 A 12 week old girl presented with increased work of breathing and poor feeding. On examination, she was tachycardic, hypoxic, and floppy with crackles and a murmur.  Initial investigations showed severe metabolic acidosis and hyperkalemia. Echocardiogram revealed congenital mitral regurgitation, severe mitral regurgitation, and multiorgan failure.  She was diagnosed with congenital mitral regurgitation and shock from cardiac decompensation, precipitated by rhinovirus infection. She required intensive care management including ventilation, fluid resuscitation, and inotropic support.

1. WELCOME TO YOUR
WORST NIGHTMARE.
The collapsed neonate.

2. SUMMARY
 Case
 Discussion
• Congenital heart disease in neonates presenting to ED

3. THE CASE
 12 week old girl, ex 32 week prem (4 weeks corrected)
• 2 days increased WOB
• Poor feeding
 Mother Burmese and speaks no english

4. THE CASE
 Initially triage 3, nurse asks for review as becoming floppy and
increased work of breathing
• Afebrile, RR 60, sats 91% RA, P 170, unable to obtain BP
• CRT 6 seconds, cool peripheries
• Floppy, grunting, no spontaneous cry, is spontaneously moving
• Asymmetry of chest
• Moderate recessions, bilateral crackles
• Grade 3 on 4 systolic murmur, loudest left sternal edge

5. ADDITIONAL
EXAMINATION FINDINGS
 ????

6. EXAM
 Trachea midline
 Equal air entry and percussion
 ? Trans-illumination (not performed)
 Hepatomegaly
 Femoral pulses not able to be palpated (however nil could be palpated)
 No signs of trauma

7. DDX
 ???

8. INITIAL INVESTIGATIONS
 ??

9. VBG (FROM HELL!)
 pH 6.70
 pCO2 58
 HCO3 7
 Na 138
 K 7.5
 Chl 114
 BSL 2.6
 Lactate 20
 Hb 78

10. CHEST XRAY

11. MANAGEMENT
 ???

12. MANAGEMENT
 A and B and C
• Neopuff assisted ventilation
• Total 20ml/kg fluid boluses
• Manage hyperkalaemia
• Calcium gluconate
• HCO3
• Salbutamol nebs
• adrenaline bolus then infusion
• Intubation
• Rocuronium 1.2 mg/kg, fentanyl 1 mcg/kg
• Size 3.5 ETT, hyperventilate
• Prostin infusion

13. MANAGEMENT
 D
• 3ml/kg 10% dextrose then infusion
• Morphine and midazolam infusion
 Other
• Ceftriaxone
• Get help

14. HELP!
 Cardiology
• ECHO = abnormal mitral valve, severe MR, severely dilated LA,
dilated and hyperdynamic LV
 PICU
• Transferred to PICU within 90 minutes of presentation

15. FINAL DIAGNOSIS
 Congenital MR
• Shock, CCF
• Multiorgan failure – liver, renal, thyroid, coagulopathic
 Collapse precipitated by inter-current illness
• Rhinovirus

16. CHD in the ED
 A practical classification
 Key presenting features
 Management principles
 Not a comprehensive review. Not discussing
 Individual lesions
 Murmurs
 Tet spells
 Arrhythmias

17. How they present
 Congestive heart failure
 Left to right shunts
 LVOTO
 Cyanosis (not responsive to oxygen)
 Right to left shunts
 Shock/collapse
 Obstructive left heart lesions
 Incidental heart mummur
 Arrhythmia

18. Fetal Circulation

19. CCF
Pathophysiology
 Left to right shunt = volume overload of the right heart and lungs
 Overtime – PVR decreases, right heart and lung flow increases
 Is responsive to oxygen
 left sided obstructive lesions
 Pressure overload left heart, back-pressure to lungs
Differential diagnosis
 ASD
 VSD
 PDA
 Endocardial cushion defects
 (left sided obstructive lesions)

20. CCF
 Timing
 First few months of life
 Symptoms
 FTT, poor weight gain
 Sweating, respiratory distress, esp with feeds
 Signs
 Sweating, wheeze, gallop, hepatomegaly, peripheral oedema
(rarely)
 May be cyanotic but oxygen responsive (Improves with crying)
 Chest x-ray
 Congestion, effusions

21. Cyanosis
 Pathophysiology
 Right to left shunt – mixing
 Flow through the lungs is either increased (eg Transposition) or decreased (eg
TOF)
 Pulmonary blood flow is duct dependent
 Is unresponsive to oxygen
 Differential diagnosis
 1T
 2T
 3T
 4T – 10% of all CHD, number 1 cause beyond infancy
 5T
 (PS)

22. Cyanosis
 Timing
 Variable
 Symptoms
 Mild, minimal respiratory distress
 Signs
 “comfortably blue”
 Central cyanosis (tongue and mucous membrane of mouth, not lips),worsens with
crying
 Hyperoxia test
 high flow O2 for 10 minutes, if sats increase > 10%, likely pulmonary cause
 Sats probe right arm (pre-ductal)
 Chest x-ray
 Variable pulmonary markings, dependent on lesion
 cardiomegaly

23. Shock
 Pathopysiology
 Left ventricular outflow tract obstruction
 Either level of heart or aorta
 Systemic blood flow is duct dependent
 Rarely severe regurgitant lesions
 May cause back pressure = CCF
 Differential diagnosis
 Hypoplastic left heart
 Critical aortic stenosis
 Interrupted aortic arch
 Coarctation

24. Shock
 Timing
 Usually within first few days of life, up to 3 weeks
 Symptoms
 Irritability, poor feeding, respiratory distress
 Signs
 Unwell, collapsed, hypotension
 Cyanosis and CCF (50%)
 Murmur (50%)
 Weak femoral pulses
 Chest x ray
 Cardiomegaly (85%)

25. Chest X-RAY
 3 key features
 Size of heart
 Shape of heart and mediastinum
 Degree of pulmonary vascular markings

29. Management Principles
 Oxygen, ABCs
 NS boluses if shocked
 10ml/kg
 Get help early
 Cardiology,+/- NICU/PICU
 Prostaglandin if shock or decompensated cyanotic heart disease
 Consider differentials
 Sepsis – Abs
 Respiratory
 Metabolic – check a BSL
 NAI

30. Prostaglandin E1
 Potent vasodilator, opens the the ductus arteriosis
 Majority of cardiac lesions occuring in the 1st 3 weeks of life presenting with
shock or cyanosis are duct dependent – should start empirically in
conjunction with Cardiology and NICU/PICU
 Remember,
 Preserves pulmonary flow in cyanotic lesions
 Preserves systemic flow in obstructive lesions
 Works within 15 minutes – either improves shock or cyanosis
 Infusion start @ 0.05 to 0.1 mcg/kg/min, up to 0.4 mcg/kg/min
 Adverse effects – apnoea (may need intubation)

31. Take home messages
 Present in 3 ways when unwell
 CCF – L to R shunt
 Cyanotic – R to L shunt
 Shock/collapse - LVOTO
 Key Sx/Sn
 DDx for the wheezing infant
 Always check the liver and femorals
 Prostaglandin E 1 can be life saving
 Get help early if CHD is suspected