Comprehensive Approach to the Management of Patients with HLHS: Analysis of 100 Consecutive Neonates.

BACKGROUND: We report our comprehensive approach to patients with hypoplastic left heart syndrome (HLHS) and describe our outcomes in 100 consecutive neonates. METHODS: One-hundred consecutive neonates (2015-2023) were stratified into 3 pathways: Pathway(1): 77/100=77% were standard-risk and underwent initial Norwood (Stage 1). Pathway(2): 10/100=10% were high-risk with noncardiac risk factors and underwent initial Hybrid Stage 1. Pathway(3): 13/100=13% were high-risk with cardiac risk factors: 10 underwent initial Hybrid Stage 1 + ventricular assist device insertion (HYBRID+VAD), while 3 underwent primary transplantation. RESULTS: One-year mortality=9/100=9%. Pathway(1): Operative Mortality for initial Norwood (Stage 1)=2/77=2.6%. Of 75 survivors of Norwood (Stage 1): 72 underwent successful Glenn, 2 underwent successful biventricular repair, and 1 underwent successful cardiac transplantation. Pathway(2): Operative Mortality for initial Hybrid Stage 1 without VAD=1/10=10%. Of 9 survivors of Hybrid (Stage 1): 4 underwent successful cardiac transplantation, 2 died while awaiting cardiac transplantation, 3 underwent Comprehensive Stage 2 (with 1 death), and 1 underwent successful biventricular repair. Pathway(3): Of 10 HYBRID+VAD: 7/10=70% underwent successful cardiac transplantation and are alive today and 3/10=30% died on VAD while awaiting transplantation. Median VAD support time=134 days (range=56-226). (Two of three patients who were bridged-to-transplant with prostaglandin underwent successful transplantation and one died while awaiting transplantation.) CONCLUSIONS: A comprehensive approach to the management of patients with HLHS is associated with Operative Mortality after Norwood of 2/77=2.6% and an overall one-year mortality of 9/100=9%. 10/100 patients=10% were stabilized with HYBRID+VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged wait times.

Epstein Barr virus-directed T-cell therapy for refractory EBV-PTLD in a toddler post Orthotopic heart transplantation.

Epstein-Barr Virus (EBV) is a ubiquitous herpes type virus that is associated with post-transplant lymphoproliferative disorder (PTLD). Usual management includes reduction or cessation of immunosuppression and in some cases chemotherapy including rituximab. However, limited therapies are available if PTLD is refractory to rituximab. Several clinical trials have investigated the use of EBV-directed T cells in rituximab-refractory patients; however, data regarding response is scarce and inconclusive. Herein, we describe a patient with EBV-PTLD refractory to rituximab after orthotopic heart transplantation (OHT) requiring EBV-directed T-cell therapy. This article aims to highlight the unique and aggressive clinical presentation and progression of PTLD with utilization of EBV-directed T-cell therapy for management and associated pitfalls.

17-Month-Old Girl With Severe, Prolonged Lethargy and Somnolence.

A 17-month-old girl arrived at the pediatric ED with decreased responsiveness. She was lethargic, localizing only to noxious stimuli with vital signs significant for fever of 103.8 °F, heart rate of 185 beats/min, respiratory rate of 12 breaths/min, blood pressure of 100/59 mmHg, and oxygen saturation level of 88% on room air. She was admitted to the pediatric intensive care unit (PICU) due to concerns of septic meningitis with altered mental status and respiratory distress, and was treated with antibiotics. A respiratory viral panel (RVP) was positive for adenovirus, resulting in all antibiotics being discontinued. She remained lethargic until day nine of illness, when she had improved almost completely to her baseline. Polymerase chain reaction (PCR) of her cerebral spinal fluid returned positive for adenovirus serotype A, thus confirming our case of transient adenovirus encephalopathy. This case illustrates the importance of keeping adenovirus in the differential for encephalopathy versus a neurologic abnormality or other malignant or infectious etiology.

Atrial fibrillation in adults with congenital heart disease following cardiac surgery in a single center: Analysis of incidence and risk factors.

OBJECTIVE: The primary aim of our work is to determine the incidence of atrial fibrillation following cardiac surgery in adults with congenital heart disease. Secondary aims include identifying risk factors predictive of developing early postoperative atrial fibrillation and morbidities associated with early postoperative atrial fibrillation. DESIGN: Retrospective analysis. SETTING: Single center, quaternary care children's hospital. PATIENTS: This review included patients at least 18 years of age with known congenital heart disease who underwent cardiac surgery requiring a median sternotomy at our congenital heart center from January 1, 2012 to December 31, 2016. INTERVENTIONS: None. OUTCOME MEASURES: The primary outcome was early postoperative atrial fibrillation. Secondary outcomes included preoperative comorbidities, preoperative echocardiographic findings, operative details, and postoperative morbidities, such length of stay, reintubation, stroke, and death. RESULTS: The incidence of early postoperative atrial fibrillation was 21%. Those who developed early postoperative atrial fibrillation were older (50 years vs 38 years, P =< .001), had a history of atrial fibrillation prior to surgery, had preoperative pulmonary hypertension, and had longer cardiopulmonary bypass times (103 minutes vs 84 minutes, P = .025) when compared to those who did not develop postoperative atrial fibrillation. Multivariate analysis identified age greater than 60, preoperative pulmonary hypertension, mitral valve intervention, and the need for postoperative inotropic support as being independent predictors of postoperative atrial fibrillation. Those who developed postoperative atrial fibrillation remained in the hospital longer (9 days vs 7 days, P =< .001). CONCLUSIONS: Atrial fibrillation is a common complication following cardiac surgery in adults with congenital heart disease. Age, preoperative comorbidities, type of surgical intervention, and the need for perioperative inotropic infusions may predict the risk of atrial fibrillation in this unique patient population.

Effects of fish oil supplementation on eicosanoid production in patients at higher risk for colorectal cancer.

Fish oil supplementation may represent a potential chemopreventive agent for reducing colorectal cancer risk. The mechanism of action of fish oil is unknown but presumed to be related to eicosanoid modification. The purpose of this study was to evaluate the effects of fish oil supplementation on the levels of urinary and rectal eicosanoids. We conducted a randomized, double-blind, controlled trial of 2.5 g of fish oil per day compared with olive oil supplementation over a 6-month period. Study participants had a history of colorectal adenomas. Randomization was stratified based on the gene variant rs174535 in the fatty acid desaturase 1 enzyme (FADS1), which affects tissue levels of arachidonic acid. A total of 141 participants were randomized. Urinary prostaglandin E2 metabolite (PGE-M) was measured at baseline, 3, and 6 months and rectal prostaglandin E2 (PGE2) at baseline and 6 months. Repeated-measures linear regression was used to determine the effect of the intervention on each outcome measure. Overall, fish oil supplementation was found to reduce urinary PGE-M production compared with olive oil (P=0.03). Fish oil did not reduce rectal PGE2 overall; however, it did significantly reduce PGE2 in the subgroup of participants not using aspirin or NSAIDs (P=0.04). FADS1 genotype did not seem to modify effects of fish oil on PGE2 production. We conclude that fish oil supplementation has a modest but beneficial effect on eicosanoids associated with colorectal carcinogenesis, particularly in those not taking aspirin or NSAIDs.

Plasma lipid levels and colorectal adenoma risk.

PURPOSE: Abnormalities in lipid levels have been associated with colorectal neoplasm risk; however, few studies have adjusted for use of cholesterol-lowering medications. The objective of this study was to determine the association of plasma lipid levels with adenoma risk while accounting for statin medication use. METHODS: We included 254 subjects with advanced adenoma, 246 with single small adenoma, 179 with multiple small adenoma cases, and 403 control participants in the Tennessee Colorectal Polyp Study who also had plasma lipid measurements performed. Data on the use of statin medications were available for 83.4% of these participants. The association between plasma lipids and adenoma risk was evaluated using logistic regression models. RESULTS: Participants in the highest quartile of HDL cholesterol (range 52-106 mg/dl) had an adjusted odds ratio of 0.49 (95% CI 0.23, 1.07), 0.35 (95% CI 0.13, 0.91), and 0.22 (95% CI 0.09, 0.54) for single small, multiple small, and advanced adenomas compared to the lowest quartile (range 12-34 mg/dl), respectively. Participants with the highest quartile of triglyceride levels (range 178-721 mg/dl) had an adjusted odds ratio of 2.40 (95% CI 1.26, 4.55), 1.67 (95% CI 0.66, 4.23), and 2.79 (95% CI 1.25, 6.23) for single small, multiple small, and advanced adenoma, respectively, compared to the lowest quartile (range 40-84 mg/dl). When restricted to individuals with known statin medication use, adjusting for statin use did not appreciably affect these results. CONCLUSION: We found a direct association between triglyceride plasma levels and an inverse association between plasma HDL cholesterol levels and adenoma risk. Both effects were not appreciably changed when accounting for the regular use of statin medication.