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.

The safe addition of nitric oxide to the sweep gas of the extracorporeal membrane oxygenation circuit in a pediatric cardiac intensive care unit.

Background: Nitric Oxide (NO) is a naturally occurring modulator of inflammation found in the human body. Several studies in the pediatric cardiothoracic surgery literature have demonstrated some beneficial clinical effects when NO is added to the sweep gas of the cardiopulmonary bypass circuit.Purpose: Our primary aim was to determine the safety of incorporating nitric oxide into the oxygenator sweep gas of the extracorporeal membrane oxygenation (ECMO) circuit. Secondarily, we looked at important clinical outcomes, such as survival, blood product utilization, and common complications related to ECMO.Methods: We performed a single center, retrospective review of all patients at our institution who received ECMO between January 1, 2017 and March 31, 2023. We began additing NO to the ECMO sweep gas in 2019. Results: There were no instances of clinically significant methemoglobinemia with the addition of NO to the sweep gas (0% vs 0%, p = 1). The median daily methemoglobin level was higher in those who received NO via the sweep gas when compared to those who did not (1.6 vs 1.1, p = <0.001). Conclusions: The addition of NO to the sweep gas of the ECMO circuit is safe.

Prevalence and Risk Factors for Cerebral Palsy in Children With Congenital Heart Disease Based on Risk of Surgical Mortality.

BACKGROUND: Children with congenital heart disease (CHD) have a higher prevalence of motor impairment secondary to brain injury, resulting in cerebral palsy (CP). The purpose of this study is to determine the prevalence of CP in CHD in a single-center cohort, stratify risk based on surgical mortality using Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) categories and identify risk factors. METHODS: Retrospective cohort study of pediatric patients registered in the University of Florida (UF) Society of Thoracic Surgeons Congenital Heart Surgery database from 2006 to 2017 with a diagnosis of CHD who continued follow-up for more than two years at UF. RESULTS: A total of 701 children with CHD met inclusion criteria. Children identified to have CP were 54 (7.7%). Most common presentation was spastic hemiplegic CP with a Gross Motor Function Classification System of level 2. Analysis of surgical and intensive care factors between the two groups showed that children with CHD and CP had longer time from admission to surgery (P = 0.003), higher STAT categories 4 and 5 (P = 0.038), and higher frequency of brain injury and seizures (P < 0.001). Developmental disabilities and rehabilitation needs were significantly greater for children with CHD and CP when compared with those with CHD alone (P < 0.001). CONCLUSIONS: In our cohort, 7.7% children with CHD develop CP; this is significantly higher than the 2010 US population estimate of 0.3%. Our study suggests higher STAT categories, brain injury, and seizures are associated with developing CP in children with CHD.

Comparative evaluation of rapid plasma reagin and ELISA with Treponema pallidum hemagglutination assay for the detection of syphilis in blood donors: a single center experience.

INTRODUCTION: The prime responsibility of blood transfusion services in India is to provide safe blood. The donated blood is tested for human immunodeficiency virus (HIV), hepatitis B virus (HBV), hepatitis C virus (HCV), malaria and syphilis. In India, the screening of donated blood for syphilis is performed by rapid plasma reagin (RPR) or venereal disease research laboratory (VDRL), whereas the World Health Organization (WHO) recommends screening of syphilis in blood donors by enzyme-linked immunosorbent assay (ELISA). Therefore, the aim of this study was to evaluate the performance of RPR and ELISA with the Treponema pallidum hemagglutination assay (TPHA - the gold standard) for the detection of syphilis in blood donors. METHODS: In this cross-sectional study, 1524 consecutive whole blood donors were screened from April to October 2022. All blood samples collected during the study period were tested by RPR, ELISA and the TPHA and the results obtained were compared. RESULTS: The seroprevalence of syphilis in blood donors in this study was 0.06% by RPR and 0.72% by ELISA and TPHA. On considering ELISA and the TPHA as the gold standard, ELISA had comparable sensitivity (100%), a higher specificity (100% vs. 99.34%), a higher positive predictive value (PPV - 100% vs. 9.1%) and no biological false positive/false negative results (0 vs. 10 false negatives) when compared to RPR. CONCLUSION: ELISA performed better as a screening assay than RPR in the detection of syphilis in blood donors, which is in agreement with the WHO recommendations for syphilis testing in blood donors with low prevalence.

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.

A single-institutional experience with 36 children less than 5 kilograms supported with the Berlin Heart: Comparison of congenital versus acquired heart disease.

OBJECTIVES: We reviewed outcomes in all 36 consecutive children <5 kg supported with the Berlin Heart pulsatile ventricular assist device at the University of Florida, comparing those with acquired heart disease (n = 8) to those with congenital heart disease (CHD) (n = 28). METHODS: The primary outcome was mortality. The Kaplan-Meier method and log-rank tests were used to assess group differences in long-term survival after ventricular assist device insertion. T-tests using estimated survival proportions were used to compare groups at specific time points. RESULTS: Of 82 patients supported with the Berlin Heart at our institution, 49 (49/82 = 59.76%) weighed <10 kg and 36 (36/82 = 43.90%) weighed <5 kg. Of 36 patients <5 kg, 26 (26/36 = 72.22%) were successfully bridged to transplantation. (The duration of support with ventricular assist device for these 36 patients <5 kg was [days]: median = 109, range = 4-305.) Eight out of 36 patients <5 kg had acquired heart disease, and all eight [8/8 = 100%] were successfully bridged to transplantation. (The duration of support with ventricular assist device for these 8 patients <5 kg with acquired heart disease was [days]: median = 50, range = 9-130.) Twenty-eight of 36 patients <5 kg had congenital heart disease. Eighteen of these 28 [64.3%] were successfully bridged to transplantation. (The duration of support with ventricular assist device for these 28 patients <5 kg with congenital heart disease was [days]: median = 136, range = 4-305.) For all 36 patients who weighed <5 kg: 1-year survival estimate after ventricular assist device insertion = 62.7% (95% confidence interval = 48.5-81.2%) and 5-year survival estimate after ventricular assist device insertion = 58.5% (95% confidence interval = 43.8-78.3%). One-year survival after ventricular assist device insertion = 87.5% (95% confidence interval = 67.3-99.9%) in acquired heart disease and 55.6% (95% confidence interval = 39.5-78.2%) in CHD, P = 0.036. Five-year survival after ventricular assist device insertion = 87.5% (95% confidence interval = 67.3-99.9%) in acquired heart disease and 48.6% (95% confidence interval = 31.6-74.8%) in CHD, P = 0.014. CONCLUSION: Pulsatile ventricular assist device facilitates bridge to transplantation in neonates and infants weighing <5 kg; however, survival after ventricular assist device insertion in these small patients is less in those with CHD in comparison to those with acquired heart disease.

Outcomes of Children Supported With Pulsatile Paracorporeal Ventricular Assist Device: Congenital Versus Acquired Heart Disease.

BACKGROUND: We reviewed the outcomes of 82 consecutive pediatric patients (less than 18 years of age) supported with the Berlin Heart ventricular assist device (VAD), comparing those with congenital heart disease (CHD; n = 44) with those with acquired heart disease (AHD; n = 37). METHODS: The primary outcome was mortality after VAD insertion. Kaplan-Meier methods and log-rank tests were used to assess group differences in long-term survival. RESULTS: Forty-four CHD patients were supported (age: median = 65 days, range = 4 days-13.3 years; weight [kg]: median = 4, range = 2.4-42.3). Ten biventricular CHD patients were supported with eight biventricular assist devices (BiVADs), one left ventricular assist device (LVAD) only, and one LVAD converted to BiVAD, while 34 univentricular CHD patients were supported with single ventricle-ventricular assist devices (sVADs). In CHD patients, duration of VAD support was [days]: median = 134, range = 4-554. Of 44 CHD patients, 28 underwent heart transplantation, 15 died on VAD, and one was still on VAD. Thirty-seven AHD patients were supported (age: median = 1.9 years, range = 27 days-17.7 years; weight [kg]: median = 11, range = 3.1-112), including 34 BiVAD and 3 LVAD. In AHD patients, duration of VAD support was [days]: median = 97, range = 4-315. Of 37 AHD patients, 28 underwent transplantation, three died on VAD, five weaned off VAD (one of whom underwent heart transplantation 334 days after weaning), and one was still on VAD. One-year survival after VAD insertion was 59.9% (95% CI = 46.7%-76.7%) in CHD and 88.6% (95% CI = 78.8%-99.8%) in AHD, P = .0004. Five-year survival after VAD insertion was 55.4% (95% CI = 40.8%-75.2%) in CHD and 85.3% (95% CI = 74.0%-98.2%) in AHD, P = .002. CONCLUSIONS: Pulsatile VAD facilitates bridge-to-transplantation in neonates, infants, and children with CHD; however, survival after VAD insertion is worse in patients with CHD than in patients with AHD.

Comparative study of quality of leukoreduced packed red blood cell units as assessed by nageotte hemocytometry and flow cytometry.

PURPOSE: Assessment of residual white blood cell (rWBC) count is vital to ascertain the quality of leukodepleted (LD) blood components. Automated cell analyzers lack the sensitivity for the assessment of very few leukocytes as found in LD blood components. Flow Cytometry (FC) based methods and Nageotte hemocytometer are the most commonly used techniques for this purpose. The objective of this study was to compare the use of Nageotte hemocytometer and FC for quality control of LD red blood cell units. MATERIALS AND METHODS: A prospective, observational study was conducted in the Department of Immunohematology and Blood Transfusion of a tertiary care center from September 2018 to September 2020. About 303 LD-packed red blood cell units were tested by FC and Nageotte hemocytometer for rWBCs. RESULTS: The number of rWBC (mean) detected by flow cytometer and Nageotte's hemocytometer was 1.06 ± 0.43 white blood cell (WBC)/µL and 0.67 ± 0.39 WBC/µL, respectively. Coefficient of variation was 58.37% by Nageotte hemocytometer method and 40.46% by FC. Linear regression analysis did not show any correlation (R2= 0.098, P = 0.001) whereas Pearson's correlation coefficient showed a weak relation (r = 0.31) between the two methods. CONCLUSION: Flow cytometric technique provides a more precise and accurate objective tool compared to Nageotte hemocytometer which is labor intensive, time consuming, and prone to errors arising out of subjectivity along with reported underestimation bias. In the absence of adequate infrastructure, resources, and trained workforce, Nageotte hemocytometer method is a reliable alternative. Nageotte's chamber could be best used in the resource-constrained setup as it offers a relatively inexpensive, simple, and viable means to enumerate rWBCs.

Support with Single Ventricle-Ventricular Assist Device (sVAD) in Patients with Functionally Univentricular Circulation Prior to Fontan Operation.

Some patients with functionally univentricular circulation develop cardiac failure refractory to maximal management and are supported with a ventricular assist device (VAD). The purpose of this manuscript is to summarize our previous publications related to single ventricle-ventricular assist device (sVAD) support in patients with functionally univentricular circulation and to describe our current institutional approach at University of Florida to sVAD support in neonates, infants, and children prior to Fontan. Our programmatic philosophy at University of Florida is to strive to identify the minority of neonates with functionally univentricular circulation who are extremely high-risk prior to initiating staged palliation and to stabilize these neonates with primary preemptive sVAD in preparation for cardiac transplantation; our rationale for this approach is related to the challenges associated with failed staged palliation and subsequent bail-out sVAD support and transplantation. A subset of extremely high-risk neonates and infants with functionally univentricular ductal-dependent circulation undergo primary preemptive sVAD insertion and subsequent cardiac transplantation. Support with VAD clearly facilitates survival on the waiting list during prolonged wait times and optimizes outcomes after Norwood (Stage 1) by providing an alternative pathway for extremely high-risk patients. Therefore, the selective utilization of sVAD in extremely high-risk neonates facilitates improved outcomes for all patients with functionally univentricular ductal-dependent circulation. At University of Florida, our programmatic approach to utilizing sVAD support as a bridge to transplantation in the minority of neonates with functionally univentricular circulation who are extremely high-risk for staged palliation is associated with Operative Mortality after Norwood (Stage 1) Operation of 2.9% (2/68) and a one-year survival of 91.1% (82/90) for all neonates presenting with hypoplastic left heart syndrome (HLHS) or HLHS-related malformation with functionally univentricular ductal-dependent systemic circulation. Meanwhile, at University of Florida, for all 82 consecutive neonates, infants, and children supported with pulsatile paracorporeal VAD: Kaplan-Meier survival estimated one year after VAD insertion = 73.3% (95% confidence interval [CI] = 64.1-83.8%), and Kaplan-Meier survival estimated five years after VAD insertion = 68.3% (95% CI = 58.4-79.8%). For all 48 consecutive neonates, infants, and children at University of Florida with biventricular circulation supported with pulsatile paracorporeal VAD: Kaplan-Meier survival estimated one year after VAD insertion = 82.7% (95% CI = 72.4-94.4%), and Kaplan-Meier survival estimated five years after VAD insertion = 79.7% (95% CI = 68.6-92.6%). For all 34 consecutive neonates, infants, and children at University of Florida with functionally univentricular circulation supported with pulsatile paracorporeal sVAD: Kaplan-Meier survival estimated one year after VAD insertion = 59.7% (95% CI = 44.9-79.5%), and Kaplan-Meier survival estimated five years after VAD insertion = 50.5% (95% CI = 35.0-73.0%). These Kaplan-Meier survival estimates for patients supported with pulsatile paracorporeal VAD are better in patients with biventricular circulation in comparison to patients with functionally univentricular circulation both one year after VAD insertion (P=0.026) and five years after VAD insertion (P=0.010). Although outcomes after VAD support in functionally univentricular patients are worse than in patients with biventricular circulation, sVAD provides a reasonable chance for survival. Ongoing research is necessary to improve the outcomes of these challenging patients, with the goal of developing strategies where outcomes after sVAD support in functionally univentricular patients are equivalent to the outcomes achieved after VAD support in patients with biventricular circulation.

A Single-Institutional Experience with 36 Children Smaller Than 5 Kilograms Supported with the Berlin Heart Ventricular Assist Device (VAD) over 12 Years: Comparison of Patients with Biventricular versus Functionally Univentricular Circulation.

OBJECTIVES: We reviewed outcomes in all 36 consecutive children <5 kg supported with the Berlin Heart pulsatile ventricular assist device (VAD) at the University of Florida, comparing those with univentricular circulation (n = 23) to those with biventricular circulation (n = 13). METHODS: The primary outcome was mortality. Kaplan-Meier methods and log-rank tests were used to assess group differences in long-term survival after VAD insertion. T-tests using estimated survival proportions and standard errors were used to compare groups at specific time points. RESULTS: Of all 82 patients ever supported with Berlin Heart at our institution, 49 (49/82 = 59.76%) weighed <10 kg and 36 (36/82 = 43.90%) weighed <5 kg. Of these 36 patients who weighed <5 kg, 26 (26/36 = 72.22%) were successfully bridged to transplantation. Of these 36 patients who weighed <5 kg, 13 (13/36 = 36.1%) had biventricular circulation and were supported with 12 biventricular assist devices (BiVADs) and 1 left ventricular assist device (LVAD) (Age [days]: median = 67, range = 17-212; Weight [kilograms]: median = 4.1, range = 3.1-4.9), while 23 (23/36 = 63.9%) had univentricular circulation and were supported with 23 single ventricle-ventricular assist devices (sVADs) (Age [days]: median = 25, range = 4-215; Weight [kilograms]: median = 3.4, range = 2.4-4.9). Of 13 biventricular patients who weighed <5 kg, 12 (12/23 = 92.3%) were successfully bridged to cardiac transplantation. Of 23 functionally univentricular patients who weighed <5 kg, 14 (14/23 = 60.87%) were successfully bridged to cardiac transplantation. For all 36 patients who weighed <5 kg: 1-year survival estimate after VAD insertion = 62.7% (95% confidence interval [CI] = 48.5%-81.2%) and 5-year survival estimate after VAD insertion = 58.5% (95% CI = 43.8%-78.3%). One-year survival after VAD insertion: 84.6% (95% CI = 67.1%-99.9%) in biventricular patients and 49.7% (95% CI = 32.3%-76.4%) in univentricular patients, P = 0.018. Three-year survival after VAD insertion: 84.6% (95% CI = 67.1%-99.9%) in biventricular patients and 41.4% (95% CI = 23.6%-72.5%) in univentricular patients, P = 0.005. CONCLUSION: Pulsatile VAD facilitates bridge to transplantation in neonates and infants weighing <5 kg; however, survival after VAD insertion in these small patients is less in those with univentricular circulation in comparison to those with biventricular circulation.

Analysis of 82 Children Supported With Pulsatile Paracorporeal Ventricular Assist Device: Comparison of Patients With Biventricular Versus Univentricular Circulation.

We reviewed outcomes in 82 consecutive children supported with the Berlin Heart pulsatile ventricular assist device (VAD), comparing those with functionally univentricular circulation (n = 34) to those with biventricular circulation (n = 48). The primary outcome was mortality. Kaplan-Meier (KM) methods and log-rank tests were used to assess group differences in long-term survival. T-tests using KM-estimated survival proportions and standard errors were used to compare groups at specific time points. 48 biventricular patients were supported (Age: median = 1.4 years, range = 17 days-17.7 years; Weight [kilograms]: median = 9.4, range = 3.1-112), including 43 BiVAD, 4 LVAD only, and 1 LVAD converted to BiVAD. In biventricular patients, duration of VAD support [days]: median = 97, range = 4-315. Of 48 biventricular patients, 35 underwent heart transplantation, 7 died on VAD, 5 weaned off VAD (1 of whom underwent heart transplantation 334 days after weaning), and 1 is still on VAD. 34 univentricular patients were supported with single VAD (sVAD) (Age: median = 38.5 days, range = 4 days-13.3 years; Weight [kilograms]: median = 3.98, range = 2.4-32.6). In univentricular patients, duration of VAD support [days]: median = 138, range = 4-554. Of 34 univentricular patients, 22 underwent transplantation, 11 died on VAD, and 1 is still on VAD. One-year survival after VAD insertion was 82.7% (95% CI = 72.4-94.4%) in biventricular patients and 59.7% (95% CI = 44.9-79.5%) in univentricular patients, p = 0.026. Five-year survival after VAD insertion was 79.7% (95% CI = 68.6-92.6%) in biventricular patients and 50.5% (95% CI = 35.0-73.0%) in univentricular patients, p = 0.010. Pulsatile VAD facilitates bridge to transplantation in neonates, infants, and children with functionally univentricular circulation; however, survival is worse than in patients with biventricular circulation.

A retrospective audit to evaluate the appropriateness and rationalization of Fresh Frozen Plasma usage in a tertiary care hospital.

Background: General guidelines describing the potential indications and contraindications of Fresh Frozen Plasma (FFP) exist. However, their implementation is inadequate, leading to inappropriate use in various clinical settings. This study aims to define the appropriateness of the FFP usage in terms of therapeutic versus prophylactic indications. Methods: Retrospective audit was conducted over one year prior to and after an educational intervention (1122 patients for 6072 FFP units and 1061 patients for 4858 FFP units, respectively). Clinical diagnosis, indication for FFP transfusion, and coagulation profile were noted, and episodes of transfusion were divided into appropriate and inappropriate based on the guidelines of the British Committee for Standards in Hematology (2004 reviewed in 2018) and College of American Pathologists (1994). Results: Initial audit found 51.8% of FFP transfusions to be inappropriate (3069 of 5922). Coagulation profile (with INR values less than 1.5 times of the normal) was the most common cause of inappropriate transfusion (15.08%). 56.7% of FFP were prophylactically transfused. Re-audit after educational interventions showed a 22.3% reduction in the number of inappropriate transfusions. Conclusion: Inappropriate, as well as high prophylactic usage of FFP, was noticed in the initial audit, which reduced significantly after educational interventions. Regular CMEs, interactive sessions with clinicians, functioning Hospital Transfusion Committees, and prospective audits can affirm, further improve and reinforce the existing Hospital Transfusion Guidelines.

Experience of hope in adult patients with advanced chronic disease and their informal caregivers: a qualitative systematic review protocol.

OBJECTIVE: This systematic review will evaluate the experience of hope in adult patients with advanced chronic diseases other than cancer, transitioning toward end-of-life. The review will also evaluate the experience of hope in informal caregivers caring for adult patients with advanced chronic diseases other than cancer as they transition toward end-of-life. INTRODUCTION: Hope is an important resource that assists patients and informal caregivers to deal with difficult and complex situations, such as living with advanced chronic disease. INCLUSION CRITERIA: The review will include studies written in English, French, and Portuguese exploring hope. Qualitative studies focusing on adult patients with advanced chronic diseases other than cancer and/or informal caregivers will be considered. Studies with children as patients or parents as caregivers will be excluded. METHODS: The review will search Embase, MEDLINE, CINAHL, PsycINFO, Web of Science, ProQuest Dissertations and Theses, DART-Europe E-theses Portal, and Google Scholar. The search will be conducted without date restrictions. Articles will be assessed against the inclusion criteria by two independent reviewers. Data will be extracted using a standard tool. The extracted findings will be synthesized using the meta-aggregation approach through assembling and categorizing data. SYSTEMATIC REVIEW REGISTRATION NUMBER: PROSPERO CRD42021266487.

Association of CD 34 positive cell dose with engraftment kinetics in autologous peripheral blood stem cell transplant patients of multiple myeloma.

Background: Treatment with high-dose chemotherapy and stem cell transplantation has prolonged survival in patients of multiple myeloma (MM). A dose-response relationship between number of CD34+ cells infused and leukocyte and platelet recovery, exists. Patients receiving dose of <2.0 × 106 CD34+ cells/kg have delayed engraftment. The level of optimal cutoff for accelerated engraftment is yet to be validated. Hence, this study was undertaken to study the association of CD 34+ cell dose with engraftment kinetics in patients of MM who underwent autolgous peripheral blood stem cell transplant (PBSCT). Methods: We retrospectively analyzed 19 patients of MM who underwent PBSCT at our center between December 2016 to December 2018. Complete blood counts were carried out daily after transplantation to record neutrophil and platelet engraftment. Results: Based on the CD34+ cell dose given : <5 × 106/kg (category 1), 5-10 × 106/kg (category 2), >5 × 106/kg (category 3), the mean (SD) neutrophil engraftment time was 11.3 (0.5) days, 10.6 (0.9) days, and 10.2 (1.3) days respectively. Platelet engraftment time was 12.4 (2.60) days, 10.6 (1.14) days, and 11.2 (1.64) days for category 1, 2, and 3 patients, respectively. Correlation co-efficient between CD 34+cell dose and days for neutrophil and platelet engraftment was found to be -0.24 and -0.20, respectively. Time for neutrophil engraftment was found to be significantly associated with CD34+ cell dose category. Conclusion: CD 34+ cell dose appears as the strongest predictor of leukocyte and platelet engraftment. CD 34+ cell dose of >5.0 × 106 cells/kg leads to an accelerated neutrophil and platelet engraftment in patients of MM.

Ventricular assist device support in neonates and infants with a failing functionally univentricular circulation.

Some neonates with functionally univentricular hearts are at extremely high risk for conventional surgical palliation. Primary cardiac transplantation offers the best option for survival of these challenging neonates; however, waitlist mortality must be minimized. We have developed a comprehensive strategy for the management of neonates with functionally univentricular hearts that includes the selective use of conventional neonatal palliation in standard-risk neonates, hybrid approaches in neonates with elevated risk secondary to a noncardiac etiology, and neonatal palliation combined with insertion of a single ventricular assist device (VAD) in neonates with elevated risk secondary to a cardiac etiology. Here we describe our selection criteria, technical details, management strategies, pitfalls, and current outcomes for neonates with functionally univentricular hearts supported with a VAD. Our experience shows that extremely high-risk neonates with functionally univentricular hearts who are poor candidates for conventional palliation can be successfully stabilized with concomitant palliation and pulsatile VAD insertion while awaiting cardiac transplantation.

A Comprehensive Approach to the Management of Patients With HLHS and Related Malformations: An Analysis of 83 Patients (2015-2021).

Background: Some patients with hypoplastic left heart syndrome (HLHS) and HLHS-related malformations with ductal-dependent systemic circulation are extremely high-risk for Norwood palliation. We report our comprehensive approach to the management of these patients designed to maximize survival and optimize the utilization of donor hearts. Methods: We reviewed our entire current single center experience with 83 neonates and infants with HLHS and HLHS-related malformations (2015-2021). Standard-risk patients (n = 62) underwent initial Norwood (Stage 1) palliation. High-risk patients with risk factors other than major cardiac risk factors (n = 9) underwent initial Hybrid Stage 1 palliation, consisting of application of bilateral pulmonary bands, stent placement in the patent arterial duct, and atrial septectomy if needed. High-risk patients with major cardiac risk factors (n = 9) were bridged to transplantation with initial combined Hybrid Stage 1 palliation and pulsatile ventricular assist device (VAD) insertion (HYBRID + VAD). Three patients were bridged to transplantation with prostaglandin. Results: Overall survival at 1 year = 90.4% (75/83). Operative Mortality for standard-risk patients undergoing initial Norwood (Stage 1) Operation was 2/62 (3.2%). Of 60 survivors: 57 underwent Glenn, 2 underwent biventricular repair, and 1 underwent cardiac transplantation. Operative Mortality for high-risk patients with risk factors other than major cardiac risk factors undergoing initial Hybrid Stage 1 palliation without VAD was 0/9: 4 underwent transplantation, 1 awaits transplantation, 3 underwent Comprehensive Stage 2 (with 1 death), and 1 underwent biventricular repair. Of 9 HYBRID + VAD patients, 6 (67%) underwent successful cardiac transplantation and are alive today and 3 (33%) died while awaiting transplantation on VAD. Median length of VAD support was 134 days (mean = 134, range = 56-226). Conclusion: A comprehensive approach to the management of patients with HLHS or HLHS-related malformations is associated with Operative Mortality after Norwood of 2/62 = 3.2% and a one-year survival of 75/83 = 90.4%. A subset of 9/83 patients (11%) were stabilized with HYBRID + VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged wait times.