Intervention for critical aortic stenosis in Hutchinson-Gilford progeria syndrome.

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultra-rare genetic premature aging disease that is historically fatal in teenage years, secondary to severe accelerated atherosclerosis. The only approved treatment is the farnesyltransferase inhibitor lonafarnib, which improves vascular structure and function, extending average untreated lifespan of 14.5 years by 4.3 years (30%). With this longer lifespan, calcific aortic stenosis (AS) was identified as an emerging critical risk factor for cardiac death in older patients. Intervention to relieve critical AS has the potential for immediate improvement in healthspan and lifespan. However, HGPS patient-device size mismatch, pervasive peripheral arterial disease, skin and bone abnormalities, and lifelong failure to thrive present unique challenges to intervention. An international group of experts in HGPS, pediatric and adult cardiology, cardiac surgery, and pediatric critical care convened to identify strategies for successful treatment. Candidate procedures were evaluated by in-depth examination of 4 cases that typify HGPS clinical pathology. Modified transcatheter aortic valve replacement (TAVR) and left ventricular Apico-Aortic Conduit (AAC) placement were deemed high risk but viable options. Two cases received TAVR and 2 received AAC post-summit. Three were successful and 1 patient died perioperatively due to cardiovascular disease severity, highlighting the importance of intervention timing and comparative risk stratification. These breakthrough interventions for treating critical aortic stenosis in HGPS patients could rewrite the current clinical perspective on disease course by greatly improving late-stage quality of life and increasing lifespan. Expanding worldwide medical and surgical competency for this ultra-rare disease through expert information-sharing could have high impact on treatment success.

Transcatheter Ductal Stents Versus Surgical Systemic-Pulmonary Artery Shunts in Neonates With Congenital Heart Disease With Ductal-Dependent Pulmonary Blood Flow: Trends and Associated Outcomes From the Pediatric Health Information System Database.

Background Surgical systemic-to-pulmonary artery shunts have been the standard approach to establish stable pulmonary blood flow in neonates with congenital heart disease with ductal-dependent pulmonary blood flow. More recently, transcatheter ductal stents have been performed as an alternative, less invasive intervention. We aimed to characterize trends in the utilization of surgical shunts versus ductal stents and compare associated outcomes. Methods and Results Using data from the Pediatric Health Information System, we retrospectively analyzed neonates with congenital heart disease with ductal-dependent pulmonary blood flow who underwent surgical shunt or ductal stent placement between January 2016 and December 2021. Patients were identified by International Classification of Diseases, Tenth Revision (ICD-10) diagnosis and procedure codes. The primary outcome was length of hospital stay. Secondary outcomes were reintervention risk and adjusted hospital costs. Of 936 patients included, 65.2% underwent a surgical shunt over the 6-year period. The proportion who underwent ductal stenting increased from 19% to 53.4% from 2016 to 2021. The median adjusted difference in postintervention length of hospital stay was 11 days greater for the surgical shunt cohort (95% CI, 7.2-14.8; P<0.001). The adjusted reintervention risks within 3 (odds ratio [OR], 3.37 [95% CI, 1.91-5.95], P<0.001) and 6 months (OR, 2.43 [95% CI, 1.62-3.64], P<0.001) were significantly greater in the ductal stent group. Median adjusted index hospital costs were $198 300 ($11 6400-$340 000) versus $120 400 ($81 800-$192 400) for the surgical shunt and ductal stent cohorts, respectively (P<0.001). Conclusions Ductal stenting has become an increasingly utilized palliative approach to secure pulmonary blood flow in neonates with congenital heart disease with ductal-dependent pulmonary blood flow in the United States. Ductal stenting is associated with decreased length of hospital stay and reduced overall cost for the index hospitalization but with a greater reintervention risk than surgical shunting.

Perioperative Anesthetic Management of the Pregnant Patient With Congenital Heart Disease Undergoing Cardiac Intervention: A Systematic Review.

Maternal congenital heart disease is increasingly prevalent, and has been associated with a significantly increased risk of maternal, obstetric, and neonatal complications. For patients with CHD who require cardiac interventions during pregnancy, there is little evidence-based guidance with regard to optimal perioperative management. The periprocedural management of pregnant patients with congenital heart disease requires extensive planning and a multidisciplinary teams-based approach. Anesthesia providers must not only be facile in the management of adult congenital heart disease, but cognizant of the normal, but significant, physiologic changes of pregnancy.

A Survey of the Congenital Cardiac Anesthesia Society on the Use and Clinical Application of Near- Infrared Tissue Oximetry in Pediatric Cardiac Surgery.

OBJECTIVE: To better understand the patterns of use and the perceived utility of tissue oximetry in pediatric cardiac surgery. DESIGN: A voluntary 32-question Research Electronic Data Capture survey instrument was sent twice via e-mail to the entire Congenital Cardiac Anesthesia Society (CCAS) membership (January 13, 2021 and March 9,2021). SETTING: International multi-institutional, universities, academic centers, and community hospitals. PARTICIPANTS: CCAS members. INTERVENTIONS: Not applicable. MEASUREMENTS AND MAIN RESULTS: The survey was completed by 185 of 1,131 members (16.4% response rate). The majority of respondents (93.5%) reported use of tissue oximetry, with 97.1% reporting use for cardiac surgery with cardiopulmonary bypass, 76.3% for cardiac surgery without cardiopulmonary bypass, 34.7% in the cardiac catheterization laboratory, and 39.3% for major noncardiac surgeries. Only 14.5% reported that their institution had a formal near-infrared spectroscopy/tissue oximetry-based protocol. The most common sensor placement configuration was bilateral cerebral. More than 90% of respondents reported having made a clinical management change based on tissue oximetry values, although there was variability as to when respondents would intervene. The majority of respondents agreed or strongly agreed that tissue oximetry adds diagnostic value to standard intraoperative monitors, validates clinical observations, and aids in guiding patient management. Most, however, felt that tissue oximetry alone is not enough to inform management changes. CONCLUSIONS: Near-infrared spectroscopy-based tissue oximetry frequently used was by CCAS members, but with significant variations in clinical application.

Elective Non-Urgent Balloon-Atrial Septostomy in Infants with d-Transposition of the Great Arteries Does Not Eliminate the Need for PGE1 Therapy at the Time of Arterial Switch Operation.

Once a mainstay in the treatment of neonates with d-transposition of the great arteries (d-TGA), the application of balloon atrial septostomy (BAS) in the d-TGA population has become more selective. Currently, there is no clear evidence for or against a selective BAS strategy. The aims of this single-center retrospective study were to determine the incidence of BAS in the neonatal d-TGA population in the current era, to measure the rate of procedural success, and to compare the outcomes and complication rates of patients who underwent BAS to those who underwent neonatal ASO alone. Between 2012 and 2018, 147 patients with d-TGA underwent initial medical management and ASO, 73 of which underwent BAS. The percentage of patients that underwent BAS decreased from 73 to 33% over the study time period. In patients with d-TGA with intact ventricular septum, 33% of patients remained off of PGE1 at the time of surgery regardless of BAS. In d-TGA with ventricular septal defect, 85.7% of those that underwent BAS and 54.1% of those who did not remained off of PGE1 at the time of surgery, however, this difference did not reach statistical significance. In this single institution retrospective cohort of patients with d-TGA, the performance of a technically successful balloon atrial septostomy did not eliminate the need for PGE1 therapy at the time of definitive ASO. This was true regardless of the presence or absence of a ventricular septal defect.

Near-Infrared Spectroscopy in Pediatric Congenital Heart Disease.

Near-infrared spectroscopy (NIRS) is widely used to monitor tissue oxygenation in the pediatric cardiac surgical population. Clinicians who use NIRS must understand the underlying measurement principles in order to interpret and use this monitoring modality appropriately. The aims of this narrative review are to provide a brief overview of NIRS technology, discuss the normative and critical values of cerebral and somatic tissue oxygen saturation and the interpretation of these values, present the clinical studies (and their limitations) of NIRS as a perioperative monitoring modality in the pediatric congenital heart disease population, and introduce the emerging and future applications of NIRS.

Elective Extracorporeal Membrane Oxygenation Support for High-Risk Pediatric Cardiac Catheterization.

OBJECTIVE: The utility of extracorporeal membrane oxygenation (ECMO) as an elective support modality for high-risk cardiac procedures is extensively described in adults, but its use in children is limited to isolated reports. The objective of this study was to analyze the outcomes of patients who underwent elective cannulation to ECMO for this purpose. DESIGN: Single-center, retrospective chart review. SETTING: Free-standing pediatric tertiary care center. PARTICIPANTS: Patients who underwent elective cannulation to ECMO for cardiorespiratory support during a high-risk cardiac catheterization procedure. INTERVENTIONS: Elective ECMO cannulation for high-risk percutaneous cardiac interventions or electrophysiology procedures. MEASUREMENTS AND MAIN RESULTS: Survival to discharge was 71.4% compared with 30% for patients who required extracorporeal cardiopulmonary resuscitation in the cardiac catheterization laboratory. The mean duration of cannulation was 137.43 hours (range 27-615 h, median 55 h). There were no major neurologic sequelae, but ECMO circuit thrombosis (57%) was relatively common. CONCLUSION: The use of elective ECMO support for high-risk pediatric cardiac catheterizations can be accomplished safely and may allow for an improved rate of survival with lower rates of severe adverse events compared with extracorporeal cardiopulmonary resuscitation as rescue therapy.

Titration of Bivalirudin Infusion in the Pediatric Cardiac Catheterization Laboratory: A Case Report.

Bivalirudin is a direct thrombin inhibitor that is used as a procedural anticoagulant during percutaneous coronary interventions and cardiac surgery for patients with heparin-resistant thrombosis or heparin-induced thrombocytopenia. There is a robust literature describing its safety and efficacy in adults; however, its use in the pediatric population is relatively rare, with dosing extrapolated from adult data. In this case report, we describe a 4-year-old with complex congenital heart disease and history of heparin-induced thrombocytopenia who required bivalirudin dose uptitration during cardiac catheterization.

Bivalirudin for Pediatric Procedural Anticoagulation: A Narrative Review.

Bivalirudin (Angiomax; The Medicines Company, Parsippany, NJ), a direct thrombin inhibitor, has found increasing utilization as a heparin alternative in the pediatric population, most commonly for the treatment of thrombosis secondary to heparin-induced thrombocytopenia. Due to the relative rarity of heparin-induced thrombocytopenia as well as the lack of Food and Drug Administration-approved indications in this age group, much of what is known regarding the pharmacokinetics and pharmacodynamics of bivalirudin in this population has been extrapolated from adult data. This narrative review will present recommendations regarding the use of bivalirudin for procedural anticoagulation in the pediatric population based on the published literature.

Delayed Presentation of Traumatic Pericardial Rupture: Diagnostic and Surgical Considerations for Treatment.

Traumatic pericardial rupture is a rare event with high mortality. We present the case of a 15-year-old boy who sustained thoracic and abdominal trauma secondary to motor vehicle collision, with a delayed diagnosis of traumatic pericardial rupture with cardiac herniation. Out of concern for torsion and hemodynamic collapse, surgical repair was advised. We have developed a novel surgical approach to this rare condition, utilizing a combination of thoracoscopic and open surgical techniques. The guiding principles of our repair include the utilization of fenestrated pieces of bovine pericardium to create a tension free repair, minimizing the likelihood of pericardial effusion, and returning the cardiac mass to normal anatomic position.

Perioperative and Anesthetic Considerations in Interrupted Aortic Arch.

Interrupted aortic arch (IAA) is defined as the loss of luminal continuity between the ascending and descending aorta and is classified based on the anatomic level of interruption. IAA is associated with a number of intracardiac anomalies with the most common being patent ductus arteriosus, ventricular septal defect, and left ventricular outflow obstruction. There is also a strong association between type B interruption and 22q11 deletion syndrome. The perioperative management of the neonate with IAA begins in the intensive care unit with optimization of end-organ perfusion and function. Survival depends on the prompt initiation of prostaglandin E1 in order to maintain ductal patency, careful management of the patient's ratio of pulmonary to systemic blood flow (Qp:Qs), and a thorough understanding of the physiologic implications of the surgical plan, type of interruption, and associated syndromes and anomalies. This review will focus on the anatomy, physiology, and perioperative anesthetic management considerations specific to the management of IAA.

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

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

Simulation-based Assessment to Reliably Identify Key Resident Performance Attributes.

BACKGROUND: Obtaining reliable and valid information on resident performance is critical to patient safety and training program improvement. The goals were to characterize important anesthesia resident performance gaps that are not typically evaluated, and to further validate scores from a multiscenario simulation-based assessment. METHODS: Seven high-fidelity scenarios reflecting core anesthesiology skills were administered to 51 first-year residents (CA-1s) and 16 third-year residents (CA-3s) from three residency programs. Twenty trained attending anesthesiologists rated resident performances using a seven-point behaviorally anchored rating scale for five domains: (1) formulate a clear plan, (2) modify the plan under changing conditions, (3) communicate effectively, (4) identify performance improvement opportunities, and (5) recognize limits. A second rater assessed 10% of encounters. Scores and variances for each domain, each scenario, and the total were compared. Low domain ratings (1, 2) were examined in detail. RESULTS: Interrater agreement was 0.76; reliability of the seven-scenario assessment was r = 0.70. CA-3s had a significantly higher average total score (4.9 ± 1.1 vs. 4.6 ± 1.1, P = 0.01, effect size = 0.33). CA-3s significantly outscored CA-1s for five of seven scenarios and domains 1, 2, and 3. CA-1s had a significantly higher proportion of worrisome ratings than CA-3s (chi-square = 24.1, P < 0.01, effect size = 1.50). Ninety-eight percent of residents rated the simulations more educational than an average day in the operating room. CONCLUSIONS: Sensitivity of the assessment to CA-1 versus CA-3 performance differences for most scenarios and domains supports validity. No differences, by experience level, were detected for two domains associated with reflective practice. Smaller score variances for CA-3s likely reflect a training effect; however, worrisome performance scores for both CA-1s and CA-3s suggest room for improvement.