1. Gavin Burgess R5, PEM

2. General  Review common presentations  Uncommon  Paediatric ECG  Congenital heart disease  Rhythm disturbances  Long QT  HOCM  Rheumatic fever  Carditis – myo, endo, peri

3. General  Innocent murmurs  Kawasaki disease

4. Fetal circulation

5. “Normal” AgeRespiratory ratePulse rateSystolic BP O-1mo30-60120-16050-70 1-12mo20-4080-14070-100 1-5y20-3080-13080-110 6-12y20-3070-11080-120 adolescents12-2060-100110-120

6. “Normal”  Ball-park BP?  Neonate?  Older?

7. “Normal”  Gestational age should equal MAP  Systolic BP = 70 + (2 x age)

8. “Normal” ECG  Typically have shorter PR, QRS, QT  RV dominance, RAD

9. RVH  Causes  Tetralogy of Fallot  PS  Coarct  ASD  TAPVD  Large VSD with Pulm HT

10. LVH  Causes  AS  VSD  PDA  Complete AV block  Cardiomyopathy

11. Diagnosis?

12. Superior or “north west” axis  Endocardial cushion defect  2% of congenital heart disease  Down syndrome account for 70%  Fatal due to pulm HT  Banding in infancy

13. Myocardial infarction  AT III  Cardiomyopathy  Congenital heart disease  CAD (ALCAPA)  Drugs (cocaine)  Homocystinuria  Hyperlipidaemia and cholesterolaemia  Kawasaki  Leukaemia  Marfans  Haemoglobinopathies  Tumours (myxoma)  Rheumatic fever  SLE

14. Diagnosis?

17. Which lesions give cyanosis?  Tetralogy of Fallot  Tricuspid atresia  Transposition of the great arteries (IDM)  Truncus arteriosus  Total anomalous pulmonary venous drainage  Hypoplastic left heart  Ebstein’s anomaly (lithium)  Pulmonary atresia/severe stenosis

18. Pulmonary markings  Decreased:  Pulmonary atresia/stenosis  Tetralogy  Tricuspid atresia  Ebstein’s anomaly  Increased:  TGA  TAPVD  Truncus

19. What’s the hyperoxia test?  ABG  Give 100% O2  Repeat ABG after 10 min  If rises by >10%, likely pulmonary lesion

20. When does the ductus close?  10-14 days after birth, it is physiologically closed

21. Neonatal and infant presentations to ED  What are the 4 presentations in and infants neonates?  1) shock  2) cyanosis  3) cardiac failure  4) murmur

22. What are the ductal- dependent lesions?  Systemic  Coarct/interrupted arch  Aortic stenosis  HLH  Pulmonary  PS/atresia  Tricuspid atresia

23. Shock  L ventricular outflow obstruction  Coarct  AS  HLH

24. Shock  Management:  ABC’s  Start prostin  CXR  ECG

25. What’s prostin?  Prostaglandin E1  Rate 0.05-0.2 mcg/kg/min  Side effects?  Apnoea  Fever  Flushing  Hypotension  Prostin has an “all or nothing” action  Should work in 15min

26. Time to presentation of cyanotic lesions AgeECGX-ray 0-1 weekTGARVHIncreased 1 st weekTAPVDRVHIncreased 1-4weeksTricuspid AtresiaLVHDecreased Severe PSRVHDecreased 1-12weeksTOFRVHDecreased Anytime in infancy Truncus arteriosusBVHIncreased

27. Cyanosis  What is a tetralogy of Fallot?  RVH  Overriding aorta  VSD  RV outflow obstruction

28. What’s a “tet spell”?  Change in the balance of pulmonary and systemic flow  Hypoxic and cyanotic event  Decreased system vascular resistance or increased RV outflow obstruction  Increasing hypoxia

29. How do I treat it?  O2  Chest-knee (why?)  Analgesia  B-blocker (why?)

30. Cardiac failure  History:  Fussy  Sweating  FTT  Short frequent meals  Physical:  HSM  Murmur  FTT  You will NOT see a JVP  AVM – auscultate the head

31. Murmurs  Features of an innocent murmur  80% of children will have a murmur at some time in their lives  All have normal ECG and X-rays  Never diastolic

32. Common innocent murmurs TypeDescriptionAge Still’sLLSB, 2/6, “twang”3-6y Pulmonary flowULSB, blowing, transmitsGone in 3-6mo Venous humSupra clavicular, rotate head, supine goes 3-6y Carotid bruitOver carotidAny age

33. Arrhythmia  SVT  Very common  Tolerated well, occasional LOC change  Child is fussy  Newborn >220 bpm  <12y often accessory pathway

34. Arrhythmia  SVT treatment  In shock vs stable  Vagal stim  Adenosine  Amiodarone,verapamil use extreme caution. Frequently develop profound hypotension and die

35. Arrhythmia  Long QT  History  Deafness  Single person MVC  Swimming syncope  Exercise syncope  Family history of sudden death  Seizure of unknown etiology  Recurrent syncope/lightheadedness  Sibling with SIDS  Physical  Infant with bradycardia

36. Arrhythmia  All first degree family members should be screened with ECG

37. HOCM  2% under 2 y, 7% under 10y  Variable history  CP  Palpitations  SOB  Syncope  Sudden death  High risk if syncope  Sudden death with strenuous exercise

38. HOCM  Physical  S4 gallop, mid systolic murmur  Increased PVR decreases murmurs

39. Rheumatic fever  Who was Jones?  What where his criteria?  What do you need to make a diagnosis?  Which valve?  Then?

40. Rheumatic fever  What about Sydenham’s chorea?  And the rash?

41. Rheumatic fever  Treatment  ASA 75-100mg/kg  Prednisone 1-2mg/kg  Benzathine (Pen G) 600 000U (27kg), 1.2 million U (27kg)  Prophylaxis  Age questioned

42. Myocarditis  Various causes, most notably viral  Coxsackie A,B, ECHO, flu’  Non-specific viral prodrome  Non-specifc fussiness, lethargy etc  Heart failure  IVIG may be indicated

43. Infective endocarditis  Rheumatic fever, congenital heart defects, catheters, IVD  S. aureus, viridans are the usual suspects  Fungi in neonates, usually in the NICU

44. Infective endocarditis  Major  2 + BC, (viridans, s. bovis, HACEK, S. aureus, enterococci  Persistently + BC (1 hr between multiple, or 12h or 3h +)  + echo mass at typical sites  Intracardiac abscess  Prosthesis failure  New regurgitant murmur

45. Infective endocarditis  Minor  Fever (38C)  Predisposing condition/IVD  Vascular phenomena  Non-specific echo findings

46. Prophylaxis -1997  High risk – amp and gent  Prosthesis  Previous IE  Complex CHD  Surgical systemic-pulmonary shunts  Medium risk - amp  Other congenital heart malformation  Acquired valve dysfunction  HOCM  MVP  Negligible risk – no Rx  Isolated secundum; repaired ASD, VSD, PDA; bypass graft; MVP (no regurge); “innocent” murmurs; KD with normal valves; RF with no valve dysfunction; pacemakers

47. Prophylaxis  High risk  Prosthesis  Previous IE  Transplants  Complex CHD  Dropped from the list…….  Moderate risk (PDA,VSD,primumASD,coarct,bicuspidAV)  Calcified AS,RF,HOCM,MVP

48. Pericarditis  Classic chest pain worse when lying flat  Radiation to L shoulder  Friction rub  Most often viral causes  Diffuse ST changes, “saddle”shaped  CXR important  Cefotaxime, ASA, prednisone, colchicine

49. Kawasaki disease  Etiology unkown, presumed infectious  More common in Asian and Pacific islanders  Peaks around 1-2years, 80% under 4y, 50% under 2y  Slight male preponderance  3mo-8y is typical range

50. Kawasaki disease  3 phases  Acute phase (10 days)  High fever for 5 days  4 of  rash (ANY rash, no bullae/vesicles),  oedema of extremities/ peeling of extremities  Non-exudative bulbar conjuctivitis  Mucosal changes (cracked lips, strawberry tongue – even on HISTORY)  Cervical LN (1.5cm)  Carditis, other organs (arthritis, pyuria, gallbladder/liver, menigitis, irritable

51. Kawasaki disease  Acute  ESR, CRP  WCC, plt  Lipids, LFTs  Echo coronary artery aneurysms unusual before 10d  Subacute phase  Desquamation  Coronary disease  Rash, fever, LN disappear  plt

52. Kawasaki disease  Convalescent phase  ESR, plt normalise  Beau’s lines

53. Kawasaki disease  Rx  IVIG  ASA  Vaccinations  Steroid of no benefit  Reduces CAD from 25% to 5%  Untreated mortality 1-5%