Anesthetic challenges in patients with multicompartmental lymphatic failure after Fontan palliation undergoing transcatheter thoracic duct decompression.

Lymphatic flow abnormalities are central to the development of protein losing enteropathy, plastic bronchitis, ascites and pleural effusions in patients palliated to the Fontan circulation. These complications can occur in isolation or multicompartmental (two or more). The treatment of multicompartmental lymphatic failure aims at improving thoracic duct drainage. Re-routing the innominate vein to the pulmonary venous atrium decompresses the thoracic duct, as atrial pressure is lower than systemic venous pressure in Fontan circulation. Transcatheter thoracic duct decompression is a new minimally invasive procedure that involves placing covered stents from the innominate vein to the atrium. Patients undergoing this procedure require multiple general anesthetics, presenting challenges in managing the sequelae of disordered lymphatic flow superimposed on Fontan physiology. We reviewed the first 20 patients at the Center for Lymphatic Imaging and Intervention at a tertiary care children's hospital presenting for transcatheter thoracic duct decompression between March 2018 and February 2023. The patients ranged in age from 3 to 26 years. The majority had failed prior catheter-based lymphatic intervention, including selective embolization of abnormal lympho-intestinal and lympho-bronchial connections to treat lymphatic failure in a single compartment. Fourteen had failure in three lymphatic compartments. Patients were functionally impaired (ASA 3-5) with significant comorbidities. Concurrent with thoracic duct decompression, three patients required fenestration closure for the resultant decrease in oxygen saturation. Ten patients had improvement in symptoms, seven had no changes and three have limited follow up. Five (25%) of these patients were deceased as of January 2024 due to non-lymphatic complications from Fontan failure.

Lymphatic imaging and intervention in a pediatric population: Anesthetic considerations.

The recent adoption of an improved lymphatic access technique coupled with Dynamic Contrast-enhanced Magnetic Resonance Lymphangiography has introduced the ability to diagnose and treat severe lymphatic disorders unresponsive to other therapies. All pediatric patients presenting for lymphatic procedures require general anesthesia presenting challenges in managing highly morbid and comorbid conditions both from logistical as well as medical aspects. General anesthesia is used because of the procedural requirement for immobility to accurately place needles and catheters, treat pain secondary to contrast and glue injections, and to accommodate additional procedures. We reviewed a one-year cohort of all pediatric patients in a newly created Center for Lymphatic Imaging and Intervention at a tertiary care children's hospital presenting for lymphatic procedures. The patients ranged in age from 4 days to 17 years and weighed from 2.5 to 92 kg. There were 106 anesthetics for 68 patients. Patients were functionally impaired (98% ASA 3 or 4) and included significant comorbidities (79.4%). Concurrent with lymphatic imaging and intervention additional procedures were frequently performed (76%). They included cardiac catheterization, bronchoscopy, endoscopy, and drain placement (thoracic or abdominal). Paralysis and controlled ventilation was used for all interventions. Reversal of paralysis and tracheal extubation occurred in all patients not previously managed by invasive respiratory support. All patients having an intervention were admitted to intensive care for observation where escalation of care or complications (fever, hypotension, bleeding, or stroke) occurred in 25% in the first 24 hours.

Reduced transfusion requirement with use of fresh whole blood in pediatric cardiac surgical procedures.

BACKGROUND: Pediatric patients undergoing cardiac operations are at high risk for blood loss and transfusion. A practice intended to reduce transfusion using a standard order of 2 units fresh whole blood (< 48 hours from donation) for elective cardiac operations in patients younger than 2 years of age was in place from 1995 to 2010. The objective of this study was to describe blood use in this population and to compare the results with those in published reports describing the use of blood components exclusively for transfusion. METHODS: Retrospective data from a surgical registry and blood bank records for 15 consecutive years were analyzed. Transfusion requirements were identified as donor exposures for the day of operation and the next postoperative day. Transfusions were fresh whole blood, packed red blood cells, platelets, and cryoprecipitate. Donor exposures for subgroups according to procedure and age were compared with those in published reports. RESULTS: The cohort consisted of 4,111 patients with a median age of 94 days and a median weight of 4.4 kg. The median donor exposure was 2 (range, 0 to 28). Younger patients having complex procedures had the most donor exposures. Fewer donor exposures were incurred in all subgroups compared with reports of component use in the literature. CONCLUSIONS: The use of fresh whole blood for cardiac operations in children younger than 2 years old reduces donor exposures compared with published reports of component use.

Evaluation of central venous pressure monitoring in children undergoing craniofacial reconstruction surgery.

BACKGROUND: Massive hemorrhage during craniofacial surgery is common and often results in hypovolemia and hypotension. We conducted this study to assess the effect of the addition of routine central venous pressure (CVP) monitoring on the incidence of intraoperative hypotension and to evaluate the relationship between CVP and hypotension in this population. METHODS: Data from our prospective craniofacial perioperative registry for children 6 to 24 months of age undergoing cranial vault reconstruction with CVP monitoring were compared with data from a historical cohort without CVP monitoring. The incidence and duration of hypotension in the 2 cohorts were compared. In the cohort of subjects with CVP monitoring who experienced hypotension, CVP at the onset of hypotension (T0) was compared with CVP 5 minutes before (T-5) and 5 minutes after (T+5) the onset of hypotension and with the baseline CVP. The amount of time spent at various CVP levels below the baseline, and the associated incidence of hypotension were also determined. RESULTS: Data from 57 registry subjects were compared with data from 115 historical cohort subjects. The median total duration of hypotension in subjects experiencing hypotension was 278 seconds in the CVP cohort versus 165 seconds in the historical cohort; the median difference was 98 seconds (95% confidence interval [CI], -45 to 345 seconds). The incidence of hypotension was 18% in the CVP cohort versus 21% in the historical cohort; the difference in the incidence of hypotension was -3% (95% CI, -10% to 15%). Analysis using a linear mixed effects model showed a significant decrease in CVP from T-5 to T0 (95% CI, -0.9 to -2.2 mm Hg), a significant increase in CVP from T0 to T+5 (95% CI, 1.0-2.4 mm Hg), no significant difference in CVP between T-5 and T+5 (95% CI, -0.9 to 0.9 mm Hg), and a significant decrease in CVP from baseline to T0 (95% CI, -3.4 to -2.1 mm Hg). CVP at T0 was less than the baseline CVP in 97% of hypotensive episodes. When all cases were examined, CVP was ≥3 mm Hg below the baseline for 16% of the total time studied, with an associated incidence of hypotension of 2%. CONCLUSIONS: The implementation of routine CVP monitoring was not associated with a decreased incidence and likely was not associated with a decreased duration of hypotension in this population experiencing massive hemorrhage. Hypotension was associated with a decrease in CVP, and resolution of hypotension was associated with an increase in CVP to prehypotensive levels. However, significant decreases in CVP below the baseline were common and usually not associated with hypotension. The routine use of CVP monitoring in these children is of questionable utility as a means to decrease the incidence and duration of hypotension.

Absence of tachycardia during hypotension in children undergoing craniofacial reconstruction surgery.

BACKGROUND: Tachycardia is a baroreceptor-mediated response to hypotension. Heart rate (HR) behavior in the setting of hypotension in anesthetized children is not well characterized. We conducted this study to assess the relationship between HR and hypotension in a population of anesthetized children experiencing massive blood loss. Our primary hypothesis was that HR would be increased with the onset of hypotension associated with hypovolemia in comparison with time points without hypotension. METHODS: We performed a query of our prospective craniofacial perioperative registry for children younger than 24 months who underwent cranial vault reconstruction surgery. Demographic and perioperative data were extracted, and the intraoperative blood loss was calculated. Vital signs were extracted from our computerized anesthesia record and analyzed. Hypotension was defined as a mean arterial blood pressure <40 mm Hg for at least 3 computerized anesthesia record entries (captured every 15 seconds). The preoperative HR, the average HR over the entire intraoperative period, the HR at the onset of hypotension, and the HR 5 minutes before and 5 minutes after the hypotensive episode were compared. RESULTS: The registry query yielded data from 57 procedures. There were 29 episodes of hypotension occurring in 10 subjects. There was no significant difference in HR at the onset of hypotension (when mean arterial blood pressure decreased below 40 mm Hg) in comparison with the preoperative HR, the average intraoperative HR, or in comparison with 5 minutes before and 5 minutes after the episode of hypotension. CONCLUSIONS: In this study of anesthetized children younger than 24 months undergoing surgery with massive blood loss, hypotension was not associated with an increased HR. HR does not appear to be a useful indicator of hypovolemia in this population.

Implications of anesthesia in children with long QT syndrome.

BACKGROUND: Patients with congenital long QT syndrome (LQTS) are susceptible to an episodic malignant ventricular tachyarrhythmia known as torsade de pointes, which can result in a cardiac arrest and death. Patients can suffer severe cardiac events resulting in syncope, seizures, and sudden cardiac death during times of physical and emotional stress and when exposed to certain drugs including anesthetics. We describe the occurrence of perioperative adverse events (AEs) related to arrhythmias in children with congenital LQTS exposed to volatile general anesthesia and describe associated risk factors. METHODS: We performed a retrospective cohort study of children with LQTS undergoing general anesthesia for noncardiac surgery or device implant, or revision for cardiac rhythm management. This study was a retrospective chart review with data collection from computerized and electronic patient medical records. RESULTS: Seventy-six patients with congenital LQTS were identified who had a total of 114 anesthetic encounters. Of the 114 anesthetic encounters, there were 3 AEs, 2 definite and 1 probable AE, for an incidence of 2.6%. The events occurred in boys (aged 11, 13, and 15 years) while undergoing noncardiac surgery under volatile general anesthesia. All were receiving ß-blocker therapy preoperatively. The AEs occurred in close proximity to the administration of reversal drugs (anticholinesterase/anticholinergic combinations) and the antiemetic ondansetron. The events occurred during emergence from anesthesia, and exclusively in the group of patients who received both reversal drugs and ondansetron. All were treated successfully with short-term antiarrhythmic drug therapy and discharged the next morning. CONCLUSIONS: There is an increased risk of AEs during periods of enhanced sympathetic activity, especially emergence. This risk seems to be further enhanced if drugs are administered at this time that are known either to prolong the corrected QT interval or the transmural dispersion of repolarization or increase the incidence of tachycardia. Restriction of medications that adversely affect ion channels and intense vigilance and monitoring during this time and in the postoperative phase could help prevent occurrence or progression of AEs.

Reconstituted blood reduces blood donor exposures in children undergoing craniofacial reconstruction surgery.

OBJECTIVE/AIMS: To assess the effect of prophylactic administration of fresh-frozen plasma (FFP) in the form of reconstituted blood in children undergoing craniofacial reconstruction. The outcomes of interest included immediate postoperative coagulation laboratory test results, postoperative surgical drain output, and the number of unique blood donor exposures incurred. BACKGROUND: We recently changed our intraoperative transfusion strategy in children undergoing craniofacial reconstruction surgery to one in which blood loss is replaced with donor-matched reconstituted blood rather than traditional blood component therapy. METHODS: We performed a query of our prospective craniofacial surgery perioperative registry for children who underwent fronto-orbital advancement or posterior cranial vault reconstruction. Registry data from this query were compared to data from a historical cohort. RESULTS: Data for 46 registry cases were compared to 150 historical cohort cases. The median number of unique donor exposures for the reconstituted blood group was 2 vs 3 in the historical cohort (P=0.004). The reconstituted blood group had a decreased incidence of postoperative derangements in soluble clotting factor tests (fibrinogen, PT, or aPTT; 2% vs 24%, P=0.001), while there was no evidence for a difference in the incidence of thrombocytopenia. There was no evidence for differences in postoperative surgical drain output in the reconstituted blood group and historical cohort over the first 12, 24, and 48 h. CONCLUSIONS: Prophylactic administration of FFP in the form of donor-matched reconstituted blood in children undergoing craniofacial reconstruction was associated with improved postoperative coagulation parameters, reduced blood donor exposures, and unchanged postoperative surgical drain output.

A comparison of the efficacy and safety of chloral hydrate versus inhaled anesthesia for sedating infants and toddlers for transthoracic echocardiograms.

BACKGROUND: Pediatric patients may need sedation for a transthoracic echocardiogram. Due to the unpredictability and safety concerns with chloral hydrate, we offered mask anesthesia as an alternate strategy. METHODS: A retrospective chart review of 507 pediatric patients sedated for transthoracic echocardiography was conducted to compare the use of oral chloral hydrate (n = 297) with the face mask administration of sevoflurane anesthesia (n = 210). RESULTS: Anesthesia had a shorter time from administration of sedation to hospital discharge (112 vs 155 minutes), largely because of a shorter, more predictable, and less variable time to achieve satisfactory study conditions. Using anesthesia, an average 43-minute difference would allow for an additional procedure using the same resources. Anesthesia was not associated with sedation failure (0% vs 6%), and the duration of examination was shorter (40 vs 46 minutes). There were no significant adverse events in either cohort. CONCLUSION: Anesthesia, although more costly, is balanced by more the efficient use of hospital and parental resources, with greater family and staff satisfaction.

Blood loss, replacement, and associated morbidity in infants and children undergoing craniofacial surgery.

BACKGROUND: Pediatric craniofacial reconstruction (CFR) procedures involve wide scalp dissections with multiple osteotomies and have been associated with significant morbidity. The aim of this study was to document the incidence of clinically important problems, particularly related to blood loss, and perform a risk factor analysis. METHODS: Records of all patients who underwent craniofacial surgery at the Children's Hospital of Philadelphia between December 1, 2001 and January 1, 2006 were reviewed. Data were collected from the electronic anesthesia record, intensive care unit (ICU) progress notes, and discharge summary. All intraoperative laboratory values and all laboratory values obtained upon arrival in the ICU were recorded. A multivariable analysis was performed to evaluate associations between elements of intraoperative management and the following clinical outcomes: intraoperative hypotension, intraoperative metabolic acidosis, presence of a postoperative coagulation test abnormality, and postoperative administration of hemostatic blood products. RESULTS: Data for 159 patients were reviewed. The mean volume of packed red blood cells transfused intraoperatively was 51 ml x kg(-1). Multivariable analysis revealed that intraoperative administration of albumin was strongly correlated with both an increased incidence of postoperative coagulation derangements and postoperative administration of hemostatic blood products (Odds Ratio 5.9, 2.8, respectively), while intraoperative fresh frozen plasma (FFP) administration was associated with an opposite effect (Odds Ratio 0.94, 0.97, respectively). CONCLUSIONS: In pediatric CFR procedures where the volume of blood loss routinely exceeds one blood volume, intraoperative administration of FFP favorably impacted postoperative laboratory coagulation parameters.

The Fontan patient.

Improved surgical and medical management has led to an increase in survival after staged univentricular palliative procedures. Subsequently, this improved survival has led to an increase in the number of patients who will present for noncardiac surgical interventions with Fontan physiology. A comprehensive understanding of normal Fontan physiology and the perturbations that the proposed surgical procedure will likely have is necessary to care for and design a comprehensive anesthetic plan that takes into account the effects of anesthetic agents, ventilation strategies, cardiovascular drugs, and various other perioperative factors. Applying the knowledge presented in this article should enable the anesthesiologist with the necessary principles to care for the patient with Fontan physiology.

Early tracheal extubation in adults undergoing single-lung transplantation for chronic obstructive pulmonary disease: pilot evaluation of perioperative outcome.

The objective of this pilot study was to evaluate the safety and success of early tracheal extubation (ETE) as compared to delayed tracheal extubation (DTE) in single-lung transplantation (SLT) for chronic obstructive pulmonary disease (COPD). This retrospective observational study was undertaken at a university hospital. Fifty-seven adult patients who underwent SLT for COPD (1998-2003) were enrolled. The study cohort was divided into an ETE subgroup (tracheal extubation in the operating room) or a DTE subgroup (tracheal extubation in the intensive care unit). There were no significant differences in perioperative outcomes between subgroups (in-hospital mortality; length of stay; prolonged mechanical ventilation; primary graft dysfunction; pneumonia; atrial fibrillation; renal dysfunction; and, sepsis). The anesthetic technique associated with ETE in SLT for COPD was characterized by limited systemic anesthetics and perioperative thoracic epidural analgesia. Appropriate ETE in SLT for COPD is not only safe but also results in equivalent perioperative outcome when compared to the traditional technique of DTE. Future studies should be powered to examine whether ETE reduces native lung complications such as hyperinflation, pneumonia and pneumothorax.

Cardiopulmonary bypass for lung transplantation in cystic fibrosis: pilot evaluation of perioperative outcome.

OBJECTIVE: The purpose of this study was to determine whether cardiopulmonary bypass (CPB) reduces the incidence of perioperative graft infection after lung transplantation in adults with cystic fibrosis (CF). DESIGN: Retrospective and observational. SETTING: University hospital. PARTICIPANTS: Adults with CF who underwent lung transplantation (1998-2003). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Cohort size was 26: group A (n = 10) who underwent CPB for implantation of both lungs, group B (n = 8) who underwent CPB only for implantation of the second lung, and group C (n = 8) who did not undergo CPB. The 3 cohort subgroups were similar (p > 0.05) in demographics, preoperative lung function, and anesthetic management. Group A had a lower incidence of perioperative pneumonia (p = 0.02). CPB exposure increased transfusion (B > A > C) of fresh frozen plasma and platelets but not packed red blood cells. There were no differences (p > 0.05) in clinical outcome as reflected by duration of mechanical ventilation, tracheal re-intubation, re-exploration for bleeding, sepsis, primary graft dysfunction, renal dysfunction, length of stay, and mortality. CONCLUSIONS: CPB is associated with decreased incidence of early graft infection after lung transplantation for adult CF when used for implantation of both lungs. This may be because of improved decontamination of the operative field before graft implantation.

Female gender increases the risk of death during hospitalization for pediatric cardiac surgery.

OBJECTIVES: The study objective was to determine whether gender is a determinant of in-hospital mortality after surgery to repair congenital heart disease in patients aged 20 years or less. Secondary objectives were to determine other factors associated with increased risk of death and whether female gender is associated with increased length of stay or total charges. METHODS: The study included a retrospective cohort consisting of all records indicating cardiac operations within the Healthcare Cost and Utilization Project Kids' Inpatient Database for the year 2000. Logistic regression was used to simultaneously evaluate the effect of gender on the risk of death while adjusting for all other factors being considered. Logistic regression was then used to evaluate possible differences in length of stay or total charges. RESULTS: Female gender was associated with increased risk of in-hospital death when all of the other measured factors were taken into consideration (odds ratio 1.31, 95% confidence interval 1.02-1.69). Other factors that were significantly associated with increased in-hospital mortality after pediatric cardiac surgery included the number of days between admission and operation; African American race; young age (neonates and infants compared with children aged > or =1 year); pulmonary hypertension; and the Norwood operation. There were no significant gender differences in risk-adjusted length of stay or total charges. CONCLUSIONS: In-hospital mortality after pediatric cardiac surgery seems to be associated with patient gender but not with the type of insurance or ability to access higher-volume pediatric facilities or teaching hospitals.