International Survey of Perfusion Practice for Pediatric and Congenital Heart Surgery: 2021 Results.

The conduct of cardiopulmonary bypass in neonatal, infant, and pediatric patients continuously evolves as new devices and innovative techniques are introduced. Since 1989, periodic pediatric perfusion surveys have been conducted to ascertain practice patterns involving demographics, equipment, and perfusion techniques. The goal of this current project is to provide an updated perspective on international pediatric and congenital perfusion practice since the last survey conducted in 2016. In July 2021, a 100-question perfusion survey was distributed to 284 pediatric cardiac surgery centers using a secure web browser-based data application. Each center was given a unique survey hyperlink to ensure one response per institution and to monitor the response rate. Centers were given 1 month to complete the survey and electronic reminders were sent weekly to nonrespondents. After the survey was closed, information from completed surveys was exported to a software program for analysis. Responses were received from 153 of 284 pediatric centers for a response rate of 54%. Sixty respondents (39%) were from North American (NA) centers while 93 respondents (61%) were from non-North American (NNA) centers. The vast majority of centers use a roller head arterial pump (93%), hollow fiber oxygenators with open reservoirs (86%), and integrated arterial line filters (73%). The use of modified ultrafiltration was reported by 76% of centers. Ninety-two percent of centers reported the use of selective antegrade cerebral perfusion for aortic arch repairs. The N + 1 staffing model was most prevalent (52%), followed by two perfusionists per case (33%). Periodic surveys continue to be a useful modality in assessing regional variation in pediatric perfusion practice. This survey marked the first time the majority of responses came from non-North American institutions. Identifying these practice patterns may aid in the development of, and adherence to, regional standards and guidelines. This would foster the reduction of variation in practice and potentially improve patient safety.

Comparison of Thromboelastography Devices TEG®6S Point of Care Device vs. TEG®5000 in Pediatric Patients Undergoing Cardiac Surgery.

Thromboelastography (TEG) can predict bleeding in pediatric patients undergoing cardiac surgery. We hypothesized that results obtained from TEG®5000 correlate with the new point-of-care TEG®6S system and that TEG®6S rewarming maximum amplitude (MA) is associated with surrogate endpoints for perioperative bleeding in pediatric patients who underwent complex cardiac surgery. We describe a retrospective study of pediatric (≤18 years) patients who underwent complex cardiac surgery on cardiopulmonary bypass. Citrate whole-blood samples were used to compared TEG®5000 vs.TEG®6S and TEG®6S-FLEV (with fibrinogen measurement) vs. Clauss-fibrinogen methods. TEG®6S parameters obtained during rewarming were compared to the surrogate endpoints for perioperative bleeding using linear regression analysis. Among 100 patients, 225 TEG®5000 vs.TEG®6S comparisons and 54 TEG®6S-FLEV were analyzed. Good correlation was observed for all parameters comparing TEG®5000 to TEG®6S and TEG®6S-FLEV to the Clauss-fibrinogen method (Pearson r ≥ .7). Similar to rewarming TEG®5000 MA, rewarming TEG®6S MA was the only parameter independently associated with risk for perioperative bleeding (median [interquartile range {IQR}] in bleeding vs. nonbleeding patients: 35 [29, 48] vs. 37 [32, 55]; p = .02). A platelet transfusion calculator was developed based on TEG®6S results by determining the relationship between platelet transfusion volume (mL/kg) and percent change in MA using linear regression analysis. TEG®6S is a good alternative point-of-care method to analyze a patient's coagulation profile and it is comparable to TEG®5000 in pediatric patients undergoing cardiac surgery on cardiopulmonary bypass. Lower TEG®6S MA during rewarming is associated with increased risk for perioperative bleeding. TEG analysis during rewarming may be useful in customizing platelet transfusion therapy by reducing the risk of bleeding while minimizing excessive blood product transfusions.

Initial experience with del Nido cardioplegia solution at a Pediatric and Congenital Cardiac Surgery Program in Brazil.

OBJECTIVE: The aim of this study was to evaluate outcome measures between our standard multidose cardioplegia protocol and a del Nido cardioplegia protocol in congenital heart surgery patients. METHODS: Retrospective single-center study including 250 consecutive patients that received del Nido cardioplegia (DN group) with a mandatory reperfusion period of 30% of cross clamp time and 250 patients that received a modified St. Thomas' solution (ST group). Groups were matched by age, weight, gender, and Risk Adjustment for Congenital Heart Surgery (RACHS-1) scores. Preoperative hematocrit and oxygen saturation were also recorded. Outcomes analyzed were the vasoactive inotropic score (VIS), lactate, ventilation time, ventricular dysfunction with low cardiac output syndrome (LCOS), intensive care unit (ICU) length of stay (LOS), hospital LOS, bypass and aortic cross-clamp times, and in-hospital mortality. RESULTS: Both groups were comparable demographically. Statistically significant differences (p ⩽ 0.05) were noted for cardiac dysfunction with LCOS, hematocrit at end of surgery (p = 0.0038), VIS on ICU admission and at end of surgery (p = 0.0111), and ICU LOS (p = 0.00118) with patients in the DN group having more desirable values for those parameters. Other outcome measures did not reach statistical significance. CONCLUSION: In our congenital cardiac surgery population, del Nido cardioplegia strategy was associated with less ventricular dysfunction with LCOS, a lower VIS and decreased ICU LOS compared with patients that received our standard myocardial protection using a modified St. Thomas' solution. Despite the limitation of this study, including its retrospective nature and cohort size, these data supported our transition to incorporate del Nido cardioplegia solution with a mandatory reperfusion period as the preferred myocardial protection method in our program.

Hybrid Left Heart Bypass Circuit for Repair of the Descending Aorta in an 8-kg Williams Syndrome Patient.

A 1-year old male patient with Williams syndrome and multiple prior interventions presented for surgical repair of his descending aorta (DA) through a left thoracotomy. Concerns for significant bleeding and spinal cord protection led the care team to consider a left heart bypass (LHB) circuit with options for pump sucker use, heat exchange capacity, and the possibility of converting to traditional cardiopulmonary bypass (CPB). A traditional CPB circuit with a roller-head arterial pump was assembled with a bypass line around the cardiotomy venous reservoir (CVR). Excluding the CVR with this line allowed for a closed LHB circuit. A second pump head was integrated to both recirculate the CVR volume and to serve as a means for controlled volume administration to the closed LHB circuit. Pump sucker return directed to the CVR could easily be transfused back to the patient. The patient was placed on the hybrid LHB circuit and cooled to 32°C. DA clamps were placed. Upper body dynamic blood pressure was managed for a target mean of 50 mmHg, the left atrial pressure (LAP) was maintained in the 5-7 mmHg range, and the nonpulsatile lower body blood pressure was targeted at 40-50 mmHg. Cerebral near-infrared spectroscopy (NIRS) helped guide volume and pressure management. The surgeons placed two long-segment patches on the DA, moving clamps as needed. The patient was rewarmed and separated from the hybrid LHB circuit after 82 minutes. Closed circuit LHB can be provided with a roller-head hybrid circuit incorporating an oxygenator for gas exchange, central cooling and warming, and arterial line filtration along with a CVR for pump sucker use and controlled transfusion to the patient.

A Multi-Mode System for Myocardial Functional and Physiological Assessment during Ex Situ Heart Perfusion.

Ex situ heart perfusion (ESHP) has proven to be an important and valuable step toward better preservation of donor hearts for heart transplantation. Currently, few ESHP systems allow for a convenient functional and physiological evaluation of the heart. We sought to establish a simple system that provides functional and physiological assessment of the heart during ESHP. The ESHP circuit consists of an oxygenator, a heart-lung machine, a heater-cooler unit, an anesthesia gas blender, and a collection funnel. Female Yorkshire pig hearts (n = 10) had del Nido cardioplegia (4°C) administered, excised, and attached to the perfusion system. Hearts were perfused retrogradely into the aortic root for 2 hours before converting the system to an isovolumic mode or a working mode for further 2 hours. Blood samples were analyzed to measure metabolic parameters. During the isovolumic mode (n = 5), a balloon inserted in the left ventricular (LV) cavity was inflated so that an end-diastolic pressure of 6-8 mmHg was reached. During the working mode (n = 5), perfusion in the aortic root was redirected into left atrium (LA) using a compliance chamber which maintained an LA pressure of 6-8 mmHg. Another compliance chamber was used to provide an afterload of 40-50 mmHg. Hemodynamic and metabolic conditions remained stable and consistent for a period of 4 hours of ESHP in both isovolumic mode (LV developed pressure: 101.0 ± 3.5 vs. 99.7 ± 6.8 mmHg, p = .979, at 2 and 4 hours, respectively) and working mode (LV developed pressure: 91.0 ± 2.6 vs. 90.7 ± 2.5 mmHg, p = .942, at 2 and 4 hours, respectively). The present study proposed a novel ESHP system that enables comprehensive functional and metabolic assessment of large mammalian hearts. This system allowed for stable myocardial function for up to 4 hours of perfusion, which would offer great potential for the development of translational therapeutic protocols to improve dysfunctional donated hearts.

Transition from Hemochron Response to Hemochron Signature Elite Activated Clotting Time Devices in a Congenital Cardiac Surgery Practice.

Heparin is the primary anticoagulant used during cardiac surgery to prevent thrombosis due to cardiopulmonary bypass (CPB)-related activation of the hemostatic system. The efficacy of heparin in the operating room is generally determined by activated clotting time (ACT) point-of-care tests performed throughout the procedure. In an effort to transition to the Hemochron Elite which requires approximately 1/10th the sampling volume of blood, we conducted a prospective study in 260 pediatric patients undergoing CPB. ACT tests were performed during CPB with a total of 260 pre-bypass and 1,117 on-bypass ACT values recorded. All samples were run simultaneously on both ACT devices. Several therapeutic cut-off possibilities ranging from >380 to >480 seconds were evaluated to ascertain the ACT level on the Elite device which best correlated with results from the Response device. Linear regression was used to determine correlation. The correlation between the two methods was moderate with a Pearson r of .6 and .4 for pre-bypass bolus ACT values and on-bypass ACT values, respectively. As the therapeutic ACT cut-off values were lowered from 480 to 380 seconds on the Elite device relative to the Response device (>480 seconds) for the on-bypass heparin samples, more patients would be under-dosed (incidence rising from 1 to 2%) and fewer patients would be overdosed (incidence decreasing from 32 to 5%) and the percent correlation between devices increased from 67 to 93%. A similar trend was observed with the pre-bypass heparin bolus samples. There was no significant effect of temperature on the ACT values comparing both devices. A therapeutic ACT value of >400 seconds for CPB with the Hemochron Elite device reasonably approximates a therapeutic ACT value of >480 seconds on the Hemochron Response device in our congenital cardiac surgery practice. Transitioning to the Elite device significantly reduces the overall sampling volume required for ACT monitoring during cardiac surgery.

A red cell preservation strategy reduces postoperative transfusions in pediatric heart surgery patients.

BACKGROUND: Blood transfusion has well-documented adverse effects. As part of a blood conservation initiative at our center, we began routine use of cell saver for all congenital heart surgery performed on cardiopulmonary bypass since 2014. AIMS: This study aimed to compare transfusion rates prior to, and in the first and second year after this initiative. We hypothesized that cell saver use would decrease transfusion requirements in second year after use of the cell saver compared to the pre cell saver group. METHODS: Consecutive patients under 18 years undergoing congenital heart surgery on cardiopulmonary bypass were retrospectively analyzed as 3 one-year cohorts defined above. We excluded patients who required mechanical support or reoperation at index admission. Baseline characteristics, and use of blood intraoperatively and postoperatively were compared between groups. RESULTS: The 3 groups had similar baseline characteristics. Blood use was significantly lower in year 2 after cell saver initiation as compared to the pre cell saver group both intra- and postoperatively. The median difference in volume of intraoperative blood transfusion was lower by 138 mL/m2 (-266, -10 mL/m2 ) in year 2 when compared to the pre cell saver group. Similarly, the proportion of subjects requiring red blood cell transfusion postoperatively on day of surgery was lower by 10% (-15%, -6%). CONCLUSION: Standardized use of cell saver significantly decreased perioperative blood use in children undergoing cardiac surgery at our center. A risk-adjusted transfusion threshold for children undergoing heart surgery needs to be developed to further decrease exposure to blood products and associated costs.

The Norwood Stage 1 procedure - conduct of perfusion: 2017 Survey results from NPC-QIC member institutions.

Hypoplastic left heart syndrome (HLHS) is a rare and severe congenital cardiac defect. Approximately 1000 infants are born with HLHS in the United States every year. Healthcare collaboratives over the last decade have focused on sharing patient experiences and techniques in an effort to improve outcomes. In 2010, cardiologists and patient families joined together to improve the care of HLHS patients by forming the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC). Sixty-six of the approximately 110 institutions caring for patients with HLHS in the United States and Canada are now members of NPC-QIC. In 2017, cardiovascular perfusionists joined the collaborative as another specialty involved in the care of HLHS patients. Perfusionists and cardiac surgeons developed the collaborative's first conduct of perfusion survey for the Norwood Stage 1 procedure, specifically targeting the provision of cardiopulmonary bypass for patients with HLHS. This manuscript discusses the results of this survey, unveiling a significant variance in the conduct of perfusion for this patient population.

Comparison of two pediatric cases requiring the use of bivalirudin during cardiopulmonary bypass.

INTRODUCTION: Comparison of two pediatric cases at our institution that utilized bivalirudin for anticoagulation on cardiopulmonary bypass (CPB); a bilateral lung transplant (BLT) and a ventricular assist device (VAD) implantation. METHODS: The same bivalirudin protocol was utilized in both cases with an initial bolus of 1 mg/kg administered by the anesthesia team, a 50 mg bolus in the pump prime at the time of the initial patient bolus and an initial infusion rate of 2.5 mg/kg/h, with titration as needed during CPB to maintain kaolin-activated clotting time (K-ACT) values >400 s. RESULTS: The BLT experienced high K-ACT levels (>720 s) for the majority of the case despite decreasing the bivalirudin infusion rate to 0.5 mg/kg/h. The VAD implantation case required the bivalirudin infusion rate to be increased to 5.0 mg/kg/h throughout the case due to low K-ACTs. CONCLUSION: The literature strongly supports a specific infusion rate1-7 (2.5 mg/kg/h) for bivalirudin anticoagulation during extracorporeal circulation. Clinicians must consider the loss of clotting factors and the administration of blood products while adjusting the bivalirudin infusion during bypass. We have now elected to maintain an infusion rate of ≥0.5 mg/kg/h for bivalirudin anticoagulation at our center, based on institutional experience, though consideration for a higher infusion rate for an added margin of safety should be considered. It is imperative to have a well-developed protocol for the management of these cardiopulmonary bypass patients and we offer our one-page timeline of events to help guide other pediatric centers looking to use bivalirudin anticoagulation.

Production Standard and Stability of Compounded Del Nido Cardioplegia Solution.

Background: The del Nido cardioplegia solution (dNCS) was originally developed for pediatric cardiac surgery, being now also used for adult patients. Hospital pharmacies frequently resort to internal dNCS production which has led to an increase in the need for validated parameters for compounding and storage. Objective: This report defines in-house production standards, as well as the stability of dNCS under optimal storage conditions. Methods: All ingredients were sterile and United States Pharmacopeia (USP)/National Formulary (NF) certified. All final bags were quarantined at 4°C for quality control, when 3 of 33 weekly bags were randomly assayed for potassium content. Each lot was only released if all 3 samples were within ±5% of target. Stability testing was performed per USP 797 guidance. Over a 6-month period, 4 different lots and 4 bags from each lot of dNCS were assayed. Each bag was assessed for physical and chemical stability while refrigerated at 4°C, at 35°C in an incubator, and at 70°C under 80% relative humidity. A light exposure arm was also set up at 25°C under 150 lumens. Calibrators of lidocaine, mannitol, and gluconate were freshly prepared and assayed with the samples by Liquid chromatography/Mass spectrometry (LC/MS). Results: Lidocaine concentrations averaged 0.117 mg/mL (95.8% of theoretical) at 4°C for 30 days. At 35°C, they decayed by 67% in 30 days, while at 70°C nearly 50% was lost after the first day. A first-order kinetics was observed with an Arrhenius activation energy of 25 kcal/mol. Degradation products identified under stress conditions were absent in the stable product. Conclusions: The dNCS is stable for at least 30 days under 4°C refrigeration in ethylene vinyl acetate (EVA) bags.

Bivalirudin Anticoagulation for a Pediatric Patient with Heparin-Induced Thrombocytopenia and Thrombosis Requiring Cardiopulmonary Bypass for Ventricular Assist Device Placement.

The direct thrombin inhibitor bivalirudin is an option for anticoagulation in patients with heparin induced thrombocytopenia (HIT) requiring cardiopulmonary bypass (CPB). There are a limited number of reports of pediatric patients in which bivalirudin has been used for anticoagulation for CPB. We present the case of an 11 year old male with acute onset heart failure secondary to idiopathic dilated cardiomyopathy that developed heparin induced thrombocytopenia with thrombosis (HITT). The patient was anticoagulated in the operating room with bivalirudin and placed on CPB for insertion of a HeartWare(®) Ventricular Assist Device (Heartware(®)). Modified techniques were utilized. This included use of the Terumo CDI 500 (Terumo Cardiovascular Systems, Inc.) in-line blood gas monitor which contains a heparin coated arterial shunt sensor. We flushed this sensor with buffered saline preoperatively and noted no significant decrease in platelet count postoperatively. The patient was successfully placed on the ventricular assist device and was subsequently listed for heart transplantation.

Arterial Limb Microemboli during Cardiopulmonary Bypass: Observations from a Congenital Cardiac Surgery Practice.

Gaseous microemboli (GME) are known to be delivered to the arterial circulation of patients during cardiopulmonary bypass (CPB). An increased number of GME delivered during adult CPB has been associated with brain injury and postoperative cognitive dysfunction. The GME load in children exposed to CPB and its consequences are not well characterized. We sought to establish a baseline of arterial limb emboli counts during the conduct of CPB for our population of patients requiring surgery for congenital heart disease. We used the emboli detection and counting (EDAC) device to measure GME activity in 103 consecutive patients for which an EDAC machine was available. Emboli counts for GME <40 µ and >40 µ were quantified and indexed to CPB time (minutes) and body surface area (BSA) to account for the variation in patient size and CPB times. Patients of all sizes had a similar embolic burden when indexed to bypass time and BSA. Furthermore, patients of all sizes saw a three-fold increase in the <40 µ embolic burden and a five-fold increase in the >40 µ embolic burden when regular air was noted in the venous line. The use of kinetic venous-assisted drainage did not significantly increase arterial limb GME. Efforts for early identification and mitigation of venous line air are warranted to minimize GME transmission to congenital cardiac surgery patients during CPB.

A Modified EXIT-to-ECMO with Optional Reservoir Circuit for Use during an EXIT Procedure Requiring Thoracic Surgery.

A 34 year old mother with a history of polyhydraminos and premature rupture of membranes presented for an ex utero intrapartum treatment (EXIT) procedure to deliver her 34 week gestation fetus. The fetus had been diagnosed with a large cervical mass which significantly extended into the right chest. The mass compressed and deviated the airway and major neck vessels posteriorly. Imaging also revealed possible tumor involvement with the superior vena cava and right atrium. The plan was for potential extracorporeal membrane oxygenation (ECMO) during the EXIT procedure (EXIT-to-ECMO) and the potential for traditional cardiopulmonary bypass (CPB) for mediastinal tumor resection. A Modified EXIT-To-ECMO with Optional Reservoir (METEOR) circuit was devised to satisfy both therapies. A fetal airway could not be established during the EXIT procedure and so the EXIT-to-ECMO strategy was utilized. The fetus was then delivered and transferred to an adjoining operating room (OR). Traditional cardiopulmonary bypass with a cardiotomy venous reservoir (CVR) was utilized during the establishment of an airway, tumor biopsy and partial resection. The patient was eventually transitioned to our institution's standard ECMO circuit and then transferred to the intensive care unit. The patient was weaned from ECMO on day of life (DOL) eight and had a successful tumor resection on DOL 11. The patient required hospitalization for numerous interventions including cardiac surgery at 4 months of age. She was discharged to home at 5 months of age.

Anesthetic Management of Cardiopulmonary Bypass in Hutchinson-Gilford Progeria Syndrome: A Case Report.

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultrarare disorder of segmental premature aging that is associated with the development of advanced atherosclerosis and significant cardiovascular and cerebrovascular disease. Treatment with lonafarnib has improved survival in patients with HGPS; however, in extended longitudinal follow-up, there has been an increase in the prevalence of rapidly progressive calcific aortic stenosis. The evolving course of HGPS has prompted reconsideration of conservative management and led to the development of strategies for anatomic treatment. In this case report, we describe the anesthetic management of patients with HGPS undergoing surgical management of aortic stenosis with cardiopulmonary bypass.

Mitochondrial transplantation preserves myocardial function and viability in pediatric and neonatal pig hearts donated after circulatory death.

OBJECTIVE: Mitochondrial transplantation has been shown to preserve myocardial function and viability in adult porcine hearts donated after circulatory death (DCD) . Herein, we investigate the efficacy of mitochondrial transplantation for the preservation of myocardial function and viability in neonatal and pediatric porcine DCD heart donation. METHODS: Circulatory death was induced in neonatal and pediatric Yorkshire pigs by cessation of mechanical ventilation. Hearts underwent 20 or 36 minutes of warm ischemia time (WIT), 10 minutes of cold cardioplegic arrest, and then were harvested for ex situ heart perfusion (ESHP). Following 15 minutes of ESHP, hearts received either vehicle (VEH) or vehicle containing isolated autologous mitochondria (MITO). A sham nonischemic group (SHAM) did not undergo WIT, mimicking donation after brain death heart procurement. Hearts underwent 2 hours each of unloaded and loaded ESHP perfusion. RESULTS: Following 4 hours of ESHP perfusion, left ventricle developed pressure, dP/dt max, and fractional shortening were significantly decreased (P < .001) in DCD hearts receiving VEH compared with SHAM hearts. In contrast, DCD hearts receiving MITO exhibited significantly preserved left ventricle developed pressure, dP/dt max, and fractional shortening (P < .001 each vs VEH, not significant vs SHAM). Infarct size was significantly decreased in DCD hearts receiving MITO as compared with VEH (P < .001). Pediatric DCD hearts subjected to extended WIT demonstrated significantly preserved fractional shortening and significantly decreased infarct size with MITO (P < .01 each vs VEH). CONCLUSIONS: Mitochondrial transplantation in neonatal and pediatric pig DCD heart donation significantly enhances the preservation of myocardial function and viability and mitigates against damage secondary to extended WIT.

Antibody depletion for the treatment of crossmatch-positive pediatric heart transplant recipients.

Sensitization to HLA is a risk factor for adverse outcomes after heart transplantation. Requiring a negative prospective CM results in longer waiting times and increased waitlist mortality. We report outcomes in a cohort of sensitized children who underwent transplant despite a positive CDC CM+ using a protocol of antibody depletion at time of transplant, followed by serial IVIG administration. All patients <21 yrs old who underwent heart transplantation at Boston Children's Hospital from 1/1998 to 1/2011 were included. We compared freedom from allograft loss, allograft rejection, and serious infection between CM+ and CM- recipients. Of 134 patients in the cohort, 33 (25%) were sensitized prior to transplantation and 12 (9%) received a CM+ heart transplant. Serious infection in the first post-transplant year was more prevalent in the CM+ patients compared with CM- patients (50% vs. 16%; p = 0.005), as was HD-AMR (50% vs. 2%; p < 0.001). There was no difference in freedom from allograft loss or any rejection. At our center, children transplanted despite a positive CM had acceptable allograft survival and risk of any rejection, but a higher risk of HD-AMR and serious infection.