Congenital Heart Disease In Pediatric Patients: Emergency Room Diagnosis & Treatment, Shock, Cyanosis, Congestive Heart Failure | EB Medicine
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Congenital Heart Disease in Pediatric Patients: Recognizing the Undiagnosed and Managing Complications in the Emergency Department (Pharmacology CME)

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Table of Contents
 
Table of Contents
  1. Abstract
  2. Case Presentations
  3. Introduction
    1. Epidemiology
    2. Presentations Of Congenital Heart Disease
  4. Critical Appraisal of the Literature
  5. Etiology And Pathophysiology
    1. Transition From Fetal To Neonatal Circulation
    2. Pathophysiology Of Clinical Presentations Of Shock
    3. Pathophysiology Of Clinical Presentations Of Cyanosis
    4. Pathophysiology Of Clinical Presentations Of Congestive Heart Failure
  6. Differential Diagnosis
  7. Prehospital Care
  8. Emergency Department Evaluation
    1. Initial Evaluation And Stabilization
    2. History
    3. Physical Examination
  9. Diagnostic Studies
    1. Laboratory Studies
    2. Electrocardiography
    3. Chest Radiography
    4. Echocardiography
  10. Treatment
    1. Shock And Congenital Heart Disease
    2. Cyanosis And Congenital Heart Disease
    3. Congestive Heart Failure And Congenital Heart Disease
      1. Medications
  11. Special Populations
    1. Complications Of Surgically Repaired/ Palliated Congenital Heart Disease
      1. Emergency Management Of Surgically Repaired/ Palliated Congenital Heart Disease Presenting With Acute Decompensation
    2. Anomalous Origin Of The Left Coronary Artery From The Pulmonary Artery
    3. Heterotaxy Syndromes
    4. Aortic Arch Abnormalities And Pulmonary Slings
    5. Pulmonary Hypertension
    6. Dysrhythmias
  12. Controversies And Cutting Edge
    1. Oxygen In Congenital Heart Disease
    2. Intubation And Positive-Pressure Ventilation
    3. Prostaglandin E1
    4. Subacute Bacterial Endocarditis And Antibiotic Prophylaxis
  13. Disposition
  14. Summary
  15. Abbreviations
  16. Risk Management Pitfalls In Pediatric Congenital Heart Disease
  17. Time- And Cost-Effective Strategies
  18. Case Conclusions
  19. Clinical Pathway For Management Of Congenital Heart Disease In The Neonate Presenting With Shock
  20. Clinical Pathway For Management Of Hypercyanotic Episode In Pediatric Patients With Tetralogy Of Fallot
  21. Clinical Pathway For Management Of Congenital Heart Disease In The Pediatric Patient Presenting With Congestive Heart Failure
  22. Tables and Figures
    1. Table 1. Absolute And Relative Frequency Of The Most Common Cardiac Defects
    2. Table 2. Clinical Presentations Of Congenital Heart Disease In Children
    3. Table 3. Genetic Defects And Syndromes Associated With Congenital Heart Disease
    4. Table 4. Risk Factors For Congenital Heart Disease
    5. Table 5. Cardiovascular Examination Findings In Specific Congenital Heart Disease
    6. Table 6. Possible Chest X-Ray And Electrocardiogram Findings In Congenital Heart Disease
    7. Table 7. Common Surgical Procedures And Their Complications In Complex Congenital Heart Disease
    8. Table 8. Indications And Antibiotics For Endocarditis Prophylaxis In Congenital Heart Disease
    9. Figure 1. Timing And Presentation Of Congenital Heart Defects
    10. Figure 2. Electrocardiogram Of 1-Week-Old Patient With Hypoplastic Left Heart Syndrome, Showing Right Ventricular Hypertrophy And Strain
    11. Figure 3. Electrocardiogram Of 6-Week-Old Patient, With Ventricular Septal Defect, Showing Left Ventricular Hypertrophy And Left Axis Deviation
    12. Figure 4. Total Anomalous Pulmonary Venous Return Initially Complicated By Multilobar Pneumonia
    13. Figure 5. Anteroposterior And Lateral Chest X-Ray Of An Infant With Anomalous Left Coronary Artery From The Pulmonary Artery
    14. Figure 6. Normal Infant Thymus May Be Mistaken For Cardiomegaly
    15. Figure 7. Electrocardiogram Of 5-Month-Old Patient With Anomalous Origin Of The Left Coronary Artery From The Pulmonary Artery
    16. Figure 8. Right-Sided Aortic Arch And Vascular Ring
  23. References

Abstract

Congenital heart disease is the most common form of all congenital malformations and, despite advances in prenatal and newborn screening, it may present undiagnosed to the emergency department. Signs and symptoms of congenital heart disease are variable and often nonspecific, making recognition and treatment challenging. Patient presentations can range from life-threatening shock or cyanosis in a neonate to respiratory distress or failure to thrive in infants. Advances in surgical techniques have improved short- and long-term survival of infants and children with congenital heart disease, but these children are at risk for a variety of complications related to the underlying or surgical anatomy and physiology. This review focuses on the recognition and initial management of patients with undiagnosed congenital heart disease presenting to the ED and touches on considerations for postoperative infants and children with complex congenital heart disease.

Case Presentations

An 8-day-old boy is brought to the ED by his mother for lethargy and “fast breathing.” She states that he has not been feeding well for the past couple of days and his breathing has become faster and more labored over the past 24 hours. This morning he became lethargic and looked pale. She denies any fever, cough, vomiting, or diarrhea. The baby was born at term and delivered at home by a midwife, and there was little prenatal care. He has been exclusively breastfed, but feeds have become progressively shorter over the preceding 48 hours. At triage, the infant appeared ashen gray and limp, with the following vital signs: temperature, 36°C; heart rate, 194 beats/min; respiratory rate, 76 breaths/min; and initial oxygen saturation, 92% on room air. He was rushed back to the resuscitation room. As you enter the room to evaluate this critically ill neonate, you consider sepsis, metabolic disease, and congenital heart disease, and wonder how you can distinguish among these potential causes of critical illness in the first weeks of life. Given the clinical picture of this neonate, you administer broad-spectrum antibiotics, begin fluid resuscitation, and consider whether to initiate empiric prostaglandin, but you are not sure if this is necessary or safe without a clear diagnosis.

A 3-month-old girl is brought to the ED by her parents in January for difficulty breathing. Her mother has noticed a gradual increase in her work of breathing over the past few days, along with poor feeding. She has a slight runny nose but no fever or cough and no vomiting or diarrhea. Her 2-year-old sibling has had a cold for the past few days. The infant was born at 37 weeks after an uncomplicated pregnancy and spontaneous vaginal delivery, and discharged at 24 hours of life. Her pediatrician noted a heart murmur at her 2-month visit and referred her to a cardiologist for further evaluation, but the appointment is not until the next week. Upon further questioning, the mother says that she has been a difficult feeder, but that she seems to be getting worse, with shorter feeds and falling asleep at the breast, and she seems sweaty during feeds. She also noted that the infant is not gaining weight. At triage, her vital signs are: temperature, 37.6°C; heart rate, 180 beats/min; respiratory rate, 60 breaths/min; and oxygen saturation, 90% on room air. She is noted to have moderate respiratory distress. On examination, you note labored breathing with scattered rales, rhonchi, and mild wheezing, making it difficult to appreciate the heart sounds. You consider bronchiolitis, but decide to obtain a chest x-ray, given her history. The x-ray revealed a large heart, patchy perihilar opacities, and some fluid in the fissures. You suspect congestive heart failure and wonder if additional tests may be helpful and what medical therapies are indicated.

Introduction

Congenital heart disease (CHD) includes a spectrum of anatomic malformations of the heart and great vessels that occur during embryologic development of the fetus and can cause a wide range of physiologic perturbations and physical signs and symptoms. While many defects are identified prenatally through fetal ultrasound (including approximately 33% of all CHD and 57%-83% of critical lesions)1 or diagnosed in the newborn period prior to discharge from the hospital, some CHD may go unrecognized and present without previous diagnosis to the emergency department (ED). The emergency clinician must maintain a high index of suspicion in these rare cases, as the clinical picture of undiagnosed CHD can be nonspecific, can mimic other common and benign childhood disease, or can present with a child in extremis. Infants and children with partially or fully corrected or palliated CHD may also present to the ED with complications related to the structural heart disease, the surgical repair, or as a result of concurrent illness in the setting of limited physiologic reserves. This review focuses primarily on the presentation, evaluation, and stabilization of undiagnosed CHD presenting to the ED, but will also touch on common emergencies in the patient with known heart defects.

Epidemiology

CHD is the most common major congenital anomaly, comprising one-third of all congenital malformations, and is the most common cause of mortality from birth defects in infants.1,2 Differing definitions of CHD and methodologies make the exact determination of birth prevalence difficult; however, a 2011 systematic review and meta-analysis of 114 articles representing more than 24,000,000 births estimates a worldwide birth prevalence of 9.1/1000.2 There is significant geographic variability, with the highest rates of CHD seen in Asia (9.3/1000), followed by Europe (8.2/1000), North America (6.9/1000), and the lowest rate noted in Africa (1.9/1000).2 The birth prevalence of CHD appears to have increased worldwide over the past century, and leveled off since the late 1990s. Possible explanations for the increased prevalence include improvements in diagnosis (eg, fetal ultrasound and echocardiography), improved prenatal care with increased survival of preterm infants, or changing social and environmental determinants of disease (eg, delayed age of maternity, medication, or toxic exposures).

The spectrum of anatomic defects associated with CHD is broad, but 8 discrete lesions comprise more than three-quarters of all defects.2 (See Table 1.) Complex and critical CHD, such as hypoplastic left heart syndrome (HLHS), total anomalous pulmonary venous return (TAPVR), and anomalous left coronary artery from the pulmonary artery (ALCAPA), are less common but important forms of CHD.

Table 1. Absolute And Relative Frequency Of The Most Common Cardiac Defects

Presentations Of Congenital Heart Disease

While reviews and textbooks often categorize CHD based on the anatomy or physiology of structural lesions, it is more useful to the emergency clinician to consider the clinical presentations of CHD. Undiagnosed CHD can present in several ways, depending on the pathophysiology of the lesion(s), although individual variations may lead to overlapping features. Cardiovascular collapse/shock is typically seen in CHD and is characterized predominantly by left outflow tract obstruction. Cyanosis may be the presenting feature in lesions with limited pulmonary blood flow or right-to-left shunting of deoxygenated blood, or both. Respiratory distress from congestive heart failure (CHF) typically results from left-to-right shunting of blood, resulting in pulmonary overcirculation. Table 2 summarizes the 3 main clinical presentations of CHD, including the symptoms, signs, and potential anatomic lesions associated with each.

Table 2. Clinical Presentations Of Congenital Heart Disease In Children

Another potentially useful way to identify CHD is by the age of presentation. Lesions that depend on the ductus arteriosus for pulmonary or systemic circulation typically present with cyanosis or shock in the first week or weeks of life as the ductus closes. Lesions that result in pulmonary overcirculation leading to CHF more often develop gradually in the second or third month of life as falling pulmonary vascular resistance increases left-to-right shunting and results in pulmonary edema. Figure 1 depicts the typical age of presentation for various types of CHD.

Two rare but important forms of CHD, ALCAPA and TAPVR, are particularly difficult to diagnose, as their presentations may vary considerably. ALCAPA can present early in the neonatal period with a shock-like state, as a result of myocardial infarction or it may present more insidiously, with recurrent periods of fussiness and gradual respiratory distress from CHF as a result of cardiac dysfunction from recurrent ischemia. Similarly, TAPVR may present in the neonate as cyanosis in cases with venous obstruction or later in infancy with CHF in cases without venous obstruction.

Critical Appraisal Of The Literature

A literature search was performed in PubMed using combinations of the search terms congenital heart disease or congenital heart defects and emergency department, epidemiology, etiology, embryology, genetics, congestive heart failure, shock, cardiogenic shock, cyanosis, prostaglandin, PGE1, and vasopressors. Only articles published in English whose subjects included children aged birth to 18 years were reviewed. Within CHD, only 25 clinical trials were available, none were conducted in the ED, and only 1 (on the use of prostaglandin E1 [PGE1]) was relevant to the acute management of infants and children with CHD. There was 1 practice guideline and evidence-based review of the management of pediatric heart failure that was not specific to CHD. The lack of high-quality evidence relevant to the ED management of infants and children with CHD is not surprising, given the rarity of ED presentation, the frequently critical nature of acute illness in these children, and the general difficulties related to clinical trials in the pediatric population. In the absence of evidence from clinical trials, the literature review was broadened to include review articles, systematic reviews, and case series related to pediatric CHD in the ED as well as the results of literature searches for specific therapies for pediatric cardiogenic shock, pediatric CHF, and complications of CHD and its surgical palliation and repair. In total, more than 70 peer-reviewed articles comprise the literature that informed this review.

Risk Management Pitfalls In Pediatric Congenital Heart Disease

  1. “This neonate had normal prenatal care, including a prenatal ultrasound, so CHD has been ruled out. There must be another cause for his shock.”
    While prenatal ultrasound has advanced significantly over recent decades, only about one-third of all CHD and 57% to 85% of critical CHD are detected before birth. Normal prenatal care and screening ultrasound do not exclude the possibility of significant CHD.

  2. “I don’t hear a murmur or a gallop, so this isn’t CHD.”
    The absence of abnormal heart sounds does not preclude underlying structural disease. A murmur requires turbulent blood flow across a defect, usually from a significant pressure gradient. In the first days of life, high pulmonary vascular resistance can minimize left-to-right shunting across a large ASD or VSD, and a murmur may not be detected prior to discharge from the nursery.

  3. “Although this 1-week-old is in shock, we can’t get an echocardiogram, and I’m not sure what is going on, so I don’t want to start PGE1 until we have more information. I’ll just fluid resuscitate….”
    In the critically ill neonate presenting with shock, PGE1 can be life-saving and should be empirically initiated if there is no response to an initial 10-mL/kg bolus of intravenous fluids. Careful monitoring of clinical response is all that is needed and the infusion can be stopped if the clinical condition worsens.

  4. “All children with significant hypoxia require 100% FiO2 to normalize oxygenation.”
    While oxygen can be beneficial and is first-line therapy for many conditions associated with hypoxia or poor perfusion, its potent pulmonary vasodilatory effects must be considered in the context of CHD with significant shunting lesions, as decreased pulmonary vascular resistance can lead to worsening pulmonary edema or decreased systemic perfusion as a result of exacerbation of left-to-right shunting. Baseline oxygen saturations should be targeted in patients with complex CHD, if the baseline is known, and oxygen saturation of 90% to 95% should be targeted if the baseline is unknown. Wean oxygen if clinical deterioration is observed after initiation of therapy.

  5. “Although he is breathing on his own, this child with a Fontan procedure and gastroen-teritis is hypoxic and tachypneic, so I should intubate. I don’t expect a difficult airway.”
    While intubation may be required for infants and children with apnea or agonal respirations, the switch to positive-pressure ventilation and the vascular and cardiac effects of preintubation medications must be carefully considered in patients with complex CHD who may be dependent on preload. In addition, airway anomalies may be associated with some CHD. Consultation with anesthesia or cardiology is recommended in all but the most emergent cases in which intubation is considered.

  6. “This is the fourth bad case of bronchiolitis I’ve had this shift! She’s getting worse despite intravenous fluids, so I’ll just admit her and try nebulized epinephrine.”
    CHD presenting with CHF can mimic common viral illness such as bronchiolitis, and, during epidemics, it is easy to overlook heart disease as a cause of respiratory distress in an infant. Worsening of clinical condition with usual treatment (such as intravenous fluids for presumed dehydration in bronchiolitis) should alert you to the possibility of CHF, for which diuretics are first-line therapy. A BNP and chest x-ray may help in these circumstances.

  7. “I’m going to start PGE1 on this neonate with suspected CoA and transfer him to a children’s hospital. I'd better intubate prior to transport in case he develops apnea, even though he is breathing well on his own now.”
    Although often recommended in textbooks, prophylactic intubation is likely not necessary in the absence of observed apnea or agonal respirations prior to transport. One study found a higher rate of adverse events among neonates on PGE1 who were prophylactically intubated compared to those who were not intubated for transport.44

  8. “The nurse got an ECG at triage for this 6-year-old with chest pain and it looks like ischemia! That’s impossible in a child with no past medical history, so it must be a technical error.”
    Although rare, children can develop myocardial ischemia or infarct from ALCAPA. Though ALCAPA typically presents in early infancy, it can escape detection and present later in life with acute myocardial infarction or progressive CHF from recurrent ischemia.

  9. “I need to refer this 2-year-old with TOF to a pediatric dentist for outpatient extraction of multiple carious teeth. Her last surgery was > 6 months ago, so I don’t think she needs antibiotic prophylaxis prior to oral surgery.”
    The 2010 American Heart Association guidelines on antibiotic prophylaxis for bacterial endocarditis eliminated many of the indications for prophylaxis, but children with cyanotic CHD and allografts are at higher risk for SBE and should receive preprocedural antibiotic prophylaxis, with a single dose of oral or parenteral antibiotics 30 to 60 minutes prior to the procedure.

  10. “This 5-month-old has a systolic ejection murmur and a slight diastolic rumble, but I think it is an innocent murmur. She doesn't require cardiology referral.”
    A systolic ejection murmur can be benign and a common finding in many children, but a diastolic murmur is usually pathologic and should be referred to a cardiologist for further evaluation.

Tables and Figures

Table 1. Absolute And Relative Frequency Of The Most Common Cardiac Defects

Table 2. Clinical Presentations Of Congenital Heart Disease In Children

References

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)
Publication Information
Authors

Pavan Judge, MD; Garth Meckler, MD, MSHS

Publication Date

May 2, 2016

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