20-Year Experience With Repair of Pulmonary Atresia or Stenosis and Major Aortopulmonary Collateral Arteries.

BACKGROUND: We have followed a consistent, albeit evolving, strategy for the management of patients with pulmonary atresia or severe stenosis and major aortopulmonary collateral arteries (MAPCAs) that aims to achieve complete repair with low right ventricular pressure by completely incorporating blood supply and relieving stenoses to all lung segments. OBJECTIVES: The purpose of this study was to characterize our 20-year institutional experience managing patients with MAPCAs. METHODS: We reviewed all patients who underwent surgery for MAPCAs and biventricular heart disease from November 2001 through December 2021. RESULTS: During the study period, 780 unique patients underwent surgery. The number of new patients undergoing surgery annually was relatively steady during the first 15 years, then increased substantially thereafter. Surgery before referral had been performed in almost 40% of patients, more often in our recent experience than earlier. Complete repair was achieved in 704 patients (90%), 521 (67%) during the first surgery at our center, with a median right ventricular to aortic pressure ratio of 0.34 (25th, 75th percentiles: 0.28, 0.40). The cumulative incidence of mortality was 15% (95% CI: 12%-19%) at 10 years, with no difference according to era of surgery (P = 0.53). On multivariable Cox regression, Alagille syndrome (HR: 2.8; 95% CI: 1.4-5.7; P = 0.004), preoperative respiratory support (HR: 2.0; 95% CI: 1.2-3.3; P = 0.008), and palliative first surgery at our center (HR: 3.5; 95% CI: 2.3-5.4; P < 0.001) were associated with higher risk of death. CONCLUSIONS: In a growing pulmonary artery reconstruction program, with increasing volumes and an expanding population of patients who underwent prior surgery, outcomes of patients with pulmonary atresia or stenosis and MAPCAs have continued to improve.

Case report: airway and concurrent hemodynamic management in a neonate with oculo-auriculo-vertebral (Goldenhar) syndrome, severe cervical scoliosis, interrupted aortic arch, multiple ventricular septal defects, and an unstable cervical spine.

We report the challenging case of a 1-week-old, term, 2.4 kg neonate with Goldenhar syndrome (including microcephaly, left microtia, left facial palsy, dextro-scoliosis of the cervical spine, and cervico-thoracic levoscoliosis), multiple ventricular septal defects, a type B interrupted aortic arch, a large patent ductus arteriosis, and radiographic and clinical signs concerning for an unstable cervical spine. Our anesthesia team was consulted for perioperative management of this patient during her surgical repair. This case report describes the use of the Air-Q size 1 laryngeal airway (LA) to assist fiberoptic intubation in an ASA 4 neonate with cardiac disease, an anticipated difficult airway with the addition of an unstable cervical spine, as well as the anesthetic techniques used to maintain hemodynamic stability while the airway was secured.

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.

Ketamine does not increase pulmonary vascular resistance in children with pulmonary hypertension undergoing sevoflurane anesthesia and spontaneous ventilation.

BACKGROUND: The use of ketamine in children with increased pulmonary vascular resistance is controversial. In this prospective, open label study, we evaluated the hemodynamic responses to ketamine in children with pulmonary hypertension (mean pulmonary artery pressure >25 mm Hg). METHODS: Children aged 3 mo to 18 yr with pulmonary hypertension, who were scheduled for cardiac catheterization with general anesthesia, were studied. Patients were anesthetized with sevoflurane (1 minimum alveolar anesthetic concentration [MAC]) in air while breathing spontaneously via a facemask. After baseline catheterization measurements, sevoflurane was reduced (0.5 MAC) and ketamine (2 mg/kg IV over 5 min) was administered, followed by a ketamine infusion (10 microg x kg(-1) x min(-1)). Catheterization measurements were repeated at 5, 10, and 15 min after completion of ketamine load. Data at various time points were compared (ANOVA, P < 0.05). RESULTS: Fifteen patients (age 147, 108 mo; median, interquartile range) were studied. Diagnoses included idiopathic pulmonary arterial hypertension (5), congenital heart disease (9), and diaphragmatic hernia (1). At baseline, median (interquartile range) baseline pulmonary vascular resistance index was 11.3 (8.2) Wood units; 33% of patients had suprasystemic mean pulmonary artery pressures. Heart rate (99, 94 bpm; P = 0.016) and Pao2 (95, 104 mm Hg; P = 007) changed after ketamine administration (baseline, 15 min after ketamine; P value). There were no significant differences in mean systemic arterial blood pressure, mean pulmonary artery pressure, systemic or pulmonary vascular resistance index, cardiac index, arterial pH, or Paco2. CONCLUSIONS: In the presence of sevoflurane, ketamine did not increase pulmonary vascular resistance in spontaneously breathing children with severe pulmonary hypertension.

Anesthetic management of infants with palliated hypoplastic left heart syndrome undergoing laparoscopic nissen fundoplication.

The safety of laparoscopic surgery in infants with single ventricle physiology has been a subject of controversy despite potential benefits over open surgery. We present the anesthetic management of five infants with palliated hypoplastic left heart syndrome that underwent laparoscopic Nissen fundoplication. After anesthetic induction and tracheal intubation, an intraarterial catheter was placed for hemodynamic monitoring. Insufflation pressure was limited to 12 mm Hg and was well tolerated by all patients. There were no intraoperative or postoperative complications. In patients with hypoplastic left heart syndrome, laparoscopic Nissen fundoplication can be safely performed with careful patient selection and close intraoperative monitoring.

Postoperative analgesia after spinal blockade in infants and children undergoing cardiac surgery.

The aim of this prospective, randomized, controlled clinical trial was to define the opioid analgesic requirement after a remifentanil (REMI)-based anesthetic with spinal anesthetic blockade (SAB+REMI) or without (REMI) spinal blockade for open-heart surgery in children. We enrolled 45 patients who were candidates for tracheal extubation in the operating room after cardiac surgery. Exclusion criteria included age <3 mo and >6 yr, pulmonary hypertension, congestive heart failure, contraindication to SAB, and failure to obtain informed consent. All patients had an inhaled induction with sevoflurane and maintenance of anesthesia with REMI and isoflurane (0.3% end-tidal). In addition, patients assigned to the SAB+REMI group received SAB with tetracaine (0.5-2.0 mg/kg) and morphine (7 mug/kg). After tracheal extubation in the operating room, patients received fentanyl 0.3 mug/kg IV every 10 min by patient-controlled analgesia for pain score = 4. Pain scores and fentanyl doses were recorded every hour for 24 h or until the patient was ready for discharge from the intensive care unit. Patients in the SAB+REMI group had significantly lower pain scores (P = 0.046 for the first 8 h; P =0.05 for 24 h) and received less IV fentanyl (P = 0.003 for the first 8 h; P = 0.004 for 24 h) than those in the REMI group. There were no intergroup differences in adverse effects, including hypotension, bradycardia, highest PaCO(2), lowest pH, episodes of oxygen desaturation, pruritus, and vomiting.

Haemodynamic changes during high spinal anaesthesia in children having open heart surgery.

BACKGROUND: This prospective series examined the haemodynamic effects of high spinal anaesthesia in combination with light general anaesthesia in infants and children undergoing open heart surgery who were candidates for immediate or early postoperative extubation. METHODS: After midazolam premedication and sevoflurane inhalation induction, 30 patients, aged 7 months to 13 years, who were undergoing open heart surgery, received spinal anaesthetics with 0.5% tetracaine D10 mixed with morphine. The spinal blocks were placed at the L2,3 or L3,4 interspace with cephalad spread being promoted by positioning the patient in 30 degrees of Trendelenburg for a minimum of 10 min. Maintenance of anaesthesia was with isoflurane 0.2-0.5% in 70% nitrous oxide to maintain heart rate and blood pressure within 20% of postinduction baseline values. Haemodynamic values were recorded at predetermined timed intervals and intraoperative events up to and including aortic cannulation. For analysis of the data, patients were divided into four age groups (< 1 years, 1-3 years, 4-6 years and > 7 years). RESULTS: Haemodynamic stability was demonstrated in all four age groups. Statistically significant slowing of the heart rate did occur in the groups older than 1 year at 25 min, although clinically significant bradycardia requiring treatment never occurred. Hypotension did occur during specific surgical manipulations but recovered spontaneously. Atropine, fluid boluses and vasopressors were never used. At the conclusion of surgery, all patients met extubation criteria and could move all four extremities. CONCLUSIONS: High spinal anaesthesia with hyperbaric tetracaine and morphine in combination with light general anaesthesia is well tolerated haemodynamically by the paediatric population studied.

The effect of baricity of intrathecal morphine in children receiving tetracaine spinal anaesthesia for cardiac surgery: a preliminary report.

BACKGROUND: This prospective, randomized study examined the effect of baricity of intrathecal preservative-free morphine on the duration of postoperative analgesia and incidence of side-effects in infants and children receiving high spinal anaesthesia with hyperbaric tetracaine in combination with a light general anaesthetic. METHODS: Fourteen infants and children, aged 7-91 months, undergoing repair of either uncomplicated atrial or ventricular septal defects, were randomized to receive either 10 microg x kg(-1) of intrathecal morphine in combination with 0.5% tetracaine D10 (hyperbaric morphine group) or intrathecal morphine mixed with saline and injected sequentially after the administration of 0.5% tetracaine D10 (hypobaric morphine group). After spinal injection, patients were positioned in 30 degrees of Trendelenburg for a minimum of 10 min. Postoperatively, patients were monitored for a minimum of 12 h. Pain scores and the incidence and severity of side-effects were recorded every 1 h. RESULTS: All patients were extubated at the conclusion of surgery without any incidence of respiratory depression. There was a decreased incidence of vomiting in the hypobaric morphine group and no significant difference in the duration of analgesia. CONCLUSIONS: When intrathecal morphine is administered in conjunction with a hyperbaric tetracaine spinal to paediatric cardiac patients in the head down position, sequential administration of the hypobaric solution may mitigate side-effects.