Successful ABO incompatible heart transplantation after desensitization therapy in an older child.

BACKGROUND: ABO-incompatible heart transplantation (HTx) has become a standard procedure for children below 2 years of age due to an immunologically immature immune system and associated low isohemagglutinin titers. METHODS: We report a case of an ABO-incompatible HTx (recipient blood group O, donor blood group A) at the age of 5 years and 11 months with a fully matured immune system and previously high isohemagglutinin titers that diminished as a result of human leucocyte antigen (HLA) desensitization therapy with rituximab and immunoglobulins. RESULTS: The anti-A titer at the time of HTx was 1:16 with post-transplant isoagglutinin titers never exceeding 1:4 without any signs of rejection with now 3 years of post-HTx follow-up. CONCLUSIONS: ABO isohemagglutinin titers should be routinely assessed in children undergoing desensitization therapy since ABOi transplantation can be considered in selected cases to expand the donor pool with the option of crossing the ABO barrier to find a better-matched allograft.

Echocardiographic Predictors of Ductal Tissue-Related Branch Pulmonary Artery Stenosis in Pulmonary Atresia.

Branch pulmonary artery (PA) stenosis due to ductus arteriosus (DA) tissue (DA-PS) contributes to the morbidity associated with pulmonary atresia (PAtr). We sought to identify preoperative echocardiographic features predictive of DA-PS. Patients consecutively encountered with PAtr and a DA-dependent pulmonary circulation at birth who underwent intervention in our program over a 5-year period were identified and records reviewed. Preoperative echocardiograms were reviewed to identify features that predicted postoperative DA-PS. Seventy patients with PAtr met inclusion criteria and 36 (51%) had DA-PS. At preoperative echocardiography, the proximal diameter of the PA ipsilateral to the DA was smaller in those with versus without DA-PS (Z-score - 4.8 ± 1.7 vs - 1.1 ± 1.7, respectively p < 0.001). PA origins could not be imaged on the same axial plane in 21/36 (58%) with versus 2/34 (6%) without DA-PS. Patients with DA-PS had an obtuse posterior angle of the PA bifurcation compared to those without (128 ± 17° and 87 ± 21°, p < 0.001), and a posterior angle of > 100° best predicted DA-PS with a sensitivity of 97% and specificity of 76%. An abnormal PA relationship and/or an obtuse posterior bifurcation angle had a sensitivity, specificity, positive and negative predictive value for DA-PS of 78%, 94%, 90% and 86%, respectively. Finally, DA insertion was into the ipsilateral PA in 26/36 (72%) of cases with DA-PS. A smaller proximal ipsilateral PA diameter, inability to image the PAs in the same plane, a posterior PA bifurcation angle of > 100°, and insertion of the DA in the ipsilateral PA demonstrated by echo are useful in identifying patients at risk for DA-PS.

Surgical repair of massive hemorrhage secondary to button battery ingestion causing aortoesophageal fistula.

BACKGROUND: In pediatrics, foreign body ingestion poses unique challenges. Each case is unique given variability in timing, type, and size of object, compounded by underlying comorbidities and age. In the mid-1990s, mortality and morbidity associated with button battery (BB) ingestion (BBI) emerged corresponding to modification in battery fabrication towards higher voltage, large-diameter lithium cells. AIMS: To describe the case and management of a BBI in a pediatric patient necessitating the use of cardiopulmonary bypass and deep hypothermic circulatory arrest (DHCA). MATERIALS AND METHODS: A 17-month-old female presented with the sudden loss of consciousness at home. Chest X-ray revealed an esophageal foreign body suspicious for BBI. A massive upper gastrointestinal bleeding was temporized with packing. The patient was urgently taken to the operating room for sternotomy, establishment of cardiopulmonary bypass (CPB) with deep hypothermic circulatory arrest (DHCA), removal of BB, repair of the left common carotid artery, esophageal, and tracheal injuries. DISCUSSION: Successful management of BBI requires coordinated care and a multidisciplinary approach. A high degree of clinical suspicion for BBI is imperative to facilitate early aggressive interventions. Lateral and anteroposterior chest films should be obtained in any suspected BBI. CONCLUSION: This case demonstrates the utility of CPB and DHCA where control of bleeding secondary to BBI is not otherwise possible.

Left ventricular fibroma presenting as esophageal spasm: A case report.

Primary tumors of the heart are rare with fibromas most commonly identified in utero or infancy and rarely developing in adulthood. Patients with cardiac fibromas are often asymptomatic until tumor enlargement results in obstructive and nonspecific symptoms. A 39-year-old female presented with 5-year history of recurrent chest pain with functional dysphagia, indicative of esophageal spasm. Imaging identified a large left ventricular (LV) fibroma compressing the esophagus provoking esophageal spasm. The fibroma was excised measuring 51 × 39 mm. This case describes presentation with esophageal spasm, contributing a novel presentation of LV fibroma to the literature.

Health-related quality of life after pediatric heart transplantation in early childhood.

BACKGROUND: There is limited information about HRQL after pediatric heart transplantation at a young age. METHODS: Prospective follow-up study of children who received a heart transplant at age ≤4 years. HRQL was assessed using the PedsQLTM 4.0 at age 4.5 years. This cohort was compared with healthy children, children with CHD, and with chronic conditions. Peri-operative factors associated with HRQL were also explored. RESULTS: Of 66 eligible patients, 15 (23%) died prior to the HRQL assessment and 2 (3%) were lost to follow-up, leaving 49 patients. Indication for transplantation was CHD in 27 (55%) and CMP in 22 (45%). Median age (IQR) at transplant was 9 (5-31) months. HRQL was significantly lower in transplanted children compared to population norms (65.3 vs 87.3, P < .0001), children with chronic conditions (65.3 vs 76.1, P = .001), and children with CHD (65.3 vs 81.1, P < .0001). Transplanted children with CHD had lower HRQL than those with a prior diagnosis of CMP (59.5 vs 72.5, P-value = .020). Higher creatinine pretransplant and higher lactate post-operatively were associated with lower HRQL. CONCLUSION: Children after heart transplant had significantly lower HRQL, as reported by their parents, than the normative population, children with chronic conditions, and children with CHD.

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.

Predicting Post-Fontan Length of Stay: The Limits of Measured Variables.

Post-operative length of stay (LOS) is an important metric for both healthcare providers and patients and their families. Predicting LOS is a challenge as it is sensitive to multitudinous patient and system factors. All subjects undergoing a Fontan from 1996-2016 who survived to hospital discharge were included. Details about the pre-operative status, operative conduct, and post-operative course of each patient were obtained. The association between patient characteristics and post-Fontan LOS were determined using stepwise multivariable regression models. Of 320 subjects who underwent a Fontan, 314 (98.1%) survived to hospital discharge. Median age at Fontan was 3.3 years (IQR 2.8, 4.0) and the most common underlying diagnosis was hypoplastic left heart syndrome (106, 33.8%). Median post Fontan LOS was 11 days (IQR 8, 17). Univariable risk factors for longer LOS included number of previous surgeries, post-Glenn LOS, cardiopulmonary bypass time, post-operative chylothorax, and failure to extubate in the operating room (all p < 0.05). In multivariable models, number of previous operations, extubation in the operating room, and postoperative complications predicted LOS (R2 = 0.5185 for full model). The proportion of patients discharged on week days (14.7-18.8% per day) was significantly higher than the proportion discharged on weekend days (5.1-9.9% per weekend day). Pre-operative variables have limited use in predicting post-Fontan length of stay. The most important predictors of post-operative LOS are extubation in the operating room and the occurrence of post-operative complications. However, a significant proportion of variability in LOS was not explained by available measurable variables.

Cardiac Surgery-Associated Kidney Injury in Children and Renal Oximetry.

OBJECTIVES: Cardiac surgery-associated acute kidney injury is common in children and associates with negative outcomes. Novel interventions to reduce cardiac surgery-associated acute kidney injury require knowledge of its pathophysiology. States of altered perfusion, oxygen delivery, and energy consumption occur during cardiopulmonary bypass and could protect against or contribute to renal cellular injury and recovery. Near-infrared spectroscopy is noninvasive technology for monitoring regional blood flow and tissue oxygenation. This study evaluated the relationship between renal regional oxygen saturation and cardiac surgery-associated acute kidney injury, using near-infrared spectroscopy monitoring before, during, and after cardiopulmonary bypass in children. DESIGN: Prospective cohort study. SETTING: Single-center, tertiary care pediatric hospital (Stollery Children's Hospital, Edmonton, AB, Canada). PATIENTS: Children less than or equal to 10 kg undergoing congenital heart disease repair with cardiopulmonary bypass. Heart transplant, preoperative dialysis, sepsis, extracorporeal life support, congenital renal disease, and preoperative nephrotoxins were exclusions. INTERVENTIONS: Renal regional near-infrared spectroscopy monitoring before, during, and after cardiopulmonary bypass. MEASUREMENTS AND MAIN RESULTS: Outcome measure was cardiac surgery-associated acute kidney injury (defined according to Kidney Disease: Improving Global Outcomes criteria). Regional oxygen saturation was measured continuously using near-infrared spectroscopy (INVOS 5100C Cerebral/Somatic Oximeter; Medronic, Troy, MI) from time of anesthesia to time of transfer to intensive care. Cardiac surgery-associated acute kidney injury occurred in 65%. Lower baseline (precardiopulmonary bypass) regional oxygen saturation was associated with decreased risk of cardiac surgery-associated acute kidney injury (p = 0.01); children with baseline regional oxygen saturation in the highest tertile were 7.14 times more likely to get cardiac surgery- associated acute kidney injury (vs lowest tertile). Area under the curve for ability of baseline regional oxygen saturation to predict cardiac surgery-associated acute kidney injury was 0.73 (95% CI, 0.60-0.85). Children with lower baseline glomerular filtration rate had lower mean renal regional oxygen saturation. CONCLUSIONS: Findings demonstrate that preoperative oxygen supply/demand balance is an important predictor of cardiac surgery-associated acute kidney injury, suggesting lower preoperative (and intraoperative) renal blood flow may be protective. There is not yet a definite link between remote ischemic preconditioning and prevention of cardiac surgery-associated acute kidney injury; however, renal protective effects of sublethal ischemia should continue to be explored.

Pediatric extracorporeal membrane oxygenation (ECMO): a guide for radiologists.

Extracorporeal membrane oxygenation (ECMO) is a life-saving treatment for pediatric patients with respiratory and/or cardiac failure. The ECMO circuit oxygenates and sometimes pumps the blood, effectively replacing lung and/or heart function temporarily. ECMO patients are clinically very complex not only because of their underlying, life-threatening pathology, but also because of the many physiological parameters that must be monitored and adjusted to maintain adequate tissue perfusion and oxygenation. Drainage and reinfusion cannulae connecting the patient to the ECMO circuit are visible on radiograph. These cannulae have different functions, different configurations, different radiographic appearances, and different positions that should be familiar to the interpreting pediatric radiologist. The primary complications of ECMO include hemorrhage, thrombosis and ischemia, as well as equipment failure and cannula malpositioning, all of which may be detected on imaging. In this pictorial essay, we discuss the basics of ECMO function and clinical management, ECMO cannula features and configurations, and the many complications of ECMO from an imaging perspective. Our goal is to educate pediatric radiologists about ECMO imaging, equipping them to properly interpret these studies and to become a useful consultant in ECMO patient care.

The development of a congenital heart programme quality dashboard to promote transparent reporting of outcomes.

In 2001, the Institute of Medicine identified healthcare transparency as a necessity for re-designing a quality healthcare system; however, despite widespread calls for publicly available transparent data, the goal remains elusive. The transparent reporting of outcome data and the results of congenital heart surgery is critical to inform patients and families who have both the wish and the ability to choose where care is provided. Indeed, in an era where data and means of communication of data have never been easier, the paucity of transparent data reporting is paradoxical. We describe the development of a quality dashboard used to inform staff, patients, and families about the outcomes of congenital heart surgery at the Stollery Children's Hospital.

Impact of Neonatal Intervention on Left Ventricular Performance in Ebstein's Anomaly and Tricuspid Valve Dysplasia.

BACKGROUND: Severe neonatal Ebstein's anomaly (EA) and tricuspid valve dysplasia (TVD) are associated with high perinatal morbidity and mortality. The authors recently demonstrated left ventricular (LV) dysfunction and dyssynchrony to be prevalent in affected newborns and to contribute to poor outcomes. The aim of this study was to investigate the impact of patent ductus arteriosus (PDA) closure, spontaneous or surgical ligation, or right ventricular exclusion (Starnes procedure) on LV performance in neonatal EA and TVD. METHODS: Neonates with EA or TVD encountered from 2004 to 2018 at three institutions were identified. Pre- and postoperative LV function was assessed using two-dimensional, Doppler-derived deformation (six-segment vector velocity imaging) and two measures of mechanical dyssynchrony (the SD of time to peak and global dyssynchrony index), and values were compared using paired t test analysis or the Wilcoxon rank sum test. RESULTS: Before the intervention, LV function was impaired in the PDA (n = 18) and Starnes (n = 6) groups and was similar between groups. After PDA closure, LV performance did not change. After the Starnes procedure, however, LV function, including synchrony, improved significantly: fractional area change from 45 ± 5% to 58 ± 8% (P = .003), global circumferential strain from -18.2 ± 5.0% to -32.5 ± 5.5% (P = .01), cardiac index from 1.9 ± 0.3 to 3.9 ± 1.5 L/min/m2 (P = .05), and circumferential strain dyssynchrony (dyssynchrony index from 0.19 ± 0.09 to 0.04 ± 0.02 [P = .009] and SD of time to peak from 59.8 ± 18.5 to 29.9 ± 8.2 [P = .02]). CONCLUSION: The Starnes procedure results in early improvements in LV dysfunction and dyssynchrony, not observed after PDA closure in neonatal severe EA and TVD, which may benefit critically unwell neonates.

Acute kidney injury and renal recovery following Fontan surgery.

Objectives: Acute kidney injury has been described after Fontan surgery, but the duration and outcomes are unknown. We sought to describe the incidence of and risk factors for acute kidney injury and the phenotype of renal recovery, and evaluate the impact of renal recovery phenotype on outcomes. Methods: All children who underwent a Fontan operation at a single center between 2009 and 2022 were included. Data collected included Fontan characteristics, vasopressor use, all measures of creatinine, and postoperative outcomes. Logistic regression models were used to assess predictors of acute kidney injury and the association between acute kidney injury and outcomes. Results: We enrolled 141 children (45% female). Acute kidney injury occurred in 100 patients (71%). Acute kidney injury duration was transient (<48 hours) in 77 patients (55%), persistent (2-7 days) in 15 patients (11%), more than 7 days in 4 patients (3%), and unknown in 4 patients (3%). Risk factors for acute kidney injury included higher preoperative indexed pulmonary vascular resistance (odds ratio, 3.90; P = .004) and higher postoperative inotrope score on day 0 (odds ratio, 1.13, P = .047). Risk factors for acute kidney injury duration more than 48 hours included absence of a fenestration (odds ratio, 3.43, P = .03) and longer duration of cardiopulmonary bypass (odds ratio, 1.22 per 15-minute interval, P = .01). Acute kidney injury duration more than 48 hours was associated with longer length of stay compared with transient acute kidney injury (median 18 days [interquartile range, 9-62] vs 10 days [interquartile range, 8-16], P = .006) and more sternal wound infections (17% vs 4%, P = .049). Conclusions: Acute kidney injury after the Fontan operation is common. The occurrence and duration of acute kidney injury have significant implications for postoperative outcomes.

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.

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.

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.

Ventricular Assist Device Use in Patients With Single-Ventricle Circulation.

Ventricular assist devices (VADs) are being increasingly used to support patients with congenital heart disease and single-ventricle physiology. Because of their unique anatomy and physiology, special consideration must be used to provide effective mechanical circulatory support for each individual patient. This can include alternative cannulation techniques, strategies to balance cardiac output to the systemic and pulmonary circulations from a single ventricle, or the use of continuous vs pulsatile VADs for better ventricular offloading. In this article we review the etiology of single-ventricle failure, VAD options for support, cannulation strategies, post-VAD management considerations, and outcomes at each of the 3 stages of palliation.

Surgical Repair of a Transannular Rupture During Transfemoral Transcatheter Aortic Valve Replacement.

Annular rupture is a rare but life-threatening complication of transcatheter aortic valve replacement (TAVR). Mortality rates are high if immediate intervention, most often necessitating surgical repair, is not performed. Herein, we describe an 87-year-old man who, after deployment of TAVR, experienced acute decompensation and required urgent conversion to a midline sternotomy to repair an aortic annular rupture. This case demonstrates an example of a rare but severe complication of TAVR. This report provides an in-depth description of the surgical approach to repair an aortic annular rupture and demonstrates the utility of performing minimally invasive procedures inside a hybrid operating room.

Berlin Heart EXCOR and ACTION post-approval surveillance study report.

BACKGROUND: The Berlin Heart EXCOR Pediatric (EXCOR) ventricular assist device (VAD) was introduced in North America nearly 2 decades ago. The EXCOR was approved under Humanitarian Device Exemption status in 2011 and received post-market approval (PMA) in 2017 from Food and Drug Administration. Since the initial approval, the field of pediatric mechanical circulatory support has changed, specifically with regard to available devices, anticoagulation strategies, and the types of patients supported. This report summarizes the outcomes of patients supported with EXCOR from the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. These data were part of the PMA surveillance study (PSS) required by the Food and Drug Administration. METHODS: ACTION is a learning collaborative of over 40 pediatric heart failure programs worldwide, which collects data for all VAD implantations as one of its initiatives. All patients in North America with EXCOR implants reported to ACTION from 2018 to 2020 (n = 72) who had met an outcome were included in the EXCOR PSS group. This was compared with a historical, previously reported Berlin Heart EXCOR study group (Berlin Heart study [BHS] group, n = 320, 2007‒2014). RESULTS: Patients in the PSS group were younger, were smaller in weight/body surface area, were more likely to have congenital heart disease, and were less likely to receive a bi-VAD than those in the BHS group. Patients in the PSS group were less likely to be in Interagency Registry for Mechanically Assisted Circulatory Support Profile 1 and were supported for a longer duration. The primary anticoagulation therapy for 92% of patients in the PSS group was bivalirudin. Success, defined as being transplanted, being weaned for recovery, or being alive on a device at 180 days after implantation, was 86% in the PSS group compared with 76% in the BHS group. Incidence of stroke was reduced by 44% and the frequency of pump exchange by 40% in the PSS group compared with those in the BHS group. Similarly, all other adverse events, including major bleeding, were reduced in the PSS group. CONCLUSIONS: The PSS data, collected through ACTION, highlight the improvement in outcomes for patients supported with EXCOR compared with the outcomes in a historical cohort. These findings may be the result of changes in patient care practices over time and collaborative learning.

Management of Critical Coarctation of Aorta in a Premature Neonate With Low Birth Weight.

Premature neonates with low birth weight have always posed a huge management dilemma, even for teams with great expertise. In this case report, we discuss a premature neonate born with extremely low birth weight diagnosed with critical coarctation of aorta and the challenges faced in stabilizing the neonate. As per our literature review, this is the smallest neonate, weighing only 680 g, to have undergone successful repair of coarctation of aorta through posterolateral thoracotomy. Identifying the ideal timing for surgery in premature neonates with low birth weight is most crucial to minimize morbidity and mortality.