The Importance of the Airway in Children Undergoing Surgery for Congenital Heart Disease

IN THIS edition of the Journal of Cardiothoracic and Vascular Anesthesia, Foz et al. reported on the risk factors, genetic syndromes, and cardiac defects associated with congenital and acquired airway disease.1Foz C.P.J. Peyton J. Staffa S.J. et al.Airway abnormalities in patients with congenital heart disease: Incidence and associated factors.J Cardiothoracic Vasc Anesth. 2020; (Accessed September 25, 2020. [e-pub ahead of print])https://doi.org/10.1053/j.jvca.2020.07.086Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar As the genetic basis for many forms of congenital heart disease (CHD) continue to be elucidated, there is a greater understanding of the associated congenital airway abnormalities found in many of these children.2Pierpont M.E. Brueckner M. Chung W.K. et al.genetic basis for congenital heart disease: Revisited: a scientific statement from the American Heart Association.Circulation. 2018; 138: e653-e711Crossref PubMed Scopus (112) Google Scholar This association is due to an intrinsic relationship between the respiratory system and cardiac structures. During embryologic development, the branchial arches are influenced by the neural crest cells, in addition to various chemical signals and growth factors, leading to the development of the heart, great vessels, and the airway.3Waldo K. Zdanowicz M. Burch J. et al.A novel role for cardiac neural crest in heart development.J Clin Invest. 1999; 103: 1499-1507Crossref PubMed Scopus (94) Google Scholar,4Evans S.M. Yelon D. Conlon F.L. et al.Myocardial lineage development.Circ Res. 2010; 107: 1428-1444Crossref PubMed Scopus (168) Google Scholar Clinically, there are many reports on the association of congenital and acquired airway abnormalities in children with CHD.5Yang L. Li S. Zhong L. et al.VACTERL association complicated with multiple airway abnormalities: A case report.Medicine (Baltimore). 2019; 98: e17413Crossref PubMed Scopus (1) Google Scholar, 6Kussman B.D. Geva T. McGowan F.X. Cardiovascular causes of airway compression.Paediatr Anaesth. 2004; 14: 60-74Crossref PubMed Scopus (128) Google Scholar, 7Hart C.K. Rutter M.J. Tracheobronchial issues in congenital heart disease.Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2015; 18: 57-61Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar, 8Ragalie W.S. Mitchell M.E. Advances in surgical treatment of congenital airway disease.Semin Thorac Cardiovasc Surg. 2016; 28: 62-68Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar, 9Matisoff A.J. Ari P. Zurakowski D. et al.Risk factors associated with thedevelopment of acquired airway disease after congenital heart surgery: A retrospective cohort study.Semin Cardiothorac Vasc Anesth. 2018; 22: 294-299Crossref PubMed Scopus (4) Google Scholar, 10Kellenberger C.J. Aortic arch malformations.Pediatr Radiol. 2010; 40: 876-884Crossref PubMed Scopus (62) Google Scholar Not infrequently, children with congenital tracheal stenosis and congenital heart disease require simultaneous airway and cardiac surgical repair on cardiopulmonary bypass soon after birth.11Xue B. Liang B. Wang S. et al.One-stage surgical correction of congenital tracheal stenosis complicated with congenital heart disease in infants and young children.J Card Surg. 2015; 30: 97-103Crossref PubMed Scopus (18) Google Scholar,12Matsuhisa H. Oshima Y. Kadowaki T. et al.Simultaneous complex single ventricle palliation and tracheoplasty for heterotaxy syndrome.Interact Cardiovasc Thorac Surg. 2012; 15: 523-524Crossref PubMed Scopus (3) Google Scholar Taking these factors into consideration, the first step in planning the delivery of safe general anesthesia for children with CHD is a detailed airway assessment. In CHD patients this assessment may require investigations before cardiac surgery, including bronchoscopy, computed axial tomography, and magnetic resonance imaging.13Chapotte C. Monrigal J.P. Pezard P. et al.Airway compression in children due to congenital heart disease: Value of flexible fiberoptic bronchoscopic assessment.J Cardiothorac Vasc Anesth. 1998; 12: 145-152Abstract Full Text PDF PubMed Scopus (26) Google Scholar, 14Goitein O. Salem Y. Jacobson J. et al.The role of cardiac computed tomography in infants with congenital heart disease.Isr Med Assoc J. 2014; 16: 147-152PubMed Google Scholar, 15Hou Q. Gao W. Zhong Y. et al.Diagnostic accuracy of three-dimensional turbo field echo magnetic resonance imaging sequence in pediatric tracheobronchial anomalies with congenital heart disease.Sci Rep. 2018; 8: 2529Crossref PubMed Scopus (3) Google Scholar Airway assessment is especially important in children with CHD, as they may be particularly vulnerable to hypoxia-associated morbidity and mortality during airway manipulation with direct laryngoscopy and endotracheal intubation. It has been found in at least one study, that children with CHD may be twice as likely to present with a Cormack Lehane Grade III and IV view during laryngoscopy and direct endotracheal intubation as compared with other pediatric patients.16Heinrich S. Birkholz T. Ihmsen H. et al.Incidence and predictors of poor laryngoscopic view in children undergoing pediatric cardiac surgery.J Cardiothorac Vasc Anesth. 2013; 27: 516-521Abstract Full Text Full Text PDF PubMed Scopus (19) Google Scholar,17Cormack R.S. Lehane J. Difficult tracheal intubation in obstetrics.Anaesthesia. 1984; 39: 1105-1111Crossref PubMed Scopus (1808) Google Scholar The most critical perioperative events in pediatric surgical patients have been found to be related to complications of the respiratory system.18Egbuta C. Mason K.P. Recognizing risks and optimizing perioperative care to reduce respiratory complications in the pediatric patient.J Clin Med. 2020; 9: 1942Crossref Scopus (3) Google Scholar It has been estimated that 75% of all perioperative adverse events involve the respiratory system, with 36% of these due to laryngospasm.18Egbuta C. Mason K.P. Recognizing risks and optimizing perioperative care to reduce respiratory complications in the pediatric patient.J Clin Med. 2020; 9: 1942Crossref Scopus (3) Google Scholar,19Tay C.L. Tan G.M. Ng S.B. Critical incidents in paediatric anaesthesia: An audit of 10 000 anaesthetics in Singapore.Paediatr Anaesth. 2001; 11: 711-718Crossref PubMed Scopus (142) Google Scholar Children with CHD remain at risk for hypoxia and cardiovascular deterioration related to airway complications during planned and unplanned endotracheal extubations in the post-anesthesia and cardiac surgery convalescent periods.20Censoplano N.M. Barrett C.S. Ing R.J. et al.Achieving sustainability in reducing unplanned extubations in a pediatric cardiac ICU.Pediatr Crit Care Med. 2020; 21: 350-356Crossref PubMed Scopus (4) Google Scholar In children undergoing anesthesia, respiratory causes are a major etiology of cardiac arrest in patients with and without heart disease, with the majority of these related to loss of airway patency.21Ramamoorthy C. Haberkern C.M. Bhananker S.M. et al.Anesthesia-related cardiac arrest in children with heart disease: Data from the Pediatric Perioperative Cardiac Arrest (POCA) registry.Anesth Analg. 2010; 110: 1376-1382Crossref PubMed Scopus (208) Google Scholar Children with CHD are also more likely to require multiple anesthetics for both cardiac and noncardiac procedures, often requiring instrumentation of the airway. Furthermore, cardiopulmonary interactions are more likely to disturb their physiology, particularly in cyanotic children with shunt-dependent or single-ventricle physiology. With many of these children presenting to the operating room critically ill or in a baseline hypoxic state, loss of the ability to oxygenate or ventilate can rapidly progress to cardiovascular collapse. This is particularly important because Fiadjoe et al. have found that the degree of airway complications increases after more than 2 endotracheal intubation attempts. Additionally, hypoxia, and cardiovascular complications occur more commonly in children weighing less than 10 kg, those with a short thyromental distance, and when 3 direct laryngoscopy attempts occur before an indirect technique is attempted.22Fiadjoe J.E. Nishisaki A. Jagannathan N. et al.Airway management complications in children with difficult tracheal intubation from the Pediatric Difficult Intubation (PeDI) registry: A prospective cohort analysis.Lancet Respir Med. 2016; 4: 37-48Abstract Full Text Full Text PDF PubMed Scopus (165) Google Scholar Previous studies have evaluated the relationship between CHD and congenital airway abnormalities, particularly in patients with genetic syndromes known to be associated with heart defects.23Sacca R. Zur K.B. Crowley T.B. et al.Association of airway abnormalities with 22q11.2 deletion syndrome.Int J Pediatr Otorhinolaryngol. 2017; 96: 11-14Crossref PubMed Scopus (22) Google Scholar, 24Hamilton J. Yaneza M.M. Clement W.A. et al.The prevalence of airway problems in children with Down's syndrome.Int J Pediatr Otorhinolaryngol. 2016; 81: 1-4Crossref PubMed Scopus (27) Google Scholar, 25Kazim R. Quaegebeur J.M. Sun L.S. The association of tracheal anomalies and tetralogy of Fallot.J Cardiothorac Vasc Anesth. 1996; 10: 589-592Abstract Full Text PDF PubMed Scopus (21) Google Scholar, 26Kazim R. Berdon W.E. Montoya C.H. et al.Tracheobronchial anomalies in children with congenital cardiac disease.J Cardiothorac Vasc Anesth. 1998; 12: 553-555Abstract Full Text PDF PubMed Scopus (17) Google Scholar Furthermore, studies have independently evaluated airway complications, and resulting morbidity and mortality, in children with and without CHD.22Fiadjoe J.E. Nishisaki A. Jagannathan N. et al.Airway management complications in children with difficult tracheal intubation from the Pediatric Difficult Intubation (PeDI) registry: A prospective cohort analysis.Lancet Respir Med. 2016; 4: 37-48Abstract Full Text Full Text PDF PubMed Scopus (165) Google Scholar,27Lee Y.S. Jeng M.J. Tsao P.C. et al.Prognosis and risk factors for congenital airway anomalies in children with congenital heart disease: A Nationwide population-based study in Taiwan.PLoS One. 2015; 10e0137437PubMed Google Scholar The study by Foz et al. is important because it evaluated a large cohort of children with heterogeneous CHD pathologies and investigated associated airway abnormalities, risk factors, and incidence of difficult intubation. Foz et al. conducted a retrospective study on 4,797 children less than 18 years of age with CHD who underwent 8,657 procedures for cardiac surgery, cardiac imaging, catheterization, and electrophysiology studies over a 7-year period. Although 1,724 (20%) patients presented for their procedure with an endotracheal tube is situ, 6,858 (79%) were intubated at the time of the procedure, with a success rate of 87.1% on the first intubation attempt, 10.2% on the second attempt, and 2.4% of patients required 3 or more intubation attempts.1Foz C.P.J. Peyton J. Staffa S.J. et al.Airway abnormalities in patients with congenital heart disease: Incidence and associated factors.J Cardiothoracic Vasc Anesth. 2020; (Accessed September 25, 2020. [e-pub ahead of print])https://doi.org/10.1053/j.jvca.2020.07.086Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar Patient demographics, cardiac diagnosis, airway abnormalities, known genetic disorders, and intubation data were collected from the electronic medical record. Specific detail also was given to verification of airway abnormalities via documented laryngoscopy, bronchoscopy, and radiologic studies. Information on difficult intubation was extracted via the Pediatric Difficult intubation Registry, of which the institution was a participant for the duration of the study. The intubation characteristics, associated genetic syndromes, and acquired or congenital abnormalities are presented as frequency and percentages. Multivariate analysis was utilized to identify independent predictors of acquired and congenital airway abnormalities.1Foz C.P.J. Peyton J. Staffa S.J. et al.Airway abnormalities in patients with congenital heart disease: Incidence and associated factors.J Cardiothoracic Vasc Anesth. 2020; (Accessed September 25, 2020. [e-pub ahead of print])https://doi.org/10.1053/j.jvca.2020.07.086Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar The authors found that 16.7% of the cohort had at least one genetic syndrome, with the most common being trisomy 21, 22q11 microdeletion, Stickler syndrome, and CHARGE syndrome. The median age of the patients was 1.3 years, with a median weight of 9.2 kg, and 23.8% of children were premature. Congenital airway abnormalities were present in 8.5% of the patients, with the most common diagnoses being laryngomalacia, bronchomalacia, tracheal disease, subglottic stenosis, and laryngeal cleft. The incidence of acquired airway abnormality was 9.7%, with the most frequent diagnoses being vocal cord paralysis, bronchomalacia, and subglottic stenosis. Independent risk factors for congenital airway abnormalities were found to be weight <10 kg, prematurity, genetic syndrome, anomalies of the aortic arch, tetralogy of Fallot, and pulmonary artery anomalies. Risk factors for acquired airway abnormalities were weight <10 kg, prematurity, genetic syndrome, anomalies of the aortic arch, hypoplastic heart, and pulmonary artery anomalies. Analysis also showed that multiple encounters did not increase the incidence of difficult intubation or causing an airway abnormality. The duration of intubation, on the other hand, was significantly associated with causing an airway abnormality. Importantly, the authors found an acquired airway abnormality rate of 68.9% among those patients with a congenital airway abnormality, with most of these acquired airway abnormalities occurring at or below the vocal cords.1Foz C.P.J. Peyton J. Staffa S.J. et al.Airway abnormalities in patients with congenital heart disease: Incidence and associated factors.J Cardiothoracic Vasc Anesth. 2020; (Accessed September 25, 2020. [e-pub ahead of print])https://doi.org/10.1053/j.jvca.2020.07.086Abstract Full Text Full Text PDF PubMed Scopus (4) Google Scholar This study by Foz et al. is an important addition to our knowledge on congenital and acquired airway abnormalities in children with congenital heart disease. Their findings reinforced the importance of detailed airway assessment in every patient, but especially in young children with congenital heart disease. Expanding our existing knowledge, along with proper assessment, planning, and preparation, increase perioperative safety for children with CHD. The authors have no conflict of interests. Airway Abnormalities in Patients With Congenital Heart Disease: Incidence and Associated FactorsJournal of Cardiothoracic and Vascular AnesthesiaVol. 35Issue 1PreviewObjectives: Airway abnormalities complicate the perioperative course of patients with congenital heart disease (CHD), leading to significant morbidity and mortality. The literature describing airway abnormalities in those patients is scarce. This study aimed to determine the incidence of airway abnormalities in CHD patients and identify associated factors, genetic syndromes, and cardiac diagnoses.Design: Retrospective study conducted after institutional review board approval.Setting: Tertiary children's hospital. Full-Text PDF