1. PEDIATRIC CARDIOLOGY in the ED Naminder Sandhu & Roger Galbraith October 27, 2011

2. OBJECTIVES Approach to the infant/neonate presenting with Shock CHF Cyanosis Approach to children presenting with Syncope Palpitations Chest pain Approach to the child with known CHD Tips for looking at CXRs and ECGs

3. Pediatric ECGs Why do we do them? – Chest pain, suspected dysrhythmia, seizure, syncope, drug exposure, electrical burns, abN lytes

4. RV dominance Normal Values by Age  

5. What are normal T waves? Upright up to first 3 days – If persists  sign of RVH Flat or inverted until pre-adolescence/adolescence

6. Back to basics: fetal circulation

7. And now for some cases... Group 1: Alyssa Jen Erik Geoff Group 2: Kristen Joe Kip Kasia Sean Group 3: Chris Jason Puja Jasmin Group 4: Ping Ashlea Michael Adam Group 5: Russ Meira Marshall Jeff

8. CASE 1 3 week old with dyspnea, poor feeding Brought to ED by mother for rapid breathing and poor breast feeding, worsening over past few days Previously well with unremarkable prenatal history Becoming more lethargic (not interested in feeding)

9. CASE 1 Afebrile, HR 180, RR 80, BP 85/50, sats 88% What is your broad ddx for neonatal shock?

10. Ddx cardiogenic shock Myocardial dysfunction – Myocarditis/pericarditis – Sepsis Arrhythmias – SVT Obstructed/impaired forward flow – AS, Ao Coarct, HLH Umm… why is the infant presenting now??

12. When does the duct close?? Functional closure within several hours; anatomic closure up to 2 – 3 weeks Term infants: – 50% by 24hrs – 90% by 48hrs by 4 days in nearly all healthy infants prems

13. What are ductal dependent lesions? Left sided obstructive lesions = shocky – AS, Ao Coarct, Hypoplastic left heart Right sided obstructive lesions = cyanotic – Tetralogy of Fallot, pulm stenosis, TGA

14. Crashing Neonate Think ductal dependent lesion! Most common lesion to present this way = Aortic Coarct

15. CASE ECG

16. Management of neonatal shock ABCs – Feel for femorals and check BP in four limbs!!! Oxygen IV access* and fluids, investigations and ?abx PGE 1 infusion Call cardiology/arrange transport Also consider Sepsis, Metabolic, NAT, etc.

17. Alprostadil Indications: – Maintenance PDA – Severe PPHTN Infusion via peripheral or central line Dilute to concentration of 10mcg/mL (in dextrose or NS) Infuse starting at 0.05mcg/kg/min – max 0.2mcg/kg/min Effect within minutes

18. Side effects? – Apnea – Fever – Flushing – Hypotension – Seizures

19. CASE 2 3 month old girl with cyanosis this AM She has had this happen before but often will resolve on own Today after feeding, seemed to be straining, noticed lips were blue Newly immigrated from Malaysia, no previous medical problems Exam: Sats 76%, RR 40, HR 166 – Irritable ++ – Deep resps with indrawing – Difficult resp/cardiac exam

20. Oxygen to the rescue!....

21. Cyanotic CHD Cyanotic heart lesions…. Do I really need to know them??? ToF10% CHD TGA5% Tricuspid atresia1-2% TAPVR1% Truncus arteriosus<1% Pulm atresia/stenosis<1% Ebstein’s anomaly<1%

22. Age of CHD presentation by lesion First Week TGA TAPVR HLH Tricuspid atresia 1-6 months (often lead to CHF) VSD PDA Coronary anomalies TOF AV canal defect First Month (ductal dependent lesions) Coarctation Severe AS Severe PS TOF with pulmonary atresia Later ASD PAPVR Valve stenosis

23. Is CXR useful? Pulmonary vascularity Shape/size of heart

24. Increased Vascularity ← Decreased Vascularity →

25. Cardiac silhouette Egg on a string = TGA Snowman = TAPVR Boot shaped = ToF

26. So what’s going on with this girl? TET SPELL

27. Tetralogy of Fallot Most common cyanotic CHD beyond infancy Sx: – Dyspnea on exertion – Cyanosis – Clubbing – Hypoxic spells – squatting

28. CASE ECG

29. Tet spell Episode of severe hypoxia due to  R to L shunting and  pulmonary blood flow Characteristics: – Paroxysm of hyperpnea – Irritability and prolonged crying – Increasing cyanosis – Decreased intensity murmur

30. Increased R → L shunting and decreased pulmonary blood flow b/c of: – Hyperpnea  increase in venous return to R heart – decrease in SVR (lower L heart pressures) – increase in PVR (higher R heart pressures) – increase in RV outflow tract obstruction  R-L shunt  pO2  pCO2  pH Hyperpnea  Systemic venous return VICIOUS CYCLE

31. Seizures Syncope Stroke DEATH

32. Management Try to calm infant- avoid pokes if you can Knee-chest position Give oxygen  R-L shunt  pO2  pCO2  pH Hyperpnea  Systemic venous return

33. Then what?? IV access & fluids Sedate with morphine Bicarbonate Phenylephrine Propranolol 0.2mg/kg over 5 min ICU/cardiology

34. CASE 3 2 month old presenting in December with cough, congestion, dyspnea, poor feeding x 4 days Hx – Difficulty feeding, decreased u/o, lethargic – Sick contacts: older brother with cold P/E – Afebrile, HR 160, RR70, BP 80/50, sats 90% – Nurses describes infant with nasal congestion, moderate indrawing Sounds like bronchiolitis….but how do you know it’s not CHF??

35. Presentation: what’s the diff? Poor feeding, failure to thrive, tachypnea, diaphoresis, wheeze Two bigs: Big heart, big liver Two fasts: Fast RR, Fast HR

36. Ddx CHF Cardiac – Structural 1)Overcirculation (L-R shunts): VSD, large PDA, AV canal defect 2)Forward flow obstruction: Ao stenosis, CoA, HLH 3)Myocardial dysfunction: anomalous coronary (Eg ALCAPA), myocarditis, cardiomyopathies – Electrical Extracardiac: AVMs

37. Causes of CHF by age < 2 weeks> 2 weeksChildAdolescent Shunts Obstructive lesions Coronary artery anomalies Cardiomyopathies Myocarditis Arrhythmias AVMs

38. L – R shunts VSD = 25% of all CHD Gradually increasing L → R shunt with drop in PVR – also present when tipped into failure by illness (e.g. bronchiolitis)

39. CASE ECG

40. CXR

41. So what’s the plan? Oxygen IV access Elevate head of bed Admit Supportive therapy for bronchiolitis (that’s a whole other talk….) Diuretics

42. CASE 4 4 year old complaining that her heart feels funny – Chest pain? Palpitations? Started this morning and ?comes and goes; URTI symptoms x 2 days Feels “dizzy” sometimes, no syncope PmHx: known to have an ASD, followed every 1 – 2 years by Cardiology On exam: HR 128, BP 80/50, RR 30, Sats 95%, afebrile – Nurse says no acute distress

43. CASE 4 Child has nasal congestion Normal heart sounds, no rubs, no extra sounds, 2/6 SEM at LUSB Clear lung fields You get an ECG… what worrisome findings are you looking for?

44. CASE ECG

45. What’s going on? Cardiac – Arrhythmia – Myocarditis/pericarditis – Ischemia, HOCM, etc etc etc Non-cardiac Then again kid says he feels funny…. You get another ECG

46. CASE ECG

47. Paediatric arrhythmias in the emergency department (Clausen et al 2011)

48. SVT In infants: – 50% = idiopathic – 25% congenital heart disease – 10-20% WPW syndrome HR >220 infants, >180 children Complications: – Syncope – Shock – CHF

49. How do you treat stable SVT? Stable – Vagal maneuvers: What can you try? Blow through plugged straw Ice on face (don’t cover mouth) – no more than 15 – 30 sec! Bear down, try to puff up stomach Stand upside down Orbital pressure = NO! – Adenosine Unstable – SVT with poor perfusion… PALS

50. Approach syncope…. Similar to adults Benign – Orthostatic, vasovagal, breath-holding Pathologic – Neuro: Seizures – Cardiac (<5%) Myocardial: HOCM, myocarditis Aortic disease: Aortic stenosis, aneurysm, dissection Conduction: LQTS, heart block, WPW, ARVD Coronary artery disease: anomalous origin, aneurysms Other: pulm HTN, electrolyte abN

51. SCD in athletes <35 yo HCM – 36% Anomalous origin of a coronary artery – 17% Myocarditis – 6% Arrhythmogenic right ventricular dysplasia – 4% MVP – 4% Aortic stenosis – 3% Coronary atherosclerosis – 3% Other – 12%

52. Cardiac warning signs History of known heart disease Sudden fainting without prodrome Syncope during exercise, emotional stimulus Incontinence during syncope Family history of sudden death (including single person MVC, drowning) Family/personal history of deafness

53. Long QTS What is prolonged QT interval in kids? – Up to 490ms in neonate  440 ms adolescents Find a LQT? – Extended electrolytes, drug history – Refer to cardiology urgently – B blockers – ICDs

54. A shout out to acquired cardiac disease Myocarditis Pericarditis Endocarditis Kawasaki disease Rheumatic heart disease

55. Myocarditis… same as in adults? Spectrum of presentation – Beware non-cardiac presentations eg. Pneumonia, wheeze not responsive to bronchodilators Most common cause dilated CM in kids Usually infectious, presents in the winter following viral prodrome

56. CASE 5 A 4 month old infant presents with increasing cyanosis Had low grade fever, cough, rhinorrhea and poor feeding for preceding 4 days Today developed increased WOB and progressively worsening cyanosis Mom says has a history of tricuspid atresia and a “BT shunt” T 38.3, HR 170, RR 70, BP 80/50, sats 75%, infant is crying, cyanotic, grunting and has retractions Other than change your pants, what should you do??? What could be going on?

57. 50% of patients with CHD that presented to the ED required admission 10% of those admitted died Patients often have complex physiology and are at risk of decompensation for a number of reasons. So, yes, you should sweat... A little

58. Most common presentations of CHD patients to the ED are for: – Respiratory tract infection – Dehydration – CHF – Arrhythmia – Tet spell – Endocarditis

59. Huh? A what shunt now?? Used primarily as palliation in defects with single ventricle pathology (ie. HLH, tricuspid atresia) – Try to bypass part of heart to offset workload on single overworked/impaired ventricle (usu RV)

60. Systemic arterial  PA shunt: Blalock Taussig Why? Increase pulmonary blood flow Subcl art  Pulm art

61. Systemic venous  PA shunt: Glenn Why? Increase pulmonary blood flow SVC  Pulm art *Dependent on passive flow to the lungs* Beware intubation/ventilation

62. Fontan procedure Why? Divert blood from RA to PA (bypass single functioning RV) IVC  Pulm art *Dependent on passive flow to the lungs*

63. Risks of shunts Clotting/ obstruction Reduced flow (from hypovolemia/dehydration) Coronary steal Over circulation in the pulmonary system – Beware too much oxygen

64. Bottom line If not sure, ask the parents what normal sats should be You probably wont have enough time or information to figure out exactly what’s causing their problem- an extensive inpatient work-up is often required Low threshold for admission, possibly ICU Low threshold for cardiology consult Don’t be stingy with fluids and oxygen, they often need one or both, just monitor their response closely

65. Take home points Shock – Do a good cardiac exam to r/o cardiogenic shock – If concerned about duct dependent lesion, think PGE1! Cyanosis – Try oxygen first… of course – You don’t need to figure out exact CHD – that’s what echo is for – If concerned about duct dependent lesion, think PGE1! CHF: – Symptoms may be subtle – exam is important JVPs don’t exist in our world – check the liver instead – Always consider on ddx for common presentations eg dyspnea – Different causes based on age (eg. L – R shunts)

66. Take home points Palpitations/chest pain/ syncope – Approach similar to adults except chest pain/syncope rarely cardiac Acquired heart disease – Most common is myocarditis – keep your eye out for it – Prevent it by looking our for Kawasaki disease Known CHD – If all else fails, ask the family and consult cardiology; low threshold for admission – Be kind with your fluids and careful with oxygen

67. Thanks! Dr. Roger Galbraith Dr. Joyce Harder Drs. Mark Bromley and Jay Green

68. References Emergency Medicine Reports, Jan 2005: 10(1) Park MK. The Pediatric Cardiology Handbook 3 rd Ed. (2003). Philadelphia: Mosby. Sharieff GQ et al. The Pediatric ECG. Emerg Med Clin N Am. 2006; 24: 195-208. Durani Y et al. Myocarditis and pericarditis in children. Pediatr Clin N Am 57. 2010; 1281– 1303.