1. Basic Approach to cyanosis in infancy Cardiology Red Cross Children's Hospital

2. “You do not really understand something unless you can explain it to your grandmother.” Dr. Albert Einstein

3.  Most common reason for referral to cardiologist in the 1 st years of life: Heart Murmur Heart Murmur Cyanosis Cyanosis Congestive Cardiac Failure Congestive Cardiac Failure Arrythmias Arrythmias Abnormal CXR Abnormal CXR Abnormal ECG Abnormal ECG

4. Cyanosis (form the Greek word meaning dark blue)  What is cyanosis? Blue discolouration of lips / tongue / extremeties Blue discolouration of lips / tongue / extremeties Oxygenated Hb is bright red Oxygenated Hb is bright red Reduced Hb is blue / purple Reduced Hb is blue / purple  Cyanosis is dependent upon the absolute concentration of reduced Hb.  T c SaO 2 3g deoxygenated Hb  If present through the entire body= CENTRAL CYANOSIS  If present only in the extremeties = PERIPHERAL CYANOSIS

5. Lees 1970

6. Making the diagnosis  Clinically: Early detection in newborns is essential Early detection in newborns is essential May be difficult to see in dark skinned individuals – tip of tongue May be difficult to see in dark skinned individuals – tip of tongue Good lighting essential Good lighting essential Thermo-neutral environment Thermo-neutral environment Is the child distressed/any other signs of cardiac abnormalities?? Is the child distressed/any other signs of cardiac abnormalities?? Does the blueness fade on pressure? Does the blueness fade on pressure? Is the child cold / poorly perfused Is the child cold / poorly perfused

7.  If in doubt – Saturation with pulse oximeter and/or arterial blood gas: Normal T c SaO 2 in newborn in room air is 92% Normal T c SaO 2 in newborn in room air is 92% Normal P a O 2 in newborn is >60mmHg (>8Kpa) Normal P a O 2 in newborn is >60mmHg (>8Kpa)

8. Transcutaneous Saturation measurement  Uses light absorption at a given wavelength  measures bound O 2  assumes a normal haemoglobin molecule  Sat of 94% - equivalent to Sa0 2 of 90%  Misses bradyarrythmias  Completely inaccurate below 70% (beware of nail polish!!!!)  Ear best in a shocked patient

9. Causes of Cyanosis

11. In a nutshell…..  Respiratory disease  Shock  metabolic derangement hypoglycaemia, hypothermia hypoglycaemia, hypothermia  congenital heart disease  methaemoglobinaemia  persistent foetal circulation

12. STEPS IN MANAGEMENT OF CYANOSED NEWBORNS

13. Steps in the management of cyanotic newborns: 1. CHEST XRAY: May reveal pulmonary cause May reveal pulmonary cause May hint to the presence or absence of cardiac lesion May hint to the presence or absence of cardiac lesion 2. ARTERIAL BLOOD GAS IN ROOM AIR: Confirm or reject cyanosis Confirm or reject cyanosis Elevated pCO 2 suggests pulmonary or CNS cause Elevated pCO 2 suggests pulmonary or CNS cause LOW pH in severe shock, sepsis, severe hypoxaemia LOW pH in severe shock, sepsis, severe hypoxaemia 3. HYPEROXIA TEST: 4. ECG: If cardiac disease suspected – will give clue to diagnosis If cardiac disease suspected – will give clue to diagnosis 5. PROSTAGLANDIN E1: If heart lesion suspected that is ductus dependent (eg pulmonary atresia,Tetralogy of Fallot, TGA, Coarctation of the aorta) If heart lesion suspected that is ductus dependent (eg pulmonary atresia,Tetralogy of Fallot, TGA, Coarctation of the aorta)

14.  Assessment of cyanosis CXR Pulmonary/Cardiac ABG Hyperoxia test Cardiac vs Pulmonary cause Sepsis Hypoglycaemia Polycythaemia Cardiac Cause PPHN Septic Screen Blood glucose ECG Echocardiogram Asphyxia CNS (hypoventilation) Hb abnormalities Metabolic causes

15. The Chest X Ray  “classical” patterns - rare  better use is to judge pulmonary flow  oligaemia - dark lung fields - tetralogy  plethora - increased flow - mixers  use to side the arch  visceral situs in complex forms

17. The hyperoxia test  Cyanosis confirmed with arterial oxygen measurement  to differentiate between respiratory and cardiac causes of cyanosis  administer oxygen at the highest concentration possible (head box)  blood gas from Right radial artery  Wait for 15 minutes – repeat ABG pO 2 < 150 mm Hg (20 kPa) - cardiac pO 2 < 150 mm Hg (20 kPa) - cardiac pO 2 > 250 mm Hg (33 kPa) - respiratory pO 2 > 250 mm Hg (33 kPa) - respiratory

18. Failures of the Hyperoxia test  Cyanotic heart defect with large pulmonary blood flow (eg TAPVD) – pO 2 may rise with O 2 administration.  Massive intrapulmonary shunts but a normal heart (eg PPHN, AVM) may not raise the pO 2 with oxygen – pO 2 wont rise with O 2.  Response to oxygen inhalation must be interpreted in the light of the clinical picture

19. BEFORE REFERRAL TO CARDIOLOGIST – ATTEMPT TO MAKE A REASONABLE DIAGOSIS

20. CXR Reduced Pulmonary Blood flow Increased Pulmonary Blood flow ECG RVH LVH CVH RVH LVH Tetralogy PAtresia Tric atresia DTGA Truncus TAPVD TGA

21. Explanation of a few common cyanotic congenital heart defects

22. TETRALOGY OF FALLOT LV LA RV RA VSD Aorta overrides septum Infundibular “PS” RVH

23. “PALLIATED” TETRAOGY RA RV LV LA Blalock-Taussig shunt

24. TETRALOGY OF FALLOT RV HYPERTROPHY Ejection systolic murmur Single S2 NO RV heave Ejection systolic murmur Single S2 NO RV heave INFUNDIBULAR STENOSIS AORTIC OVERRIDE VSD NO CYANOSIS MILD CYANOSIS

25. TETRALOGY OF FALLOT ‘ HYPERCYANOTIC SPELL’ CYANOSIS ACIDOSIS Tachypnoea NO ejection systolic murmur Death Tachypnoea NO ejection systolic murmur Death POSITIVE FEEDBACK

26. TRANSPOSITION OF THE GREAT ARTERIES RA LA LV RV Aorta from RV Pulmonary Artery from LV

27. TGA SEVERECYANOSIS Two separate parallel circuits Incompatible with life No murmurs Two separate parallel circuits Incompatible with life No murmurs LA ENLARGEMENT LV ENLARGEMENT CCF AortaPA RVLV

28. TGA IVC PDA Rashkind atrial septostomy Followed by : Arterial switch or Mustard operation Followed by : Arterial switch or Mustard operation

29. TRICUSPID ATRESIA RA LV LA Atretic TV VSD

30. TRUNCUS ARTERIOSUS LV RV VSD “Truncus” Aorta Pulmonary artery RA

31. after referral?  ECG can give clues to the diagnosis  echocardiography - main diagnostic tool  catheterisation - particularly to assess pulmonary artery structure and size  balloon septostomy in TGA  use of IV prostaglandin to keep the duct open  surgery

33. Pre Muscle resection Post Muscle resection

34. Post VSD Repair

35. Thanks!!!!!