Related to anatomy & physiology of the heart, eazy explaination

1. My Heart Will Go On

2. Disclosure
• None

3. Paediatric Cardiac Emergencies in ED
• Neonatal
• Duct-dependent congenital heart disease
• Infants & older children
• Hypercyanotic spells
• Supraventricular tachycardia
• Pericardial tamponade

4. Case 1
• Term baby, delivered in a specialist hospital
• Birth weight 3.2 kg, good Apgar
• Uneventful postnatal period
• Discharged home at 24 hours of life
• Came to ED on day 3 of life because mother noted child to be
“blue”

5. Case 1
• Alert, good cry
• SpO2 65 – 70%
• No respiratory distress
• Heart: S1 S2, no heart murmur
• Lungs: clear
• SpO2 improved to 80% after giving 2L/min oxygen via nasal cannula

7. Quiz
Could he has cyanotic heart disease?
• Yes
• Unlikely; his SpO2 improved with oxygen supplementation suggestive of lung
pathology
• Unlikely; there was no heart murmur
• Unlikely; there was no respiratory distress
• Unlikely; he should have presented much earlier during postnatal period
before home discharge

8. Echocardiogram
Pulmonary atresia intact ventricular septum

9. Duct-Dependent Pulmonary Circulation
• Critically obstructed pulmonary flow
• Pulmonary circulation relies on PDA
• Examples
• PAIVS
• TOF pulmonary atresia
• Critical PS
• Univentricular heart with pulmonary
atresia

10. Duct-Dependent Pulmonary Circulation

11. Parallel Circulation
Transposition of great arteries

12. Why Missed During Newborn Check?
• Cyanosis is easily missed during early
postnatal period
• Haemoglobin-oxygen dissociation
curve
• Fetal haemoglobin
• Presence of PDA
• Absence of heart murmur
• Absence of respiratory distress

13. Cyanosis: Heart vs Lungs
Heart Lungs
History None
Fever, cough, rapid breathing, poor
feeding
Clinical signs Cyanosis
Chest recession, tachypnoea,
abnormal auscultatory findings
Chest X-ray
Normal; sometimes
oligaemic lung fields
Abnormal lung fields
Arterial blood gas
↓ PaO2
Normal PaCO2
↓ PaO2
Variable PaCO2
Response to O2
supplementation
Poor response Good response

14. Management
• Airway, breathing, circulation
• IV Prostaglandin E2 infusion
• Emergency balloon atrial septostomy for
transposition of great arteries
• Definitive surgery or catheter intervention after
stabilization
• Early diagnosis is the key!

15. Case 2
• Term baby, delivered in a district hospital
• Birth weight 3.5 kg, good Apgar
• Uneventful postnatal period
• Discharged home at 24 hours of life
• Came to ED on day 3 of life as mother noticed poor feeding

16. Case 2
• Mottled, poor perfusion
• SpO2 poor signal, heart rate 180 bpm
• Peripheral pulses not palpable
• Grunting cry, marked chest recession
• No heart murmur
• ABG: pH 6.9 pO2 58 pCO2 56 BE – 23.9

17. Quiz
What is your immediate management?
• Intubate and start ambu-bagging with 100% oxygen
• Obtain venous access and administer 20 ml/kg of fluid bolus
• Start IV Dopamine infusion
• Given IV NaCO3 to correct metabolic acidosis
• Do septic workout and start IV antibiotics
• Perform bedside echocardiogram

18. Echocardiogram
Severe coarctation of aorta

19. Duct-Dependent Systemic Circulation
• Critically obstructed aortic outflow
• PDA provides adequate systemic flow
in-utero/ early postnatal life
• Problems starts when PDA constricts

20. Duct-Dependent Systemic Circulation
Clinical presentations mimic sepsis
• Unwell, poor feeding
• Poor perfusion, tachycardia
• Respiratory distress
• Hypotension, metabolic acidosis, oliguria, hypoglycaemia
• Cardiorespiratory failure

21. Duct-Dependent Systemic Circulation
Interrupted aortic arch Hypoplastic left heart syndrome

22. Management
• High index of suspicion
• Early diagnosis by echocardiogram is the key!
• Start IV Prostaglandin E2 infusion
• Intubation, mechanical ventilation
• Fluid resuscitation
• Inotropic support
• Correction of hypoglycaemia, acidosis, electrolytes
• Empirical IV antibiotics

23. Critical Congenital Heart Disease
Duct-dependent pulmonary circulation
Duct-dependent systemic circulation
Parallel circulation
Obstructed pulmonary venous drainage

24. Critical Congenital Heart Disease
• Only 45% of all CHD had abnormal finding
on routine neonatal examination
• Heart murmur is not a reliable sign
• Sensitivity rate = 44% (CI 31 – 51%)
• Positive predictive value = 54% (CI 39
– 69%)
1. Wren C et al. Presentationof congenital heart disease in infancy: implications for routine examination. Arch Dis Child Fetal Neonatal Ed 1999;80:F49–53
2. Ainsworth SB et al. Prevalence and clinical significance of cardiac murmurs in neonates. Arch Dis Child Fetal Neonatal Ed 1999;80:F43-F45

25. Case 3
• 5 years old girl
• History of fever & cough for 3 days
• Given cough mixture by GP
• Complains of chest discomfort this morning
• No past history of heart problem
• Heart rate 200/min on cardiac monitor
• BP stable, good perfusion

27. Quiz
What is your diagnosis?
• Sinus tachycardia
• Atrioventricular re-entrant tachycardia
• Atrial flutter
• Junctional tachycardia
• Ventricular tachycardia

28. Case 3
• Carotid massage → no response
• IV Adenosine 0.2 mg/kg rapid bolus → no response also !!

29. Quiz
What is your diagnosis now?
• Sinus tachycardia
• Atrioventricular re-entrant tachycardia
• Atrial flutter
• Junctional tachycardia
• Ventricular tachycardia

31. Atrioventricular Reentry Tachycardia
• Most common supraventricular tachycardia
in paediatric
• Substrate: accessory pathway connecting
atrium with adjacent ventricular myocardium
• Reentry circuit formed by
• Atrium
• AV node
• Ventricle
• Accessory pathway

32. Atrioventricular Reentry Tachycardia
SA node
AV node
Accessory
pathway
Ventricular premature
contraction

33. Clinical Presentation
• Older children & adults
• Paroxysmal, abrupt onset and termination
• Palpitation, chest discomfort, pre-syncope
• Usually well tolerated
• Infants
• Inability to express
• Non-specific symptoms of irritability, poor feeding, diaphoresis
• SVT may persists for days leading to heart failure

34. ECG
• Rate depends on age
• 300/min in neonates, 250/min in infancy, 200/min in older children
• Narrow QRS complex
• Except in pre-existing bundle branch block or rate-related aberrant
conduction
• P wave difficult to visualize
• Constant rate, no beat-to-beat variation
• Abrupt onset, abrupt termination (all or none phenomenon)

35. ECG

36. ECG
• Sometimes P wave can be seen immediately after QRS (best seen
on lead V1)
• Short RP tachycardia

37. IV Adenosine
• IV Adenosine terminate the tachycardia
• Results in non-conducted P wave, transient junctional escape
rhythm followed by restoration of sinus rhythm

38. Case 3
When tachycardia is “not responding” to IV Adenosine
• Inadequate dose
• Suboptimal administration (not fast enough, small peripheral vein)
• Wrong diagnosis of AVRT
• AVRT stopped by IV Adenosine but restarted soon after

40. IV Adenosine as Diagnostic Tool
Important to have continuous ECG recording when administering IV
Adenosine
4 patterns of response
• Tachycardia terminated
• Tachycardia stopped but restart shortly
• Tachycardia slowed down transiently then speed up
• Tachycardia continues in the presence of AV block

41. IV Adenosine as Diagnostic Tool
• Tachycardia terminated
→ AVRT, AVNRT
• Tachycardia stops transiently, then restart
→ AVRT, PJRT

42. IV Adenosine as Diagnostic Tool
• Tachycardia slows transiently and then speed up
→ sinus tachycardia, atrial ectopic tachycardia
• Tachycardia continues in the presence of AV block
→ atrial flutter, atrial ectopic tachycardia

43. Management
• In haemodynamically stable patients
• Vagal maneuvers
• Diving reflex (ice bag/cold clothe on forehead)
• Valsalva maneuvers
• Carotid massage
• IV Adenosine
• 0.1 mg/kg rapid IV bolus, double the dosage until termination or maximal
dose 0.6 mg/kg 1
1. Dixon J et al. Guidelines and adenosine dosing in supraventricular tachycardia. Arch Dis Child (2005) 90:1190-1

44. Management
• Initial dose of 0.2 mg/kg was found to be more effective 1
• Important technical issues
• As fast as possible
• Saline flush
• Large cannula
• As close to central vein
1. Dixon J et al. Guidelines and adenosine dosing in supraventricular tachycardia. Arch Dis Child (2005) 90:1190-1

45. AVRT Management
If haemodynamically unstable/decompensated heart failure
• IV Adenosine (up to 1 mg/kg) if venous access available
• DC cardioversion (0.5 – 1 J/kg)
• Avoid IV Verapamil, especially in infants (cardiovascular collapse) 1, 2
1. Epstein ML et al. Cardiac decompensation following verapamil therapy in infants with supraventricular tachycardia. Pediatrics (1985) 75:737-40
2. Kirk CR et al. Cardiovascular collapse after verapamil in supraventricular tachycardia. Arch Dis Child (1987) 62:1265-85

46. AVRT Management
If recurrence soon after acute termination
• IV Digoxin; loading dose of 15 mcg/kg followed by 5 mcg/kg 6 hours later
• IV Propranolol: 0.02 mg/kg test dose, then 0.1 mg/kg over 10 mins
• IV Amiodarone 25 mcg/kg/min for 4 hour followed by 5 to 15 mcg/kg/min until
conversion
Repeat IV Adenosine dosing may work after initial “priming” with
another anti-arrhythmics

47. Case 4
• 3 months old boy
• Known case of Tetralogy of Fallot, planned for elective surgical
repair at 1 year old
• Sudden onset of bluish discoloration of lips and finger tips this
morning after defaecation
• Associated with ↓ conscious level & breathing difficulty
• No fever
• Was still active and feeding well last nigh

48. Case 4
• SpO2 40 – 50%
• Drowsy
• Tachypnoea, hyperventilating
• Grade 2 systolic murmur upper left sternal border
• Lungs: clear
• No clubbing

50. Quiz
What is the diagnosis?
• Status epilepticus
• Breath holding attack
• Sepsis
• Hypercyanotic spell

51. Tetralogy of Fallot
• Commonest cyanotic heart disease
• Constellation of
• RV outflow tract obstruction
• Large VSD
• Overriding of aorta
• RV hypertrophy

52. Hypercyanotic Spell
• Peak incidence 3 – 6 months
• Period of uncontrolled crying/irritability
• Worsening cyanosis
• Rapid & deep breathing
• Lethargy, limpness, coma, seizure
• Reduced murmur intensity
Clinical diagnosis!

53. Pathophysiology

54. Management
• Oxygen
• Nasal cannula/non-rebreather mask
• Calm the child
• Knee-chest position
• Morphine
• Intramuscular/intravenous
• 0.1 mg/kg
• May be repeated but monitor for
respiratory depression

55. Management
• 20 ml/kg IV normal saline bolus
• IV NaHCO3 1 to 2 mmol/kg if severe metabolic acidosis (pH < 7.1)
• IV Ketamine 1 mg/kg
• Beta blockers
• IV Propranolol 0.1 mg/kg slow bolus
• IV Esmolol 0.5 mg/kg bolus, followed by infusion 50 - 1000 mcg/kg/min
• Systemic vasoconstrictors
• IV Phenylephrine 0.1 mg/kg slow bolus, followed by infusion
• IV Noradrenaline infusion 0.01 – 1 mcg/kg/min

56. Management
• Intubation & general anaesthesia
if fail all the above measures
• Emergency surgical BT shunt

57. Case 5
7 years old girl
• Known case of large ASD
• Underwent surgical ASD closure at IJN 3 weeks ago
• Uneventful postoperative recovery
• Postop echocardiogram: no residual ASD
• Discharged home on POD5

58. Case 5
• Abdominal pain x 2 days
• Afebrile, normal vital signs
• Sternotomy wound clean & healed
• S1, S2, no murmur
• Lungs clear
• Abdomen soft, non tender
• Prescribed some antacids, discharged home

59. Case 5
3 days later
• Shortness of breath
• Came to ED again
• Collapsed while doing CXR
• Immediate CPR, return of circulation

61. What Is the Diagnosis?

62. Echocardiogram

63. Diagnosis
• Cardiac tamponade
• Post-pericardiotomy pericardial effusion

64. Pericardial Effusion
• Common after any open heart
surgery
• 1 to 6 weeks after surgery
• Majority are small and self-limiting
• Occasionally can cause cardiac
tamponade

65. Pericardial Effusion
Causes
• Post open heart surgery
• Infective – pyogenic, tuberculous
• Connective tissue disease – SLE
• Malignancy
• Central line extravasation
• Post-traumatic

66. Pericardial Effusion
Non-specific symptoms
• Chest pain
• Abdominal
pain/discomfort/fullness
• Dyspnoea
Clinical signs
• Muffled heart sounds
• Pericardial rub
• Jugular vein distension
• Pulses paradoxus
• Hypotension

67. Management
• Bed-side ultrasound for early
confirmation of diagnosis
• Emergency pericardiocentesis if
haemodynamic compromise