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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 25, No 4, July/August 2014
AFRICA
Case Report Anesthetic management of a newborn with trisomy 18 undergoing closure of patent ductus arteriosus and pulmonary artery banding Oguzha Arun, Bahar Oc, Mehmet Oc, Ates Duman
Abstract Background: Peri-operative management of infants with trisomy 18 syndrome is challenging due to various congenital cardiac and facial anomalies. Case report: We report the anaesthetic management of a 13-day-old neonate with 1 540 g body weight, undergoing closure of patent ductus arteriosus and pulmonary artery banding. Anaesthesia was induced with sevoflurane, fentanyl and rocuronium. Despite dysmorphic facial features, ventilation and endotracheal intubation were achieved uneventfully. Anaesthesia was maintained with sevoflurane and fentanyl and was uneventful. The patient was transferred to the neonatal ICU intubated and with ventilatory support. The baby was extubated on the second day postoperatively. Conclusion: Our knowledge of the proper anaesthetic technique for children undergoing palliative or corrective surgery is limited. Further case reports will increase our experience in peri-operative management of children with trisomy 18. Keywords: Edwards syndrome, trisomy 18, newborn, anaesthesia Submitted 28/6/13, accepted 6/5/14 Cardiovasc J Afr 2014; 25: e10–e12
www.cvja.co.za
DOI: 10.5830/CVJA-2014-024
Trisomy 18 syndrome, first described by Edwards in 1960, is characterised by an autosomal chromosomal disorder with multiple congenital anomalies.1 The syndrome consists of major and minor anomalies, prenatal and postnatal growth deficiency, and an increased risk of neonatal and infant mortality. It is the second most common autosomal trisomy syndrome after trisomy 21, and the live-birth prevalence of trisomy 18 is estimated at between 1/3 600 and 1/10 000. The overall prevalence is higher Department of Anesthesiology and Reanimation, Faculty of Medicine, Selcuk University, Konya, Turkey Oguzhan Arun, MD, [email protected] Bahar Oc, MD Ates Duman, MD
Department of Cardiovascular Surgery, Faculty of Medicine, Selcuk University, Konya, Turkey Mehmet Oc, MD
(1/2 500–1/2 600) due to the high frequency of foetal loss and pregnancy termination after prenatal diagnosis.2 Structural heart defects occur in over 90% of infants with the syndrome, and the most common cardiac lesions are atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA) and polyvalvular disease.3 There is some controversy regarding offering cardiac surgery to patients with trisomy 18 because of the elevated risk of mortality during surgery and in the first month of life, and the presence of significant developmental disability in surviving children. If scheduled for surgery, the peri-operative management of these children is challenging. We report on the anaesthetic management of a newborn with Edwards syndrome undergoing closure of patent ductus arteriosus and pulmonary artery banding.
Case report The patient was a 13-day-old girl born at 36 weeks and 4 days of gestation with 1 540 g body weight. She had three healthy sisters and there was not any consanguinity between her parents. Foetal growth retardation, single umbilical artery, and polyhydramnios were detected with ultrasonography during the second and third trimester follow ups. After a successful caesarean section, the first-, fifth-, and tenth-minute APGAR scores were 5, 7 and 10, respectively. At birth she had dysmorphic facial features including microcephaly, malformed ears, micrognathia, redundant skin at the neck, and hypotonia (Figs 1, 2). Karyotype analysis revealed trisomy 18. Structural heart defects such as atrial and ventricular septal defects and patent ductus arteriosus; bilateral hydronephrosis and hydro-ureter in the right kidney, and mega cisterna magna variation in the central nervous system were also present. She was transferred to the neonatal intensive care unit (NICU) and given daily digitalis and antibiotic therapy. Despite two doses of intravenous indomethacin treatment, the patient’s pulmonary pressure increased and her general condition gradually worsened. Echocardiography revealed a left atrium/aorta ratio (LA/Ao) of 1.4, PDA diameter of 3–4 mm, systolic pulmonary arterial pressure of 55 mmHg and enlargement of the left ventricle. Cardiovascular surgeons, paediatric cardiologists and the patient’s parents were invited for consultation. Closure of the PDA and pulmonary banding were expected to reduce pulmonary blood flow and pulmonary artery pressure. The parents were informed that closure of the PDA and pulmonary
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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 25, No 4, July/August 2014
Fig. 1. Dysmorphic face of the baby.
artery banding would be a palliative intervention and the probable survival outcome was not expected to change. The family insisted on the surgical procedure, and according to the local medical laws, the patient was scheduled for surgery. The baby was accepted for anaesthesia as class ASA IV. The parents were informed about the risks and a written consent was obtained. Initial laboratory investigations showed abnormalities such as aspartate aminotransferase (AST) 86 μg/l, indirect bilirubin 7.7 mg/dl, potassium 6.0 meq/l, albumin 2.4 g/dl, and platelet 55 000 per mcl. Pre-operative laboratory investigation was normal. No premedication was given, and the baby was transferred to the operating room with oxygen supply in the transport incubator. After the application of standard non-invasive monitoring, including ECG, SpO2 and NIBP, due to the problem of existing vascular access, general anaesthesia was induced with sevoflurane 8% in 50:50% oxygen in air. We refrained from using a high concentration of oxygen due to the risk of retinopathy of prematurity. Peripheral venous access was achieved with a 24-G iv catheter at the dorsum of the left hand. In order to facilitate endotracheal intubation, 2 mcg/kg of fentanyl and 0.5 mg/kg of rocuronium bromide were given intravenously. After two minutes of ventilation with 100% O2 via a face mask, a laryngoscopy was performed using a size 0 Miller blade. Laryngoscopy revealed grade II Cormach-Lahane, and intubation was achieved easily with a 3.0 mm uncuffed endotracheal tube. Central venous and arterial accesses were achieved via the right femoral route with 4-F double-lumen 5-cm and 22-G singlelumen 5-cm catheters, respectively. Anaesthesia was maintained using controlled ventilation with sevoflurane 1.5–2% in 50:50% oxygen in air; 1 mcg/kg of fentanyl and 0.2 mg/kg of rocuronium were added as needed. Pre-banding invasive blood pressure, which was 66/34 (45) mmHg, increased to 84/48 (60) mmHg following PDA ligation and banding. Also SpO2 decreased from 99 to 95% after banding (FiO2 = 0.5). The operation lasted for 35 minutes without any surgical and/ or anaesthetic problems. After pulmonary banding, 5 mcg/kg/
e11
Fig. 2. Malformed ear of the baby.
min dopamine infusion was started for haemodynamic support. After an uneventful closure of the PDA and pulmonary artery banding, the baby was transferred to the NICU intubated with mechanical ventilation support. The baby was extubated on the second day postoperatively. She died in the NICU 10 weeks later after an episode of resistant bradycardia despite proper resuscitation.
Discussion The clinical pattern of trisomy 18 is characterised by growth deficiency that starts in the prenatal period; specific craniofacial features such as dolichocephaly, short palpebral fissures, micrognathia, external anomalies of the ears, and redundant skin at the back of the neck; and marked psychomotor and cognitive developmental delay. The presence of major systemic malformations is common, and any organ and system can be affected. Prenatal and early postnatal infant mortality rates are high when compared to the general population.4 The postnatal median survival of children with trisomy 18 is three to 4.5 days; approximately 50% of babies with trisomy 18 live longer than one week and only five to 10% of children survive beyond the first year. One of the major causes of death is cardiac failure due to congenital heart defect-related problems.5 The severity of the cardiac and extra-cardiac lesions is an important factor for the timing and extent of cardiac surgery. Kaneko et al. hypothesised that trisomy 18 patients who underwent intra-cardiac repair had more complicated cardiac lesions and, therefore, mortality rates were increased.6 We believe similarly that postponement or abandonment of the cardiac operation can have a negative impact on survival because of longer exposure to excessive pulmonary blood flow and heart failure. Therefore palliative cardiac surgery may be sufficient to relieve symptoms, prevent heart-related death, and improve life expectancy when performed at an early stage. Due to the limited data regarding anaesthesia techniques in patients with trisomy 18, it is impossible to suggest a
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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 25, No 4, July/August 2014
definite anaesthesia protocol. Anaesthetic goals in paediatric patients with congenital heart disease include preservation of haemodynamic stability with minimal depression of contractility, avoiding arrhythmias, and the maintenance of homeostasis between systemic and pulmonary circulations. One of the routes used for induction in cardiac paediatric patients is via inhalational agents. Although induction with volatile anaesthetics in cardiac paediatric patients can have potentially deleterious effects due to compromised haemodynamic stability, of these agents, sevoflurane appears to have less cardiac depressant effects than other halogenated agents.7 Courreges et al. reported that the anaesthetic management for a Cohen procedure in a girl with Edwards syndrome,8 and longer duration of induction (8 min) with sevoflurane was linked to a change in the ventilation/perfusion ratio caused by the cardiac disease. In our patient, due to the problem of existing vascular access, we chose sevoflurane and performed an uneventful and regular duration of induction. Although pancuronium may be preferred for neuromuscular blockade of patients undergoing cardiac surgeries, it has a slow onset and prolonged duration of action, and is eliminated mainly by renal excretion. Instead we preferred rocuronium, a non-depolarising NMB agent structurally related to pancuronium, based on its rapid onset, intermediate duration of action, and non-histamine release property with a metabolism rate of less than 1%, taken up by the liver and excreted into bile.9 We also preferred fentanyl because it improves the protein metabolism of premature neonates enduring the stress of a PDA and is considered to be the first drug choice in children with pulmonary hypertension.10 In the early postoperative course of congenital cardiac surgery, more than 20% of patients exhibit low cardiac output syndrome (LCOS), characterised by poor systemic perfusion and high vasoactive drug requirements.11 We transferred our patient with a 5-µg/kg/min dopamine infusion to the neonatal ICU.
AFRICA
of the proper anaesthetic technique for children undergoing palliative or corrective surgery is limited. Further case reports will increase our experience in peri-operative management of children with trisomy 18.
References 1.
Edwards JH, Harnden DG, Cameron AH, Crosse VM, Wolff OH. A new trisomic syndrome. Lancet 1960; 1: 787–789.
2.
Embleton ND, Wyllie JP, Wright MJ, Burn J, Hunter S. Natural history of trisomy 18. Arch Dis Child 1996; 75: 38–41.
3.
Kaneko Y, Kobayashi J, Achiwa I, Yoda H, Tsuchiya K, Nakajima Y, et al. Cardiac surgery in patients with trisomy 18. Pediatr Cardiol 2009; 30(6): 729–734.
4.
Yamanaka M, Setoyama T, Igarashi Y, Kurosawa K, Itani Y, Hashimoto S, et al. Pregnancy outcome of fetuses with trisomy 18 identified by prenatal sonography and chromosomal analysis in a perinatal center. Am J Med Genet 2006; 140: 1177–1182.
5.
Goldstein H, Nielsen KG. Rates and survival of individuals with trisomy 18 and 13. Clin Genet 1988; 34: 366–372.
6.
Kaneko Y, Kobayashi J, Yamamoto Y, Yoda H, Kanetaka Y, Nakajima Y, et al. Intensive cardiac management in patients with trisomy 13 or trisomy 18. Am J Med Genet 2008; 146: 1372–1380.
7.
Rivenes SM, Lewin MB, Stayer SA, Bent ST, Schoenig HM, McKenzie ED, et al Cardiovascular effects of sevoflurane, isoflurane, halothane, and fentanylmidazolam in children with congenital heart disease: an echocardiographic study of myocardial contractility and hemodynamics. Anesthesiology 2001; 94: 223–229.
8.
Courrèges P, Nieuviarts R, Lecoutre D. Anaesthetic management for Edward’s syndrome. Paediatr Anaesth 2003; 13(3): 267–269.
9.
Sparr HJ, Beaufort TM, Fuchs-Buder T. Newer neuromuscular blocking agents: How do they compare with established agents? Drugs 2001; 61: 919–942.
10. Shew SB, Keshen TH, Glass NL, Jahoor F, Jaksic T. Ligation of a patent ductus arteriosus under fentanyl anesthesia improves protein metabo-
Conclusion Neonates with trisomy 18 are an uncommon subgroup of cardiac surgery patients with a short life expectancy. Our knowledge
lism in premature neonates. J Pediatr Surg 2000; 35(9): 1277–1281. 11. Tweddell JS, Hoffman GM. Postoperative management in patients with complex congenital heart disease. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2002; 5: 187–205.
CARDIOVASCULAR JOURNAL OF AFRICA • Volume 25, No 4, July/August 2014
AFRICA
Case Report Anesthetic management of a newborn with trisomy 18 undergoing closure of patent ductus arteriosus and pulmonary artery banding Oguzha Arun, Bahar Oc, Mehmet Oc, Ates Duman
Abstract Background: Peri-operative management of infants with trisomy 18 syndrome is challenging due to various congenital cardiac and facial anomalies. Case report: We report the anaesthetic management of a 13-day-old neonate with 1 540 g body weight, undergoing closure of patent ductus arteriosus and pulmonary artery banding. Anaesthesia was induced with sevoflurane, fentanyl and rocuronium. Despite dysmorphic facial features, ventilation and endotracheal intubation were achieved uneventfully. Anaesthesia was maintained with sevoflurane and fentanyl and was uneventful. The patient was transferred to the neonatal ICU intubated and with ventilatory support. The baby was extubated on the second day postoperatively. Conclusion: Our knowledge of the proper anaesthetic technique for children undergoing palliative or corrective surgery is limited. Further case reports will increase our experience in peri-operative management of children with trisomy 18. Keywords: Edwards syndrome, trisomy 18, newborn, anaesthesia Submitted 28/6/13, accepted 6/5/14 Cardiovasc J Afr 2014; 25: e10–e12
www.cvja.co.za
DOI: 10.5830/CVJA-2014-024
Trisomy 18 syndrome, first described by Edwards in 1960, is characterised by an autosomal chromosomal disorder with multiple congenital anomalies.1 The syndrome consists of major and minor anomalies, prenatal and postnatal growth deficiency, and an increased risk of neonatal and infant mortality. It is the second most common autosomal trisomy syndrome after trisomy 21, and the live-birth prevalence of trisomy 18 is estimated at between 1/3 600 and 1/10 000. The overall prevalence is higher Department of Anesthesiology and Reanimation, Faculty of Medicine, Selcuk University, Konya, Turkey Oguzhan Arun, MD, [email protected] Bahar Oc, MD Ates Duman, MD
Department of Cardiovascular Surgery, Faculty of Medicine, Selcuk University, Konya, Turkey Mehmet Oc, MD
(1/2 500–1/2 600) due to the high frequency of foetal loss and pregnancy termination after prenatal diagnosis.2 Structural heart defects occur in over 90% of infants with the syndrome, and the most common cardiac lesions are atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA) and polyvalvular disease.3 There is some controversy regarding offering cardiac surgery to patients with trisomy 18 because of the elevated risk of mortality during surgery and in the first month of life, and the presence of significant developmental disability in surviving children. If scheduled for surgery, the peri-operative management of these children is challenging. We report on the anaesthetic management of a newborn with Edwards syndrome undergoing closure of patent ductus arteriosus and pulmonary artery banding.
Case report The patient was a 13-day-old girl born at 36 weeks and 4 days of gestation with 1 540 g body weight. She had three healthy sisters and there was not any consanguinity between her parents. Foetal growth retardation, single umbilical artery, and polyhydramnios were detected with ultrasonography during the second and third trimester follow ups. After a successful caesarean section, the first-, fifth-, and tenth-minute APGAR scores were 5, 7 and 10, respectively. At birth she had dysmorphic facial features including microcephaly, malformed ears, micrognathia, redundant skin at the neck, and hypotonia (Figs 1, 2). Karyotype analysis revealed trisomy 18. Structural heart defects such as atrial and ventricular septal defects and patent ductus arteriosus; bilateral hydronephrosis and hydro-ureter in the right kidney, and mega cisterna magna variation in the central nervous system were also present. She was transferred to the neonatal intensive care unit (NICU) and given daily digitalis and antibiotic therapy. Despite two doses of intravenous indomethacin treatment, the patient’s pulmonary pressure increased and her general condition gradually worsened. Echocardiography revealed a left atrium/aorta ratio (LA/Ao) of 1.4, PDA diameter of 3–4 mm, systolic pulmonary arterial pressure of 55 mmHg and enlargement of the left ventricle. Cardiovascular surgeons, paediatric cardiologists and the patient’s parents were invited for consultation. Closure of the PDA and pulmonary banding were expected to reduce pulmonary blood flow and pulmonary artery pressure. The parents were informed that closure of the PDA and pulmonary
AFRICA
CARDIOVASCULAR JOURNAL OF AFRICA • Volume 25, No 4, July/August 2014
Fig. 1. Dysmorphic face of the baby.
artery banding would be a palliative intervention and the probable survival outcome was not expected to change. The family insisted on the surgical procedure, and according to the local medical laws, the patient was scheduled for surgery. The baby was accepted for anaesthesia as class ASA IV. The parents were informed about the risks and a written consent was obtained. Initial laboratory investigations showed abnormalities such as aspartate aminotransferase (AST) 86 μg/l, indirect bilirubin 7.7 mg/dl, potassium 6.0 meq/l, albumin 2.4 g/dl, and platelet 55 000 per mcl. Pre-operative laboratory investigation was normal. No premedication was given, and the baby was transferred to the operating room with oxygen supply in the transport incubator. After the application of standard non-invasive monitoring, including ECG, SpO2 and NIBP, due to the problem of existing vascular access, general anaesthesia was induced with sevoflurane 8% in 50:50% oxygen in air. We refrained from using a high concentration of oxygen due to the risk of retinopathy of prematurity. Peripheral venous access was achieved with a 24-G iv catheter at the dorsum of the left hand. In order to facilitate endotracheal intubation, 2 mcg/kg of fentanyl and 0.5 mg/kg of rocuronium bromide were given intravenously. After two minutes of ventilation with 100% O2 via a face mask, a laryngoscopy was performed using a size 0 Miller blade. Laryngoscopy revealed grade II Cormach-Lahane, and intubation was achieved easily with a 3.0 mm uncuffed endotracheal tube. Central venous and arterial accesses were achieved via the right femoral route with 4-F double-lumen 5-cm and 22-G singlelumen 5-cm catheters, respectively. Anaesthesia was maintained using controlled ventilation with sevoflurane 1.5–2% in 50:50% oxygen in air; 1 mcg/kg of fentanyl and 0.2 mg/kg of rocuronium were added as needed. Pre-banding invasive blood pressure, which was 66/34 (45) mmHg, increased to 84/48 (60) mmHg following PDA ligation and banding. Also SpO2 decreased from 99 to 95% after banding (FiO2 = 0.5). The operation lasted for 35 minutes without any surgical and/ or anaesthetic problems. After pulmonary banding, 5 mcg/kg/
e11
Fig. 2. Malformed ear of the baby.
min dopamine infusion was started for haemodynamic support. After an uneventful closure of the PDA and pulmonary artery banding, the baby was transferred to the NICU intubated with mechanical ventilation support. The baby was extubated on the second day postoperatively. She died in the NICU 10 weeks later after an episode of resistant bradycardia despite proper resuscitation.
Discussion The clinical pattern of trisomy 18 is characterised by growth deficiency that starts in the prenatal period; specific craniofacial features such as dolichocephaly, short palpebral fissures, micrognathia, external anomalies of the ears, and redundant skin at the back of the neck; and marked psychomotor and cognitive developmental delay. The presence of major systemic malformations is common, and any organ and system can be affected. Prenatal and early postnatal infant mortality rates are high when compared to the general population.4 The postnatal median survival of children with trisomy 18 is three to 4.5 days; approximately 50% of babies with trisomy 18 live longer than one week and only five to 10% of children survive beyond the first year. One of the major causes of death is cardiac failure due to congenital heart defect-related problems.5 The severity of the cardiac and extra-cardiac lesions is an important factor for the timing and extent of cardiac surgery. Kaneko et al. hypothesised that trisomy 18 patients who underwent intra-cardiac repair had more complicated cardiac lesions and, therefore, mortality rates were increased.6 We believe similarly that postponement or abandonment of the cardiac operation can have a negative impact on survival because of longer exposure to excessive pulmonary blood flow and heart failure. Therefore palliative cardiac surgery may be sufficient to relieve symptoms, prevent heart-related death, and improve life expectancy when performed at an early stage. Due to the limited data regarding anaesthesia techniques in patients with trisomy 18, it is impossible to suggest a
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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 25, No 4, July/August 2014
definite anaesthesia protocol. Anaesthetic goals in paediatric patients with congenital heart disease include preservation of haemodynamic stability with minimal depression of contractility, avoiding arrhythmias, and the maintenance of homeostasis between systemic and pulmonary circulations. One of the routes used for induction in cardiac paediatric patients is via inhalational agents. Although induction with volatile anaesthetics in cardiac paediatric patients can have potentially deleterious effects due to compromised haemodynamic stability, of these agents, sevoflurane appears to have less cardiac depressant effects than other halogenated agents.7 Courreges et al. reported that the anaesthetic management for a Cohen procedure in a girl with Edwards syndrome,8 and longer duration of induction (8 min) with sevoflurane was linked to a change in the ventilation/perfusion ratio caused by the cardiac disease. In our patient, due to the problem of existing vascular access, we chose sevoflurane and performed an uneventful and regular duration of induction. Although pancuronium may be preferred for neuromuscular blockade of patients undergoing cardiac surgeries, it has a slow onset and prolonged duration of action, and is eliminated mainly by renal excretion. Instead we preferred rocuronium, a non-depolarising NMB agent structurally related to pancuronium, based on its rapid onset, intermediate duration of action, and non-histamine release property with a metabolism rate of less than 1%, taken up by the liver and excreted into bile.9 We also preferred fentanyl because it improves the protein metabolism of premature neonates enduring the stress of a PDA and is considered to be the first drug choice in children with pulmonary hypertension.10 In the early postoperative course of congenital cardiac surgery, more than 20% of patients exhibit low cardiac output syndrome (LCOS), characterised by poor systemic perfusion and high vasoactive drug requirements.11 We transferred our patient with a 5-µg/kg/min dopamine infusion to the neonatal ICU.
AFRICA
of the proper anaesthetic technique for children undergoing palliative or corrective surgery is limited. Further case reports will increase our experience in peri-operative management of children with trisomy 18.
References 1.
Edwards JH, Harnden DG, Cameron AH, Crosse VM, Wolff OH. A new trisomic syndrome. Lancet 1960; 1: 787–789.
2.
Embleton ND, Wyllie JP, Wright MJ, Burn J, Hunter S. Natural history of trisomy 18. Arch Dis Child 1996; 75: 38–41.
3.
Kaneko Y, Kobayashi J, Achiwa I, Yoda H, Tsuchiya K, Nakajima Y, et al. Cardiac surgery in patients with trisomy 18. Pediatr Cardiol 2009; 30(6): 729–734.
4.
Yamanaka M, Setoyama T, Igarashi Y, Kurosawa K, Itani Y, Hashimoto S, et al. Pregnancy outcome of fetuses with trisomy 18 identified by prenatal sonography and chromosomal analysis in a perinatal center. Am J Med Genet 2006; 140: 1177–1182.
5.
Goldstein H, Nielsen KG. Rates and survival of individuals with trisomy 18 and 13. Clin Genet 1988; 34: 366–372.
6.
Kaneko Y, Kobayashi J, Yamamoto Y, Yoda H, Kanetaka Y, Nakajima Y, et al. Intensive cardiac management in patients with trisomy 13 or trisomy 18. Am J Med Genet 2008; 146: 1372–1380.
7.
Rivenes SM, Lewin MB, Stayer SA, Bent ST, Schoenig HM, McKenzie ED, et al Cardiovascular effects of sevoflurane, isoflurane, halothane, and fentanylmidazolam in children with congenital heart disease: an echocardiographic study of myocardial contractility and hemodynamics. Anesthesiology 2001; 94: 223–229.
8.
Courrèges P, Nieuviarts R, Lecoutre D. Anaesthetic management for Edward’s syndrome. Paediatr Anaesth 2003; 13(3): 267–269.
9.
Sparr HJ, Beaufort TM, Fuchs-Buder T. Newer neuromuscular blocking agents: How do they compare with established agents? Drugs 2001; 61: 919–942.
10. Shew SB, Keshen TH, Glass NL, Jahoor F, Jaksic T. Ligation of a patent ductus arteriosus under fentanyl anesthesia improves protein metabo-
Conclusion Neonates with trisomy 18 are an uncommon subgroup of cardiac surgery patients with a short life expectancy. Our knowledge
lism in premature neonates. J Pediatr Surg 2000; 35(9): 1277–1281. 11. Tweddell JS, Hoffman GM. Postoperative management in patients with complex congenital heart disease. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann. 2002; 5: 187–205.
