Connecting Transcriptomics with Computational Modeling to Reveal Developmental Adaptations in the Human Pediatric Myocardium.

Background: Nearly 1% or 1.3 million babies are born with congenital heart disease (CHD) globally each year - many of whom will require palliative or corrective heart surgery within the first few years of life. A detailed understanding of cardiac maturation can help to expand our knowledge on cardiac diseases that develop during gestation, identify age-appropriate cardiovascular drug therapies, and inform clinical care decisions related to surgical repair, myocardial preservation, or postoperative management. Yet, to date, our knowledge of the temporal changes that cardiomyocytes undergo during postnatal development is largely limited to animal models. Methods: Right atrial tissue samples were collected from n=117 neonatal, infant, and pediatric patients undergoing correct surgery due to (acyanotic) CHD. Patients were stratified into five age groups: neonate (0-30 days), infant (31-364 days), toddler to preschool (1-5 years), school age (6-11 years), and adolescent to young adults (12-32 years). We measured age-dependent adaptations in cardiac gene expression, and used computational modeling to simulate action potential and calcium transients. Results: Enrichment of differentially expressed genes (DEG) was explored, revealing age-dependent changes in several key biological processes (cell cycle, cell division, mitosis), cardiac ion channels, and calcium handling genes. Gene-associated changes in ionic currents exhibited both linear trends and sudden shifts across developmental stages, with changes in calcium handling ( I NCX ) and repolarization ( I K1 ) most strongly associated with an age-dependent decrease in the action potential plateau potential and increase in triangulation, respectively. We also note a shift in repolarization reserve, with lower I Kr expression in younger patients, a finding likely tied to the increased amplitude of I Ks triggered by elevated sympathetic activation in pediatric patients. Conclusion: This study provides valuable insights into age-dependent changes in human cardiac gene expression and electrophysiology among patients with CHD, shedding light on molecular mechanisms underlying cardiac development and function across different developmental stages.

Enhancing Congenital Cardiac Postprocedural Care: A QR Code-Enabled Medical Bracelet Pilot Study.

The study aimed to evaluate the feasibility of using QR code-enabled medical bracelets for congenital heart disease (CHD) patients after hospital discharge to ensure quick communication of vital information to other medical personnel in emergency situations. A prospective study was conducted where QR code-enabled medical bracelets were given to families of postoperative pediatric cardiac patients. The QR code linked to a secure medical information sheet detailing the patient's cardiac history. Post-study surveys were completed by providers and families to assess their experiences with the bracelet. Of the 20 participants enrolled, 65% used the QR bracelet when seeking medical care. 55% found the bracelet useful, and 70% rated their experience as either "positive" or "very positive". Additionally, 80% recommended the bracelet for other patients undergoing cardiac procedures. The use of QR code bracelets for postoperative CHD patients has shown high levels of satisfaction from families and providers, potentially reducing medical errors and treatment delays.

Impact of new allocation policy on waitlist and transplant outcomes of adult congenital heart patients supported with ECMO.

BACKGROUND: The use of ECMO as a bridge to heart transplantation has been growing rapidly in all heart transplant recipients since the implementation of the new UNOS allocation policy; however, the impact on adult congenital heart disease (ACHD) patients is not known. METHODS: We analyzed the UNOS data (2015-2021) for ACHD patients supported with extracorporeal membrane oxygenation (ECMO) during the waitlist, before and after October 2018, to assess the impact on the waitlist and posttransplant outcomes. We compared the characteristics and outcomes of ACHD patients with or without ECMO use during the waitlist and pre- and postpolicy changes. RESULTS: A total of 23 821 patients underwent heart transplantation, and only 918 (4%) had ACHD. Out of all ACHD patients undergoing heart transplants, 6% of patients in the prepolicy era and 7.6% in the postpolicy era were on ECMO at the time of listing. Those on ECMO were younger and sicker compared to the rest of the ACHD cohort. Those on ECMO had similar profiles pre- and postpolicy change; however, there was a very significant decrease in the waitlist time [136 days (IQR 29-384) vs. 38 days (IQR 11-108), p = 0.01]. There was no difference in waitlist mortality; however, competing risk analyses showed a higher likelihood of transplantation (51% vs. 29%) and a lower likelihood of death or deterioration (31% vs. 42%) postpolicy change. Long-term outcomes posttransplant for those supported with ECMO compared to the non-ECMO cohort are similar for ACHD patients, although there was higher attrition in the first year for the ECMO cohort. CONCLUSION: The new allocation policy has resulted in shorter waitlist times and a higher likelihood of transplantation for ACHD patients supported by ECMO. However, the appropriate use of ECMO and the underuse of durable circulatory support devices in this population need further exploration.

Real-Time Detection of Circulating Thrombi in an Extracorporeal Circuit Using Doppler Ultrasound: In-Vitro Proof of Concept Study.

BACKGROUND: Thromboembolic stroke continues to be by far the most common severe adverse event in patients supported with mechanical circulatory assist devices. Feasibility of using Doppler ultrasound to detect circulating thrombi in an extracorporeal circuit was investigated. METHODS: A mock extracorporeal circulatory loop of uncoated cardiopulmonary bypass tubing and a roller pump was setup. A Doppler bubble counter was used to monitor the mean ultrasound backscatter signal (MUBS). The study involved two sets of experiments. In Scenario 1, the circuit was sequentially primed with human blood components, and the MUBS was measured. In Scenario 2, the circuit was primed with heparinized fresh porcine blood, and the MUBS was measured. Fresh blood clots (diameter <1,000 microns, 1,000-5,000 microns, >5,000 microns) were injected into the circuit followed by protamine administration. RESULTS: In Scenario 1 (n = 3), human platelets produced a baseline MUBS of 1.5 to 3.5 volts/s. Addition of packed human red blood cells increased the baseline backscatter to 17 to 21 volts/s. Addition of fresh frozen plasma did not change the baseline backscatter. In Scenario 2 (n = 5), the blood-primed circuit produced a steady baseline MUBS. Injection of the clots resulted in abrupt and transient increase (range: 3-30 volts/s) of the baseline MUBS. Protamine administration resulted in a sustained increase of MUBS followed by circuit thrombosis. CONCLUSIONS: Doppler ultrasound may be used for real-time detection of circulating solid microemboli in the extracorporeal circuit. This technology could potentially be used to design safety systems that can reduce the risk of thromboembolic stroke associated with mechanical circulatory support therapy.

In vitro investigation of axial mechanical support devices implanted in the novel convergent cavopulmonary connection Fontan.

OBJECTIVES: The 2 opposing inflows and 2 outflows in a total cavopulmonary connection make mechanical circulatory support (MCS) extremely challenging. We have previously reported a novel convergent cavopulmonary connection (CCPC) Fontan design that improves baseline characteristics and provides a single inflow and outflow, thus simplifying MCS. This study aims to assess the feasibility of MCS of this novel configuration using axial flow pumps in an in vitro benchtop model. METHODS: Three-dimensional segmentations of 12 single-ventricle patients (body surface area 0.5-1.75 m2) were generated from cardiovascular magnetic resonance images. The CCPC models were designed by connecting the inferior vena cava and superior vena cava to a shared conduit ascending to the pulmonary arteries, optimized in silico. The 12 total cavopulmonary connection and their corresponding CCPC models underwent in vitro benchtop characterization. Two MCS devices were used, the Impella RP® and the PediPump. RESULTS: MCS successfully and symmetrically reduced the pressure in both vena cavae by >20 mmHg. The devices improved the hepatic flow distribution balance of all CCPC models (Impella RP®P = 0.045, PediPump P = 0.055). CONCLUSIONS: The CCPC Fontan design provides a feasible MCS solution for a failing Fontan by balancing hepatic flow distribution and symmetrically decompressing the central venous pressure. Cardiac index may also improve with MCS. Additional studies are needed to evaluate this concept for managing Fontan failure.

Perioperative Morbidity and Outcomes in Pediatric Patients Transitioned From Extracorporeal Membrane Oxygenation to Ventricular Assist Device Support: A Study of the Society of Thoracic Surgeons Congenital Heart Surgery Database.

As a bridge to transplant strategy, children transitioned from extracorporeal membrane oxygenation (ECMO) to ventricular assist device (VAD) have higher waitlist mortality compared with those who receive de novo VAD. However, the contribution of the immediate perioperative period and differences in the two groups are not well studied. We performed a nested case-control study between children receiving de novo VAD (group 1) and those transitioned from ECMO to VAD (group 2) between 2014 and 2019 using The Society of Thoracic Surgeons (STS) database. A total of 735 children underwent VAD placement with 498 in group 1 and 237 in group 2. Patients in group 2 were significantly younger, smaller, and significantly sicker, were twice as likely to transition to biventricular VAD and need unplanned reoperations. Overall mortality was 16% for group 1 and 34% for group 2 ( p < 0.01). Regression analysis showed that ECMO use (odds ratio [OR], 2.17 [1.3-3.4]), ventilator need (OR, 2.2 [1.3-3.9]), and cardiogenic shock (OR, 1.8 [1.2-2.8]) were all independent preoperative predictors of VAD mortality while dialysis need (OR, 25.5 [8.6-75.3]), stroke (OR, 6.2 [3.1-12.6]), and bleeding (OR, 1.9 [1.1-3.4]) were independent postoperative predictors of VAD mortality within 30 days (all p < 0.05). The study demonstrated significant baseline differences between the two cohorts, warranting avoidance of comparison. Early elective VAD placement in this cohort of patients should be sought to avoid interim ECMO and high post-VAD mortality.

Clinical Profile and Outcomes of Rheumatic Heart Disease Patients Undergoing Surgical Valve Procedures in Uganda.

Background: Chronic valvular heart disease is a well-known, long-term complication of acute rheumatic fever (ARF), which remains a major public health problem in low- and middle-income countries. Access to surgical management remains limited. Outcomes of the minority proportion of patients that access surgery have not been described in Uganda. Objectives: To describe the volume and type of rheumatic heart disease (RHD) valvular interventions and the outcomes of operated patients in the Uganda RHD registry. Methods: We performed a retrospective cohort study of all valve surgery procedures identified in the Uganda RHD registry through December 2021. Results: Three hundred and sixty-seven surgical procedures were performed among 359 patients. More than half were among young (55.9% were ≤20 years of age), female (59.9%) patients. All patients were censored at 15 years. The median (IQR) follow up period was 43 (22,79) months. Nearly half of surgeries (46.9%) included interventions on multiple valves, and most valvular operations were replacements with mechanical prostheses (96.6%). Over 70% of the procedures were sponsored by charity organizations. The overall mortality of patients who underwent surgery was 13% (47/359), with over half of the mortalities occurring within the first year following surgery (27/47; 57.4%). Fifteen-year survival or freedom from re-operation was not significantly different between those receiving valve replacements and those receiving valve repair (log-rank p = 0.76). Conclusions: There has been increasing access to valve surgery among Ugandan patients with RHD. Post-operative survival is similar to regionally reported rates. The growing cohort of patients living with prosthetic valves necessitates national expansion and decentralization of post-operative care services. Major reliance on charity funding of surgery is unsustainable, thus calling for locally generated and controlled support mechanisms such as a national health insurance scheme. The central illustration (Figure 1) provides a summary of our findings and recommendations.

Children with single ventricle heart disease have a greater increase in sRAGE after cardiopulmonary bypass.

INTRODUCTION: Reducing cardiopulmonary bypass (CPB) induced inflammatory injury is a potentially important strategy for children undergoing multiple operations for single ventricle palliation. We sought to characterize the soluble receptor for advanced glycation end products (sRAGE), a protein involved in acute lung injury and inflammation, in pediatric patients with congenital heart disease and hypothesized that patients undergoing single ventricle palliation would have higher levels of sRAGE following bypass than those with biventricular physiologies. METHODS: This was a prospective, observational study of children undergoing CPB. Plasma samples were obtained before and after bypass. sRAGE levels were measured and compared between those with biventricular and single ventricle heart disease using descriptive statistics and multivariate analysis for risk factors for lung injury. RESULTS: sRAGE levels were measured in 40 patients: 19 with biventricular and 21 with single ventricle heart disease. Children undergoing single ventricle palliation had a higher factor and percent increase in sRAGE levels when compared to patients with biventricular circulations (4.6 vs. 2.4, p = 0.002) and (364% vs. 181%, p = 0.014). The factor increase in sRAGE inversely correlated with the patient's preoperative oxygen saturation (Pearson correlation (r) = -0.43, p = 0.005) and was positively associated with red blood cell transfusion (coefficient = 0.011; 95% CI: 0.004, 0.017; p = 0.001). CONCLUSIONS: Children with single ventricle physiology have greater increase in sRAGE following CPB as compared to children undergoing biventricular repair. Larger studies delineating the role of sRAGE in children undergoing single ventricle palliation may be beneficial in understanding how to prevent complications in this high-risk population.

Cardiac surgical outcomes of patients with heterotaxy syndrome.

Objectives: The study objectives were to analyze the outcomes of pediatric patients with heterotaxy syndrome undergoing cardiovascular surgery and to determine the predictors of mortality. Methods: A retrospective analysis of 82 patients diagnosed with heterotaxy syndrome who underwent cardiovascular surgery between January 2008 and December 2017 was performed. Univariate and multivariable Cox regression analyses to determine risk factors for mortality and Kaplan-Meier analysis for survival were performed. Results: Patient mortality in the cohort was 34% (28/82), including 36% (20/55) for single ventricle palliation and 30% (8/27) for biventricular repair. At 5 years, the probability of survival did not differ between the groups by log-rank testing (P = .829). Multivariable analysis found extracorporeal membrane oxygenation support (hazard ratio, 10.4; 95% confidence interval, 4.3-25.4; P < .001), total anomalous pulmonary venous return (hazard ratio, 4.3; 95% confidence interval, 1.7-10.8; P = .002), and birth weight 2.5 kg or less (hazard ratio, 2.4; 95% confidence interval, 1.0-5.4; P = .041) to be independent risk factors for mortality in all-comers. Pulmonary vein stenosis was a univariate predictor of mortality among all patients with heterotaxy (hazard ratio, 3.0; 95% confidence interval, 1.4-6.4; P = .005) and in the subgroup of patients with single ventricles (hazard ratio, 4.0; 95% confidence interval, 1.7-9.7; P = .002). Overall survival was 66% (54/82) at a median follow-up time of 2.2 years (0.4-4.1) from the initial surgery. Conclusions: Outcomes of children with heterotaxy syndrome, irrespective of the operative pathway, remain suboptimal in the current era. Risk factors for mortality in this population include birth weight 2.5 kg or less, extracorporeal membrane oxygenation, pulmonary vein stenosis, and total anomalous pulmonary venous return, which may help to further optimize surgical decision making. Multiorgan system involvment is frequently encountered in these patients.

Fate of aortic regurgitation after isolated repair of ventricular septal defect with concomitant aortic regurgitation in children.

Objectives: The aim of the study was to evaluate the course of aortic valve regurgitation in patients with preoperative aortic valve regurgitation and ventricular septal defect who underwent repair of the ventricular septal defect without aortic valve repair. Methods: A total of 37 consecutive patients with a ventricular septal defect and aortic regurgitation who underwent surgery between April 2007 and March 2016 were included in the study. Demographic, echocardiographic, operative, and clinical data were reviewed. Early and late mortality and morbidity were analyzed. Aortic regurgitation grade, left ventricular function, and dimensions were compared between the preoperative transesophageal echocardiography and postoperative transthoracic echocardiogram at last follow-up. Multivariate logistic regression analysis was performed to determine factors associated with improvement of aortic valve function. Results: There was no early or late mortality. No reoperations or reinterventions were required. A total of 17 patients had mild or greater aortic regurgitation preoperatively. Only 5 patients had mild or greater aortic regurgitation at follow-up of 4.3 years (0.5-10.1). Twenty-eight (76%) of the 37 patients showed an improvement in their aortic regurgitation grade. Left ventricular end-systolic and end-diastolic diameter z-scores were significantly lower at follow-up (P = .007 and P = .001, respectively). Multivariable logistic regression identified low preoperative left ventricular ejection fraction as the only predictor of nonimprovement of aortic regurgitation (95% confidence interval, 0.732-0.999, P = .002). Conclusions: Repair of a ventricular septal defect with accompanying aortic regurgitation can be performed with excellent results without surgical intervention on the aortic valve. Accompanying aortic regurgitation, especially trivial to mild, at the time of ventricular septal defect repair improves in the majority of cases. Low preoperative left ventricular ejection fraction is predictive of nonimprovement of aortic regurgitation grade.

The convergent cavopulmonary connection: A novel and efficient configuration of Fontan to accommodate mechanical support.

Objective: The current total cavopulmonary connection Fontan has competing inflows and outflows, creating hemodynamic inefficiencies that contribute to Fontan failure and complicate placement and efficiency of mechanical circulatory support. We propose a novel convergent cavopulmonary connection (CCPC) Fontan design to create a single, converged venous outflow to the pulmonary arteries, thus increasing efficiency and mechanical circulatory support access. We then evaluate the feasibility and hemodynamic performance of the CCPC in various patient sizes using computational fluid dynamic assessments of computer-aided designs. Methods: Cardiac magnetic resonance imaging data from 12 patients with single ventricle (10 total cavopulmonary connection, 2 Glenn) physiology (body surface area, 0.5-2.0 m2) were segmented to create 3-dimensional replicas of all thoracic structures. Surgically feasible CCPC shapes within constraints of anatomy were created using iterative computational fluid dynamic and clinician input. Designs varied based on superior and inferior vena cava conduit sizes, coronal attachment height, coronal entry angle, and axial entry angle of the superior vena cava to the inferior vena cava. CCPC designs were optimized based on efficiency (indexed power loss), risk of arteriovenous malformations (hepatic flow distribution), and risk of flow stasis (% nonphysiologic wall shear stress). Results: All CCPC designs met hemodynamic performance thresholds for indexed power loss and hepatic flow distribution. CCPC designs showed improvements in reducing % nonphysiologic wall shear stress and balancing HFD. Conclusions: CCPC is physiologically and surgically feasible in various patient sizes using validated computational fluid dynamic models. CCPC configuration has analogous indexed power loss, hepatic flow distribution, and % nonphysiologic wall shear stress compared with total cavopulmonary connection, and the single inflow and outflow may ease mechanical circulatory support therapies. Further studies are required for design optimization and mechanical circulatory support institution.

Modifications to therapeutic plasma exchange to achieve rapid exchange on cardiopulmonary bypass prior to pediatric cardiac transplant.

BACKGROUND: Cardiac transplants increasingly occur following placement of ventricular assist devices (VADs). A strong association exists between human leukocyte antigen (HLA) sensitization and VAD placement; however, desensitization protocols that utilize therapeutic plasma exchange (TPE) are fraught with technical challenges and are at increased risk of adverse events. In response to increased VAD utilization in our pre-transplant population, we developed a new institutional standard for TPE in the operating room. METHODS: Through a multidisciplinary effort, we developed an institutional protocol for intraoperative TPE immediately prior to cardiac transplantation after cannulation onto cardiopulmonary bypass (CPB). All procedures used the standard TPE protocol on the Terumo Optia (Terumo BCT, Lakewood, CO, USA), but incorporated multiple modifications to limit patients' bypass times, and to coordinate with the surgical teams. These modifications included deliberate misidentification of replacement fluid and maximization of the citrate infusion rate. RESULTS: These adjustments allowed the machine to run at maximal inlet speeds, minimizing duration of TPE. To date, 11 patients have been treated with this protocol. All survived their cardiac transplantation operation. Hypocalcemia and hypotension were noted; however, none of these adverse events appeared to have clinical impact. Technical complications included unexpected fibrin deposition in the TPE circuit and air in the inlet line due to surgical manipulation of the CPB cannula. No thromboembolic complications occurred in any patient. CONCLUSION: We feel that this procedure can be rapidly and safely performed in HLA sensitized pediatric patients on CPB to limit the risk of antibody mediated rejection of their heart transplant.

Transcatheter Closure of a Coronary Artery Fistula in a Neonate with Tetralogy of Fallot.

Coronary artery-to-pulmonary artery fistulae are a not uncommon finding in patients with Tetralogy of Fallot (TOF) and collateral-dependent pulmonary blood flow. Management for these fistulae is often primary surgical ligation or unifocalization at the time of complete repair, dependent on the presence of dual blood flow to the involved areas. We present the case of a 32-week premature boy weighing 1.79 kg with TOF, confluent branch pulmonary arteries, major aortopulmonary collaterals, and right coronary artery to main pulmonary artery fistula. The patient demonstrated evidence of coronary steal into the pulmonary vasculature with an elevation in the troponin level without hemodynamic instability, and subsequently underwent successful transcatheter occlusion of the fistula via right common carotid access using a Medtronic 3Q microvascular plug. This case demonstrates the realistic potential for early coronary steal in this physiology and possibility of transcatheter therapy even in a small neonate.

Influence of Body Mass Index in Donor-Recipient Size Mismatch in Pediatric Heart Transplantation.

BACKGROUND: Body weight is the traditional metric for matching donor and recipient size for pediatric heart transplantation (pHT). We hypothesized that mismatch in body mass index (BMI) or body surface area (BSA) rather than weight is better associated with outcomes of transplantation and therefore should be used for donor-recipient size matching. METHODS: Analysis of the United Network for Organ Sharing database limited to pHT recipients was performed. Donor and recipient mismatch groups were created for weight, BMI, and BSA ratios. Differences in recipient characteristics between each cohort and the impact of mismatch on outcomes were statistically analyzed. RESULTS: A total of 4,465 patients were included in the analysis of which 43% had congenital heart disease (CHD). There were significant differences in patient characteristics by matching, independent of the matching parameter. Multivariable regression analysis showed that a low donor-recipient BMI ratio (compared to normal) (CHD OR 1.70; non-CHD 2.78) was a predictor of one-year mortality (all P < .001) in both CHD and non-CHD cohorts. Low BMI ratio was also associated with worse long-term survival in non-CHD groups, but not in the CHD cohort. Weight and BSA ratio did not predict one year or long-term survival. CONCLUSION: The use of low BMI donors compared to recipient may predict poor early and long-term survival and therefore should be avoided in pHT. The use of BMI matching may improve donor-recipient matching in pHT.

Systematic Review of Slide Tracheoplasty Outcomes.

OBJECTIVE: To identify factors predicting success in slide tracheoplasty surgery at a regional children's hospital and compare with available published literature. MEASURES: Retrospective chart review comparing demographics (age, weight) and clinical (operative and hospital course, need for additional airway intervention) factors experienced with slide tracheoplasty. Findings were compared with a systematic review of published literature. RESULTS: Of the 16 tracheal stenosis patients in our cohort, 13 (81.3%) presented with an additional congenital or cardiovascular anomaly. When adjusted for cardiovascular anomalies, congenital tracheal stenosis patients had a mean age of 5.2 months (range 6 days-17 months), mean weight of 5.04 kg, and average ICU and hospital length of stay of 31.5 and 36.0 days, respectively. Tracheostomy was required for 4 patients and no early deaths were recorded. Of the 391 children in the grouped cohort, mean age and weight was older at 7.67 months and larger at 5.70 kg. Length of stay in both ICU and overall hospital course was 31.6 and 43.5 days, respectively. Mortality etiology for 44 patients was reported: 17 (38.6%) cardiac-related and 28 (63.6%) late mortalities. Our overall calculated mortality risk of 1.26 (P < .05) was lower than reported ratios of 2.0+. CONCLUSION: Despite the numerous institutional studies involving tracheal stenosis, mortality and surgical challenges remain high. Future studies with the inclusion of specific perioperative data can prove to further evaluate correlations between presentation characteristics and mortality.

Predicting Cardiac Anatomy, Physiology, and Surgical Management Based on Fetal Echocardiography in Heterotaxy Syndrome.

OBJECTIVE: Heterotaxy syndrome (HS) is often associated with complex congenital heart disease (CHD). While fetal echocardiography (FE) permits accurate prenatal identification of most CHD, the high level of disease complexity in HS may pose challenges in predicting postnatal findings and outcomes. This study aimed to define the accuracy of FE in predicting postnatal anatomy, physiology, and surgical management of CHD in the setting of HS. STUDY DESIGN: Retrospective single-center cohort study including all patients with a prenatal diagnosis of HS from 2003 to 2018. Anatomic diagnoses from FE reports were compared with postnatal echocardiogram, catheterization, and operative reports. Prenatal predictions were compared with postnatal outcomes with a focus on ductal dependence, time to first intervention (immediate, neonatal period, 1-6 months, or older than 6 months), and surgical approach (single or biventricular). RESULTS: There were 102 pregnancies with fetal HS resulting in 21 terminations, 5 fetal losses, and 76 live births. Of the live births, 55 had significant CHD and available postnatal data for review. Among this group, survival to 1 year was 62% and was no different comparing single versus biventricular surgical approach. FE diagnostic accuracy varied by anatomic feature and was the lowest for diagnosis of venous anatomy. Determination of postnatal care was most accurate for predicting single versus biventricular surgical approach (91%), followed by ductal dependence (75%). Accuracy for predicting time to first intervention was the lowest at 69%. The most common reason for an incorrect prediction was difficulty in assessing the severity of pulmonary stenosis. CONCLUSION: FE permits accurate predictions regarding surgical approach. Characterizing systemic and pulmonary veins is challenging, as is predicting ductal dependence and time to first intervention. These data suggest that despite the high diagnostic accuracy of CHD in HS, a circumspect approach may be reasonable with regard to predicting some anatomic details and postnatal management decisions. KEY POINTS: · In HS, FE was most accurate for intracardiac anatomy.. · Diagnostic accuracy of venous anatomy was less reliable.. · Predicting surgical approach (single ventricle vs. biventricular) was highly accurate.. · Predicting ductal dependence and time-to-intervention were more challenging in some instances..

Effect of preoperative extracorporeal membrane oxygenation therapy on postventricular assist device outcomes: an analysis of the STS Pedimacs database.

OBJECTIVES: Extracorporeal membrane oxygenation (ECMO) support prior to ventricular assist device (VAD) therapy is frequently used for stabilizing INTERMACS 1 and 2 paediatric patients. Data regarding outcomes with this strategy is limited. METHODS: Patient characteristics and outcomes post-VAD therapy with and without preop ECMO support were compared. Survival and risk factor analysis was performed for all patients and INTERMACS profile 1 subgroup. RESULTS: Of 541 INTERMACS 1 and 2 patients enrolled in Paediatric Interagency Registry for Mechanical Circulatory Support registry, 391 received primary VAD implantation and ECMO prior to VAD therapy was utilized in 150 patients. Younger age (P < 0.0001) and shock (P < 0.0001) were more common in group 2, with lower survival at 1, 6 and 12 months compared to group 1 (P < 0.0001). Freedom from infection (P = 0.03) was higher in group 1. Freedom from stroke (P = 0.7) was similar. Paracorporeal continuous flow devices led to poor survival in both groups 1 and 2 (P = 0.4). Implantable continuous flow (P < 0.0001) and paracorporeal pulsatile devices (P = 0.007) had better survival in primary VAD group. INTERMACS profile 1, lower weight, hepatic dysfunction, renal failure, paracorporeal continuous and percutaneous devices were associated with higher mortality. Preop ECMO therapy was not associated with higher mortality (P = 0.12). Survival of INTERMACS profile 1 patient was similar at 1, 6 and 12 months in both groups (P = 0.1). CONCLUSIONS: INTERMACS profile 1, lower weight, hepatic dysfunction, renal failure and use of paracorporeal continuous flow or percutaneous assist devices are associated with a higher postoperative mortality. Preop ECMO therapy however is not independently associated with higher postop mortality.

A Smartphone application to provide real-time cardiopulmonary resuscitation quality feedback.

BACKGROUND: Quality of cardiopulmonary resuscitation (CPR) contributes significantly to outcomes. Key determinants of CPR quality pertaining to chest compressions are compression rate, compression depth, duration of interruptions, and chest recoil. Several studies have demonstrated that real-time audiovisual feedback improves CPR quality. We hypothesize that a mobile application using sensor data from built-in accelerometers in smartphones can provide accurate chest compression quality feedback in real time. This study aims to develop and validate an application for smartphone which can provide real-time audiovisual and haptic feedback on determinants of CPR quality. METHODS: A mobile application was developed to detect the compression depth and compression rate in real time using data captured from a smartphone's intrinsic accelerometer. The mobile device was placed on an adult manikin's chest at the point of compressions. In a simulated environment, data obtained using the application was compared directly to data obtained from a validated standard CPR quality tool. RESULTS: CPR quality parameters were obtained from the application and industry standard for 60, 30s-long sessions. Bland-Altman plot analysis for compression depth showed agreement between the app measurements and standard within ±4 mm (<10% error). The interclass correlation for agreement in the measurement of compression count was 0.92 (95% CI: 0.88-0.95), indicative of very strong agreement. CONCLUSIONS: Smart device applications using acceleration sensor data derived from smart phones can accurately provide real-time CPR quality feedback. With further development and validation, they can provide a ubiquitously available CPR feedback tool valuable for out-of-hospital arrests and in-hospital arrests in under-privileged areas.