Skolyoz Ameliyatı Yapılan Goldenhar Sendromlu Çocuk Olguda Anestezi Yönetimi
Özet
Goldenhar sendromu (okülo-aurikülo-vertebral spektrum), göz, vertebra, kalp, akciğer, gastrointestinal, ürolojik ve otorinolojik anomalilerle karakterize, oldukça heterojen, çoğu kez sporadik olan bir sendromdur. Sıklığı 56.000 canlı doğumda 1 olarak değerlendirilmektedir. Okülo-airukülo-vertebral spektrumun (OAVS), birinci ve ikinci faringeal arkus türevlerinin anormal gelişimi nedeniyle geliştiği bilinmektedir. Mezenkimal hücrelerden oluşan bu faringeal arkuslar gebeliğin dördüncü haftasında gelişir. OAVS’un türevleri sinir ve kas-iskelet sistemi unsurlarını içerir. Kranial nöral krest hücrelerinin oluşumu ve ventrolateral migrasyonu, kraniofasiyal bölgelerin gelişiminden sorumludur. Bu nöral krest hücrelerinin oluşumu, göçü, çoğalması ve hayatta kalmasındaki herhangi bir kusur veya genetik ve çevresel faktörlerin varlığı OAVS’nin oluşmasında önemli bir mekanizma olarak kabul edilmektedir. OAVS'li hastaların fenotipik spektrumu oldukça heterojendir. OAVS'de çoklu sistem tutulumu yaygındır. Muayene bulguları ve semptomları ilgili organ sistemine göre değişebilir. OAVS'nin klasik üçlüsü fasiyal asimetri, okülo-auriküler malformasyonlar ve vertebral anormalliklerle birlikte mandibular hipoplaziyi içerir. Nadir olarak da kalp, akciğer, ürolojik organ tutulumu da eşlik edebilir.
Onüç yaş, Goldenhar sendromu tanısı olan erkek çocuk, OAVS’un bir komponenti olan vertebra deformitesi nedeniyle ilk kez skolyoz ameliyatına alındı. Hastanın doğumsal kalp hastalığı mevcut olup 6 yıl önce modifiye Fontan ameliyatı geçirmiş ve sonrasında her yıl kontrol amaçlı çok kez kateter anjiyografi olmuş. Hastanın periferik oksijen satürasyon değerleri %90 düzeylerinde olmasından dolayı hastanemiz pediatrik kardiyoloji, pediatrik kardiyovasküler cerrahi (KVC) yoğun bakım, ortopedi ve anesteziyoloji kliniği olarak multidisipliner bir ekip şeklinde çalışıldı. Bu yazımızda, konjenital kalp hastalığının eşlik ettiği, skolyoz ameliyatı yapılan Goldenhar sendromlu çocuk hastada anestezi yönetimimizi anlattık.
Goldenhar syndrome (oculo-auriculo-auriculo-vertebral spectrum) is a highly heterogeneous, often sporadic syndrome characterized by ocular, vertebral, cardiac, pulmonary, gastrointestinal, urological and otorhinolaryngological anomalies. The frequency is estimated to be 1 in 56,000 live births. The oculo-airuculo-vertebral spectrum (OAVS) is known to develop due to abnormal development of the first and second pharyngeal arch derivatives. These pharyngeal arches, composed of mesenchymal cells, develop in the fourth week of gestation. The derivatives of the OAVS include nervous and musculoskeletal elements. The formation and ventrolateral migration of cranial neural crest cells is responsible for the development of craniofacial regions. Any defect in the formation, migration, proliferation and survival of these neural crest cells or the presence of genetic and environmental factors is considered to be an important mechanism in the formation of OAVS. The phenotypic spectrum of patients with OAVS is quite heterogeneous. Multiple system involvement is quite common in OAVS. Examination findings and symptoms can be divided according to the organ system involved. The classic triad of OAVS includes facial asymmetry, oculo-auricular malformations and mandibular hypoplasia with vertebral abnormalities. Rarely, cardiac, pulmonary and urologic organ involvement may also be associated. A 13-year-old boy with Goldenhar syndrome underwent scoliosis surgery for the first time due to vertebral deformity, a component of OAVS. The patient had congenital heart disease and underwent modified Fontan surgery 6 years ago, after which he underwent coronary angiography multiple times for follow-up every year. Since the patient's oxygen saturation values were around 90%, we worked as a multidisciplinary team including pediatric cardiology, pediatric cardiovascular intensive care, orthopedics and anesthesiology clinic. In this article, we describe our anesthesia management in a child with Goldenhar syndrome who underwent scoliosis surgery for congenital heart disease.
Referanslar
Singh M, Kaur M, Grewal AM, Yangzes S, Yadav D, Zadeng Z, et al. Ophthalmic characteristics and management outcomes of 30 children with Goldenhar syndrome. International Ophthalmol.2020 March; 40 (3):667-675. [ PubMed ]
Beleza-Meireles A, Clayton-Smith J, Saraiva JM, Tassabehji M. Oculo-auriculo-vertebral spectrum: a review of the literature and genetic update. J Med Genet. 2014 Oct; 51 (10):635-45. [ PubMed ]
Bogusiak K, Puch A, Arkuszewski P. Goldenhar syndrome: current perspectives. World J Pediatr. October 2017; 13 (5):405-415. [ PubMed ]
Tasse C, Böhringer S, Fischer S, Lüdecke HJ, Albrecht B, Horn D, et al. Oculo-auriculo-vertebral spectrum (OAVS): Clinical evaluation and severity scoring of 53 patients and proposal for a new classification. Eur J Med Genet. 2005 October-December; 48 (4):397 411. [ PubMed ]
Strömland K, Miller M, Sjögreen L, Johansson M, Joelsson BM, Billstedt E, et al. Oculo-auriculo-vertebral spectrum: associated anomalies, functional deficits, and possible developmental risk factors. Am J Med Genet A. 2007 June 15;143A (12):1317-25. [ PubMed ]
A L Petrin , Lam Machado-Paula , A Hinkle , L Hovey , W Awotoye , M Chimenti, et al.: Whole genome sequencing of a family with autosomal dominant features within the oculoauriculovertebral spectrum. medRxiv. 2024 Feb 7:2024.02.07.24301824. doi: 10.1101/2024.02.07.24301824. [Pubmed]
Vendramini-Pittoli S, Kokitsu-Nakata NM. Oculoauriculovertebral spectrum: Report of nine familial cases with evidence of autosomal dominant inheritance and review of the literature. Clin Dysmorphol. April 2009; 18 (2):67-77. [ PubMed ]
Tuğ E, Atasoy HI, Koybaşı Sanal S. Thrombophilia gene mutations in the oculoauriculovertebral spectrum. Genet Couns. 2012; 23 (1):65-72. [ PubMed ]
Descartes M. Oculoauriculovertebral spectrum with 5p15.33-pter deletion. Clin Dysmorphol. July 2006; 15 (3):153-154. [ PubMed ]
Josifova DJ, Patton MA, Marks K. An oculoauriculovertebral spectrum phenotype caused by an unbalanced t(5;8) (p15.31;p23.1) rearrangement. Clin Dysmorphol. July 2004;13 (3):151-153. [ PubMed ]
Ala-Mello S, Siggberg L, Knuutila S, von Koskull H, Taskinen M, Peippo M. Further evidence for an association between chromosome region 5p15 and oculoauriculovertebral anomaly. Am J Med Genet A. 2008 Oct 01; 146A (19):2490-4. [ PubMed ]
Ingeborg Barisiç , Ljubica Odak , Maria Kredi , Ester Garne , Diana Wellesley , Elisa Calzolari and et.al. Prevalence, prenatal diagnosis, and clinical features of oculo-auriculo-vertebral spectrum: A registry-based study in Europe. Eur J Hum Genet 2014 Aug;22(8):1026-33. doi: 10.1038/ejhg.2013.287. Epub 2014 Jan 8. [Pubmed]
Rosa RF, Graziadio C, Lenhardt R, Alves RP, Paskulin GA, Zen PR. Central nervous system abnormalities in patients with oculo-auriculo-auriculo-vertebral spectrum (Goldenhar syndrome). Arq Neuropsiquiatr. February 2010; 68 (1):98-102. [ PubMed ]
Fischer S, Lüdecke HJ, Wieczorek D, Böhringer S, Gillessen-Kaesbach G, Horsthemke B. Allelic expression imbalance of BAPX1 in patients with oculo-auriculo-vertebral spectrum due to histone acetylation. Hum Mol Genet. February 15, 2006;15 (4):581 7. [ PubMed ]
Hongjuan Zhao , Chen Du , Guang Yang , Yu Wang. Diagnosis, treatment, and research status of rare diseases related to birth defects. Intractable Rare Dis Res. 2023 Aug;12(3): 148-160.doi: 10.5582/irdr.2023.01052.
Tripathy K, Sharma YR, Chawla R, Basu K, Vohra R, Venkatesh P. Triads in Ophthalmology: A Comprehensive Review. Semin Ophthalmol. 2017; 32 (2):237-250. [ PubMed ]
Martelli H, Miranda RT, Fernandes CM, Bonan PR, Paranaíba LM, Graner E, et al. Goldenhar syndrome: clinical features with orofacial accentuation. J Appl Oral Sci. December 2010;18 (6):646-9. [ PubMed ]
Renkema RW, Caron CJJM, Mathijssen IMJ, Wolvius EB, Dunaway DJ, Forrest CR, et al. Vertebral anomalies in craniofacial microsomia: a systematic review. Int J Oral Maxillofacial Surgery. October 2017; 46 (10):1319-1329. [ PubMed]
İsmail Balaban, Meltem Ceyhan Bilgici, Kemal Baysal. A new association of oculoauriculovertebral spectrum and persistent fifth aortic arch-double lumen aorta: a case report.BMC Pediatr. 2022 Feb 21;22(1):102. doi:10.1186/s12887 02031370.
John Richard McNamara , Aisling McMahon , Michael Griffin Perioperative Management of the Fontan Patient for Cardiac and Noncardiac Surgery Cardiothorac Vasc Anesth 2022 Jan;36(1): 275-285.doi: 10.1053/j.jvca.2021.04.021. Epub 2021 Apr 20.
Eagle SS, Daves SM. The adult with Fontan physiology: systematic approach to perioperative management for noncardiac surgery. J Cardio- thorac Vasc Anesth2011; 25:320–34.
Egbe AC, Khan AR, Ammash NM, et al. Predictors of procedural complications in adult Fontan patients undergoing noncardiac procedures. Heart 2017; 103:181320.
Lui GK, Saidi A, Bhatt AB, et al. Diagnosis and management of noncardiac complications in adults with congenital heart disease: a scientific statement from the American Heart Association. Circulation 2017;136: e348–92.
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guidelines for the management of adults with congenital heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019;73: e81–192.
Larsen JR, Torp P, Norrild K, Sloth E: Propofol reduces tissue-Doppler markers of left ventricle function: a transthoracic echocardiographic study. Br J Anaesth 2007;98: 183−188.
Wappler F, Rossaint R, Baumert J et al.: Multicenter randomized com- parison of xenon and isoflurane on left ventricular function in patients undergoing elective surgery. Anesthesiology 2007; 106: 463−471.
Hasija S, Chauhan S, Jain P, Choudhury A, Aggarwal N, Pandey RK: Com- parison of speed of inhalational induction in children with and without congenital heart disease. Ann Card Anaesth 2016; 19: 468−474. doi: 10.4103/0971 9784.185531.
Rothrock SG, Pagane J Pediatric rapid sequence intubation incidence of reflex bradycardia and effects of pretreatment with atropine. Pediatr Emerg Care 2005;21: 637−638.
Naguib AN, Tobias JD, Hall MW et al. The role of different anesthetic techniques in altering the stress response during cardiac surgery in children: A prospective, double-blinded, randomized study. Pediatr Crit Care Med 2013; 14: 481−490.doi: 10.1097/PCC.0b013e31828a742c.
Shields M, Giovannelli M, Mirakhur RK, Moppett I, Adams J, Hermens Y: Org 25969 (sugammadex), a selective relaxant binding agent for antagonism of prolonged rocuronium-induced neuromuscular block. Br J Anaesth 2006; 96:36−43.
Groudine SB, Soto R, Lien C, Drover D, Roberts K: A randomized, dose- finding, phase II study of the selective relaxant binding drug, Sugam- madex, capable of safely reversing profound rocuronium-induced neuromuscular block. Anesth Analg2007; 104: 555−562.
Beers R, Camporesi E: Remifentanil update: clinical science and utility. CNS Drugs 2004; 18: 1085−1104.
Bailey PD Jr, Jobes DR. The Fontan Patient. Anesthesiol Clin 2009; 27:285–300.
Heggie J, Karski J. Anesthesiologist’s role in adults with congenital heart disease. Cardiol Clin 2006; 24:571–85.
Hedequist DJ, Emans JB, Hall JE, et al. Operative treatment for scoliosis in patients with Fontan circulation. Spine (Phila Pa 1976) 2006; 31:202-5.
d’Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton GR, et al. Rede-fining expectations of long-term survival after the Fontan procedure: Twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation. 2014 Sep;130(11 Suppl 1): S32–8.
Bigelow AM, Ghanayem NS, Thompson NE et al. Safety and efficacy of vasopressin after Fontan procedure: A randomized pilot study. Ann Thorac Surg2019; 108:1865–74.
Currigan DA, Hughes RJA, Wright CE, et al. Vasoconstrictor responses to vasopressor agents in human pulmonary and radial arteries: An in vitro study.Anaesthesiology 2014; 121:930–6.
Windsor J, Townsley MM, Briston D et al. Fontan palliation for single-ventricle physiology: perioperative management for noncardiac surgery and analysis of outcomes. J Cardiothorac Vasc Anesth 2017; 31:2296–303.
Roeleveld PP, de Klerk JCA. The perspective of the intensivist on inotropes and postoperative care following pediatric heart surgery: an institutional survey and systematic review of the literature. World J Pediatr Congenit Heart Surg 2018; 9:10–21.
Jolley M, Colan SD, Rhodes J, et al. The Fontan physiology has been revisited. Anesth Analg 2015; 121:172–82.
Mittnacht AJC. Pro: Early extubation following surgery for congenital heart disease. J Cardiothorac Vasc Anesth 2011; 25:874–6.
Lofland GK. Enhancement of hemodynamic performance in the Fontan circulation using pain-free spontaneous ventilation. Eur J Cardiothorac Surg 2001; 20:114–9.
Lovell AT. Anesthetic implications of congenital heart disease. Br J Anaesth 2004; 93:129–39
Price S, Jaggar SI, Jordan S et al. Adult congenital heart disease: Intensive care management and outcome prediction. Intensive Care Med 2007; 33:652–9.
Referanslar
Singh M, Kaur M, Grewal AM, Yangzes S, Yadav D, Zadeng Z, et al. Ophthalmic characteristics and management outcomes of 30 children with Goldenhar syndrome. International Ophthalmol.2020 March; 40 (3):667-675. [ PubMed ]
Beleza-Meireles A, Clayton-Smith J, Saraiva JM, Tassabehji M. Oculo-auriculo-vertebral spectrum: a review of the literature and genetic update. J Med Genet. 2014 Oct; 51 (10):635-45. [ PubMed ]
Bogusiak K, Puch A, Arkuszewski P. Goldenhar syndrome: current perspectives. World J Pediatr. October 2017; 13 (5):405-415. [ PubMed ]
Tasse C, Böhringer S, Fischer S, Lüdecke HJ, Albrecht B, Horn D, et al. Oculo-auriculo-vertebral spectrum (OAVS): Clinical evaluation and severity scoring of 53 patients and proposal for a new classification. Eur J Med Genet. 2005 October-December; 48 (4):397 411. [ PubMed ]
Strömland K, Miller M, Sjögreen L, Johansson M, Joelsson BM, Billstedt E, et al. Oculo-auriculo-vertebral spectrum: associated anomalies, functional deficits, and possible developmental risk factors. Am J Med Genet A. 2007 June 15;143A (12):1317-25. [ PubMed ]
A L Petrin , Lam Machado-Paula , A Hinkle , L Hovey , W Awotoye , M Chimenti, et al.: Whole genome sequencing of a family with autosomal dominant features within the oculoauriculovertebral spectrum. medRxiv. 2024 Feb 7:2024.02.07.24301824. doi: 10.1101/2024.02.07.24301824. [Pubmed]
Vendramini-Pittoli S, Kokitsu-Nakata NM. Oculoauriculovertebral spectrum: Report of nine familial cases with evidence of autosomal dominant inheritance and review of the literature. Clin Dysmorphol. April 2009; 18 (2):67-77. [ PubMed ]
Tuğ E, Atasoy HI, Koybaşı Sanal S. Thrombophilia gene mutations in the oculoauriculovertebral spectrum. Genet Couns. 2012; 23 (1):65-72. [ PubMed ]
Descartes M. Oculoauriculovertebral spectrum with 5p15.33-pter deletion. Clin Dysmorphol. July 2006; 15 (3):153-154. [ PubMed ]
Josifova DJ, Patton MA, Marks K. An oculoauriculovertebral spectrum phenotype caused by an unbalanced t(5;8) (p15.31;p23.1) rearrangement. Clin Dysmorphol. July 2004;13 (3):151-153. [ PubMed ]
Ala-Mello S, Siggberg L, Knuutila S, von Koskull H, Taskinen M, Peippo M. Further evidence for an association between chromosome region 5p15 and oculoauriculovertebral anomaly. Am J Med Genet A. 2008 Oct 01; 146A (19):2490-4. [ PubMed ]
Ingeborg Barisiç , Ljubica Odak , Maria Kredi , Ester Garne , Diana Wellesley , Elisa Calzolari and et.al. Prevalence, prenatal diagnosis, and clinical features of oculo-auriculo-vertebral spectrum: A registry-based study in Europe. Eur J Hum Genet 2014 Aug;22(8):1026-33. doi: 10.1038/ejhg.2013.287. Epub 2014 Jan 8. [Pubmed]
Rosa RF, Graziadio C, Lenhardt R, Alves RP, Paskulin GA, Zen PR. Central nervous system abnormalities in patients with oculo-auriculo-auriculo-vertebral spectrum (Goldenhar syndrome). Arq Neuropsiquiatr. February 2010; 68 (1):98-102. [ PubMed ]
Fischer S, Lüdecke HJ, Wieczorek D, Böhringer S, Gillessen-Kaesbach G, Horsthemke B. Allelic expression imbalance of BAPX1 in patients with oculo-auriculo-vertebral spectrum due to histone acetylation. Hum Mol Genet. February 15, 2006;15 (4):581 7. [ PubMed ]
Hongjuan Zhao , Chen Du , Guang Yang , Yu Wang. Diagnosis, treatment, and research status of rare diseases related to birth defects. Intractable Rare Dis Res. 2023 Aug;12(3): 148-160.doi: 10.5582/irdr.2023.01052.
Tripathy K, Sharma YR, Chawla R, Basu K, Vohra R, Venkatesh P. Triads in Ophthalmology: A Comprehensive Review. Semin Ophthalmol. 2017; 32 (2):237-250. [ PubMed ]
Martelli H, Miranda RT, Fernandes CM, Bonan PR, Paranaíba LM, Graner E, et al. Goldenhar syndrome: clinical features with orofacial accentuation. J Appl Oral Sci. December 2010;18 (6):646-9. [ PubMed ]
Renkema RW, Caron CJJM, Mathijssen IMJ, Wolvius EB, Dunaway DJ, Forrest CR, et al. Vertebral anomalies in craniofacial microsomia: a systematic review. Int J Oral Maxillofacial Surgery. October 2017; 46 (10):1319-1329. [ PubMed]
İsmail Balaban, Meltem Ceyhan Bilgici, Kemal Baysal. A new association of oculoauriculovertebral spectrum and persistent fifth aortic arch-double lumen aorta: a case report.BMC Pediatr. 2022 Feb 21;22(1):102. doi:10.1186/s12887 02031370.
John Richard McNamara , Aisling McMahon , Michael Griffin Perioperative Management of the Fontan Patient for Cardiac and Noncardiac Surgery Cardiothorac Vasc Anesth 2022 Jan;36(1): 275-285.doi: 10.1053/j.jvca.2021.04.021. Epub 2021 Apr 20.
Eagle SS, Daves SM. The adult with Fontan physiology: systematic approach to perioperative management for noncardiac surgery. J Cardio- thorac Vasc Anesth2011; 25:320–34.
Egbe AC, Khan AR, Ammash NM, et al. Predictors of procedural complications in adult Fontan patients undergoing noncardiac procedures. Heart 2017; 103:181320.
Lui GK, Saidi A, Bhatt AB, et al. Diagnosis and management of noncardiac complications in adults with congenital heart disease: a scientific statement from the American Heart Association. Circulation 2017;136: e348–92.
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guidelines for the management of adults with congenital heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol 2019;73: e81–192.
Larsen JR, Torp P, Norrild K, Sloth E: Propofol reduces tissue-Doppler markers of left ventricle function: a transthoracic echocardiographic study. Br J Anaesth 2007;98: 183−188.
Wappler F, Rossaint R, Baumert J et al.: Multicenter randomized com- parison of xenon and isoflurane on left ventricular function in patients undergoing elective surgery. Anesthesiology 2007; 106: 463−471.
Hasija S, Chauhan S, Jain P, Choudhury A, Aggarwal N, Pandey RK: Com- parison of speed of inhalational induction in children with and without congenital heart disease. Ann Card Anaesth 2016; 19: 468−474. doi: 10.4103/0971 9784.185531.
Rothrock SG, Pagane J Pediatric rapid sequence intubation incidence of reflex bradycardia and effects of pretreatment with atropine. Pediatr Emerg Care 2005;21: 637−638.
Naguib AN, Tobias JD, Hall MW et al. The role of different anesthetic techniques in altering the stress response during cardiac surgery in children: A prospective, double-blinded, randomized study. Pediatr Crit Care Med 2013; 14: 481−490.doi: 10.1097/PCC.0b013e31828a742c.
Shields M, Giovannelli M, Mirakhur RK, Moppett I, Adams J, Hermens Y: Org 25969 (sugammadex), a selective relaxant binding agent for antagonism of prolonged rocuronium-induced neuromuscular block. Br J Anaesth 2006; 96:36−43.
Groudine SB, Soto R, Lien C, Drover D, Roberts K: A randomized, dose- finding, phase II study of the selective relaxant binding drug, Sugam- madex, capable of safely reversing profound rocuronium-induced neuromuscular block. Anesth Analg2007; 104: 555−562.
Beers R, Camporesi E: Remifentanil update: clinical science and utility. CNS Drugs 2004; 18: 1085−1104.
Bailey PD Jr, Jobes DR. The Fontan Patient. Anesthesiol Clin 2009; 27:285–300.
Heggie J, Karski J. Anesthesiologist’s role in adults with congenital heart disease. Cardiol Clin 2006; 24:571–85.
Hedequist DJ, Emans JB, Hall JE, et al. Operative treatment for scoliosis in patients with Fontan circulation. Spine (Phila Pa 1976) 2006; 31:202-5.
d’Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton GR, et al. Rede-fining expectations of long-term survival after the Fontan procedure: Twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation. 2014 Sep;130(11 Suppl 1): S32–8.
Bigelow AM, Ghanayem NS, Thompson NE et al. Safety and efficacy of vasopressin after Fontan procedure: A randomized pilot study. Ann Thorac Surg2019; 108:1865–74.
Currigan DA, Hughes RJA, Wright CE, et al. Vasoconstrictor responses to vasopressor agents in human pulmonary and radial arteries: An in vitro study.Anaesthesiology 2014; 121:930–6.
Windsor J, Townsley MM, Briston D et al. Fontan palliation for single-ventricle physiology: perioperative management for noncardiac surgery and analysis of outcomes. J Cardiothorac Vasc Anesth 2017; 31:2296–303.
Roeleveld PP, de Klerk JCA. The perspective of the intensivist on inotropes and postoperative care following pediatric heart surgery: an institutional survey and systematic review of the literature. World J Pediatr Congenit Heart Surg 2018; 9:10–21.
Jolley M, Colan SD, Rhodes J, et al. The Fontan physiology has been revisited. Anesth Analg 2015; 121:172–82.
Mittnacht AJC. Pro: Early extubation following surgery for congenital heart disease. J Cardiothorac Vasc Anesth 2011; 25:874–6.
Lofland GK. Enhancement of hemodynamic performance in the Fontan circulation using pain-free spontaneous ventilation. Eur J Cardiothorac Surg 2001; 20:114–9.
Lovell AT. Anesthetic implications of congenital heart disease. Br J Anaesth 2004; 93:129–39
Price S, Jaggar SI, Jordan S et al. Adult congenital heart disease: Intensive care management and outcome prediction. Intensive Care Med 2007; 33:652–9.
