Analgesia, Sedation, and Anesthesia for Neonates With Cardiac Disease.

Analgesia, sedation, and anesthesia are a continuum. Diagnostic and/or therapeutic procedures in newborns often require analgesia, sedation, and/or anesthesia. Newborns, in general, and, particularly, those with heart disease, have an increased risk of serious adverse events, including mortality under anesthesia. In this section, we discuss the assessment and management of pain and discomfort during interventions, review the doses and side effects of commonly used medications, and provide recommendations for their use in newborns with heart disease. For procedures requiring deeper levels of sedation and anesthesia, airway and hemodynamic support might be necessary. Although associations of long-term deleterious neurocognitive effects of anesthetic agents have received considerable attention in both scientific and lay press, causality is not established. Nonetheless, an early multimodal, multidisciplinary approach is beneficial for safe management before, during, and after interventional procedures and surgery to avoid problems of tolerance and delirium, which can contribute to long-term cognitive dysfunction.

Bronchopulmonary dysplasia.

Bronchopulmonary dysplasia is the most frequent adverse outcome of prematurity. Before implementation of antenatal steroids and surfactant therapy, bronchopulmonary dysplasia was mostly characterized by fibrotic, scarred, and hyper-inflated lungs due to pulmonary injury following mechanical ventilation and oxygen toxicity. With advances in neonatal medicine, this "old" bronchopulmonary dysplasia has changed to a "new" bronchopulmonary dysplasia characterized by an arrest in lung growth, leading to alveolar simplification and pulmonary vascular dysangiogenesis. While the old definition was based on the need for oxygen supplementation at a postnatal age of 28 days or at a corrected gestational age of 36 weeks, the newer definition looks at the mode of respiratory support required (eg, invasive versus noninvasive) and is then graded as mild, moderate, or severe. Patients with bronchopulmonary dysplasia may present with significantly impaired pulmonary function, reactive airway disease, or exercise intolerance. Over time, these patients may develop asthma or chronic obstructive pulmonary disease. The most serious long-term complication is the development of pulmonary vascular disease and pulmonary hypertension. Medical treatment often includes diuretics, steroids, bronchodilators, or oxygen supplementation and in the presence of pulmonary hypertension medication to decrease the pulmonary vascular resistance. Perioperative anesthetic risk is increased in children with pulmonary hypertension. These patients might require additional diagnostic imaging and plans for increased resource allocation such as postoperative intensive care admission.

Impact of Modified Anesthesia Management for Pediatric Patients With Williams Syndrome.

OBJECTIVE: This study compared the percent change in systolic blood pressure and the incidence of adverse cardiac events (ACEs; defined as cardiac arrest, cardiopulmonary resuscitation, arrhythmias, or ST-segment changes) during anesthesia induction in patients with Williams syndrome (WS) before and after implementation of a perioperative management strategy. DESIGN: Retrospective observational cohort study. SETTING: Single quaternary academic referral center. PARTICIPANTS: The authors reviewed the records of all children with WS at the authors' institution who underwent general anesthesia for cardiac catheterization, diagnostic imaging, or any type of surgery between November 2008 and August 2019. The authors identified 142 patients with WS, 48 of whom underwent 118 general anesthesia administrations. A historic group (HG) was compared with the intervention group (IG). INTERVENTIONS: Change in perioperative management (three-stage risk stratification: preoperative intravenous hydration, intravenous anesthesia induction, and early use of vasoactives). MEASUREMENTS AND MAIN RESULTS: The authors determined event rates within 60 minutes of anesthesia induction. Standardized mean difference (SMD) was calculated (SMD >0.2 suggests clinically meaningful difference). Sixty-seven general anesthesia encounters were recorded in the HG (mean age, 4.8 years; mean weight, 16.3 kg) and 51 in the IG (mean age, 6.0 years; mean weight, 18.2 kg). The change in systolic blood pressure was -17.5% (-30.0, -5.0) in the HG versus -9% (-18.0, 5.0) in the IG (p = 0.015; SMD = 0.419), and the incidence of ACEs was 6% in the HG and 2% in the IG (p = 0.542; SMD = 0.207). CONCLUSIONS: Preoperative risk stratification, preoperative intravenous hydration, intravenous induction, and early use of continuous vasoactives resulted in greater hemodynamic stability, with a 2% incidence of ACEs.

Pharmacokinetics of Cefazolin and Vancomycin in Infants Undergoing Open-Heart Surgery With Cardiopulmonary Bypass.

BACKGROUND: Gram-positive bacteria account for nearly three-quarters of all surgical site infections. Antibiotic prophylaxis against these bacteria with cephalosporins or, in select circumstances, with vancomycin is considered standard of care for prevention of surgical site infections. There is little evidence to describe the optimal dosing regimen for surgical site infection prophylaxis in infants undergoing cardiac surgery, and a great deal of institutional variability exists in dosing prophylactic antibiotics. We designed this study to describe an optimal dose regimen for cephalosporin and vancomycin based on pharmacokinetic evidence for infant open-heart surgery on cardiopulmonary bypass. METHODS: Two separate cohorts of infants undergoing cardiac surgery with cardiopulmonary bypass were evaluated. Plasma concentrations of vancomycin (cohort 1, N = 10) and cefazolin (cohort 2, N = 10) were measured, and mixed-effects pharmacokinetic models were constructed for each drug. Simulations of various dosing regimens were performed to describe an appropriate dosing regimen necessary to maintain antibiotic concentrations above the susceptibility cutoff for staphylococci. RESULTS: Both cefazolin and vancomycin plasma concentration versus time profiles were characterized by a 2-compartment model. Subject weight was a significant covariate for V1 for vancomycin. Subject age was a significant covariate for V1 for cefazolin. Cardiopulmonary bypass did not influence concentration versus time profiles. Simulations demonstrated that a 1-hour vancomycin infusion (15 mg·kg), repeated every 12 hours and a 10-minute infusion of cefazolin (30 mg·kg), repeated every 4 hours maintained plasma concentrations above 4 µg·mL and 16 µg·mL, for vancomycin and cefazolin, respectively. Both concentrations are above the minimum inhibitory concentration 90 for most susceptible staphylococci. CONCLUSIONS: Prophylactic treatment of vancomycin 15 mg·kg infused >1 hour with 12-hour redosing and cefazolin 30 mg·kg infused >10 minutes with 4-hour redosing will maintain serum levels of each antibiotic above the susceptibility cut-offs for susceptible staphylococci in infants undergoing cardiac surgery. Cefazolin levels may be adequate for some, but not all, Gram-negative bacteria. The effect of cardiopulmonary bypass on pharmacokinetics is negligible.

Perioperative management of pediatric en-bloc combined heart-liver transplants: a case series review.

BACKGROUND: Combined heart and liver transplantation (CHLT) in the pediatric population involves a complex group of patients, many of whom have palliated congenital heart disease (CHD) involving single ventricle physiology. OBJECTIVE: The purpose of this study was to describe the perioperative management of pediatric patients undergoing CHLT at a single institution and to identify management strategies that may be used to optimize perioperative care. METHODS: We did a retrospective database review of all patients receiving CHLT at a children's hospital between 2006 and 2014. Information collected included preoperative characteristics, intraoperative management, blood transfusions, and postoperative morbidity and mortality. RESULTS: Five pediatric CHLTs were performed over an 8-year period. All patients had a history of complex CHD with multiple sternotomies, three of whom had failing Fontan physiology. Patient age ranged from 7 to 23 years and weight from 29.5 to 68.5 kg. All CHLTs were performed using an en-bloc technique where both the donor heart and liver were implanted together on cardiopulmonary bypass (CPB). The median operating room time was 14.25 h, median CPB time was 3.58 h, and median donor ischemia time was 4.13 h. Patients separated from CPB on dopamine, epinephrine, and milrinone infusions and two required inhaled nitric oxide. All patients received a massive intraoperative blood transfusion post CPB with amounts ranging from one to three times the patient's estimated blood volume. The patient who required the most transfusions was in decompensated heart and liver failure preoperatively. Four of the five patients received an antifibrinolytic agent as well as a procoagulant (prothrombin complex concentrate or recombinant activated Factor VII) to assist with hemostasis. There were no 30-day thromboembolic events detected. Postoperatively the median length of mechanical ventilation, ICU stay and stay to hospital discharge was 4, 8, and 37 days, respectively. All patients are alive and free from allograft rejection at this time. CONCLUSION: Combined heart and liver transplantation in the pediatric population involves a complex group of patients with unique perioperative challenges. Successful management starts with thorough preoperative planning and communication and involves strategies to deal with massive intraoperative hemorrhage and coagulopathy in addition to protecting and supporting the transplanted heart and liver and meticulous surgical technique. An integrated multidisciplinary team approach is the cornerstone for successful outcomes.

Factors contributing to adverse perioperative events in adults with congenital heart disease: a structured analysis of cases from the closed claims project.

OBJECTIVE: Prior investigations have suggested that the rapidly growing population of adults with congenital heart disease is at increased risk of perioperative morbidity and mortality, but information is limited on the nature of those perioperative factors that may relate to adverse outcomes. We sought to use a national claims database to describe the contribution of perioperative factors to adverse outcomes and compare contributing factors in cardiac vs. noncardiac operations. DESIGN: The study is a retrospective in-depth structured analysis of cases from the Anesthesia Closed Claims Project database. SETTING: We examined the largest national anesthesia malpractice claims database. PATIENTS: We included all claims cases involving adult patients with congenital heart disease (CHD). INTERVENTIONS: Patients in this retrospective analysis were classified by type of surgery (cardiac or noncardiac). OUTCOME MEASURES: Perioperative factors contributing to an adverse event were assessed by an expert panel of cardiac anesthesiologists. RESULTS: Of 21 confirmed cases, 11 (52%) involved cardiac procedures and 10 (48%) noncardiac procedures. The most common factors contributing to the adverse event in cardiac cases were surgical technique (73% of cases) and intraoperative anesthetic care (55%), whereas in noncardiac cases, postoperative monitoring/care (50%), CHD (50%) and preoperative assessment or optimization (40%) were most common. The factors contributing to the patient injury differed similarly: in cardiac cases, the most common factors were intraoperative anesthetic care (55%) and surgical technique (45%) compared with postoperative monitoring/care (50%) and CHD (50%) in noncardiac cases. CONCLUSIONS: Within the limitations of a small number of events in a claims-based database, this study offers advantages of being a national, structured analysis of real cases to provide detailed information on phenomena that are otherwise abstract and hypothesized by expert opinion. These results should help affirm the role of anesthesiologists in acquiring and executing expertise as consultants in perioperative medicine for adults with congenital heart disease patients.

Knowledge and attitudes of anesthesia providers about noncardiac surgery in adults with congenital heart disease.

OBJECTIVE: To examine the knowledge and attitudes of anesthesia providers in relation to the care of adult congenital heart disease (ACHD) patients presenting for noncardiac surgery. DESIGN/SETTING: A novel survey was designed and administered to 168 anesthesiologists across a single academic department in a range of practice environments. INTERVENTIONS: None. OUTCOME MEASURES: Survey responses, including true/false, multiple choice, and Likert scale questions. RESULTS: A total of 118 anesthesiologists (response rate = 70%) completed the survey. Knowledge scores ranged from 0 to 19 (median [interquartile range] = 7 [5-13]) out of a possible maximum of 20. Total knowledge scores differed significantly by fellowship background (P = .004), with higher scores in those with cardiac (11 [7-15], P = .005) and pediatric (12 [6-15], P = .001) fellowship training, but not in those with critical care, obstetric, regional, or pain management training. Scores also differed by frequency of providing care for cardiopulmonary bypass cases and frequency of providing care for patients under 2 years of age (P < .001 for both), but not by gender or years removed from residency. Respondents reported only moderate levels of comfort with a range of questions about providing perioperative or obstetric care to ACHD patients, with decreasing levels of comfort reported in patients with more complex lesions. CONCLUSIONS: Within the context of the limitations of a single-institution survey design, the low levels of knowledge and comfort we observed suggest that providers may benefit from improved training and protocols for ensuring adequate preparedness for the care of ACHD patients.

Home monitoring program reduces interstage mortality after the modified Norwood procedure.

BACKGROUND: From 2002 to 2005, the interstage mortality after a modified Norwood procedure was 7% in our program. An interstage home monitoring program (HMP) was established to identify Norwood procedure patients at increased risk of decompensation and to reduce interstage mortality. METHODS: Results of the first 5 years of the Norwood HMP were reviewed retrospectively. Interstage was defined as the time between Norwood hospital discharge and admission for second stage surgical palliation. In the HMP, families documented oxygen saturation, heart rate, weight, and feedings daily. Nurse practitioners called each family at least weekly, and when issues arose, action plans were determined based on symptom severity. RESULTS: Between October 2005 and October 2010 there were 46 Norwood procedure patients who survived to hospital discharge. All were enrolled in the HMP. Forty-five patients had a Norwood procedure with right ventricle to pulmonary artery conduit, and 1 patient had a modified Blalock-Taussig shunt. Interstage survival was 100%. Nineteen patients (41%) were admitted interstage; 5 patients were admitted twice, 1 patient was admitted 4 times. Seventeen patients (37%) required interstage interventions. Eight patients (17%) required major interventions: conduit stenting, aortic arch balloon angioplasty, emergent shunt, or early Glenn surgery. Minor interventions included supplemental oxygen, blood transfusion, intravenous hydration, diuresis, anti-arrhythmic therapy, or feeding adjustments. CONCLUSIONS: In the first 5 years of the HMP, all infants discharged after a modified Norwood procedure survived the interstage period. The HMP altered clinical management in 37% of patients. Home monitoring of oxygen saturation, heart rate, weight, and feedings, along with comprehensive care coordination, allowed timely interventions and reduced interstage mortality from 7% to 0%.

Evaluation of pediatric near-infrared cerebral oximeter for cardiac disease.

BACKGROUND: Cerebral hypoxia-ischemia remains a complication in children with congenital heart disease. Near-infrared spectroscopy can be utilized at the bedside to detect cerebral hypoxia-ischemia. This study aimed to calibrate and validate an advanced technology near-infrared cerebral oximeter for use in children with congenital heart disease. METHODS: After institutional review board approval and parental consent, 100 children less than 12 years and less than 40 kg were enrolled. Phase I (calibration) measured arterial and jugular venous saturation (SaO(2), SjO(2)) by co-oximetry simultaneously with device signals to calibrate an algorithm to determine regional cerebral saturation against a weighted average cerebral saturation (0.7 SjO(2) + 0.3 SaO(2)). Phase II (validation) evaluated regional cerebral saturation from the algorithm against the weighted average cerebral saturation by correlation, bias, precision, and A(Root Mean Square) assessed by linear regression and Bland-Altman analysis. RESULTS: Of 100 patients, 86 were evaluable consisting of 7 neonates, 44 infants, and 35 children of whom 55% were female, 79% Caucasian, and 41% with cyanotic disease. The SaO(2) and regional cerebral saturation ranged from 34% to 100% and 34% to 91%, respectively. There were no significant differences in subject characteristics between phases. For the entire cohort, A(RMS), bias, precision, and correlation coefficient were 5.4%, 0.5%, 5.39%, and 0.88, respectively. Age, skin color, and hematocrit did not affect these values. CONCLUSIONS: This cerebral oximeter accurately measures the absolute value of cerebral saturation in children over a wide range of oxygenation and subject characteristics, offering advantages in assessment of cerebral hypoxia-ischemia in congenital heart disease.

Perioperative management of the morbidly obese adolescent with heart failure undergoing bariatric surgery.

The incidence and prevalence of adolescent obesity and adolescent heart failure are increasing, and anesthesiologists increasingly will encounter patients with both conditions. A greater understanding of the physiologic challenges of adolescent heart failure as they relate to the perioperative stressors of anesthesia and bariatric surgery is necessary to successfully manage the perioperative risks faced by this growing subpopulation. Here, we present a representative case of a morbidly obese adolescent with heart failure who underwent a laparoscopic bariatric operation and review the limited available literature on perioperative management in this age group. Specifically, we review evidence and offer recommendations related to preoperative evaluation, venous thromboembolism prophylaxis, positioning, induction, airway management, monitoring, anesthetic maintenance, ventilator management, and adverse effects of the pneumoperitoneum, rhabdomyolysis, and postoperative care.

Cerebral autoregulation in neonates with a hemodynamically significant patent ductus arteriosus.

OBJECTIVE: Very low birth weight (VLBW) preterm infants are at risk for impaired cerebral autoregulation with pressure passive blood flow. Fluctuations in cerebral perfusion may occur in infants with a hemodynamically significant patent ductus arteriosus (hsPDA), especially during ductal closure. Our goal was to compare cerebral autoregulation using near-infrared spectroscopy in VLBW infants treated for an hsPDA. STUDY DESIGN: This prospective observational study enrolled 28 VLBW infants with an hsPDA diagnosed by echocardiography and 12 control VLBW infants without an hsPDA. Near-infrared spectroscopy cerebral monitoring was applied during conservative treatment, indomethacin treatment, or surgical ligation. A cerebral pressure passivity index (PPI) was calculated, and PPI differences were compared using a mixed-effects regression model. Cranial ultrasound and magnetic resonance imaging data were also assessed. RESULTS: Infants with surgically ligated hsPDAs were more likely to have had a greater PPI within 2 hours following ligation than were those treated with conservative management (P=.04) or indomethacin (P=.0007). These differences resolved by 6 hours after treatment. CONCLUSIONS: Cerebral autoregulation was better preserved after indomethacin treatment of an hsPDA compared with surgical ligation. Infants requiring surgical hsPDA ligation may be at increased risk for cerebral pressure passivity in the 6 hours following surgery.

Optimal flow rate for antegrade cerebral perfusion.

OBJECTIVE: Antegrade cerebral perfusion is widely used in neonatal heart surgery, yet commonly used flow rates have never been standardized. The objective of this study was to determine the antegrade cerebral perfusion flow rate that most closely matches standard cardiopulmonary bypass conditions. METHODS: Nine neonatal piglets underwent deep hypothermic cardiopulmonary bypass at a total body flow of 100 mL/kg/min (baseline). Antegrade cerebral perfusion was conducted via innominate artery cannulation at perfusion rates of 10, 30, and 50 mL/kg/min in random order. Cerebral blood flow was measured using fluorescent microspheres. Regional oxygen saturation and cerebral oxygen extraction were monitored. RESULTS: Cerebral blood flow was as follows: baseline, 60 +/- 17 mL/100 g/min; antegrade cerebral perfusion at 50 mL/kg/min, 56 +/- 17 mL/100 g/min; antegrade cerebral perfusion at 30 mL/kg/min, 36 +/- 9 mL/100 g/min; and antegrade cerebral perfusion at 10 mL/kg/min, 13 +/- 6 mL/100 g/min. At an antegrade cerebral perfusion rate of 50 mL/kg/min, cerebral blood flow matched baseline (P = .87), as did regional oxygen saturation (P = .13). Antegrade cerebral perfusion at 30 mL/kg/min provided approximately 60% of baseline cerebral blood flow (P < .002); however, regional oxygen saturation was equal to baseline (P = .93). Antegrade cerebral perfusion at 10 mL/kg/min provided 20% of baseline cerebral blood flow (P < .001) and a lower regional oxygen saturation than baseline (P = .011). Cerebral oxygen extraction at antegrade cerebral perfusion rates of 30 and 50 mL/kg/min was equal to baseline (P = .53, .48) but greater than baseline (P < .0001) at an antegrade cerebral perfusion rate of 10 mL/kg/min. The distributions of cerebral blood flow and regional oxygen saturation were equal in each brain hemisphere at all antegrade cerebral perfusion rates. CONCLUSION: Cerebral blood flow increased with antegrade cerebral perfusion rate. At an antegrade cerebral perfusion rate of 50 mL/kg/min, cerebral blood flow was equal to baseline, but regional oxygen saturation and cerebral oxygen extraction trends suggested more oxygenation than baseline. An antegrade cerebral perfusion rate of 30 mL/kg/min provided only 60% of baseline cerebral blood flow, but cerebral oxygen extraction and regional oxygen saturation were equal to baseline. An antegrade cerebral perfusion rate that closely matches standard cardiopulmonary bypass conditions is between 30 and 50 mL/kg/min.

Perioperative complications in children with pulmonary hypertension undergoing general anesthesia with ketamine.

BACKGROUND: Pulmonary arterial hypertension (PAH) is associated with significant perioperative risk for major complications in children, including pulmonary hypertensive crisis and cardiac arrest. Uncertainty remains about the safety of ketamine anesthesia in this patient population. AIM: Retrospectively review the medical records of children with PAH to ascertain the nature and frequency of peri-procedural complications and to determine whether ketamine administration was associated with peri-procedural complications. METHODS: Children with PAH (mean pulmonary artery pressure > or =25 mmHg and pulmonary vascular resistance index > or =3 Wood units) who underwent general anesthesia for procedures during a 6-year period (2002-2008) were enrolled. Details about the patient, PAH, procedure, anesthetic and postprocedural course were noted, including adverse events during or within 48 h of the procedure. Complication rates were reported per procedure. Association between ketamine and peri-procedural complications was tested. RESULTS: Sixty-eight children (median age 7.3 year, median weight 22 kg) underwent 192 procedures. Severity of PAH was mild (23%), moderate (37%), and severe (40%). Procedures undertaken were major surgery (n = 20), minor surgery (n = 27), cardiac catheterization (n = 128) and nonsurgical procedures (n = 17). Ketamine was administered during 149 procedures. Twenty minor and nine major complications were noted. Incidence of cardiac arrest was 0.78% for cardiac catheterization procedures, 10% for major surgical procedures and 1.6% for all procedures. There was no procedure-related mortality. Ketamine administration was not associated with increased complications. CONCLUSIONS: Ketamine appears to be a safe anesthetic option for children with PAH. We report rates for cardiopulmonary resuscitation and mortality that are more favorable than those previously reported.

Deep brain hyperthermia while rewarming from hypothermic circulatory arrest.

BACKGROUND: Neurologic injury is a feared and serious long-term complication of cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). Postoperative hyperthermia was found to enhance postischemic neurologic injury. The use of core temperature as the reference point through CPB assumes parallel changes in brain temperature. We tested the hypothesis that regional and deep brain temperature (DBT) differ during cooling, DHCA, and rewarming. METHODS: Neonatal piglets (n = 9) were subject to CPB and cooled to rectal temperature (RT) of 18 degrees C, 30 minutes of DHCA were initiated, and subsequently the piglets were rewarmed to RT of 36.5 degrees C and weaned from CPB. Temperature probes were inserted into the DBT targeting the caudate and thalamic nuclei, their position confirmed by pathology. Superficial brain temperature was measured by a temperature probe inserted extradurally. RT, nasopharyngeal (NPT), and tympanic (TT) temperatures were recorded. RESULTS: During cooling the deep brain cooled faster and to lower temperatures compared to RT and TT; NPT reflected DBT accurately. During rewarming DBT was significantly higher than RT and TT. By the end of rewarming the difference between the deep brain and the RT reached statistical significance (30 minutes: 35.1 +/- 0.7 vs. 32.3 +/- 0.7 p < 0.05, respectively, 40 minutes: 37.5 +/- 0.3 vs. 34.7 +/- 0.8 p < 0.05, respectively). CONCLUSION: Deep brain hyperthermia routinely occurs during the last stages of rewarming following DHCA. DBT is accurately reflected by NPT and is directly correlated with inflow temperature. Therefore, during rewarming inflow temperatures should not exceed 36 degrees C and NPT should be closely monitored.

Dynamics of human myocardial progenitor cell populations in the neonatal period.

BACKGROUND: Pluripotent cardiac progenitor cells resident in myocardium offer a potentially promising role in promoting recovery from injury. In pediatric congenital heart disease (CHD) patients, manipulation of resident progenitor cells may provide important new approaches to improving outcomes. Our study goals were to identify and quantitate populations of progenitor cells in human neonatal myocardium during the early postnatal period and determine the proliferative capacity of differentiated cardiac myocytes. METHODS: Immunologic markers of cell lineage (stage-specific embryonic antigen 4 [SSEA-4], islet cell antigen 1 [Isl1], c-kit, Nkx2.5, sarcoplasmic reticulum calcium-regulated ATPase type 2 [SERCA2]) and proliferation (Ki67) were localized in right ventricular biopsies from 32 CHD patients aged 2 to 93 days. RESULTS: Neonatal myocardium contains progenitor cells and transitional cells expressing progenitor and differentiated myocyte marker proteins. Some cells expressed the pluripotent cell marker c-kit and also coexpressed the myocyte marker SERCA2. Multipotent progenitor cells, identified by the expression of Isl1, were found. Ki67 was expressed in some myocytes and in nonmyocyte cells. A few cells expressing SSEA-4 and Isl1 were observed during the early postnatal period. Cells expressing c-kit, the premyocyte marker Nkx2.5, and Ki67 were found throughout the first postnatal month. A progressive decline in cell density during the first postnatal month was observed for c-kit+ cells (p = 0.0013) and Nkx2.5+ cells (p = 0.0001). The percentage of cells expressing Ki67 declined during the first 3 postnatal months (p = 0.0030). CONCLUSIONS: Cells in an incomplete state of cardiomyocyte differentiation continue to reside in the infant heart. However, the relative density of progenitor cells declines during the first postnatal month.

A randomized, controlled trial of aprotinin in neonates undergoing open-heart surgery.

BACKGROUND: Neonates undergoing open-heart surgery are especially at risk for massive bleeding and pronounced inflammation. The efficacy of aprotinin, a serine protease inhibitor, at ameliorating these adverse effects of cardiopulmonary bypass has not been clearly demonstrated in neonates. METHODS: Term neonates were enrolled and randomly assigned in a blinded fashion to receive saline (group P, placebo) or high-dose aprotinin (group A). Intraoperative management was standardized: surgeon, anesthesia, cardiopulmonary bypass and hemostasis therapy. Patients were admitted postoperatively to a pediatric cardiac intensive care unit. Primary outcome measure of efficacy was duration of the postoperative mechanical ventilation. Secondary outcome measures were total volume and units of blood products transfused intraoperatively and for 24 h after surgery, duration of chest tube in situ, and intensive care and hospital stays after surgery. RESULTS: Twenty-six neonates were enrolled; 13 received aprotinin and 13 received placebo. The study was halted prematurely because of US Food and Drug Administration's concerns about aprotinin's safety. Baseline patient, surgery and cardiopulmonary bypass characteristics were similar between groups. No outcome variables differed between groups (P > 0.05). Duration of postoperative ventilation was 115 +/- 139 h (group A); 126 +/- 82 h (group P); P = 0.29, and total blood product exposure was 8.2 +/- 2.6 U (group A); 8.8 +/- 1.4 U (group P); P = 0.1. Postoperative blood creatinine values did not differ between groups. In-hospital mortality rate was 4%. CONCLUSIONS: Aprotinin was not shown to be efficacious in neonates undergoing open-heart surgery. It is unclear whether adult aprotinin safety data are relevant to neonates undergoing open-heart surgery.

Thoracoscopic repair of a type D esophageal atresia in a newborn with complex congenital heart disease.

This report describes a case of thoracoscopic repair of esophageal atresia with a rare type D tracheoesophageal fistula in a child with complex congenital heart disease. We demonstrate the feasibility of thoracoscopic repair and anesthetic management in a child with complex congenital heart disease.

Children with cardiomyopathy: complications after noncardiac procedures with general anesthesia.

BACKGROUND: Children with cardiomyopathy (CM) often undergo procedures that require general anesthesia (GA) but little is known about anesthesia-related adverse events or postprocedural outcomes. METHODS: After approval, all children with CM who underwent nonopen heart surgical procedures and/or diagnostic imaging under GA at a tertiary children's hospital during January 2002 to May 2005 were identified from a clinical database. Based on their preprocedure fractional shortening (FS) on echocardiogram, systemic ventricular dysfunction was categorized as mild (FS 23-28%), moderate (FS 16-22%), or severe (FS < 16%) and those with normal (FS > 28%) were excluded from review. RESULTS: Twenty-six patients underwent 34 procedures under GA, of whom 13 (38%) had mild or moderate ventricular dysfunction and 21 (62%) had severe dysfunction. Common procedures included pacer/defibrillator placement (43%) and imaging studies (18%). Eighteen complications were noted in 12 patients. Fifteen (83%) complications occurred in patients with severe ventricular dysfunction. One patient with severe ventricular dysfunction died (3% mortality). Hypotension requiring inotropic support was the most frequent complication (61%). Children with severe ventricular dysfunction often required hospital support pre- and postprocedure with 67% requiring intensive care. Hospital stay was longer for patients with severe ventricular dysfunction compared with children with mild or moderate ventricular dysfunction (P = 0.006). CONCLUSIONS: The 30-day mortality rate was low but complications were common, especially in patients with severe ventricular dysfunction. For these patients, we recommend early consideration of perioperative intensive care support to optimize cardiovascular therapy and monitoring.

Outcomes after laparoscopic surgery in neonates with hypoplastic heart left heart syndrome.

BACKGROUND/PURPOSE: Laparoscopy has advanced the care of children for a variety of pediatric surgical diseases. However, complication rates for laparoscopic interventions in neonates with hypoplastic left heart syndrome (HLHS) have not been well described. The purpose of this study is to present the largest reported series of laparoscopic surgery performed in patients with HLHS. METHODS: We conducted a single-institution, retrospective chart review for all neonates with HLHS who underwent a laparoscopic procedure from September 2002 to March 2005. Data regarding patient characteristics, intraoperative monitoring, previous cardiac surgery, perioperative complications, and postoperative mortality were assessed. RESULTS: Twelve patients with HLHS underwent a total of 13 operations during the study period (8 combined Nissen fundoplication and gastrostomy tubes, 3 isolated gastrostomy tubes, 1 Ladd procedure, and 1 combined Nissen fundoplication and gastrocutaneous fistula closure). All cases were completed laparoscopically. Patients had undergone palliative cardiac surgery but were not completely corrected; therefore, they were cyanotic. Perioperative complications were observed in 6 patients (3 gastrostomy tube site infections, 1 small bowel obstruction, 1 postoperative sepsis, and 1 urinary tract infection). There was no mortality in this series. CONCLUSIONS: From this experience, it appears that laparoscopy can be performed safely and with satisfactory outcomes in patients with HLHS. However, a multidisciplinary approach, including the availability of a skilled and experienced cardiac anesthesia team, is believed to be critical to optimize outcomes in these critically ill children.