. Final visual outcome was excellent despite no globe repair was performed., (Published by Elsevier Inc.)

Background: Although interstage mortality for infants with hypoplastic left heart syndrome has declined within the National Pediatric Cardiology Quality Improvement Collaborative, variation across centres persists. It remains unclear whether centres with lower interstage mortality have lower-risk patients or whether differences in care may explain this variation. We examined previously established risk factors across National Pediatric Cardiology Quality Improvement Collaborative centres with lower and higher interstage mortality rates., Methods: Lower-mortality centres were defined as those with >25 consecutive interstage survivors. Higher-mortality centres were defined as those with cumulative interstage mortality rates >10%, which is a collaborative historic baseline rate. Baseline risk factors and perioperative characteristics were compared., Results: Seven lower-mortality centres were identified (n=331 patients) and had an interstage mortality rate of 2.7%, as compared with 13.3% in the four higher-mortality centres (n=173 patients, p<0.0001). Of all baseline risk factors examined, the only factor that differed between the lower- and higher-mortality centres was postnatal diagnosis (18.4 versus 31.8%, p=0.001). In multivariable analysis, there remained a significant mortality difference between the two groups of centres after adjusting for this variable: adjusted mortality rate was 2.8% in lower-mortality centres compared with 12.6% in higher-mortality centres, p=0.003. Secondary analyses identified multiple differences between groups in perioperative practices and other variables., Conclusions: Variation in interstage mortality rates between these two groups of centres does not appear to be explained by differences in baseline risk factors. Further study is necessary to evaluate variation in care practices to identify targets for improvement efforts.

Background: Interstage outcomes for single ventricle infants following Norwood operation have been well studied, showing significant mortality. Other single ventricle infants require only an aortopulmonary shunt. The aim of the study was to describe the interstage outcomes of this group compared to Norwood patients and identify risk factors for mortality., Methods: A single-center retrospective cohort review was performed in patients who underwent a Norwood operation (Norwood) or aortopulmonary shunt (Shunt) during 2000 to 2011 and survived to discharge. Hybrid or pulmonary artery banding patients were excluded. Univariate comparison was made between Norwood and Shunt patients as well as a Shunt subgroup analysis., Results: A total of 486 patients (368 Norwood and 118 Shunt) were included. Norwood and Shunt patients were similar in terms of preterm birth, surgery weight, and stage 1 complications. Shunt patients were more likely to be female, have an extracardiac or genetic anomaly, and have a shorter hospital length of stay compared to the Norwood patients (all P < .0001). No significant difference in interstage mortality was seen between the Shunt and Norwood patients (6.8% vs 11.1%, respectively; P = .17). Stage 2 mortality was also similar (Shunt 4.6% vs Norwood 7.8%; P = .25). In the Shunt patients, those who died during interstage weighed less at surgery (2.7 [0.7] kg vs 3.3 [0.7] kg, P = .03) and were more likely to have arrhythmias (50% vs 12%, P = .01), compared to survivors., Conclusions: Shunt patients have an interstage mortality that is not significantly less than Norwood patients. Lower weight at surgery and arrhythmias are risk factors for interstage death in Shunt patients.

Background: Single ventricle (SV) patients with total anomalous pulmonary venous connection (TAPVC) are at high risk. Given the limited published data available, we examined outcomes and the implications of a prenatal diagnosis of SV/TAPVC., Methods: A single-center, retrospective review was performed in neonates with SV/TAPVC from 1998 to 2014, identified through institutional databases. Patient demographic, perioperative, and follow-up data were collected., Results: Thirty-four eligible infants with SV/TAPVC were identified (mean birth weight: 3.0 kg). The TAPVC types were supracardiac (59%), infracardiac (21%), mixed (12%), and cardiac (9%). Heterotaxy syndrome was present in 25 (74%) infants. A prenatal diagnosis of SV was made in 26 (76%) infants, with TAPVC identified in 12 (35%). Seventeen (50%) had obstructed TAPVC within the first 48 hours of life; 7 of these patients had obstructed TAPVC identified prenatally. There were two preoperative deaths. Overall survival for the cohort was 65% at 1 year and 50% at 3 years. Survival in the obstructed group was significantly worse compared to the unobstructed group (47% vs 81% at 1 year; 27% vs 73% at 3 years, P = .01). Obstructed TAPVC and a prenatal prediction of obstructed TAPVC were significantly associated with postoperative mortality ( P = .01 and .03, respectively)., Conclusions: Patients with SV/TAPVC remain a high-risk group, with obstructed TAPVC a significant risk factor for mortality. Prenatal diagnosis of TAPVC in SV patients is challenging, but given those with obstructed TAPVC are especially at high risk, improved prenatal diagnostic techniques in this group may enhance counseling/delivery planning.

Background: Neurodevelopmental impairment is increasingly recognised as a potentially disabling outcome of CHD and formal evaluation is recommended for high-risk patients. However, data are lacking regarding the proportion of eligible children who actually receive neurodevelopmental evaluation, and barriers to follow-up are unclear. We examined the prevalence and risk factors associated with failure to attend neurodevelopmental follow-up clinic after infant cardiac surgery., Methods: Survivors of infant (<1 year) cardiac surgery at our institution (4/2011-3/2014) were included. Socio-demographic and clinical characteristics were evaluated in neurodevelopmental clinic attendees and non-attendees in univariate and multivariable analyses., Results: A total of 552 patients were included; median age at surgery was 2.4 months, 15% were premature, and 80% had moderate-severe CHD. Only 17% returned for neurodevelopmental evaluation, with a median age of 12.4 months. In univariate analysis, non-attendees were older at surgery, had lower surgical complexity, fewer non-cardiac anomalies, shorter hospital stay, and lived farther from the surgical center. Non-attendee families had lower income, and fewer were college graduates or had private insurance. In multivariable analysis, lack of private insurance remained independently associated with non-attendance (adjusted odds ratio 1.85, p=0.01), with a trend towards significance for distance from surgical center (adjusted odds ratio 2.86, p=0.054 for ⩾200 miles)., Conclusions: The majority of infants with CHD at high risk for neurodevelopmental dysfunction evaluated in this study are not receiving important neurodevelopmental evaluation. Efforts to remove financial/insurance barriers, increase access to neurodevelopmental clinics, and better delineate other barriers to receipt of neurodevelopmental evaluation are needed.

Children with single-ventricle disease experience high mortality and complex care. In other life-limiting childhood illnesses, paediatric palliative care may mitigate maternal stress. We hypothesised that early palliative care in the single-ventricle population may have the same benefit for mothers. In this pilot randomised trial of early palliative care, mothers of infants with prenatal single-ventricle diagnoses completed surveys measuring depression, anxiety, coping, and quality of life at a prenatal visit and neonatal discharge. Infants were randomised to receive early palliative care - structured evaluation, psychosocial/spiritual, and communication support before surgery - or standard care. Among 56 eligible mothers, 40 enrolled and completed baseline surveys; 38 neonates were randomised, 18 early palliative care and 20 standard care; and 34 postnatal surveys were completed. Baseline Beck Depression Inventory-II and State-Trait Anxiety Index scores exceeded normal pregnant sample scores (mean 13.76±8.46 versus 7.0±5.0 and 46.34±12.59 versus 29.8±6.35, respectively; p=0.0001); there were no significant differences between study groups. The early palliative care group had a decrease in prenatal to postnatal State-Trait Anxiety Index scores (-7.6 versus 0.3 in standard care, p=0.02), higher postnatal Brief Cope Inventory positive reframing scores (p=0.03), and a positive change in PedsQL Family Impact Module communication and family relationships scores (effect size 0.46 and 0.41, respectively). In conclusion, these data show that mothers of infants with single-ventricle disease experience significant depression and anxiety prenatally. Early palliative care resulted in decreased maternal anxiety, improved maternal positive reframing, and improved communication and family relationships.

Objectives: To describe our 20-year experience with intraoperative pulmonary artery (PA) stent placement and evaluate long-term patient outcomes, specifically the need and risk factors for reintervention., Background: Intraoperative PA stent placement is an alternative to surgical patch arterioplasty and percutaneous angioplasty or stent placement to treat branch PA stenosis., Methods: We performed a retrospective review of all intraoperative PA stents placed at our institution from 1994-2013. Patient and stent characteristics and outcome data were collected. Risk factors associated with reintervention were identified using univariate cox regression analysis., Results: Eighty-one PA stents were placed in 68 patients. The procedural complication rate was 4.4%. During a median follow-up period of 6 years (interquartile range [IQR] 0.9-12.7), 30 patients (44%) underwent reintervention on the stented PA with a median time to first reintervention of 2.6 years (IQR 0.7-4.4 years). The first reintervention was surgical in 30% and catheter-based in 70%. Risk factors for reintervention included age < 18 months (Hazard ratio [HR] 2.97, P = 0.005) and body surface area < 0.47 m 2 (HR 3.20, P = 0.003) at the time of stent implantation, and the presence of multiple aortopulmonary collaterals in patients with tetralogy of Fallot (HR 4.61, P = 0.003)., Conclusions: Intraoperative PA stent implantation is a safe and effective alternative to percutaneous stent implantation and offers several advantages, including the ability to implant adult-size stents in small patients while avoiding injury to peripheral vessels, to position stents to facilitate future percutaneous stent redilation, and to access the PAs directly, which eliminates radiation exposure. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Background: Congenital cardiac magnetic resonance is a limited resource because of scanner and physician availability. Missed appointments decrease scheduling efficiency, have financial implications and represent missed care opportunities., Objective: To characterize the rate of missed appointments and identify modifiable predictors., Materials and Methods: This single-center retrospective study included all patients with outpatient congenital or pediatric cardiac MR appointments from Jan. 1, 2014, through Dec. 31, 2015. We identified missed appointments (no-shows or same-day cancellations) from the electronic medical record. We obtained demographic and clinical factors from the medical record and assessed socioeconomic factors by U.S. Census block data by patient ZIP code. Statistically significant variables (P<0.05) were included into a multivariable analysis., Results: Of 795 outpatients (median age 18.5 years, interquartile range 13.4-27.1 years) referred for congenital cardiac MR, a total of 91 patients (11.4%) missed appointments; 28 (3.5%) missed multiple appointments. Reason for missed appointment could be identified in only 38 patients (42%), but of these, 28 (74%) were preventable or could have been identified prior to the appointment. In multivariable analysis, independent predictors of missed appointments were referral by a non-cardiologist (adjusted odds ratio [AOR] 5.8, P=0.0002), referral for research (AOR 3.6, P=0.01), having public insurance (AOR 2.1, P=0.004), and having scheduled cardiac MR from November to April (AOR 1.8, P=0.01)., Conclusion: Demographic factors can identify patients at higher risk for missing appointments. These data may inform initiatives to limit missed appointments, such as targeted education of referring providers and patients. Further data are needed to evaluate the efficacy of potential interventions.

Background: Infants who require extracorporeal membrane oxygenation (ECMO) support after a Norwood operation are at increased risk for early and late death compared with patients who do not require ECMO post-Norwood. Little is known about the effect that ECMO post-Norwood has on functional status and quality of life among long-term survivors., Methods: We prospectively evaluated functional status and health-related quality of life in 12 surviving patients (cases) and 19 corresponding patients (controls) from a previous retrospective case-control assessment of long-term survival in patients requiring ECMO post-Norwood. Functional status was assessed with the Vineland Adaptive Behavior Scale-II, and health-related quality of life was assessed with the Pediatric Quality of Life Inventory (PedsQL) core and cardiac modules., Results: There were no differences in demographics, extracardiac or genetic anomalies, or age at follow-up assessment between ECMO cases and non-ECMO controls. The Vineland Adaptive Behavior Scale-II scores were comparable between groups, with both groups demonstrating function in the normal range in all four domains tested. The only difference in PedsQL scores between cases and controls was perceived physical appearance, which was lower among ECMO survivors by both patient and proxy report. PedsQL scores of both groups were comparable to published scores for patients with single-ventricle congenital heart disease but generally lower than scores for the healthy population., Conclusions: The requirement for ECMO support after a Norwood operation does not appear to significantly affect functional status or quality of life among the subset of patients who achieve long-term survival., (Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: With improvements in early survival following congenital heart surgery, it has become increasingly important to understand longer-term outcomes; however, routine collection of these data is challenging and remains very limited. We describe the development and initial results of a collaborative programme incorporating standardised longitudinal follow-up into usual care at the Children's Hospital of Philadelphia (CHOP) and University of Michigan (UM)., Methods: We included children undergoing benchmark operations of the Society of Thoracic Surgeons. Considerations regarding personnel, patient/parent engagement, funding, regulatory issues, and annual data collection are described, and initial follow-up rates are reported., Results: The present analysis included 1737 eligible patients undergoing surgery at CHOP from January 2007 to December 2014 and 887 UM patients from January 2010 to December 2014. Overall, follow-up data, of any type, were obtained from 90.8% of patients at CHOP (median follow-up 4.3 years, 92.2% survival) and 98.3% at UM (median follow-up 2.8 years, 92.7% survival), with similar rates across operations and institutions. Most patients lost to follow-up at CHOP had undergone surgery before 2010. Standardised questionnaires assessing burden of disease/quality of life were completed by 80.2% (CHOP) and 78.4% (UM) via phone follow-up. In subsequent pilot testing of an automated e-mail system, 53.4% of eligible patients completed the follow-up questionnaire through this system., Conclusions: Standardised follow-up data can be obtained on the majority of children undergoing benchmark operations. Ongoing efforts to support automated electronic systems and integration with registry data may reduce resource needs, facilitate expansion across centres, and support multi-centre efforts to understand and improve long-term outcomes in this population.

Left ventricular (LV) ejection fraction (EF) and right ventricular (RV) ejection fraction by cardiovascular magnetic resonance (CMR) are associated with functional health status in patients with repaired tetralogy of Fallot (TOF) in cross-sectional studies, but few longitudinal data are available. This study aimed to determine predictors of subsequent decrease in functional health status in midterm follow-up. Patients with repaired TOF who had previously completed CMR and assessment with the Short Form 36 version 2 (SF-36) were recruited for repeat CMR, SF-36, and exercise test, if they had not had interval pulmonary valve replacement (PVR). Patients from the same cohort who had undergone PVR were recruited for repeat SF-36. A total of 19 patients (median 33.5 years old, interquartile range [IQR] 26-42 years, 53 % male) had not undergone PVR and were enrolled at a median of 5.0 years (IQR 4.8-5.3) since prior CMR and SF-36. LVEF and RVEF did not change from baseline, while RV end-diastolic volume increased (138 ± 34 vs. 126 ± 31 ml/m(2), p = 0.02). In the overall cohort, SF-36 scores remained stable. However, higher baseline RV end-systolic volume and pulmonary regurgitant fraction correlated with subsequent decreases in SF-36 scores. In 9 patients post-PVR (median 35.9 years old, IQR 24-43), physical functioning increased compared to those without PVR (change in z-score +0.59 ± 0.59 vs. -0.26 ± 0.72, p = 0.005). In adults with repaired TOF who do not undergo PVR, LVEF, RVEF, and functional health status remain stable in midterm follow-up. However, baseline RV end-systolic volume correlates with subsequent change in functional health status, underscoring its importance in prognostication and timing of intervention in this population.

In pediatric heart transplant recipients, elevated pulmonary capillary wedge pressure (PCWP) is associated with rejection and coronary artery vasculopathy. This study aimed to evaluate which echocardiographic parameters track changes in PCWP and predict adverse outcomes (rejection or coronary artery vasculopathy). This prospective single-center study enrolled 49 patients (median 11.4 years old, interquartile range 7.4 to 16.5) at time of cardiac catheterization and echocardiography. Median follow-up was 2.4 years (range 1.2 to 3.1 years), with serial testing per clinical protocol. Ratio of early mitral inflow to annular velocity (E/E'), left atrial (LA) distensibility, peak LA systolic strain, E/left ventricular (LV) diastolic strain, and E/LV diastolic strain rate were measured from echocardiograms. Increase in PCWP ≥3 mm Hg was associated with changes in LA distensibility, E/E', and E/LV diastolic strain, with highest area under the receiver operating characteristic curve for E/LV diastolic strain (0.76). In 9 patients who subsequently developed rejection or coronary artery vasculopathy, E/LV diastolic strain rate at baseline differed from patients without events (median 57.0 vs 43.6, p = 0.02). On serial studies, only change in LV ejection fraction differed in patients with events (median -10% vs -1%, p = 0.01); decrease in LV ejection fraction of -19% had a specificity of 100% and sensitivity of 44%. In conclusion, LV diastolic strain and strain rate measurements can track changes in PCWP and identify patients at risk for subsequent rejection or coronary artery vasculopathy. Further studies are necessary to confirm these data in a larger cohort., (Copyright © 2016 Elsevier Inc. All rights reserved.)

The increasing use of serial multimodality imaging in the management of pediatric osteosarcoma raises concern of over exposure to ionizing radiation in children, especially from repeated computed tomographic (CT) scans. This study reviews the utilization of multimodality imaging in patients with osteosarcoma at our institution and analyzes any potential radiation-related complications. Twenty-eight patients were identified. Three patients developed late complications-acute myeloid leukemia, myelodysplastic syndrome, and early menopause. Using the patient's age and body part imaged, CT dose length product and effective dose was estimated with the use of a conversion factor for 19 patients. The effective doses were higher in the 3 patients with late complications than the other patients in the cohort (P=0.018). These results suggest an increased risk for adverse effects with higher CT exposures and effective doses. On the basis of our data and published data, methods to decrease the doses of radiation from medical imaging need to be explored. The number of CT scans may be limited. Implementing the Image Gently concept to decrease radiation exposure can be beneficial in modification of CT acquisition parameters.

Objectives: Patients who require venoarterial extracorporeal membrane oxygenation because of cardiac failure frequently have supranormal blood oxygen tensions (hyperoxia). Recent studies have suggested worse outcomes in patients with hyperoxia after resuscitation from cardiac or respiratory arrests, presumably because of oxidative stress. There are limited data regarding the effect of hyperoxia on outcomes in pediatric patients on venoarterial extracorporeal membrane oxygenation., Design: Retrospective chart review., Setting: Pediatric cardiothoracic ICU., Patients: Cardiac surgery patients less than 1 year old requiring venoarterial extracorporeal membrane oxygenation in the postoperative period from 2007 to 2013., Measurements and Main Results: In 93 infants (median time on extracorporeal membrane oxygenation, 5 d), mortality at 30 days post surgery (primary outcome) was 38%. Using a receiver operating characteristic curve, a mean PaO2 of 193 mm Hg in the first 48 hours of extracorporeal membrane oxygenation was determined to have good discriminatory ability with regard to 30-day mortality. Univariate analysis identified a mean PaO2 greater than 193 mm Hg (p = 0.001), longer cardiopulmonary bypass times (p = 0.09), longer duration of extracorporeal membrane oxygenation (p < 0.0001), and higher extracorporeal membrane oxygenation pump flows (p = 0.052) as possible risk factors for 30-day mortality. In multivariable analysis controlling for the variables listed above, a mean PaO2 greater than 193 mm Hg remained an independent risk factor for mortality (p = 0.03). In addition, a mean PaO2 greater than 193 mm Hg was associated with the need for renal dialysis (p = 0.02) but not with neurologic injury (p = 0.41) during the hospitalization., Conclusions: In infants with congenital heart disease who are placed on venoarterial extracorporeal membrane oxygenation postoperatively, hyperoxia (defined as a mean PaO2 > 193 mm Hg in the first 48 hr of extracorporeal membrane oxygenation) was an independent risk factor for 30-day mortality after surgery. Future studies are needed to delineate the causative or associative role of hyperoxia with outcomes, especially in children with baseline cyanosis who may be more susceptible to the effects of oxidative stress.

Objectives: The aim of the study was to evaluate outcomes with an examination of individual predictors influencing survival at a single institution., Methods: This was a retrospective review of the 28 pediatric osteosarcoma patients diagnosed and studied from 2000 through 2012. Twenty-eight patient charts and imaging studies were reviewed for age, race, sex, location, extent of disease at presentation, imaging results, histology, treatment options, and overall survival., Results: Of the 28 patients who were identified, the median age at diagnosis was 14 years. The majority of the patients were male African Americans with the tumor located in the lower long bones and most had conventional osteosarcoma histology. Four patients had metastasis at diagnosis. Of the 28 patients, 16 patients underwent limb salvage surgery, 6 underwent amputation, 4 had biopsy only, 1 had hip disarticulation, and 1 moved out of state and had no information available. All 28 patients received chemotherapy. Four patients received additional radiation therapy. On follow-up, 15 patients were still alive at last clinical contact and 13 died. Of the deceased, the median survival time was 2.3 years. The patient who lived the longest survived 8.3 years. Metastasis at diagnosis was associated with poorer outcome (P = 0.002). The 5-year overall survival rate was 40% (95% confidence interval 18-62) for our entire population of patients., Conclusions: Survival in our patient cohort tended to be at the lower end of the spectrum reported by other contemporary treatment centers of excellence or Surveillance, Epidemiology, and End Results databases probably because of the large number of African American patients with associated poor socioeconomic status. Future studies should be conducted to explore biological and nonbiological factors that may affect the prognosis in this disease.

Background: An intraoperative decline in regional cerebral oxygen saturation (rSO2) has been associated with postoperative injury to the central nervous system. Wide individual variation in steady-state cerebral oxygen saturation limits the clinical use of rSO2 to monitoring during anesthesia and surgical procedures. Recently, low preoperative rSO2 has been proposed as a predictor of adverse postoperative outcomes in cardiovascular operations. We compared the sensitivity and specificity of preinduction rSO2 as a predictor of adverse operative events and compared this to the widely accepted risk index developed by the Society for Thoracic Surgeons., Methods: 2,097 consecutive white patients who underwent cardiac operations from 2010 through 2012 were included. In 1,496 patients (group 1) the preinduction rSO2 was equal to or greater than 60%, whereas in the remaining 601 patients (group 2) it was below 60%. We compared the predictive accuracy of preinduction rSO2 with that of the STS mortality risk score by means of standard statistical techniques, including a receiver operating curve characteristic analysis., Results: Patients with a preinduction rSO2 below 60% had significantly higher STS mortality risk scores than did patients with an rSO2 equal to or greater than 60% (2.0 vs 4.0, p<0.001). Those with an rSO2 below 60% experienced higher operative mortality (p<0.001) and after adjustment this determination emerged as an independent predictor of increased mortality (p<0.001). Receiver operating characteristic curve analysis demonstrated that the rSO2 was slightly less accurate as a mortality predictor (area under the curve: 0.71 vs 0.85)., Conclusions: Measurement of rSO2 is considerably less complex than calculation of the STS score and is only slightly less accurate as a predictor of operative mortality. It may be useful when the STS mortality risk score cannot be calculated., (Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The diagnosis and the management of traumatic thoracic aortic injuries have undergone significant changes due to new technology and improved prehospital care. Most of the discussions have focused on descending aortic injuries. In this review, we discuss the recent management of ascending aortic injuries., Methods: We found 5 cohort studies on traumatic aortic injuries and 11 case reports describing ascending aortic injuries between 1998 to the present through Medline research., Results: Among case reports, 78.9% of cases were caused by motor vehicle accidents (MVA). 42.1% of patients underwent emergent open repair and the operative mortality was 12.5%. 36.8% underwent delayed repair. Associated injuries occurred in 84.2% of patients. Aortic valve injury was concurrent in 26.3% of patients. The incidence of ascending aortic injury ranged 1.9-20% in cohort studies., Conclusions: Traumatic injuries to the ascending aorta are relatively uncommon among survivors following blunt trauma. Aortography has been replaced by computed tomography and echocardiography as a diagnostic tool. Open repair, either emergent or delayed, remains the treatment of choice., (© 2013 Wiley Periodicals, Inc.)

We present an 11-month-old girl with congenital ocular motor apraxia (COMA) and Joubert syndrome found to have a compound heterozygous mutation in the NPHP1 gene that is responsible for juvenile nephronophthisis type 1. The association of congenital ocular motor apraxia and juvenile nephronophthisis is reviewed. The patient does not currently manifest signs of renal failure, although her mutation indicates that she is at risk for the development of juvenile nephronophthisis type 1., (Published by Mosby, Inc.)

Skeletal muscle hypertrophy and increases in muscular function have been observed following low intensity/load exercise with blood flow restriction (BFR). The mechanisms behind these effects are largely unknown, but have been hypothesized to include a metabolic accumulation induced increase in muscle activation, elevations in growth hormone, and improvements in muscle protein balance. However, many of the aforementioned mechanisms are not present with BFR in the absence of exercise. In these situations, signaling through the β2 adrenoceptor has been hypothesized to possibly contribute to the positive muscle adaptions, possibly in concert with muscle cell swelling. Signaling through the β2 adrenoceptor has been shown to stimulate both muscle protein synthesis and an inhibition of protein degradation through increasing cyclic adenosine monophosphate (cAMP) or signaling via the Gβγ subunit, especially in situations where the basal rates of protein synthesis are already reduced. Every study that has investigated the catecholamine response to BFR in the absence of exercise or in combination with exercise has shown a significant increase above resting conditions. However, from the available evidence, it is unlikely that the norepinephrine response from BFR, particularly with exercise, is playing a prominent role with muscle adaptation in skeletal muscle that is not immobilized by a cast or joint injury., (© Georg Thieme Verlag KG Stuttgart · New York.)

Purpose: Report of an unusual case of Aicardi syndrome., Methods: Case report of an infant with severe chorioretinal lacunae and Aicardi syndrome., Results: Despite severe chorioretinal lacunae, a 25-month-old white girl with infantile spasms/seizures had better visual acuity than expected., Conclusion: The extent of chorioretinal lesions is not necessarily a prognostic factor for visual function.

Introduction: Acute aortic dissection (AAD) is one of the most common aortic emergencies that vascular specialists are asked to manage. Traditional surgical interventions for cases complicated by malperfusion have resulted in significant morbidity and mortality. With increasing availability of thoracic endografts, endovascular interventions for complicated AAD have become more acceptable. We reviewed our experience with endovascular treatment of AAD since January 2005., Methods: Medical records of patients admitted for AAD from January 1, 2005, to December 31, 2008, were entered into our vascular registry and analyzed for risk factors, extent of dissection, type of management, fate of the false lumen, complications, and survival. There were 249 admissions for aortic dissections during the study period. Our study group included 28 patients with complicated AAD who underwent endovascular intervention., Results: During the study interval, 28 patients (16 male) underwent 44 procedures. The average age was 54 years. Risk factors differed from the typical atherosclerotic patient and were dominated by an 89.3% incidence of hypertension. Five patients (17.9%) presented with a history of recent cocaine use. The average length of stay was 25.1 days (range, 1-196 days). Stanford type B dissections were present in all but one patient. Twenty-six thoracic endografts were placed in 25 patients. Eight patients required multiple procedures in addition to a thoracic endograft. Morbidity occurred in 17 (60.7%) patients, with renal insufficiency occurring in 11 patients (39.3%) and one requiring permanent dialysis. Four neurologic events occurred: three strokes (10.7%) and one patient (3.6%) with temporary paraplegia. Three patients (10.7%) died in the periprocedural period, with ruptured dissection in one and pericardial tamponade in another. Eight of 10 computed tomography scans (80%) available for review in follow-up showed complete thrombosis of the thoracic false lumen., Conclusions: Complicated AAD remains a challenging problem, with significant morbidity and mortality rates. However, our early experience with endovascular management offers a favorable reduction in mortality from historic controls., (Copyright © 2011. Published by Mosby, Inc.)

In the general population, African Americans experience atrial fibrillation (AF) less frequently than European Americans. This difference could also exist in the incidence of this arrhythmia after cardiac surgery, but this possibility has been insufficiently examined. To test the association of such an ethnic difference, we compared the incidence of postoperative AF in a consecutive series of 2,312 African Americans and 6,054 European Americans who underwent isolated coronary artery bypass grafting from July 2000 to June 2007. Raw differences between the cohorts in the incidence of new AF were adjusted to take into account the baseline differences. Postoperatively, new-onset AF developed in 504 (22%) of 2,312 African-American patients and in 1,838 (30%) of 6,054 European-American patients (p <0.01). After adjustment with logistic regression analysis for numerous baseline differences, African Americans remained less likely to develop AF (odds ratio 0.63, 95% confidence interval 0.55 to 0.72; p <0.001). Risk was also adjusted using propensity matching. In that analysis, 457 (22%) of 2,059 African-American patients had postoperative AF, as did 597 (29%) of 2,059 matched European-American patients (p <0.01). In conclusion, AF was significantly less common among African-American patients than among European-American patients after coronary artery bypass grafting., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Objective: To examine the long-term best-corrected visual acuity (BCVA) in children who, during the amblyogenic period, underwent cataract extraction with intraocular lens (IOL) implantation and to see if initial postoperative refractive error following cataract extraction correlates with long-term BCVA., Design: Retrospective chart review., Participants: Thirty-six eyes of 26 patients were included in the study. At least 2 years of follow-up, an age , 8.5 years at time of IOL implantation, an ability to participate in subjective visual acuity measurement, and an absence of congenital glaucoma and significant trauma were required for inclusion in the study., Methods: Retrospective review of pseudophakic patients' charts at a tertiary care centre, with attention to initial and long-term pseudophakic refractive error, long-term BCVA, total myopic shift, length of follow-up, age at IOL implantation, and unilateral or bilateral cataract extraction with IOL implantation., Results: Initial pseudophakic spherical equivalent (SE) showed a significant nonlinear relationship with most recentBCVAin unilateral cases and no relationship in bilateral cases (interaction p 0.001). Unilateral cases with initial pseudophakic SE between +1.75 D and +5.00 D showed better long-term BCVA than those with values below +1.75 D or above +5.00 D; this was irrespective of the refractive error of their fellow eye., Conclusions: In patients receiving lens implants in the first 8 years of life, we recommend aiming for low early postoperative hyperopia, taking into consideration each patient's situation and age.

Background: Early readmission after coronary artery bypass grafting (CABG) is an expensive adverse outcome. Although the perioperative experience of high-risk CABG patients has been studied extensively, little attention has been paid to low-risk CABG patients. The primary goal of this study was to identify the preoperative characteristics and to define risk predictors of readmission and preventive factors for readmission in low-risk isolated-CABG patients., Methods: We identified 2157 patients who underwent CABG between January 2000 and December 2005 at Washington Hospital Center, Washington, DC, and defined as low risk patients who had a Parsonnet bedside risk score lower than the 25th percentile. Patients who were rehospitalized within 30 days after surgery were compared with those who were not rehospitalized during this period., Results: The overall readmission rate for this study cohort was 6.3%. Compared with non-readmitted patients, early-readmitted patients were more likely to have diabetes mellitus (27.94% versus 20.88%, P = .05) and less likely to have hypertension (42.65% versus 51.36%, P = .05). Blood product transfusion (P < .01), postoperative length of intensive care unit stay (P = .01), and length of hospital stay (P = .05) were all significantly increased in the readmitted patients. The use of beta-blockers (P = .03) and angiotensin-converting enzyme inhibitors (P = .04) was significantly lower at discharge in this group of patients; however, multivariate regression analysis demonstrated diabetes (odds ratio, 1.59; 95% confidence interval, 1.08-2.42) to be the only independent predictor of early readmission., Conclusions: For low-risk CABG patients, diabetes mellitus is the risk predictor of early readmission. Early discharge was not associated with early readmission.

Objective: Postoperative vasoplegic syndrome (PVS) is a potentially lethal condition with increased mortality and other postoperative morbidities. Many previous studies have examined the outcomes associated with on-pump coronary artery bypass grafting (CABG) surgery, little is known about the incidence of PVS after off-pump CABG., Methods: From November 21, 2005 to June 9, 2006, 334 patients underwent isolated on-pump CABG and 362 had off-pump CABG surgery. Perioperative variables were retrospectively compared between on-pump and off-pump CABG surgery using univariate analysis. Significant variables were included into a stepwise regression model to ascertain their independent impact on the incidence of PVS., Results: The incidence of PVS in isolated on-pump CABG was 6.9%; in off-pump CABG was 2.8% (p=0.01). However, in multivariable models adjusted for confounders, on-pump CABG did not reach statistical significance as a risk factor of PVS (OR=2.3, 95% CI 0.94-5.78; p=0.07). In on-pump CABG, preoperative left ventricular EF less than 35% (OR=3.6; p=0.02) and increased body mass index (OR=1.1; p=0.04) were identified as risk predictors of PVS; whereas elective surgery (OR=0.2; p=0.02) and preoperative use of beta-blockers (OR=0.21; p=0.02) were associated with a decreased rate of PVS. PVS was associated with longer ICU stay (OR=6.0; p<0.01), postoperative ventilation (OR=4.6; p<0.01), and hospital stay (OR=2.62; p=0.03). There was a stronger association between preoperative ACE inhibitors therapy and increased risk of PVS in off-pump CABG surgery (OR=4.52, 95% CI 0.95-21.67; p=0.06) than in on-pump CABG surgery (OR=1.06, 95% CI 0.35-3.19; p=0.91), but neither of them reaches statistical significance., Conclusions: The incidence of PVS after off-pump CABG surgery was significantly lower than after on-pump CABG surgery.

Anomalies of the coronary arteries can be benign or life threatening. The prevalence of these anomalies is reported to be approximately 0.3% to 1%, however, this may be an underestimation as conventional angiography may not allow correct identification of these abnormalities. Morphologic variations can arise in the origin, course, or termination of coronary arteries. These variations may be related to other congenital abnormalities or isolated. Some anomalies can lead to myocardial ischemia and have been implicated in episodes of sudden death in young adults. Noninvasive imaging modalities such as multidetector computerized tomography provide an efficient method of evaluating coronary artery anomalies by allowing more complete visualization of chest, mediastinal, and vascular structures. We describe a case of anomalous origin of the right coronary artery originating from the main pulmonary trunk demonstrated by computerized tomography in a patient with exertional ischemia. In the past, this diagnosis has only been made by angiography, echocardiography, and at autopsy.

Aim: To review the medical records of seven children with a delayed diagnosis of cerebral visual impairment., Methods: The charts of a consecutive series of children examined in a university based ophthalmology clinic with a delayed diagnosis of cerebral visual impairment were reviewed. Their neuroimaging studies were evaluated by a paediatric neuroradiologist., Results: The seven patients ranged in age from 2 years to 10 years at the time of presentation and had acuities ranging from 20/40 to 20/400. Their visual fields ranged from full visual fields to homonymous hemianopias. Neuroimaging findings ranged from subtle occipital lobe abnormalities to obvious cerebral volume loss., Conclusions: Cerebral visual impairment can be cryptic in children with mild visual impairment. Neuroimaging studies and visual field testing can help to differentiate this condition from other causes of visual impairment.

Many children with accommodative esotropia are able to successfully discontinue spectacle wear, while others require spectacle correction into adulthood. Parents often ask about the likelihood of glasses being required on a long-term basis and whether use of spectacles will cause dependency. Most infants are hyperopic and gradually become emmetropic. The extent to which accommodation and spectacle use affect this process is still debated. However, certain characteristics, such as degree of hyperopia, can help predict long-term spectacle requirement.

We report the use of clonidine in an infant as an adjunct to sedation and analgesia for 4.5 months in the critical care setting. Advantages, potential side effects, and dosing for multiple modes of delivery are discussed.

The purpose of this part of a longitudinal study was to examine whether medication therapy for older adults with heart failure predicted days to readmission post-hospital discharge. Using a prospective, predictive design, a convenience sample included 127 older adults with heart failure who had been recently discharged from two hospitals in northeastern Ohio. One hundred five patients were prescribed diuretics, 49 angiotensin-converting enzyme inhibitors, 23 b blockers, and 47 digoxin. There were no significant differences between readmitted and non-readmitted patients with regard to the use of the specific classes of cardiac medications. None of the specific classes of cardiac medications predicted the number of days between the initial hospital discharge and readmission 3 months later. The use of a small, non-probability sample and exclusion of variables limit the results of the study. Effective case management with teaching about heart failure must address changes involved with heart failure and the use of medication therapy. More research is needed about treatment protocols in various regions of the United States.

Background: Aortic valve replacement (AVR) through a partial sternotomy (mini-AVR) has been suggested to significantly reduce postoperative morbidity compared with conventional AVR. This study sought to investigate whether mini-AVR patients require fewer transfusions than patients who had conventional AVR., Methods: Of 511 patients who had AVR, 56 had mini-AVR and 455 had conventional AVR. A matched-case logistic regression analysis was used to adjust for these imbalances between groups., Results: No patient in the mini-AVR cohort required conversion to a conventional AVR. Cardiopulmonary bypass time was longer in the mini-AVR group compared with the conventional AVR group, with a median of 102 minutes (range, 78 to 119 minutes) versus 75 minutes (range, 61 to 96 minutes; p < 0.01) in the conventional AVR group. A total of 31 patients (55%) in the mini-AVR group and 336 patients (74%) in the conventional sternotomy group required transfusions during their hospital stay (p < 0.01). After adjusting for differences in preoperative risk factors, year of operation, and surgeon, by matching on propensity score, the differences were not statistically significant (odds ratio = 0.84, 95% confidence interval = 0.40 to 1.75, p = 0.63)., Conclusions: Mini-AVR produces better wound cosmesis and less surgical trauma but requires more time to perform. Matched-case analysis failed to show a significant difference in blood transfusion requirements after mini-AVR compared with the conventional AVR approach.

Study Objectives: Atrial fibrillation (AF) is a common occurrence after cardiac surgery (10 to 53%) that contributes to increased length of stay and hospital cost. Recent evidence suggests that treatment with amiodarone may provide safe and effective prophylaxis against AF in many patients undergoing cardiac operations. This study sought to investigate whether oral amiodarone administered postoperatively would reduce the incidence of postoperative AF., Design: Prospective nonrandomized cohort study., Patients and Participants: In this prospective study, 1,196 consecutive patients who underwent various open-heart procedures with cardiopulmonary bypass between July 1999 and February 2000 received oral amiodarone, 400 mg bid, from the transfer to the cardiovascular recovery room until the day of hospital discharge, or up to 7 days postoperatively. The incidence of AF in this group of patients was compared with a group of 1,246 patients who underwent cardiac surgery with cardiopulmonary bypass in the preceding 8-month period (November 1998 to June 1999) at the same institution without receiving amiodarone postoperatively., Setting: Tertiary health-care center., Measurement and Results: AF developed in 294 patients (25%) in amiodarone-treated group and in 385 patients (31%) in the control group (p = 0.001). In multivariate logistic regression analysis, oral amiodarone treatment emerged as an independent predictor of lower risk of AF (odds ratio, 0.7; 95%; 95% confidence interval, 0.6 to 0.9; p = 0.002) and shorter hospital length of stay (odds ratio, 0.8; 95% confidence interval, 0.5 to 0.9; p = 0.006)., Conclusions: Postoperative oral amiodarone treatment is a safe and effective regimen associated with a reduced incidence of new-onset AF and decreased length of hospital stay. Prospective randomized trials are needed to evaluate the benefits of amiodarone treatment relative to its side effect profiles.

The UDP-glucuronosyltransferase UGT2B7 is an important human UGT isoform that catalyzes the conjugation of many endogenous and exogenous compounds, among them opioids, resulting in the formation of D-glucuronides. The binding site of the aglycone is located in the N-terminal half of the protein. In this study, we demonstrate that the opioid binding site in UGT2B7 is within the first 119 amino-terminal amino acids. Two maltose binding protein fusion proteins, 2B7F1 and 2B7F2, incorporating the first 157 or 119 amino acids, respectively, of UGT2B7 were expressed in Escherichia coli and purified by affinity chromatography. NMR spectroscopy using one-dimensional spectra, the inversion recovery method, and the transferred nuclear Overhauser effect spectroscopy was used to study the binding properties of opioids to the fusion proteins. Morphine was found to bind at a single site within the first 119 amino acids and to undergo a conformational change upon binding, as demonstrated by transferred nuclear Overhauser effect spectroscopy. Dissociation constants were obtained for morphine, naloxone, buprenorphine, and zidovudine, and the results were confirmed by equilibrium dialysis determinations. Two possible opioid binding sites, based on the nearest neighbors from opioid binding to the micro-receptor and to cytochrome 2D6, are proposed.