Congenital Tracheal Stenosis Patients Undergoing Modified Slide Tracheoplasty: Single-Centre Technique and Long-Term Morbidity and Mortality.

Background: Congenital malformations of the trachea are rare but often life-threatening. Limited data have been published on the outcomes of tracheal reconstruction for congenital tracheal stenosis. We sought to describe the outcomes of patients undergoing tracheal reconstruction over 10 years at our centre. Methods: All paediatric patients who underwent long-segment tracheal or bronchial reconstruction from January 1, 2012, to August 31, 2022, were included. The primary outcome was mortality, and secondary outcomes included reoperation and postoperative morbidity. Patients were followed up to 10 years. Results: Thirty-three patients with ages ranging from 1 day to 12 years (mean 8.5 months) at the time of tracheoplasty or bronchoplasty were included, with 5 patients undergoing off-pump tracheal reconstruction. The most common preoperative comorbidities included patent ductus arteriosus (30.3%), atrial septal defect (27.3%), and prematurity (24.2%). There were no deaths postoperatively within the follow-up period. All patients experienced successful reconstruction with no patients requiring reoperation of the trachea. A total of 14 patients (42.4%) required postoperative balloon dilation, 3 (9.1%) required bronchial repair after tracheal repair, and 2 (6.1%) required bronchoscopic tracheal debridement. Conclusions: This single-centre retrospective study provides a large cohort of congenital tracheal reconstruction patients with a survival rate of 100%, experiencing no mortality during follow-up. The majority of patients had preoperative comorbidities and concomitant congenital cardiac defects. Although tracheal reconstruction continues to be complex with significant postoperative morbidity and mortality, the results of our single-centre study demonstrate the continual advancement of this field and the evolving improvement of postoperative outcomes for these patients.

Contexte: Les malformations congénitales de la trachée sont rares, mais elles menacent souvent le pronostic vital. Peu de données ont été publiées sur les résultats cliniques de la reconstruction trachéale dans les cas de sténose trachéale congénitale. Notre objectif était de décrire les résultats obtenus par les patients ayant subi une reconstruction trachéale dans notre établissement sur une période de 10 ans. Méthodologie: Les données de tous les enfants ayant subi une reconstruction d'un long segment trachéal ou bronchique entre le 1er janvier 2012 et le 31 août 2022 ont été incluses dans l'étude. Le critère principal d'évaluation était la mortalité et les critères secondaires d'évaluation incluaient la réintervention chirurgicale et la morbidité postopératoire. Les patients ont été suivis pendant 10 ans. Résultats: Trente-trois patients âgés de 1 jour à 12 ans (moyenne de 8,5 mois) au moment de la trachéoplastie ou de la bronchoplastie ont été inclus dans l'étude, dont 5 ayant subi une reconstruction trachéale sans circulation extracorporelle (off-pump). Avant l'intervention, les affections concomitantes les plus fréquentes étaient la persistance du canal artériel (30,3 %), la communication interatriale (27,3 %) et la prématurité (24,2 %). Aucun décès postopératoire n'est survenu au cours de la période de suivi. La reconstruction a été un succès chez tous les patients, et aucun d'entre eux n'a dû subir de nouvelle opération trachéale. Une dilatation postopératoire par ballon a été nécessaire chez 14 patients (42,4 %), 3 d'entre eux (9,1 %) ont subi une correction chirurgicale des bronches après la reconstruction trachéale, et 2 (6,1 %) ont subi un débridement trachéal par bronchoscopie. Conclusions: Notre étude rétrospective monocentrique dresse le portrait d'une vaste cohorte de patients ayant subi une reconstruction de la trachée en raison d'une malformation congénitale, parmi laquelle le taux de survie était de 100 %, sans aucun décès au cours de la période de suivi. Par ailleurs, la majorité des patients présentaient, avant l'intervention, des affections et des malformations cardiaques concomitantes. La reconstruction trachéale demeure malgré tout complexe et les taux de mortalité et de morbidité postopératoires restent importants, mais les résultats de notre étude monocentrique témoignent des progrès continuels dans ce domaine et de l'amélioration des résultats postopératoires pour ces patients.

Predicting High-Risk Fetal Cardiac Disease Anticipated to Need Immediate Postnatal Stabilization and Intervention with Planned Pediatric Cardiac Operating Room Delivery.

BACKGROUND: Distances between delivery and cardiac services can make the care of fetuses with cardiac disease at risk of acute cardiorespiratory instability at birth a challenge. In 2013 we implemented a fetal echocardiography-based algorithm targeting fetuses considered high risk for acute cardiorespiratory instability at ≤2 hours of birth for delivery in our pediatric cardiac operating room of our children's hospital, and, herein, examine our experience. METHODS AND RESULTS: We reviewed maternal and postnatal medical records of all fetuses with cardiac disease encountered January 2013 to March 2022 considered high risk for acute cardiorespiratory instability. Secondary analysis was performed including all fetuses with diagnoses of d-transposition of the great arteries/intact ventricular septum (d-TGA/IVS) and hypoplastic left heart syndrome (HLHS) encountered over the study period. Forty fetuses were considered high risk for acute cardiorespiratory instability: 15 with d-TGA/IVS and 7 with HLHS with restrictive atrial septum, 4 with absent pulmonary valve syndrome, 3 with obstructed anomalous pulmonary veins, 2 with severe Ebstein anomaly, 2 with thoracic/intracardiac tumors, and 7 others. Pediatric cardiac operating room delivery occurred for 33 but not for 7 (5 with d-TGA/IVS, 2 with HLHS with restrictive atrial septum). For high-risk cases, fetal echocardiography had a positive predictive value of 50% for intervention/extracorporeal membrane oxygenation/death at ≤2 hours and 70% at ≤24 hours. Of "low-risk" cases, 6/46 with d-TGA/IVS and 0/45 with HLHS required intervention at ≤2 hours. Fetal echocardiography for predicting intervention/extracorporeal membrane oxygenation/death at ≤2 hours had a sensitivity of 67%, specificity 93%, and positive and negative predictive values of 80% and 87%, respectively, for d-TGA/IVS, and 100%, 95%, 71%, and 100% for HLHS, respectively. CONCLUSIONS: Fetal echocardiography can predict the need for urgent intervention in a majority with d-TGA/IVS and HLHS and in half of the entire spectrum of high-risk cardiac disease.

Tracheal Reconstruction for Congenital Tracheal Stenosis: A 950-Gram Neonate.

Congenital tracheal stenosis is a rare but life-threatening malformation of the trachea. Surgical reconstruction is high risk, and not frequently performed in neonates born of extreme prematurity and low birth weight. We present the case of an extremely premature 950-gram neonate with severe congenital tracheal stenosis who underwent tracheal reconstruction. Complete repair, with no residual stenosis, was achieved with slide tracheoplasty without the need for cardiopulmonary bypass.

Paracorporeal Support in Pediatric Patients: The Role of the Patient-Device Interaction.

BACKGROUND: Ventricular assist devices are important in the treatment of pediatric heart failure. Although paracorporeal pulsatile (PP) devices have historically been used, there has been increased use of paracorporeal continuous (PC) devices. We sought to compare the outcomes of children supported with a PP or PC, or combination of devices. METHODS: A retrospective review (2005 to 2019) was made of patients less than 19 years of age from a single center who received a PC, PP, or combination of devices. Patient characteristics were compared between device strategies, and Kaplan-Meier survival analysis was performed. RESULTS: Sixty-six patients were included: 62% male; 62% non-congenital heart disease; median age 0.9 years (interquartile range, 0.2 to 4.9); and median weight 8.5 kg (interquartile range, 4.3 to 17.7 kg). The PC devices were used in 45% of patients, PP in 35%, and a combination in 20%. Patients on PC devices had a lower median weight (P = .02) and a higher proportion of congenital heart disease (P = .02), and more patients required pre-ventricular assist device dialysis (P = .01). There was no difference in pre-ventricular assist device extracorporeal membrane oxygenation use (P = .15). There was a difference in survival among the three device strategies (P = .02). CONCLUSIONS: Differences in survival were evident, with patients on PC support having worse outcomes. Transition from PC to a PP devices was associated with a survival advantage. These findings may be driven by differences in patient characteristics across device strategies. Further studies are required to confirm these findings and to better understand the interaction between patient characteristics and device options.

Discharge and Readmission to the Pediatric Cardiac ICU in Pediatric Patients With Durable Ventricular Assist Devices.

OBJECTIVES: Pediatric patients implanted with a durable ventricular assist device are initially managed in the pediatric cardiac ICU but are eligible for discharge to the ward. Our objectives were to characterize discharge and readmission of ventricular assist device patients to the pediatric cardiac ICU, identify risk factors for readmission, and determine whether discharge or readmission is associated with mortality. DESIGN: Retrospective study. SETTING: Stollery Children's Hospital. PATIENTS: Patients implanted with a durable ventricular assist device at less than 18 years old between 2005 and 2016. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 44 patients who underwent ventricular assist device implantation at a median age of 3.7 years (interquartile range, 0.6-9.0 yr), with the most common etiology being noncongenital heart disease (76.7%). Median time of total ventricular assist device support was 110.0 days (interquartile range, 42.3-212.3 d) with the median index pediatric cardiac ICU stay being 34.0 days (interquartile range, 19.8-81.0 d). Thirty patients (68.0%) were discharged to the ward with 18 (60.0%) having at least one readmission. The median time to first readmission was 18.0 days (interquartile range, 14.8-109.8 d) with a median of two readmissions per patient (interquartile range, 1.0-3.0). The most common reason for readmission was pump thrombosis (30.4%), followed by neurologic dysfunction (23.9%). There were no statistically significant pre- or post-implant factors associated with readmission, and readmission was not associated with mortality (p = 0.600). Univariate Kaplan-Meier survival analysis indicated that use of pre-implant extracorporeal membrane oxygenation, post-implant continuous renal replacement therapy, as well as failure to be discharged from the index pediatric cardiac ICU stay were associated with mortality. CONCLUSIONS: Readmissions to the pediatric cardiac ICU occurred in 60.0% of pediatric patients on durable ventricular assist devices with the first readmission occurring within a month of discharge from the index pediatric cardiac ICU stay. While readmission was not associated with mortality, lack of discharge from index pediatric cardiac ICU stay was likely due to a worse pre-implant clinical status.

End-Stage Liver Disease Models and Outcomes in Pediatric Patients Supported With Short-Term Continuous-Flow Ventricular Assist Devices.

Short-term continuous-flow ventricular assist devices (STCF-VADs) are increasingly being utilized in pediatrics. End-stage liver disease (ELD) models have been associated with outcomes in adult patients on mechanical circulatory support. We sought to determine the relationship between outcomes in children on STCF-VADs and three ELD models: model for end-stage liver disease-excluding international normalized ratio (MELD-XI; all) and MELD-XI (> 1 year), PELD, and a novel score, PedMELD-XI. All patients (< 19 years) supported with STCF-VADs, between June 2009 and December 2016 were included. The MELD-XI, PELD, and PedMELD-XI scores were calculated and their association with adverse events and a composite measure of death, major bleeding, and neurologic dysfunction was analyzed. Of 32 patients, median age was 0.57 years (interquartile range [IQR], 0.10-4.43), median weight was 7.15 kg (IQR, 3.68-16.53), 53.1% had congenital heart disease, and 53.1% were male. In total, 78.1% patients experienced an adverse event (78.1% a major bleed, 25.0% neurologic dysfunction, and 15.6% death). The median MELD-XI score was 11.17 (IQR, 9.44-30.01), MELD-XI (>1 year) 9.44 (IQR, 9.44-24.33), PELD 6.00 (IQR, 4.00-13.75), and PedMELD-XI -14.91 (IQR, -18.85 to -12.25). A higher MELD-XI for all ages (13.80 vs. 9.44, p = 0.037) and less negative PedMELD-XI (-14.16 vs. -19.34, p = 0.028) scores were significantly associated with bleeding and the composite outcome. PedMELD-XI was significantly associated with death (-12.87 vs. -16.84, p = 0.041) while a trend was seen for increased MELD-XI in all ages being associated with death (31.52 vs. 10.11, p = 0.051). Last, there was no association with the models and neurologic events. MELD-XI and PedMELD-XI were significantly associated with major bleeding and the composite endpoints with PedMELD-XI also being associated with death. These results suggest that ELD models can be used to predict outcomes in this specific patient population, however, further analysis in a larger population is required.

Neonatal Tracheal and Intracardiac Repair in a High-Risk Premature Infant Requiring Preoperative ECMO Transport.

Congenital tracheal stenosis is an uncommon malformation that portends a poor outcome in children who are symptomatic in the neonatal period. Over time, the management of significant tracheal disease has been consolidated at high-volume centers, and increasingly complex patients have undergone surgical repair. We present a premature newborn boy who was diagnosed with critical multi-level airway and cardiac disease who decompensated at a remote site, requiring extracorporeal membrane oxygenation support for transport. He underwent a complete repair including a slide tracheoplasty and was successfully discharged home, with no residual stenosis at follow-up.

Successful Treatment of Pediatric Ventricular Assist Device Thrombosis.

Pump thrombosis represents a significant cause of morbidity and mortality in patients on continuous flow ventricular assist devices (CF-VAD). Pump thrombosis in the pediatric CF-VAD population has been reported between 11% and 44%, with the largest reported series from the PediMACS registry reporting a rate of approximately 15%. We report our early experience with four pediatric patients who developed pump thrombosis on a CF-VAD. Our limited experience suggests that the treatment of pediatric VAD thrombosis can be approached with similar principles to the adult population. Our current strategy includes:i. Initiating treatment with bivalirudin for an isolated rise in lactate dehydrogenase (LDH) with no corresponding rapid rise in plasma-free hemoglobin which may prevent further progression.ii. Treatment with a low-dose systemic tissue plasminogen activator (TPA) protocol as opposed to targeted therapy via catheter intervention if bivalirudin fails.iii. If there are concerns with respect to the impact of hemolysis on kidney function or the patient is close to a previous surgery, device exchange can be considered.The balance between achieving appropriate anticoagulation/antiplatelet therapy in the face of bleeding/hemorrhagic complications remains a challenge. There is a need for larger studies in the pediatric population to outline an algorithm for the definitive management of VAD thrombosis.

Hyperoxia Reduces Oxygen Consumption in Children with Pulmonary Hypertension.

High inspired oxygen concentration (FiO2 > 0.85) is administered to test pulmonary vascular reactivity in children with pulmonary hypertension (PH). It is difficult to measure oxygen consumption (VO2) if the subject is breathing a hyperoxic gas mixture so the assumption is made that baseline VO2 does not change. We hypothesized that hyperoxia changes VO2. We sought to compare the VO2 measured by a thermodilution catheter in room air and hyperoxia. A retrospective review of the hemodynamic data obtained in children with PH who underwent cardiac catheterization was conducted between 2009 and 2014. Cardiac index (CI) was measured by a thermodilution catheter in room air and hyperoxia. VO2 was calculated using the equation CI = VO2/arterial-venous oxygen content difference. Data were available in 24 subjects (males = 10), with median age 8.3 years (0.8-17.6 years), weight 23.3 kg (7.5-95 kg), and body surface area 0.9 m2 (0.4-2.0 m2). In hyperoxia compared with room air, we measured decreased VO2 (154 ± 38 to 136 ± 34 ml/min/m2, p = 0.007), heart rate (91 [Formula: see text] 20 to 83 [Formula: see text] 21 beats/minute, p=0.005), mean pulmonary artery pressure (41 [Formula: see text] 16 to 35 [Formula: see text] 14 mmHg, p=0.024), CI (3.6 [Formula: see text] 0.8 to 3.3 [Formula: see text] 0.9 L/min/m2, p = 0.03), pulmonary vascular resistance (9 [Formula: see text] 6 to 7 [Formula: see text] 3 WU m2, p = 0.029), increased mean aortic (61 [Formula: see text] 11 to 67 [Formula: see text] 11 mmHg, p = 0.005), pulmonary artery wedge pressures (11 [Formula: see text] 8 to 13 [Formula: see text] 9 mmHg, p = 0.006), and systemic vascular resistance (12 [Formula: see text] 6 to 20 [Formula: see text] 7 WU m2, p=0.001). Hyperoxia decreased VO2 and CI and caused pulmonary vasodilation and systemic vasoconstriction in children with PH. The assumption that VO2 remains unchanged in hyperoxia may be incorrect and, if the Fick equation is used, may lead to an overestimation of pulmonary blood flow and underestimation of PVRI.

A simple meningococcal sepsis prognostic score: focusing on the human animal.

A simple cheap meningococcal sepsis prognostic score based on readily available, rapid, objective laboratory base excess and platelet count was developed and validated retrospectively. This BEP score should facilitate sepsis clinical trials, allowing study of the relevant human animal model.

The diagnosis of myocardial infarction in critically ill patients: an agreement study.

PURPOSE: The aim of the study was to assess agreement among 4 intensivists in diagnosing myocardial infarction (MI) in critically ill patients based on screening electrocardiograms (ECGs) and cardiac troponin (cTn) levels. METHODS: Consecutive patients admitted to a medical-surgical intensive care unit (ICU) underwent systematic screening with 12-lead ECGs and cTn measurements throughout their ICU stay. Independently, 4 raters interpreted the ECGs assessing for changes indicative of ischemia and then classified each patient as to whether they met diagnostic criteria for MI based on the screening cTn measurements and ECG results. A priori, 2 raters were designated the primary adjudicators, and their consensus was used as the reference for the agreement statistics. Agreement on MI diagnosis was calculated for the 4 raters and expressed as raw agreement, kappa (chance-corrected agreement) and varphi (chance-independent agreement, calculated using pairs). RESULTS: Among 103 enrolled patients, 37 (35.9%) had MI according to the primary adjudicators. The raw agreement for diagnosing MI was 79% (substantial), kappa was 0.24 (fair), and varphi ranged from 0.12 to 0.73 (slight to substantial). CONCLUSIONS: Diagnosing MI in the ICU remains a challenge due to variable agreement in 12-lead ECG interpretation. Such variation in practice may contribute to underrecognition of MI during critical illness.

Detecting myocardial infarction in critical illness using screening troponin measurements and ECG recordings.

INTRODUCTION: To use screening cardiac troponin (cTn) measurements and electrocardiograms (ECGs) to determine the incidence of elevated cTn and of myocardial infarction (MI) in patients admitted to the intensive care unit (ICU), and to assess whether these findings influence prognosis. This is a prospective screening study. MATERIALS AND METHODS: We enrolled consecutive patients admitted to a general medical-surgical ICU over two months. All patients underwent systematic screening with cTn measurements and ECGs on ICU admission, then daily for the first week in ICU, alternate days for up to one month and weekly thereafter until ICU death or discharge, for a maximum of two months. Patients without these investigations ordered during routine clinical care underwent screening for study purposes but these results were unavailable to the ICU team. After the study, all ECGs were interpreted independently in duplicate for ischaemic changes meeting ESC/ACC criteria supporting a diagnosis of MI. Patients were classified as having MI (elevated cTn and ECG evidence supporting diagnosis of MI), elevated cTn only (no ECG evidence supporting diagnosis of MI), or no cTn elevation. RESULTS: One hundred and three patients were admitted to the ICU on 112 occasions. Overall, 37 patients (35.9 per cent) had an MI, 15 patients (14.6 per cent) had an elevated cTn only and 51 patients (49.5 per cent) had no cTn elevation. Patients with MI had longer duration of mechanical ventilation (p < 0.0001), longer ICU stay (p = 0.001), higher ICU mortality (p < 0.0001) and higher hospital mortality (p < 0.0001) compared with those with no cTn elevation. Patients with elevated cTn had higher hospital mortality (p = 0.001) than patients without cTn elevation. Elevated cTn was associated with increased hospital mortality (odds ratio 27.3, 95 per cent CI 1.7 - 449.4), after adjusting for APACHE II score, MI and advanced life support. The ICU team diagnosed 18 patients (17.5 per cent) as having MI on clinical grounds; four of these patients did not have MI by adjudication. Thus, screening detected an additional 23 MIs not diagnosed in practice, reflecting 62.2 per cent of MIs ultimately diagnosed. Patients with MI diagnosed by the ICU team had similar outcomes to patients with MI detected by screening alone. CONCLUSION: Systematic screening detected elevated cTn measurements and MI in more patients than were found in routine practice. Elevated cTn was an independent predictor of hospital mortality. Further research is needed to evaluate whether screening and subsequent treatment of these patients reduces mortality.