Patient-tailored platelet transfusion practices for children supported by extracorporeal membrane oxygenation.

BACKGROUND AND OBJECTIVES: Extracorporeal membrane oxygenation (ECMO) serves as cardiopulmonary therapy in critically ill patients with respiratory/heart failure and often necessitates multiple blood product transfusions. The administration of platelet transfusions during ECMO is triggered by the presence or risk of significant bleeding. Most paediatric ECMO programmes follow guidelines that recommend a platelet transfusion threshold of 80-100 × 109/L. To reduce exposure to platelets, we developed a practice to dynamically lower the threshold to ~20 × 109/L. We describe our experience with patient-tailored platelet thresholds and related bleeding outcomes. MATERIALS AND METHODS: We retrospectively evaluated our platelet transfusion policy, bleeding complications and patient outcome in 229 ECMO-supported paediatric patients in our unit. RESULTS: We found that more than 97.4% of patients had a platelet count <100 × 109/L at some point during their ECMO course. Platelets were transfused only on 28.5% of ECMO days; and 19.2% of patients never required a platelet transfusion. The median lowest platelet count in children who had bleeding events was 25 × 109/L as compared to 33 × 109/L in children who did not bleed (p < 0.001). Our patients received fewer platelet transfusions and did not require more red blood cell transfusions, nor did they experience more haemorrhagic complications. CONCLUSION: We have shown that a restrictive, 'patient-tailored' rather than 'goal-directed' platelet transfusion policy is feasible and safe, which can greatly reduce the use of platelet products. Although there was a difference in the lowest platelet counts in children who bled versus those who did not, the median counts were much lower than current recommendations.

Three-Patch Reconstruction of Hourglass Supravalvar Pulmonary Artery Stenosis Mid-Term Results.

BACKGROUND: Some patients with pulmonary stenosis present with a complex multilevel disease that involves the valve, the leaflets, and the sinotubular junction (STJ) forming an hourglass appearance. We herein report the mid-term results of our experience with the reconstruction of the supravalvar narrowing using three pericardial patches. MATERIAL AND METHODS: Retrospective analysis of patient charts and echocardiography studies of patients who underwent three-patch reconstruction of the pulmonary valve (PV) from 2013 to 2022. After PV transection distal to STJ, vertical incisions into the sinuses were performed, and leaflets were trimmed and thinned. The three sinuses were augmented using three pericardial patches. RESULTS: Nineteen patients underwent repair of hourglass supravalvar pulmonary stenosis. Mean weight at surgery was 9.4 kg (median 7.2, range 4.7-35); 16 patients underwent previous catheterization with unsuccessful balloon dilatation of the PV (13 pts.). Preoperative aortic/pulmonary annulus ratio was 1.02 (median 1, range 0.89-1.25). After surgery, gradients across the PV were significantly reduced (94 ± 26 vs 29 ± 9 mm Hg, P = .02). Postoperatively, 14 patients had mild or no pulmonary insufficiency (PI) and five had mild to moderate PI. At a mean follow-up of 71 months (median 78 months, range 8-137), gradients continued to decrease (29 ± 9 vs 15 ± 5 mm Hg, P < .001). CONCLUSION: The three-patch technique for the repair of supravalvar pulmonary stenosis is simple, reproducible, and achieves excellent and long-standing relief of the right ventricular outflow tract gradient.

Pediatric Mechanical Circulatory Support: Introduction and Schneider's Experience.

BACKGROUND: Ventricular assist devices (VADs) play a critical and increasing role in treating end-stage heart failure in pediatric patients. A growing number of patients are supported by VADs as a bridge to heart transplantation. Experience with VADs in the pediatric population is limited, and experience in Israel has not been published. OBJECTIVES: To describe this life-saving technology and our experience with VAD implantation in children with heart failure, including characteristics and outcomes. METHODS: We conducted a retrospective chart review of all patients who underwent VAD implantation at Schneider Children's Medical Center from 2018 to 2023. RESULTS: We analyzed results of 15 children who underwent VAD implantation. The youngest was 2.5 years old and weighed 11 kg at implantation. In eight patients, HeartMate 3, a continuous-flow device, was implanted. Seven patients received Berlin Heart, a pulsatile-flow device. Three children required biventricular support; 11 underwent heart transplants after a median duration of 169 days. Two patients died due to complications while awaiting a transplant; two were still on VAD support at the time of submission of this article. Successful VAD support was achieved in 86.6% of patients. In the last 5 years,79% of our heart transplant patients received VAD support prior to transplant. CONCLUSIONS: Circulatory assist devices are an excellent bridge to transplantation for pediatric patients reaching end-stage heart failure. VADs should be carefully selected, and implantation techniques tailored to patient's weight and diagnosis at a centralized pediatric cardiac transplantation center. Israeli healthcare providers should be cognizant of this therapeutic alternative.

Low Risk of Inappropriate Shock Among Pediatric Patients With an Implantable Cardioverter Defibrillator: A Single Center Experience.

Implantable cardioverter-defibrillators (ICD) are increasingly being used among the pediatric population for indications of both primary and secondary prevention. There is limited long-term data on the outcomes of pediatric patients following ICD implantation. The aim of this study was to investigate the characteristics of this population, burden of appropriate and inappropriate shock and complication rate in a large tertiary pediatric medical center. Included were children under the age of 18 years who underwent ICD implantation and had clinical follow up at our center. Data were retrospectively collected between study period 2005-2020. Primary outcome was the incidence of ICD shock appropriate and inappropriate. Secondary outcome was defining our patient population characteristics. Our cohort included 51 patients who underwent ICD implantation. Mean age at implantation was 10.9 ± 4.7 years and average follow-up time was 67 months. Diagnoses of implanted patients were: 28 (55%) patients with syndromes with risk for sudden death, cardiomyopathy in 14 patients (27%) and congenital heart disease (CHD) in 9 patients (18%). Forty-two (82%) patients had an ICD implanted for secondary prevention after experiencing a life-threatening arrhythmia and 9 (18%) for primary prevention. An endocardial system was implanted in 39 (76%) patients and an epicardial systems in 12 (24%) patients. A total of 20 (39%) patients received appropriate shocks for ventricular fibrillation(VF). 5 patients received inappropriate shocks, 4 due to sinus tachycardia and 1 due to rapidly conducted atrial fibrillation. Those who received an inappropriate shock had a significantly shorter ICD-programmed VF detection cycle length compared to those who did not receive an inappropriate shock (320 ms versus 270 ms, p = 0.062). This single center study demonstrates a high rate of appropriate ICD shocks (39%) and a low rate of inappropriate ICD shocks. Accurate programming of ICD devices in the pediatric population is paramount to avoid inappropriate ICD shocks.

The Amplatzer duct occluder (ADOII) and Piccolo devices for patent ductus arteriosus closure: a large single institution series.

Purpose: Evaluate Piccolo and ADOII devices for transcatheter patent ductus arteriosus (PDA) closure. Piccolo has smaller retention discs reducing risk of flow disturbance but residual leak and embolization risk may increase. Methods: Retrospective review of all patients undergoing PDA closure with an Amplatzer device between January 2008 and April 2022 in our institution. Data from the procedure and 6 months follow-up were collected. Results: 762 patients, median age 2.6 years (range 0-46.7) years and median weight 13 kg (range 3.5-92) were referred for PDA closure. Overall, 758 (99.5%) had successful implantation: 296 (38.8%) with ADOII, 418 (54.8%) with Piccolo, and 44 (5.8%) with AVPII. The ADOII patients were smaller than the Piccolo patients (15.8 vs. 20.5 kg, p < 0.001) and with larger PDA diameters (2.3 vs. 1.9 mm, p < 0.001). Mean device diameter was similar for both groups. Closure rate at follow-up was similar for all devices ADOII 295/296 (99.6%), Piccolo 417/418 (99.7%), and AVPII 44/44 (100%). Four intraprocedural embolizations occurred during the study time period: two ADOII and two Piccolo. Following retrieval the PDA was closed with an AVPII in two cases, ADOI in one case and with surgery in the fourth case. Mild stenosis of the left pulmonary artery (LPA) occurred in three patients with ADOII devices (1%) and one patient with Piccolo device (0.2%). Severe LPA stenosis occurred in one patient with ADOII (0.3%) and one with AVPII device (2.2%). Conclusions: ADOII and Piccolo are safe and effective for PDA closure with a tendency to less LPA stenosis with Piccolo. There were no cases of aortic coarctation related to a PDA device in this study.

Preceding Clinical Events in High-risk, Postoperative, Pediatric Cardiac Patients-A Novel Association With Bacteremia.

Objective: Blood stream infections (BSIs) are well described in pediatric cardiac intensive care units (PCICU). We noted that postoperative high-risk patients may develop BSI after a preceding clinical event (PCE). The study aim was to investigate whether high-risk patients who developed bacteremia experienced more PCEs than a similar group of high-risk patients. Design: Retrospective case-control study. Setting: Referral pediatric center. Patients: We enrolled patients who developed bacteremia from March 2010 to November 2019, after undergoing open-heart surgery at a pediatric center. The control group was comprised of case-matched patients with immediate consecutive same surgery. Interventions: None. Measurements: We recorded operative data, common risk factors, postoperative indicators of organ dysfunction, mortality, and PCEs 72 to 24 h before bacteremia emerged. Main results: A total of 200 patients were included (100 with bacteremia and 100 controls). Key demographic and operative parameters were matched. Bacteremia emerged on average on postoperative day 12.8. Skin-associated Gram-positive bacteria were cultured in 10% and Gram-negative bacteria in 84% of the patients. Average central-venous lines (CVL) duration was 9.5 ± 8.4 days. Postoperatively (72 h), indicators of organ dysfunction were significantly worse in patients with bacteremia, with a higher rate of postoperative complications during PCICU length-of-stay (LOS). In the bacteremia group, 72 to 24 h prior to the development of bacteremia, 92 (92%) PCEs were recorded, as compared to 21 (21%) in controls during their entire LOS (odds ratio [OR] 43.3, confidence interval [CI] 18.2-103.1, P < .0001). Conclusions: We propose a 3-hit model demonstrating that high-risk patients undergoing open-heart surgery have significantly higher risk for bacteremia after a PCE.

Transthoracic intracardiac lines-A double edged sword.

OBJECTIVE: Routine use of central venous access is needed in children undergoing open heart surgery for pressure monitoring and inotrope infusion. We sought to evaluate the efficiency and safety of routine use of transthoracic intracardiac lines (ICLs) in patients undergoing cardiac surgery and to compare them to patients who have been previously treated with traditional central venous lines (non-ICLs). METHODS: Retrospective review of charts of all patients who underwent cardiac surgery and had an ICL inserted in the operating room. Case control matching was done with similar patient in which ICL was not inserted. Patients characteristics, diagnosis, operative, and intensive care data were collected for each patient and analyzed. RESULTS: A total number of 376 patient records were reviewed (198 ICL patients and 178 non-ICL patients). Umbilical line and non-ICL durations were longer in the non-ICL group. ICL duration was the longest of all lines, averaging 12.87 ± 10.82 days. The necessity for multiple line insertions (˃2 insertions) was significantly higher in the non-ICL group, with a relative risk ratio of 3.24 (95% confidence interval: 1.617-6.428). There was no statistical difference of infections rate and line complications between the two groups. CONCLUSION: ICLs are safe in infants undergoing cardiac surgery and can be kept in place for a long period of time with a low rate of line complications and infection. Routine use of ICLs reduces the number of central venous catheter placement in this complex patient population.

CMR Imaging 6 Months After Myocarditis Associated with the BNT162b2 mRNA COVID-19 Vaccine.

Temporal association between BNT162b2 mRNA COVID-19 vaccine and myocarditis (PCVM) has been reported. We herein present early and 6-month clinical follow-up and cardiac magnetic resonance imaging (CMR) of patients with PVCM. A retrospective collection of data from 15 patients with PCVM and abnormal CMR was performed. Clinical manifestation, laboratory data, hospitalizations, treatment protocols, and imaging studies were collected early (up to 2 months) and later. In nine patients, an additional CMR evaluation was performed 6 months after diagnosis. PCVM was diagnosed in 15 patients, mean age 17 ± 1 (median 17.2, range 14.9-19 years) years, predominantly in males. Mean time from vaccination to onset of symptoms was 4.4 ± 6.7 (median 3, range 0-28) days. All patients had CMR post diagnosis at 4 ± 3 (median 3, range 1-9) weeks, 4/5 patients had hyper enhancement on the T2 sequences representing edemaQuery, and 12 pathological Late glandolinium enhancement. A repeat scan performed after 5-6 months was positive for scar formation in 7/9 patients. PCVM is a rare complication, affecting predominantly males and appearing usually within the first week after administration of the second dose of the vaccine. It usually is a mild disease, with clinical resolution with anti-inflammatory treatment. Late CMR follow up demonstrated resolution of the edema in all patients, while some had evidence of residual myocardial scarring.

Mid-term results of vascular ring surgery.

BACKGROUND: Vascular rings cause respiratory symptoms in children. Treatment consists of surgical division; however, data regarding mid-term results are scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery. METHODS: Retrospective chart review of consecutive patients who underwent vascular ring surgery. Mid-term follow-up consisted of clinic visits and telephone questionnaire over a 1-year period sampling at five points in time. RESULTS: Follow-up of 85 patients who underwent vascular rings surgery revealed significant symptomatic improvement within 6 months. In total, 50% were symptomatic to some degree at their last documented clinic visit complaining of stridor (36.8%), cough (34.2%), wheezing (10.5%), dyspnoea on exertion (10.5%), or recurrent respiratory infections (23.7%). By telephone questionnaires, 83% described a significant improvement in respiratory symptoms, 63.4% had some residual symptoms, 36.6% stridor, 38.8% chronic cough, 32.4% bronchodilator use,18.3% had at least one episode of pneumonia per year, 31% of children had dyspnoea or respiratory limitation, and 12.7% while doing physical activities. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. CONCLUSIONS: Surgical division of vascular rings results in significant clinical improvement within 1 year; nevertheless, some patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. Further evaluation is warranted to assess the nature of residual symptoms and explore whether anatomical causes can be identified leading to surgical modifications.

Aerodigestive Clinics as Emerging Pediatric Care Model: The First 100 Patients in Israel.

BACKGROUND: Aerodigestive clinics are run by interdisciplinary medical and surgical teams, and provide complex care coordination and combined endoscopies. OBJECTIVES: To describe the design and patient population of the first pediatric aerodigestive center in Israel. METHODS: A retrospective single-center cohort study was conducted describing patients followed in the aerodigestive clinic of Schneider Children's Medical Center of Israel, a tertiary pediatric hospital, between its inception in January 2017 and June 2020. RESULTS: During the study period, 100 patients were seen at the combined respiratory and digestive (NoAM) clinic, with a total of 271 visits. Median age at first assessment was 29.5 months (range 3-216). Fifty-six patients (56%) had esophageal atresia and tracheoesophageal fistula. Thirty-nine patients had an identified genetic disorder, 28 had a primary airway abnormality, 28 were oxygen dependent, and 21 were born premature. Fifty-two patients underwent triple endoscopy, consisting of flexible bronchoscopy, rigid bronchoscopy, and gastroscopy. In 33 patients, esophageal dilatation was necessary. Six patients underwent posterior tracheopexy at a median of 6 months of age (range 5 days to 8 years) all with ensuing symptom improvement. The total mean parental satisfaction score on a Likert-type scale of 1-5 (5 = highest satisfaction) was 4.5. CONCLUSIONS: A coordinated approach is required to provide effective care to the growing population of children with aerodigestive disorders. The cross fertilization between multiple disciplines offers a unique opportunity to develop high quality and innovative care. Outcome measures must be defined to objectively measure clinical benefit.

Clinical Features and Comparison of Kingella and Non-Kingella Endocarditis in Children, Israel.

Kingella spp. have emerged as an important cause of invasive pediatric diseases. Data on Kingella infective endocarditis (KIE) in children are scarce. We compared the clinical features of pediatric KIE cases with those of Streptococcus species IE (StIE) and Staphylococcus aureus IE (SaIE). A total of 60 patients were included in the study. Throughout the study period, a rise in incidence of KIE was noted. KIE patients were significantly younger than those with StIE and SaIE, were predominately boys, and had higher temperature at admission, history of oral aphthae before IE diagnosis, and higher lymphocyte count (p<0.05). Pediatric KIE exhibits unique features compared with StIE and SaIE. Therefore, in young healthy children <36 months of age, especially boys, with or without a congenital heart defect, with a recent history of oral aphthae, and experiencing signs and symptoms compatible with endocarditis, Kingella should be suspected as the causative pathogen.

Transposition of the Great Arteries-Are We Doing Better? Correlating Outcome to Change in Renal Function Over 2 Decades of Arterial Switch Operation.

OBJECTIVES: It is believed that management of neonates with dextro-transposition of the great arteries is constantly improving. Renal function may play a role in the prognosis of patients after congenital heart surgery. The aim of this study was to describe the outcome of neonates who underwent arterial switch operation during the past 2 decades using renal function as a surrogate marker for morbidity and mortality. DESIGN: Retrospective cohort study. SETTING: Dedicated cardiac ICU of a university-affiliated pediatric medical center. PATIENTS: Infants who underwent arterial switch surgery in 1993-2015. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The cohort included 336 infants who underwent arterial switch operation for dextro-transposition of the great arteries (n = 169, 50%), transposition of the great arteries/ventricular septal defect (n = 133, 40%), or Taussig-Bing anomaly (n = 34, 10%). Between 1993-1998 and 2012-2015, the mean minimal postoperative estimated glomerular filtration rate rose from 30 mL/min/1.73 m to 40 mL/min/1.73 m (p < 0.05), and the proportion of patients with estimated glomerular filtration rate less than 30 mL/min/1.73 m decreased from 56% to 23% (p < 0.05). The daily furosemide dosage decreased from 4 mg/kg/d to 0.5 mg/kg/d (p < 0.05). Urinary output on operative day 0 decreased over time, but urinary output on operative day 2 significantly increased. Maximal lactate levels and time to lactate normalization decreased steadily. Dialysis was performed in only a few patients in the early periods, and in none in the last 6 years. The mean mortality rate of patients with dextro-transposition of the great arteries and transposition of the great arteries/ventricular septal defect decreased to 2.7% in the last 6 years. The odds ratio of a prolonged hospital stay (≥ 28 d) in a patient with estimated glomerular filtration rate less than 30 mL/min/1.73 m was 18.79, and in a patient with transposition of the great arteries/ventricular septal defect, 3.39. The odds ratio of dying after Rashkind atrial septostomy was 4.42. CONCLUSIONS: During the past 2 decades, there has been significant improvement in outcome of patients undergoing transposition of the great arteries repair. Renal function was found to be a good prognostic marker of morbidity and mortality.

The Utility of Albumin Level as a Marker of Postoperative Course in Infants Undergoing Repair of Congenital Heart Disease.

We sought to examine the role of preoperative and 2nd postoperative day albumin levels as predictors for postoperative course in infants undergoing repair of congenital heart disease. This retrospective, single-center, observational study comprised consecutive infants younger than 1 year who had undergone repair of tetralogy of Fallot, ventricular septal defect, complete atrioventricular canal or transposition of the great arteries over a 25 months period. We correlated preoperative and postoperative day (POD) #2 albumin level to vaso-inotropic score (VIS) and intensive care unit (ICU) length of stay (LOS) as markers for degree and duration of postoperative cardiac support. A composite outcome was defined as maximal vaso-inotropic score of > 10 and ICU LOS > 96 h. Preoperative albumin level negatively correlated with VIS and ICU LOS. Compared to preoperative albumin level of > 4 g/dL, the relative risk of meeting composite criteria was 1.5 for preoperative albumin of 3.1-4 g/dL and 2.6 for preoperative albumin ≤ 3 g/dL. Compared to POD#2 albumin level > 3 g/dL, the relative risk of meeting composite criteria was 1.8 for albumin of 2.6-3 g/dL, and 2.5 for albumin ≤ 2.5 g/dL. In summary, we found that preoperative and POD#2 albumin levels predicted prolonged and complicated postoperative course. These finding may help clinicians to inform the patient's parents, early in the ICU hospitalization, as to the predicted risks and difficulties of their infant's postoperative course.

Pediatric Extracorporeal Membrane Oxygenation Reach-Out Program: Successes and Insights.

The shortage of dedicated pediatric extracorporeal membrane oxygenation (ECMO) centers and the expanding indications for pediatric ECMO necessitate a regional program for transport of ECMO-supported patients. Data about feasibly and safety of pediatric ECMO transport are scarce. Our aim is to describe our experience with a pediatric ECMO reach-out program and review pertinent literature. Demographic, clinical, and outcome data were collected retrospectively from the charts of all patients cannulated onto ECMO at referring centers and transported to our center from 2003 to 2018. Similar data were recorded for patients who were referred for ECMO support from within the hospital. The cohort included 80 patients cannulated at 17 referring centers. The transport team included a senior pediatric cardiac surgeon and an ECMO specialist. All transfers but one were done by special emergency medical service ambulance. No major complications or deaths occurred during transport, and all patients were stable upon arrival to our unit. Mortality was lower in the ECMO reach-out cohort than in-house patients referred for ECMO support. This is the first study from Israel and one of the largest to date describing a dedicated pediatric ECMO transport program. Extracorporeal membrane oxygenation transport appears to be feasible and safe when conducted by a small, highly skilled mobile team. Successful reach-out program requires open communication between the referring physician and the accepting center. As survival correlates with ECMO volume, maintaining a large ECMO center with 24/7 retrieval capabilities may be the best strategy for pediatric mechanical circulatory support program.

Vacuum-Assisted Closure for the Treatment of Deep Sternal Wound Infection After Pediatric Cardiac Surgery.

OBJECTIVES: Vacuum-assisted closure is being increasingly used to treat deep sternal wound infection following cardiac surgery, but most of the data refer to adults. This study investigated the safety and efficacy of vacuum-assisted closure in pediatric patients. DESIGN: Retrospective file review. SETTING: Tertiary pediatric medical center. PATIENTS: All children with deep sternal wound infection treated with vacuum-assisted closure in 2003-2016. INTERVENTIONS: Epidemiological, clinical, and microbiological data were collected from the medical records. MEASUREMENTS AND MAIN RESULTS: The cohort included 50 patients (0.9% of cardiac patients operated during the study period) of median age 6.5 months (interquartile range, 2-12.75 mo; range, 1 wk to 14 yr) and median weight 5.1 kg (interquartile range, 4-9.75 kg). The most frequent heart defects were tetralogy of Fallot (22%) and ventricular septal defect (20%); 38% of patients had cyanotic heart disease. Deep sternal wound infections appeared a median of 10 days postoperatively (interquartile range, 7-14 d; range 3-100 d). Vacuum-assisted closure was applied a median of 13 days postoperatively (interquartile range, 10-18.5 d; range, 5-103 d) for a median duration of 10 days (interquartile range, 7-13.25 d; range, 1-21 d). Wound cultures were positive in 48 patients (96%); most isolates were Gram-positive (76%). The main bacterial pathogen was methicillin-susceptible Staphylococcus aureus (61%). Most patients were treated with cloxacillin for a median of 38 days (interquartile range, 28-42 d; range, 9-189 d). There were no statistically significant differences in clinical or treatment characteristics between bacteremic (56%) and nonbacteremic patients. Compared with older patients, infants less than 3 months old (36%) had a significantly longer hospitalization time (41 vs 25 d; p = 0.001) and higher Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Mortality Category (3 vs 2; p = 0.003). All cases except one (contact dermatitis) were uneventful. In 10 patients, wounds were closed surgically after vacuum-assisted closure. Two patients required a pectoralis flap, both treated before 2005. One of the two deaths was infection-related. CONCLUSIONS: Vacuum-assisted closure is a feasible treatment option of deep sternal wound infection after pediatric cardiac surgery and was not associated with independent morbidity.

Alveolar Dead-Space Fraction and Arterial Saturation Predict Postoperative Course in Fontan Patients.

OBJECTIVES: Fontan surgery, the final surgical stage in single ventricle palliation, redirects systemic venous blood into the pulmonary circulation for gas exchange. A decrease in pulmonary blood flow can lead to major complications and grave outcomes. Alveolar dead-space fraction represents the portion of inhaled air that does not participate in gas exchange and hence quantifies ventilation-perfusion abnormalities in the lung. Increased alveolar dead-space fraction has been associated with prolonged mechanical ventilation and worse outcome after congenital heart surgery. The association of alveolar dead-space fraction with clinical outcomes in patients undergoing Fontan operation has not been reported. INTERVENTIONS: None. DESIGN, SETTING, AND PATIENTS: A retrospective charts review of all pediatric patients who underwent Fontan surgery during June 2010-November 2018 in a tertiary-care pediatric hospital. Associations between alveolar dead-space fraction and arterial oxyhemoglobin saturation to a composite outcome (surgical or catheter-based intervention, extracorporeal membrane oxygenation use, prolonged ventilation, prolonged hospital length of stay, or death) were explored. Secondary endpoints were parameters of severity of illness, chest drainage duration, and length of stay. MEASUREMENTS AND MAIN RESULTS: Of 128 patients undergoing Fontan operation, 34 met criteria for composite outcome. Alveolar dead-space fraction was significantly higher in the composite (0.33 ± 0.14) versus control (0.25 ± 0.26; p = 0.016) group. Alveolar dead-space fraction greater than or equal to 0.29 indicated a 37% increase in risk to meet composite criteria. Admission arterial oxygen saturation was significantly lower in composite versus control group (93.4% vs 97.1%; p = 0.005). Alveolar dead-space fraction was significantly associated with increased durations of mechanical ventilation, ICU length of stay, duration of thoracic drainage, and parameters of severity of illness. CONCLUSIONS: Alveolar dead-space fraction and arterial saturation may predict complicated postoperative course in patients undergoing the Fontan operation.

Urgent Surgical Treatment of Aortic Endocarditis in Infants and Children.

Infective endocarditis (IE) in the pediatric population can present as a life-threatening condition. Optimal timing for surgical intervention should consider surgical risks versus the risk of neurologic complications. We herein report our experience with this group of critically ill children. Retrospective analysis of patient charts of all patients who underwent urgent surgical treatment of aortic IE from 1994 to 2014 was performed. Nine patients with acute storming IE of the aortic valve or the ascending aorta were urgently operated (eight normal heart, one congenital aortic stenosis), age ranged from 8 weeks to 4.2 years. Causative organisms were Staphylococcus aureus (2), Staphylococcus coagulase negative (1), Kingella kingae (2), Streptococcus pneumoniae (2), or culture negative (2). Presenting symptoms other than hemodynamic instability were neurologic decompensation (5) coronary embolization (1) and cardiogenic shock due to scalded skin syndrome (1). CT and MRI demonstrated significant brain infarcts in four patients. Operations performed were the Ross operation (7) and ascending aortic reconstruction (2). There were no operative deaths. At a median follow-up of 6 years (range 2-196 months), all patients are alive and well. Re-intervention included homograft replacement (2) and transcatheter Melody valve implantation (1). At their last follow-up, the neo-aortic valve was functional in all patients with minimal regurgitation and all had full resolution of the neurological deficits. Urgent surgical treatment for aortic valve IE in infants is challenging. Although surgery is complex and pre-disposing conditions such as sepsis, neurologic and cardiac decompensations are prevalent, operative results are excellent and gradual and significant neurologic improvement was noted over time.

Streptobacillus moniliformis Endocarditis: An Unusual Case of Pulmonary Valve Erosion Resulting in Free Pulmonary Regurgitation and Aneurysm.

Endocarditis is a consideration in the differential diagnosis when masses are seen on echocardiography in a patient with congenital heart disease. We present a case of insidious development of endocarditis caused by Streptobacillus moniliformis in a seven-month-old baby after a rat bite, when the baby was three months of age.

Computer-generated real-time digital holography: first time use in clinical medical imaging.

AIMS: Assessment of the feasibility of creating real-time interactive 3D digital holograms in a standard catheterization laboratory. 3D medical images are typically displayed and interacted with on 2D screens limiting their usefulness. A digital computer-generated real-time holographic display of patient's 3D data could provide a spatially accurate image with all the depth cues and afford interaction within the image. METHODS AND RESULTS: We performed a feasibility study of creating real-time interactive 3D digital holograms with a purpose-built prototype using intraprocedural data from 3D rotational angiography and live 3D transesophageal echocardiography. The primary objective was to demonstrate that all the anatomical landmarks identified on standard imaging can be similarly identified using dynamic and static holographic images. The secondary objective was to demonstrate the usability of interactions with the image. Parameters were assessed by a rating scale. Eight patients were enrolled of whom five underwent transcatheter ASD closure using 3DTEE and three patients were evaluated by 3D rotational angiography. In all cases dynamic real-time and static 3D holograms were created in standard cath lab conditions. Four individual observers identified all anatomical landmarks on the holographic display independently from the 2D display. Interactions with the hologram including marking, cropping and rotation were performed. There were no adverse events. CONCLUSIONS: This study demonstrates, for the first time, the feasibility of generating high quality, clinically relevant, 3D real-time colour dynamic holograms in a standard clinical setting with real patient volumetric data. The impact of computer-generated holography needs to be evaluated in controlled clinical trials.

Neonatal Cardiac Surgery in the New Era: Lessons learned from 1000 Consecutive Cases.

BACKGROUND: neonatal cardiac surgery has evolved over the last 50 years with a large percentage of the patients achieving complete physiological repair in the neonatal period. The remaining patients achieve staged palliation with an increasing amount of success. OBJECTIVES: To report our experience with 1000 neonatal cardiac surgical procedures performed in the last 10 years. METHODS: We conducted a retrospective analysis of surgical outcome in all neonatal patients who underwent cardiac surgery between January 2007 and July 2016 at Schneider Children's Medical Center of Israel. RESULTS: A total of 1003 neonates aged < 30 days underwent surgery for congenital heart defects at our center. Neonatal surgery accounted for 22.5% of all cardiac surgeries. Neonatal operative mortality was 7.3%, Operative mortality for individual lesions were: simple aortic coarctation (CoA) (198 patients, 2.5%), CoA with hypoplastic arch (24, 4%), CoA with ventricular septal defect (VSD) (84, 2.3%), transposition of the great arteries (TGA, simple and complex, 185, 6.3%), TGA with VSD (37, 0%), truncus arteriosus (26, 3.8%), interrupted aortic arch (25, 4%), Norwood Sano (71, 19.7%), neonatal tetralogy of Fallot (41, 0%), and shunt (131 patients, 12%). CONCLUSIONS: Neonatal surgical capabilities have improved substantially over the last decades. Excellent results can be expected for lesions that can be repaired to create biventricular circulation. Improved results can be attributed in part to the evolution of surgical strategies and assistive technologies, but essential is the collaborative effort of surgeons, cardiologists, anesthesiologists, and intensive care specialists acting as a cohesive team whose performance far exceeds the sum of its individual members' contributions.