Left Superior Vena Cava Draining to Left Atrium: A Case Report, Review of the Literature, and Classification.

Persistent left superior vena cava (PLSVC) draining to the left atrium (LA) is a rare congenital abnormality that is often asymptomatic and found incidentally on imaging. PLSVC is usually described alongside other congenital defects, such as septal defects, tetralogy of fallot, and aortic coarctation. PLSVC to LA with an atrial septal defect is known as Raghib syndrome, but to our knowledge PLSVC to LA without an atrial septal defect or right superior vena cava has not been described in the literature. Here, we report the presentation of a patient with PLSVC-LA without ASD and propose a classification system for this subset of congenital heart defects to help guide clinical and surgical management of these patients.

Growing Heart Valve Implants for Children.

The current standard of care for pediatric patients with unrepairable congenital valvular disease is a heart valve implant. However, current heart valve implants are unable to accommodate the somatic growth of the recipient, preventing long-term clinical success in these patients. Therefore, there is an urgent need for a growing heart valve implant for children. This article reviews recent studies investigating tissue-engineered heart valves and partial heart transplantation as potential growing heart valve implants in large animal and clinical translational research. In vitro and in situ designs of tissue engineered heart valves are discussed, as well as the barriers to clinical translation.

Partial Heart Transplantation in Adult Cardiac Surgery.

Many young adults require heart valve replacements. Current options for valve replacement in adults include mechanical valves, bioprosthetic valves, or the Ross procedure. Of these, mechanical and bioprosthetic valves are the most common options, although mechanical valve usage predominates in younger adults due to durability, while bioprosthetic valve usage predominates in older adults. Partial heart transplantation is a new method of valvular replacement that can deliver durable and self-repairing valves and allow adult patients freedom from anticoagulation therapy. This procedure involves transplantation of donor heart valves only, permitting expanded utilization of donor hearts as compared with orthotopic heart transplantation. In this review, we discuss the potential benefits of this procedure in adults who elect against the anticoagulation regimen required of mechanical valve replacements, although it has not yet been clinically established. Partial heart transplantation is a promising new therapy for the treatment of pediatric valvular dysfunction. This is a novel technique in the adult population with potential utility for valve replacement in young patients for whom anticoagulation therapy is problematic, such as women who wish to become pregnant, patients with bleeding disorders, and patients with active lifestyles.

Relationship Between Palliative Care Consults and Outcomes of Pediatric Surgical Patients During Terminal Admissions.

Background: Pediatric patients often undergo surgery during terminal admissions. However, the involvement and timing of palliative care consults in caring for these patients has not been readily described. Objective: To describe the presence and timing of palliative care consults for pediatric patients who undergo surgical procedures during terminal admissions. Design: Retrospective cohort study using data from the electronic health record. Setting and Participants: Pediatric patients who underwent at least one surgical procedure during a terminal admission at an urban, quaternary hospital in the United States from January 1, 2016 to December 31, 2021. Main Outcomes and Measures: Patients' medical, surgical, and admission-level characteristics were abstracted. Associations were evaluated between these characteristics and the occurrence and timing of a palliative care consult relative to surgery and death. Results: Of 134 patients, 84% received a palliative care consult during their terminal admission. Approximately 36% of consults occurred before surgery, and 12% were within one day of death. Children without a palliative care consult were more likely than children with a consult to die during surgery (19.1% vs. 2.7%, p = 0.02), have surgery within 24 hours of death (52.4% vs. 15.9%, p < 0.001), and undergo a full resuscitation attempt (47.6% vs. 12.4%, p = 0.002). Receipt of a palliative care consultation did not differ by patient sex, reported race and ethnicity, language, insurance, or income level. Conclusions and Relevance: Palliative care consults support high-quality end-of-life care for children and impact perioperative outcomes, including intensity of surgical care and resuscitation in the final hours of life.

Devices for donor lung preservation.

INTRODUCTION: Lung transplantation is the gold standard for the treatment of end stage lung disease but is limited by donor availability. Recently, the donor pool has seen significant expansion with liberalization of donor criteria. However, extended criteria donors can require additional time to prepare for implantation, necessitating additional preservation time of donor lungs. AREAS COVERED: We present a review of current lung transplant storage strategies including new methodologies and technological advancements. The current standard, static cold storage, is a simple and cost-effective method of preserving grafts, but offers little flexibility with limited ability to mitigate ischemic-reperfusion injury, inflammation, and hypothermic tissue damage. Novel ex vivo lung perfusion (EVLP) devices, TransMedics OCS and XVIVO perfusion systems, extend preservation time by perfusing, and ventilating donor lungs while simultaneously allowing for evaluation of lung viability. Perfusate, preservation solutions, additives, temperature regulation, and assessment of organ damage are all critical components when evaluating the success and outcomes of these devices. EXPERT OPINION: EVLP devices are more costly and often require additional resources and personnel support compared to static cold storage, but may provide the opportunity to extend preservation time, perform functional assessment, mitigate ischemic injury, and optimize extended criteria donors.

Partial heart transplantation can ameliorate donor organ utilization.

BACKGROUND: The treatment of babies with unrepairable heart valve dysfunction remains an unsolved problem because there are no growing heart valve implants. However, orthotopic heart transplants are known to grow with recipients. AIM: Partial heart transplantation is a new approach to delivering growing heart valve implants, which involves transplantation of the part of the heart containing the valves only. In this review, we discuss the benefits of this procedure in children with unrepairable valve dysfunction. CONCLUSION: Partial heart transplantation can be performed using donor hearts with poor ventricular function and slow progression to donation after cardiac death. This should ameliorate donor heart utilization and avoid both primary orthotopic heart transplantation in children with unrepairable heart valve dysfunction and progression of these children to end-stage heart failure.

Population pharmacokinetics of crenolanib in children and young adults with brain tumors.

PURPOSE: Crenolanib, an oral inhibitor of platelet-derived growth factor receptor, was evaluated to treat children and young adults with brain tumors. Crenolanib population pharmacokinetics and covariate influence were characterized in this patient population. METHODS: Patients enrolled on this phase I study (NCT01393912) received oral crenolanib once daily. Serial single-dose and steady-state serum pharmacokinetic samples were collected and analyzed using a validated LC-ESI-MS/MS method. Population modeling and covariate analysis evaluating demographics, laboratory values, and comedications were performed. The impact of significant covariates on crenolanib exposure was further explored using model simulations. RESULTS: Crenolanib serum concentrations were analyzed for 55 patients (2.1-19.2 years-old) and best fitted with a linear two-compartment model, with delayed absorption modeled with a lag time. A typical patient [8-year-old, body surface area (BSA) 1 m2] had an apparent central clearance, volume, and absorption rate of 41 L/h, 54.3 L, and 0.19 /h, respectively. Patients taking acid reducers (histamine H2 antagonists or proton pump inhibitors) concomitantly exhibited about 2- and 1.7-fold lower clearance and volume (p < 0.0001 and p = 0.018, respectively). Crenolanib clearance increased with BSA (p < 0.0001), and absorption rate decreased with age (p < 0.0001). Model simulations showed cotreatment with an acid reducer was the only covariate significantly altering crenolanib exposure and supported the use of BSA-based crenolanib dosages vs flat-dosages for this population. CONCLUSIONS: Crenolanib pharmacokinetics were adequately characterized in children and young adults with brain tumors. Despite marked increased drug exposure with acid reducer cotreatment, crenolanib therapy was well tolerated. No dosing adjustments are recommended for this population.