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<< Congenital Heart Disease In Pediatric Patients: Recognizing The Undiagnosed And Managing Complications In The Emergency Department


Evidence-based medicine requires a critical appraisal of the literature based upon study methodology and number of subjects. Not all references are equally robust. The findings of a large, prospective, randomized, and blinded trial should carry more weight than a case report.

To help the reader judge the strength of each reference, pertinent information about the study, such as the type of study and the number of patients in the study are included in bold type following the references, where available. The most informative references cited in this paper, as determined by the authors, are noted by an asterisk (*) next to the number of the reference.

  1. van der Bom T, Zomer AC, Zwinderman AH, et al. The changing epidemiology of congenital heart disease. Nat Rev Cardiol. 2011;8(1):50-60. (Review article)

  2. van der Linde D, Konings EE, Slager MA, et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol. 2011;58(21):2241- 2247. (Systematic review and meta-analysis; 114 studies)

  3. Fahed AC, Roberts AE, Mital S, et al. Heart failure in congenital heart disease: a confluence of acquired and congenital. Heart Fail Clin. 2014;10(1):219-227. (Review article)

  4. Bajolle F, Zaffran S, Bonnet D. Genetics and embryological mechanisms of congenital heart diseases. Arch Cardiovasc Dis. 2009;102(1):59-63. (Review article)

  5. * Jenkins KJ, Correa A, Feinstein JA, et al. Noninherited risk factors and congenital cardiovascular defects: current knowledge: a scientific statement from the American Heart Association Council on Cardiovascular Disease in the Young: endorsed by the American Academy of Pediatrics. Circulation. 2007;115(23):2995-3014. (Scientific statement)

  6. Miller A, Riehle-Colarusso T, Siffel C, et al. Maternal age and prevalence of isolated congenital heart defects in an urban area of the United States. Am J Med Genet A. 2011;155a(9):2137-2145. (Cohort study; 5289 infants and 1,301,143 live births)

  7. Fung A, Manlhiot C, Naik S, et al. Impact of prenatal risk factors on congenital heart disease in the current era. J Am Heart Assoc. 2013;2(3):e000064. (Case control study; 2339 cases and 199 controls)

  8. Dedieu N, Burch M. Understanding and treating heart failure in children. Paediatr Child Health.23(2):47-52. (Review article)

  9. * Brown K. The infant with undiagnosed cardiac disease in the emergency department. Clin Pediatr Emerg Med. 2005;6(4):200-206. (Review article)

  10. Sinha IP, Mayell SJ, Halfhide C. Pulse oximetry in children. Arch Dis Child Educ Pract Ed. 2014;99(3):117-118. (Review article)

  11. Fouzas S, Priftis KN, Anthracopoulos MB. Pulse oximetry in pediatric practice. Pediatrics. 2011;128(4):740-752. (Review article)

  12. Yee L. Cardiac emergencies in the first year of life. Emerg Med Clin North Am. 2007;25(4):981-1008. (Review article)

  13. Kleinman ME, Chameides L, Schexnayder SM, et al. Pediatric advanced life support: 2010 American Heart Association guidelines for cardiopulmonary resuscitation and emergency cardiovascular care. Pediatrics. 2010;126(5):e1361-e1399. (Guidelines)

  14. Gerritse BM, Draaisma JM, Schalkwijk A, et al. Should EMS-paramedics perform paediatric tracheal intubation in the field? Resuscitation. 2008;79(2):225-229. (Prospective observational study of pediatric intubations by EMS vs specialized air transport team)

  15. Wang HE, Lave JR, Sirio CA, et al. Paramedic intubation er¬rors: isolated events or symptoms of larger problems? Health Aff (Millwood). 2006;25(2):501-509. (Retrospective study of pediatric EMS intubations)

  16. Gausche M, Lewis RJ, Stratton SJ, et al. Effect of out-of-hospital pediatric endotracheal intubation on survival and neurological outcome: a controlled clinical trial. JAMA. 2000;283(6):783-790. (Controlled prospective trial of intuba¬tion vs BVM; 830 consecutive pediatric patients treated by EMS)

  17. Young KD, Gausche-Hill M, McClung CD, et al. A prospective, population-based study of the epidemiology and outcome of out-of-hospital pediatric cardiopulmonary arrest. Pediatrics. 2004;114(1):157-164. (Secondary analysis of data; 599 patients from above prospective interventional trial of pediatric EMS airway management)

  18. Lee C, Mason LJ. Pediatric cardiac emergencies. Anesthesiol Clin North America. 2001;19(2):287-308. (Review article)

  19. * Szlam S, Dejanovich B, Ramirez R, et al. Congenital heart disease: complications before and after surgical repair. Clin Pediatr Emerg Med. 2012;13(2):65-80. (Review article)

  20. Orr RA, Felmet KA, Han Y, et al. Pediatric specialized transport teams are associated with improved outcomes. Pediatrics. 2009;124(1):40-48. (Prospective single-center cohort study; 1085 pediatric transports)

  21. Edge WE, Kanter RK, Weigle CG, et al. Reduction of morbid¬ity in interhospital transport by specialized pediatric staff. Crit Care Med. 1994;22(7):1186-1191. (Concurrent prospective comparison of 2 centers with different transport teams and 141 total transports)

  22. Singh JM, MacDonald RD, Ahghari M. Critical events during land-based interfacility transport. Ann Emerg Med. 2014;64(1):9-15. (Retrospective cohort study; 5144 transports)

  23. Lim MT, Ratnavel N. A prospective review of adverse events during interhospital transfers of neonates by a dedicated neonatal transfer service. Pediatr Crit Care Med. 2008;9(3):289- 293. (Prospective observational study; 346 pediatric transports)

  24. * Lee YS, Baek JS, Kwon BS, et al. Pediatric emergency room presentation of congenital heart disease. Korean Circ J. 2010;40(1):36-41. (Retrospective study; 368 pediatric patients)

  25. Tibbles CD, Bouton M, Lucas JM, et al. Emergency department management of pediatric patients with cyanotic heart disease and fever. J Emerg Med. 2013;44(3):599-604. (Retrospective review; 809 pediatric patients)

  26. * Savitsky E, Alejos J, Votey S. Emergency department presentations of pediatric congenital heart disease. J Emerg Med. 2003;24(3):239-245. (Retrospective review; 77 pediatric patients)

  27. * Wren C, Richmond S, Donaldson L. Presentation of congeni¬tal heart disease in infancy: implications for routine examination. Arch Dis Child Fetal Neonatal Ed. 1999;80(1):F49-F53. (Retrospective review; 1590 infants)

  28. Thangaratinam S, Brown K, Zamora J, et al. Pulse oximetry screening for critical congenital heart defects in asymptomatic newborn babies: a systematic review and meta-analysis. Lancet. 2012;379(9835):2459-2464. (Systematic review)

  29. Crossland DS, Furness JC, Abu-Harb M, et al. Variability of four limb blood pressure in normal neonates. Arch Dis Child Fetal Neonatal Ed. 2004;89(4):F325-F327. (Prospective study; 40 neonates)

  30. Sharieff GQ, Wylie TW. Pediatric cardiac disorders. J Emerg Med. 2004;26(1):65-79. (Review article)

  31. Colletti JE, Homme JL, Woodridge DP. Unsuspected neonatal killers in emergency medicine. Emerg Med Clin North Am. 2004;22(4):929-960. (Review article)

  32. Koulouri S, Acherman RJ, Wong PC, et al. Utility of B-type natriuretic peptide in differentiating congestive heart failure from lung disease in pediatric patients with respiratory distress. Pediatr Cardiol. 2004;25(4):341-346. (Prospective study; 49 infants and children)

  33. Maher KO, Reed H, Cuadrado A, et al. B-type natriuretic peptide in the emergency diagnosis of critical heart disease in children. Pediatrics. 2008;121(6):e1484-e1488. (Prospective case control study; 33 patients with CHD and 70 controls)

  34. Rossano JW, Shaddy RE. Heart failure in children: etiology and treatment. J Pediatr. 2014;165(2):228-233. (Review article)

  35. * Chan TC, Sharieff GQ, Brady WJ. Electrocardiographic manifestations: pediatric ECG. J Emerg Med. 2008;35(4):421-430. (Review article)

  36. O’Connor M, McDaniel N, Brady WJ. The pediatric electrocardiogram. Part I: age-related interpretation. Am J Emerg Med. 2008;26(2):221-228. (Review article)

  37. O’Connor M, McDaniel N, Brady WJ. The pediatric electrocardiogram. Part III: congenital heart disease and other cardiac syndromes. Am J Emerg Med. 2008;26(4):497-503. (Review article)

  38. Tonkin IL. Imaging of pediatric congenital heart disease. J Thorac Imaging. 2000;15(4):274-279. (Review article)

  39. Russell J, Justino H, Dipchand A, et al. Noninvasive imaging in congenital heart disease. Curr Opin Cardiol. 2000;15(4):224- 237. (Review article)

  40. Ferguson EC, Krishnamurthy R, Oldham SA. Classic imaging signs of congenital cardiovascular abnormalities. Radiographics. 2007;27(5):1323-1334. (Review)

  41. Bernstein D. Laboratory evaluation. In: Kliegman RM, Stanton BF, St. Geme JW, et al, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011. (Textbook)

  42. * Gazit AZ, Oren PP. Pharmaceutical management of decompensated heart failure syndrome in children: current state of the art and a new approach. Curr Treat Options Cardiovasc Med. 2009;11(5):403-409. (Review article)

  43. Lewis AB, Freed MD, Heymann MA, et al. Side effects of therapy with prostaglandin E1 in infants with critical congenital heart disease. Circulation. 1981;64(5):893-898. (Case report review; 492 infants)

  44. * Meckler GD, Lowe C. To intubate or not to intubate? Transporting infants on prostaglandin E1. Pediatrics. 2009;123(1):e25-e30. (Retrospective review; 202 neonatal transports receiving PGE)

  45. Kothari SS. Mechanism of cyanotic spells in Tetralogy of Fallot--the missing link? Int J Cardiol. 1992;37(1):1-5. (Review)

  46. BMJ Best Practice. Tetralogy of Fallot (management of hypercyanotic (tet) spells). Available at: http://bestpractice.bmj.com/best-practice/monograph/701/treatment/step-by-step.html. Accessed January 15, 2016. (Guidelines)

  47. Apitz C, Webb GD, Redington AN. Tetralogy of Fallot. Lancet. 2009;374(9699):1462-1471. (Review article)

  48. Barata IA. Cardiac emergencies. Emerg Med Clin North Am. 2013;31(3):677-704. (Review)

  49. Tsze DS, Vitberg YM, Berezow J, et al. Treatment of Tetralogy of Fallot hypoxic spell with intranasal fentanyl. Pediatrics. 2014;134(1):e266-e269. (Case report)

  50. * Faris R, Flather MD, Purcell H, et al. Diuretics for heart fail¬ure. Cochrane Database Syst Rev. 2006(1):CD003838. (Systematic review)

  51. Kantor PF, Lougheed J, Dancea A, et al. Presentation, diagnosis, and medical management of heart failure in children: Canadian Cardiovascular Society guidelines. Can J Cardiol. 2013;29(12):1535-1552. (Professional society practice guide-lines)

  52. Beggs S, Thompson A, Nash R, et al. Cardiac failure in children. 17th Expert Committee on the Selection and Use of Essential Medicines. Geneva, Switzerland: World Health Organization; 2009. (Expert committee systematic review and recommendations)

  53. Thackray S, Easthaugh J, Freemantle N, et al. The effectiveness and relative effectiveness of intravenous inotropic drugs acting through the adrenergic pathway in patients with heart failure-a meta-regression analysis. Eur J Heart Fail. 2002;4(4):515-529. (Systematic review and meta-analysis)

  54. Cuffe MS, Califf RM, Adams KF, Jr., et al. Short-term intravenous milrinone for acute exacerbation of chronic heart failure: a randomized controlled trial. JAMA. 2002;287(12):1541- 1547. (Prospective randomized double-blind placebo-controlled multicenter study; 951 patients)

  55. Hoffman TM, Wernovsky G, Atz AM, et al. Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heart disease. Circulation. 2003;107(7):996-1002. (Prospective double-blind placebo-controlled parallel group trial; 238 patients)

  56. Buck ML. Clinical experience with spironolactone in pediatrics. Ann Pharmacother. 2005;39(5):823-828. (Prospective observational study; 100 consecutive pediatric patients)

  57. Hsu DT, Pearson GD. Heart failure in children: part II: diagnosis, treatment, and future directions. Circ Heart Fail. 2009;2(5):490-498. (Review article)

  58. Bruns LA, Chrisant MK, Lamour JM, et al. Carvedilol as therapy in pediatric heart failure: an initial multicenter experience. J Pediatr. 2001;138(4):505-511. (Retrospective multicenter study)

  59. Shaddy RE, Boucek MM, Hsu DT, et al. Carvedilol for children and adolescents with heart failure: a randomized controlled trial. JAMA. 2007;298(10):1171-1179. (Prospective randomized, double-blind, placebo-controlled multicenter study; 161 pediatric patients)

  60. Frobel AK, Hulpke-Wette M, Schmidt KG, et al. Beta-blockers for congestive heart failure in children. Cochrane Database Syst Rev. 2009(1):CD007037. (Systematic review)

  61. Hood WB Jr, Dans AL, Guyatt GH, et al. Digitalis for treatment of congestive heart failure in patients in sinus rhythm. Cochrane Database Syst Rev. 2004(2):CD002901. (Systematic review)

  62. Gaca AM, Jaggers JJ, Dudley LT, et al. Repair of congenital heart disease: a primer-part 2. Radiology. 2008;248(1):44-60. (Review article)

  63. Gaca AM, Jaggers JJ, Dudley LT, et al. Repair of congenital heart disease: a primer-part 1. Radiology. 2008;247(3):617-631. (Review article)

  64. * Woods WA, McCulloch MA. Cardiovascular emergencies in the pediatric patient. Emerg Med Clin North Am. 2005;23(4):1233-1249. (Review article)

  65.  Bernstein D. Anomalous origin of the coronary arteries. In: Kliegman RM, Stanton BF, St. Geme JW, et al, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011. (Textbook)

  66. Frazier A, Hunt EA, Holmes K. Pediatric cardiac emergencies: children are not small adults. J Emerg Trauma Shock. 2011;4(1):89-96. (Review article)

  67. Bernstein D. Abnormal positions of the heart and heterotaxy syndromes. In: Kliegman RM, Stanton BF, St. Geme JW, et al., eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011. (Textbook)

  68. Healy F, Hanna BD, Zinman R. Pulmonary complications of congenital heart disease. Paediatr Respir Rev. 2012;13(1):10-15. (Review article)

  69. Bernstein D. Pulmonary hypertension. In: Kliegman RM, Stanton BF, St. Geme JW, et al., eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, PA: Elsevier Saunders; 2011. (Textbook)

  70. Kleinman ME, Chameides L, Schexnayder SM, et al. Part 14: pediatric advanced life support: 2010 American Heart Association guidelines for cardiopulmonary resuscitation and emergency cardiovascular care. Circulation. 2010;122(18 Suppl 3):S876-S908. (Professional society practice guidelines)

  71. Yun SW. Congenital heart disease in the newborn requiring early intervention. Korean J Pediatr. 2011;54(5):183-191. (Review article)

  72. Tallman TA, Peacock WF, Emerman CL, et al. Noninvasive ventilation outcomes in 2,430 acute decompensated heart failure patients: an ADHERE Registry Analysis. Acad Emerg Med. 2008;15(4):355-362. (Retrospective study; 2430 patients)

  73. * Kantor PF, Mertens LL. Clinical practice: heart failure in children. Part I: clinical evaluation, diagnostic testing, and initial medical management. Eur J Pediatr. 2010;169(3):269- 279. (Review article)

  74. Huang FK, Lin CC, Huang TC, et al. Reappraisal of the prostaglandin E1 dose for early newborns with patent ductus arteriosus-dependent pulmonary circulation. Pediatr Neonatol. 2013;54(2):102-106. (Retrospective review; 33 patients)

  75. Sharma M, Sasikumar M, Karloopia S, et al. Prostaglandins in congenital heart disease. Med J Armed Forces India. 2001;57(2):134-138. (Review article)

  76. * Rushani D, Kaufman JS, Ionescu-Ittu R, et al. Infective endocarditis in children with congenital heart disease: cumulative incidence and predictors. Circulation. 2013;128(13):1412-1419. (Population based study; 47,518 children in Quebec)

  77. Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007;116(15):1736-1754. (Professional society practice guidelines)

  78. Allen UD, Canadian Paediatric Society. Infective endocarditis: updated guidelines Paediatr Child Health. 2010 (reaffirmed 2014);15(4):205-208. (Professional society guidelines)

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