1. Pediatric Cardiac Emergencies Gavin Greenfield Peggy Thomsen

2. ► 4 year old female presents with fast breathing, “grunting”, cough x 5 days ► seen 2 days earlier and started on steroids and bronchodilators ► initial vitals: HR 150, BP 100/85, RR 36, T 37.5

3. ► 1 month old with irritability, poor feeding (fatigues), failure to thrive, fast breathing ► no fever or runny nose ► physical exam: HR 160, RR 60 with minimal respiratory distress, gallop rhythm, rales

4. Infant Cardiac Disease Leading to ER Presentation ► Congenital ► Acquired  Cardiomyopathy  Myocarditis (usually with CHF)  Dysrhythmias

5. Congestive Heart Failure ► the physiologic state in which cardiac output is unable to meet tissue metabolic demands (Rosen) ► CO = HR x SV ► SV dependent upon preload, afterload, contractility

6. CHF - Presentation ► infants: irritable, poor feeding (early fatigue), failure to thrive, respiratory symptoms ► always consider in patients with respiratory symptoms  often misdiagnosed as respiratory illness / infection

7. CHF - Etiology ► Increased Preload  L to R shunts (VSD, PDA, AV fistula)  severe anemia ► Increased Afterload  HTN  Congenital (aortic stenosis, coarctation of aorta) ► Decreased Contractility  myocarditis, pericarditis with tamponade  cardiomyopathy (dilated or hypertrophic)  Kawasaki syndrome (early phase)  metabolic: electrolyte, hypothyroid  myocardial contusion  toxins: dig, calcium channel blockers, beta blockers ► Dysrhythmia

8. CHF - Etiology ► presents immediately at birth  anemia, acidosis, hypoxia, hypoglycemia, hypocalcemia, sepsis ► presents at 1 day (congenital)  PDA in premature infants ► presents in first month (congenital)  HPLV, aortic stenosis, coarctation, VSD presents later ► presents later (acquired)  myocarditis, cardiomyopathy (dilated or hypertrophic), SVT, severe anemia, rheumatic fever

9. Myocarditis ► leading cause of dilated cardiomyopathy and one of the most common causes of CHF in children ► etiology: idiopathic, viral, bacterial, parasitic ► hallmark is CHF ► failure to respond to bronchodilators in wheezing child ► treatment includes inotropes, afterload reduction, diuretics, antibiotics, antivirals

10. Pericarditis ► sharp stabbing precordial pain ► worse with supine and better leaning forward ► no sensory innervation of the pericardium  pain referred from diaphragmatic and pleural irritation

11. Etiology ► infectious  viral  bacterial  TB  fungal  parasitic ► Connective tissue  RA  Rheumatic fever  SLE ► Metabolic / Endocrine  uremia  hypothyroid ► Hematology / Oncology  bleeding diathesis  malignancy ► Trauma ► Iatrogenic

12. Pericarditis ► usually a benign course ► virulent bacteria (H. flu, E. coli) can cause constrictive pericarditis and subsequent tamponade – may need urgent pericardiocentesis ► uncomplicated pericarditis usually responds to rest and anti-inflammatories

13. Chest Pain ► 4% of children will have a cardiac origin ► remainder: MSK, pulmonic (asthma, bronchitis, pneumonia), GI ► Cardiac causes: myocarditis, pericarditis, structural abnormalities such as congenital heart disease or hypertrophic cardiomyopathy

14. ► 14 year old male collapses at school while in class ► non-responsive for one minute ► feels fine in the department ► Approach?

16. Syncope ► 20-50% of adolescents experience at least one episode of syncope  most cases benign ► Pathophysiology  vascular  orthostatic, hypovolemia  neurally mediated  hypoxia: PE, CNS depression from OD, CO  cardiac

17. Cardiac Syncope ► Dysrhythmias  tachy  brady ► Outflow obstruction ► Myocardial Dysfunction ► cardiac syncope often precedes future sudden cardiac death

18. Sudden Cardiac Death ► includes those causes that directly relate to cardiovascular dysfunction ► one third of all sudden deaths

19. Sudden Cardiac Death ► Etiology  myocarditis  cardiomyopathy (hypertrophic)  cyanotic and noncyanotic congenital heart disease  valvular heart disease  congenital complete heart block  WPW  long QT syndrome  Marfan syndrome  coronary artery disease  anomalous coronary arteries

20. Risk Factors for Serious Cause of Syncope ► history of cardiac disease in patient ► FH of sudden death, cardiac disease, or deafness ► recurrent episodes ► recumbent episode ► exertional ► prolonged loss of consciousness ► associated chest pain or palpitations ► medications that can alter cardiac conduction

21. What to look for in the Department: EKG ► Long QT syndrome  congenital or acquired  get paroxysmal v tach with torsades de pointes  congenital long QT associated with hypertrophic cardiomyopathy  long QT defined as corrected QT longer than 0.44 s  T wave alternans sometimes present  can have normal ECG in the department  two clinical syndromes not associated with structural heart disease: Romano-Ward and Jervell-Lange-Nielsen

22. Other dysrhythmias ► WPW and other SVT’s ► AV block  usually acquired, rarely congenital ► Sick sinus syndrome

23. Idiopathic Hypertrophic Cardiomyopathy ► aka IHSS ► both a fixed and dynamic subvalvular obstruction ► characterized by ventricular hypertrophy with principle involvement of the ventricular septum ► associated with long QT ► autosomal dominant ► often presents with exertional syncope ► 10 year mortality is 50% for children diagnosed by age 14

24. Other structural cardiac diseases ► dilated cardiomyopathy  usually secondary to myocarditis  syncope and death secondary to ventricular dysrhythmias or severe myocardial dysfunction ► arrhythmogenic RV dysplasia ► congenital cyanotic and non-cyanotic heart disease ► valvular diseases  aortic stenosis ► coronary artery anomalies  exertional syncope or sudden death  aberrant artery passes between aorta and pulmonary artery

25. ► 2 week old infant brought in by parents with difficulty breathing ► HR 180, BP 50/P, RR 80, T 37.5 ► history and physical ► investigations ► repeat vitals: HR 30, no BP, RR 12 ► “definitive treatment”:

26. ► 4 year old male presents with 2 weeks history of cough, fast breathing, fatigue, decreased exercise tolerance, “puffy eyes” ► On exam: tachypneic, moderate respiratory distress, O2 sats 92%, bilateral crackles

27. ► 6 month male presents with failure to thrive, fast breathing, blue lips ► On exam tachypnea but no respiratory distress, lips and extremities blue, oxygen saturations 70%

28. Congenital Heart Disease ► Fetal to Neonatal Circulation

30. Classification

31. Congenital Heart Disease Classification ► pink (in failure) ► blue (no distress) ► gray (in shock)

32. Anatomic Classification; 4 groups ► Right to Left Shunt  Tetralogy of Fallot  Transposition of the Great Arteries  Tricuspid Atresia ► Left to Right Shunt  ASD  VSD  PDA ► Stenotic  Aortic valve stenosis  Pulmonic valve stenosis  Aortic coarctation ► Mixing  Truncus  Total Anomalous Pulmonary Venous Return  Hypoplastic left heart syndrome

33. CHD Classified as Cyanotic vs. Acyanotic ► Cyanotic (R to L shunt and mixing lesions)  tetralogy of Fallot  transposition of great vessels  tricuspid atresia  total anomalous pulmonary venous return  truncus arteriosus  hypoplastic left heart syndrome

34. CHD Classified as Cyanotic vs. Acyanotic ► Acyanotic (L to R shunts, stenotic lesions)  ASD  VSD  PDA  aortic valve stenosis  pulmonic valve stenosis  aortic coarctation

35. Cyanosis ► Classified as central or peripheral ► Central cyanosis (always abnormal)  mucous membranes, trunk, extremities  classified as cardiac (R to L shunt) or pulmonary ► Peripheral cyanosis (acrocyanosis)  no involvement of mucous membranes  involves hands, feet, circumoral area  common in neonates from vasomotor instability  CHF, PVD, shock, cold extremities

36. Congenital Heart Disease ► History  feeding difficulties  tachypnea  diaphoresis  syncope  cyanotic episodes  failure to thrive

37. Congenital Heart Disease ► Physical Examination  colour: pink, blue, gray  vitals: tachypnea, tachycardia, BP  symptoms suggestive of infection  palpation and auscultation of precordium  chest auscultation  survey for organomegaly  pulses in all extremities

38. Cyanotic Congenital Heart Disease ► R to L shunts ► mixing lesions

39. Tetralogy of Fallot – the classic cyanotic lesion ► RV outflow obstruction ► RVH ► VSD ► overriding aorta ► CXR reveals boot shaped heart with decreased pulmonary blood flow

41. ► 2 month old female with known tetralogy of Fallot brought in with 24 hour history of vomiting and diarrhea ► On exam: moderate dehydration ► during IV attempts patient becomes irritable and cyanotic

43. Treatment of Tet Spell ► quiet, calm environment ► knee-chest or squatting position  increases afterload thus decreasing R to L shunting ► Oxygen ► Morphine  to treat hyperpnea and decrease systemic catecholamines ► Phenylephrine  increases afterload thereby decreasing R to L shunt ► Manual external aortic compression below level of renal arteries ► Propranolol  to block beta receptors in infundibulum therefore lessening RV outflow obstruction

44. Consider ► consider small volume challenge (5-10 cc/kg) to increase preload and reduce dynamic outflow obstruction ► ?NaHCO3 for correction of acidosis ► may need general anesthesia if severe and/or prolonged spell ► interim prophylactic treatment with propranolol while awaiting surgery

45. Acyanotic Congenital Heart Disease ► L to R shunts ► stenotic lesions

46. VSD Most common congenital lesion Large VSD’s may be silent and become symptomatic in first few weeks as pulmonary resistance  SOB and diaphoresis w feeds Poor weight gain Systolic murmur CXR demonstrates CHF

47. ► 2 week old infant brought in by parents with difficulty breathing ► HR 180, BP 50/P, RR 80, T 37.5 ► history and physical ► investigations ► repeat vitals: HR 30, no BP, RR 12 ► “definitive treatment”:

48. Differential Dx of Infant Shock ► infection (septic shock/ meningitis)  bacterial: GBS, E. coli, S. aureus  virus: enteroviruses, H. simplex ► metabolic: amino/organic acidopathies, urea cycle defect ► ‘hypoxic shock’: eg. RSV, C.N.S. depression ► heart disease: congenital or acquired

49. LV Outflow Obstruction

50. ► Aortic coarctation ► Hypoplastic left heart syndrome ► Aortic stenosis (presents later) ► Rosen: “any neonate in shock that does not respond to fluids or pressors has LV outflow obstruction until proven otherwise” ► complete obstruction incompatible with life unless there is shunting

51. Coarctation of the aorta Most often distal to L subclavian Can be diagnosed anytime Neonates present as acutely ill, gray shocky (from DA closure) Systolic murmur at the back Hepatomegaly Diminished femoral pulses BP difference b/t arms and legs CXR demonstrates CHF Treatment of CHF Prostaglandin E1

52. Duct Dependant Lesions ► Duct needed to perfuse lungs or periphery ► Lungs  Tetralogy of Fallot, transposition of great arteries, tricuspid or pulmonary atresia ► a patent ductus arteriosus results in preserved pulmonary blood flow ► Periphery  Aortic coarctation (severe) and Hypoplastic left heart

53. Treatment of acute decline in patients with ductal dependant lesions ► Open the closed duct ► Prostaglandin E1 0.1 ug/kg/min infusion ► reduce dosage as perfusion and colour return ► Rosen: “any infant in the first week of life with decreased perfusion, hypotension, or acidosis should be considered a candidate for PGE1 administration”

54. What do you need to know about PGE ? ► it functions by dilating vascular smooth muscle, both systemically and in the pulmonary vascular bed ► it’s use in CHD pts’ is to maintain patency of the PDA, whether to maintain PBF or to maintain systemic blood flow past a

55. Classification Review ► pink child in respiratory distress suggests acyanotic chd (L to R shunt, coarct, aortic stenosis) ► blue cyanotic child in little respiratory distress suggests R to L shunt or mixing lesions ► gray, shocky baby suggests outflow tract obstruction

56. Bradyarrhythmias ► Etiology  hypoxia, acidosis, hypoglycemia  excess vagal stimulation (ex. intubation) ► Treatment  Epinephrine  Atropine if known vagally mediated or heart block

57. Congenital Bradyarrhythmias ► complete AV block  autoimmune injury to fetal conduction system secondary to maternal autoimmune disease  atropine, isoproteronol, epinephrine may be tried temporarily prior to pacing

58. Tachyarrhythmias ► Supraventricular Tachycardia  re-entrant with accessory pathway (AV nodal or WPW)  re-entrant without accessory pathway (re-entry occurs within sinus node or within atrium)  ectopic ► nonspecific presentations in infants

59. Murmurs ► Areas  aortic: R 2 nd intercostal space  pulmonic: L 2 nd intercostal space  mitral: apex  tricuspid and VSD: L lower sternal border ► Pathologic  diastolic, holosystolic, late systolic, continuous

60. Innocent Heart Murmurs ► History  normal growth and development, normal exercise tolerance  no history of cyanosis ► Physical Examination  Grade II or less, localized  varies with position (decreased with upright posture)  normal precordium  normal pulses ► Lab  normal EKG, normal CXR

61. 3 innocent murmurs ► Still’s  short ejection systolic murmur  musical or vibratory quality  heard best between apex and left sternal border ► physiologic pulmonary flow murmur  harsh, located at pulmonic area ► peripheral arterial stenosis  low-intensity systolic ejection murmur best heard in axilla and back

62. ► 8 year old male presents with fever, arthralgias ► mother mentions that he had a sore throat 3 weeks ago for a few days with spontaneous resolution ► a throat swab was done and positive for GAS but patient better so did not take the prescribed antibiotics

63. Acute Rheumatic Fever ► school aged children ► associated with certain strains of Group A beta- hemolytic streptococcal infections ► the streptococcal organism stimulated antibody production to host tissues  CT of heart, joints, CNS, subcutaneous tissues, skin ► carditis is an endomyocarditis with valvulitis involving mitral and aortic valves ► 2 to 6 weeks post streptococcal pharyngitis

64. Jones Criteria ► Major  carditis ► new or changing murmur ► cardiomegaly, CHF ► pericarditis  migratory polyarthritis  chorea  erythema marginatum  subcutaneous nodules ► Minor  fever  arthralgia  history of previous ARF  elevated ESR, CRP  prolonged PR on EKG  Rising titer of antistreptococcal antibodies

65. Erythema Marginatum

66. ER Treatment ► management of complicating features of carditis (CHF)  significant carditis or CHF managed with glucocorticoids ► high-dose ASA 75-100 mg/kg/day ► pencillin ► long term management of rheumatic heart disease

67. Pediatric EKG’s General Principles ► RV Dominance at birth; gradually changes to LV dominance ► axis up to +180 in normal newborn ► T waves negative in right precordial leads until adolescence (except they are upright in first week of life)