This document provides an overview of cyanotic heart disease in newborns. It discusses the causes, types, and approach to evaluating and managing cyanosis. Key points include: - Cyanosis is a bluish discoloration of the skin from low oxygen levels in the blood. It is detectable at oxygen saturations below 85%. - Cardiac causes of cyanosis include decreased or increased pulmonary blood flow from conditions like tetralogy of Fallot or transposition of the great arteries. - Evaluation involves history, physical exam, chest X-ray, ECG, pulse oximetry, blood gases and hyperoxic challenge test, and echocardiogram. - Management focuses on maintaining ductus arter

1. Approach To Cyanotic
Heart Disease in
Newborn

2. OVERVIEWOVERVIEW
• CHD is the most common birth defect
• Incidence –varies from 4 to 50/1000 live birth
• Sabah – 80 to 1000 cases per year base on 20000
live birth
• Critical CHD about 25%
• 2/3 need surgery
• 1/3 need transcatheter intervention

3. CyanosisCyanosis
• Derived from the colour ‘cyan’ which comes
from the word ‘kyanous’, Greek word for
blue
• Bluish or purplish discoloration of skin and
mucous membrane due to increased
amount of deoxygenated Hb
• > 3g deoxygenated Hb
• Visible if SPO2 < 85% ( in pt with normal Hb)

4. The arterial oxygen saturation level at which cyanosis is
detectable at different total hemoglobin concentrations is
illustrated above. The solid red portion of each bar represents 3
gm/dL reduced hemoglobin.

5. CyanosisCyanosis
Central Cyanosis – cyanosis entire body

6. CyanosisCyanosis
Peripheral Cyanosis – cyanosis only in extremities
Causes
vasomotor instability
vasoconstriction caused by exposure to cold
venous obstruction
elevated venous pressure
polycythemia
low cardiac output,

7. CyanosisCyanosis
Acrocyanosis
•cyanosis of the hands, feet and around mouth in neonate
•Mucous membrane remain pink
•Not pathological
o part of normal transition
o may last 72hr

8. Causes of CyanosisCauses of Cyanosis
Cardiac and Non Cardiac

9. Causes of CyanosisCauses of Cyanosis
• Respiratory – upper or lower
• Cardiac
• CNS
• Metabolic – hypoglycaemia
• Shock
• PPHN

10. Cardiac CausesCardiac Causes
• Decreased pulmonary blood flow-
o TOF/Severe PS
o VSD/PA
o Critical valvular PS
o PAIVS
• Increased pulmonary blood flow-
o TGA
o Truncus arteriosus
o TAPVD
• Severe heart failure- low cardiac output
o Hypoplastic left heart syndrome
o Coarctation of the aorta
o Interrupted aortic arch
o Critical valvular aortic steanosis

11. Approach to CyanosisApproach to Cyanosis
1. History and physical examination
2. Investigations – ABG, CXR, ECG
3. SPO2 and Hyperoxic test
4. ECHO

12. AimAim
• Differentiate physiologic from pathologic
cyanosis
• Differentiate cardiac from non- cardiac cause of
cyanosis
• Find cause which needs urgent treatment or
referral

13. Perinatal historyPerinatal history
Drug intake
Causing neonatal depression
Lithium- Ebstein anomaly
Phenytoin- PS and AS Fetal hydantoin synd-
Fetal alcohol- VSD,ASD
Maternal diabetes-
TGA, ventricular septal defect (VSD), and
hypertrophic cardiomyopathy
Connective tissue disorder- Heart blocks associated with
anti-Ro/SSA and anti-La/SSB antibodies.
Congenital intrauterine infections cytomegalovirus,
herpesvirus, rubella, or coxsackie virus can lead to cardiac
structural abnormalities or functional impairment
Antenatal fetal echocardiography

14. Onset of cyanosis in cardiac lesionsOnset of cyanosis in cardiac lesions
Age on admission Diagnosis
newborn PAIVS
Severe TOF
HLHS, Critical CoA
Triscupid Atresia
1st
week of life TGA
CoA
VSD/PA
TAPVD
1 month of life TGA
TOF
TAPVD

15. Ductal DependentDuctal Dependent
CirculationCirculation

16. Ductal DependentDuctal Dependent
Pulmonary CirculationPulmonary Circulation
• Pulmonary Atresia/Intact
ventricular septum
• Triscupid Atresia
• Critical pulmonary stenosis
• TOF with severe PS
• VSD/PS
• Ebsteins anomaly

17. Ductal dependent SystemicDuctal dependent Systemic
CirculationCirculation
• Severe Coarctation of Aorta
• Interrupted Aortic Arch
• Critical Aortic Stenosis
• Hypoplastic Left Heart Syndrome

18. Physical ExaminationPhysical Examination
Points suggestive of Cardiac Cyanosis
1)Central Cyanosis
2)Reduced or absent femoral pulses
3)Cardiac murmur
4)Shock – poor pulse volume, reduced perfusion

19. Physical ExaminationPhysical Examination
Downs syndrome – TOF, AVSD
DiGeorge syndrome – Truncus arteriosus
Cat Cry Sundrome – Triscupid Atresia
Turners - CoA

20. Investigation - CXRInvestigation - CXR
• Cardiomegaly
• Oligemic lung – reduced PBF to lung
• Plethoric – increased PBF to lung
• To identify pulmonary causes of cyanosis:
pneumothorax, pulmonary hypoplasia, diaphragmatic
hernia, pulmonary edema, pleural effusion, etc.

21. CXRCXR
Increased PBF, Oligemic lung – TAPVD, Critical CoA,
IAA
Decreased PBF, Plethoric Lung – TOF, PAIVS, VSD/PA

22. CXRCXR
"Egg Shaped” in TGA

23. CXRCXR
“Boot- Shaped” in TOF

24. CXRCXR
“Snowman” in TAPVD

25. CXRCXR
Wall to wall heart – extreme cardiomegaly in Ebsteins
Anomaly

26. ECG IN CCHDECG IN CCHD
• DETERMINATION OF VENTRICULAR HYPERTROPHY AND QRS
AXIS DEVIATION AIDS IN DIAGNOSIS

29. ECG IN CCHDECG IN CCHD
CCHDCCHD RADRAD LADLAD RAERAE LAELAE RVHRVH LVHLVH RBBBRBBB
TOFTOF ++ ++
+post+post
repairrepair
PA+IVSPA+IVS ++ ++ ++
TR.ATRETR.ATRE
SIASIA
++ ++ ++
EBSTEINEBSTEIN’’
SS
++ ++
D-TGAD-TGA
+VSD+VSD
++ ++ ++ ++
TRUNCUTRUNCU
SS
++ ++
TAPVRTAPVR ++ ++

30. ECGECG
RVH – TOF, TGA, TAPVD, PPHN

31. ECGECG
Cyanosis with LVH/LAD – Pulm Atresia, Triscupid
Atresia,

32. TRICUSPID ATRESIA - LVH

33. Pre and Post Ductal PulsePre and Post Ductal Pulse
OximeterOximeter
SPO2 on Rt UL and Rt LL
•Positive if SPO2 difference > 5%
•Identifies Rt to Lt shunt at PDA level
•PPHN, CoA, IAA

34. Hyperoxic TestHyperoxic Test
Helps to differentiate between cardiac
and non cardiac cause of cyanosis
•Initial ABG on Rt Arm
•100% O2 for 10 – 20 min
•Repeat ABG at Rt Arm

35. Hyperoxia testHyperoxia test
Disease Result- Increase
in PaO2
Lung disease is more likely than
CHD
>150 mmHg
Cyanotic CHD <50 to 60 mmHg
In lesions with intracardiac
mixing and increased pulmonary
blood flow such as truncus
arteriosus-
>75 to 150
mmHg

36. Failure of Hyperoxic TestFailure of Hyperoxic Test
1) Failure to raise PO2 despite normal heart
- Massive intrapulmonary shunting – AVM
- shunt at PDA level in normal Heart – PPHN
2) Cyanotic heart disease with large Pulm blood plow
may rise the PO2 with O2
- TAPVD, Truncus arteriosus

37. Differentiate PulmonaryDifferentiate Pulmonary
and Cardiac Cyanosisand Cardiac Cyanosis

38. WHAT’S NEXTWHAT’S NEXT
1. History and physical examination
2. Investigations – ABG, CXR, ECG
3. SPO2 and Hyperoxic test
ECHO

39. TREATMENTTREATMENT
If Clinically suggestive of
DUCT DEPENDENT LESIONS
- TOF/PA, PAIVS, SEVERE PS, IAA, Critical CoA,
PA,TA
START IV PROSTAGLANDIN
( BEFORE ECHO )
REFER PEDIATRIC
CARDIOLOGIST

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

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

42. TreatmentTreatment
• Goals-
o Provide adequate tissue oxygen and CO2 removal
• Principles-
o Establish airway
o Ensure oxygenation
o Ensure adequate ventilation
o Correct metabolic abnormalities
o Alleviate the cause of respiratory distress

43. ManagementManagement
• PGE1 infusion to Open/Maintain Duct
o 10ng/kg/min and taper down gradually
• In doubt – start PGE1 till definitive ECHO
diagnosis
• Aim for percutaneous sats of 75 – 85%
• Adequate urine output
• Absence of metabolic acidosis
• ventilation for airway control/systemic collapse

44. DEFINITIVEDEFINITIVE
MANAGEMENTMANAGEMENT
Depends on the etiology
1)PDA Stenting – PAVSD, PAIVS, PA
2)Balloon valvuloplasty – severe PS, critical AS
3)BT shunt – severe ebsteins, TOF/PS, PA,TA
4)Corrective surgery – TGA, Truncus, TAPVD

45. THANK YOU