Cyanosis is a bluish discoloration of the skin and mucous membranes caused by low oxygen levels. It can be due to pulmonary or cardiac issues. Pulmonary causes include problems leading to ventilation-perfusion mismatching or low oxygen exchange in the lungs. Cardiac causes are congenital heart defects causing right-to-left shunts, reducing oxygenated blood to the body. Diagnosis involves physical exam, blood tests, chest x-ray, and oxygen responsiveness testing to distinguish between cardiac and pulmonary etiologies. Initial management focuses on stabilization, monitoring, supportive care like oxygen, and treating any underlying issues like sepsis.

1. CYANOSIS
By
P.Padma Priyanka

2. Contents
 Introduction
 Factors affecting detection of cyanosis
 Etiology
 Types
 Cardiac vs pulmonary
 Approach
 Conclusion

3. Introduction
 Cyanosis is the bluish discoloration of the skin and
mucous membranes due to increased concentration of
reduced hemoglobin to about >5g/100 mL in the
cutaneous veins
 Desaturation of arterial blood
 Increased extraction of oxygen by peripheral tissue in
the presence of normal arterial saturation
 Detected –lips,fingernails,oral mucous
membranes,conjuctiva and tip of tongue

4. Factors affecting detection of
cyanosis in newborn
 Hemoglobin concentration
 Fetal hemoglobin
 Skin pigmentation

5. Hemoglobin
concentration
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.

6. Fetal hemoglobin
The oxygen-dissociation curve of human blood and the effects of
changes in the H+ ion concentration, Pco2 temperature and level of
2, 3-diposphoglycerate (2,3-DPG) are depicted above. For fetal
hemoglobin, the normal curve (a) is shifted to the left (b).

7. Skin pigmentation
 Less apparent in the skin of babies with darker
pigmentation.
 Examination should include the nail beds, tongue,
and mucous membranes, which are less affected by
pigmentation.

8. Cyanosis
Pulmonary
Central CNS
depression
Local
Ventilation-
perfusion
mismatch
Alveolar
hypoventilation
Diffuse
impairment
Cardiac
Increased
pulmonary
vascularity
Decreased
pulmonary
vascularity
Hemoglobi-
nopathies

9. Ventilation/perfusion mismatch
 Airway disease: transient tachypnea of the newborn
(TTN), respiratory distress syndrome (RDS),
pneumonia, aspiration (meconium, blood, amniotic
fluid), atelectasis, diaphragmatic hernia, pulmonary
hypoplasia, pulmonary hemorrhage, CCAM
 Extrinsic compression of the lungs: pneumothorax,
pleural effusion, hemothorax,

10. Alveolar hypoventilation
 CNS depression: asphyxia, maternal sedation,
intraventricular hemorrhage, seizure, meningitis,
encephalitis
 Airway obstruction: choanal atresia, laryngomalacia,
Pierre Robin syndrome
 Neuromuscular disease: phrenic nerve inury, neonatal
myasthenia gravis

11. Diffusion impairment
 Pulmonary edema: left-sided obstructive cardiac
disease, cardiomyopathy
 Pulmonary fibrosis
 Congenital lymphangiectasia

12. Cardiac causes
 Decreased pulmonary blood flow-
 Tetralogy of Fallot
 Tricuspid valve anomaly
 Pulmonary valve atresia
 Critical valvular pulmonary steanosis
 Increased pulmonary blood flow-
 Transposition of great arteries
 Truncus arteriosus
 Total anomalous pulmonary venous connection

13.  Cardiac causes- "five Ts" of cyanotic CHD:
 Transposition of the great arteries
 Tetralogy of Fallot
 Truncus arteriosus
 Total anomalous pulmonary venous connection
 Tricuspid valve abnormalities.
 A sixth "T" is often added for "tons" of other diseases,
such as double outlet right ventricle, pulmonary atresia,
multiple variations of single ventricle, hypoplastic left
heart syndrome, or anomalous systemic venous
connection (left superior vena cava connected to the
left atrium).

14. Hemoglobinopathies
 Hereditary < exposure to toxic substances
 >15%- cyanosis
 >70% -lethal
 Remain chocolate brown-even with full oxygenation or
long exposure to room air

15. Central cyanosis
Inadequate alveolar
ventilation
CNS depression
Inadequate ventilatory drive
Obstruction
Structural changes
Muscle weakness
Desaturated blood
bypassing alveolar units
Intracardiac R-L
Intrapulmonary shunt
Pulmonary hypertension
with R-L shunt

16. Peripheral cyanosis
 Peripheral cyanosis, involves a bluish discoloration of the
skin but sparing of the mucus membranes & tongue. In this
type, a normal PaO2 value is detected
 Increased oxygen extraction due to sluggish movement
through the capillaries leads to increased deoxygenated
blood on the venous side
 Vasomotor instability,vasoconstriction caused by cold, low
cardiac output, venous obstruction, elevated venous
pressure and polycythemia

18. Acrocyanosis
 Bluish discoloration of fingers seen in neonates and infants
due to vasoconstriction as a result of transient hypothermia
 No clinical significance unless associated with circulatory
shock
Circum-oral cyanosis
 Healthy child with fair skin due to sluggish blood flow
with vasoconstriction
 No clinical significance unless associated with low
cardiac output

19. Cardiac vs Pulmonary
 Hyperoxiatest-
 Response of arterial PaO2 to 100%oxygen inhalation
Result in PaO2 Disease
>100mm Hg Lung disease
Large pulmonary blood flow
(TAPVR)
<100mm Hg Massive intra-pulmonary shunt
with normal heart
<10-30mm Hg increase
(<100)
Intra-cardiac right to left shunt

21. Approach to cyanotic neonate

22. Antenatal history
 Fetal ultrasound scans- congenital heart disease,
diaphragmatic hernia and congenital cystic
adenomatoid malformation (CCAM).
 Family history of CHD

24. Physical examination
 Vitals
 R/o choanal atresia
 Respiratory system
 Cardiovascular system
 Abdomen
 Neurological disorders

25. Vitals
 Vital signs-
 signs of respiratory distress such as tachypnea,
retractions, nasal flaring & grunting usually indicate a
respiratory problem
 congenital heart disease is often accompanied by absent
or effortless tachypnea.
 Sepsis often has the following findings: peripheral
cyanosis, HR, RR, BP, / temp

26. R/o choanal atresia
 Cyanosis decreases during crying
 Confirmed by failure to pass a soft No. 5F to 8F
catheter through each nostril

27. Respiratory system
 Inspiratory stridor-
 upper airway obstruction
 Chest-
 Asymmetric chest movement combined with severe
distress-
 alarming sign for tension pneumothorax, diaphragmatic
hernia
 Transillumination of the chest-
 Pneumothorax

28. Cardiovascular system
 A systolic murmur audible in most forms of cyanotic
CHD (exception: d-TGA with intact ventricular septum &
no pulmonary stenosis).
 Respirations often are unlabored unless there is
pulmonary congestion or complicated by the
development of heart failure or acidosis, which will
affect the respiratory pattern

29. Per abdomen
 Scaphoid abdomen-congenital diaphragmatic hernia

30. Neurological disorders
 Observe for apnea and periodic breathing, which may
be related to immaturity of the nervous system.
 Seizures can cause cyanosis if the infant fails to
breathe during the episodes.

31. Investigations
• CBC
• Serum glucose
• ABG
 Chest X-ray films,ECG
 Arterial PaO2 in preductal and postductal arteries
 Hyperoxitest

32.  CBC & diff :
 or WBC  sepsis
 hematocrit > 65%  polycythemia
 Serum glucose: to detect hypoglycemia
 Arterial Blood Gases (ABGs):
 Arterial PO2: to confirm central cyanosis SaO2 not as good an indicator
due to fetal Hb affinity for O2 (left-shift)
 PaCO2: may indicate pulmonary or CNS disorders, heart failure
 pH: sepsis, circulatory shock, severe hypoxemia
 Methemoglobinemia: SaO2, normal PaO2, chocolate-brown blood

33. X-ray -Increased pulmonary
vascularity
 RVH on ECG
 D-TGA
 TAPVR with obstruction
 DORV with subpulmonary VSD
 PPHN
 LVH/BVH on ECG
 Persistent truncus arteriosus
 Single ventricle
 TGA and VSD
 Polysplenia syndrome

34. X-ray -Decreased pulmonary
vascularity
 RVH on ECG
 TOF
 DORV with PS
 Asplenia syndrome
 RBBB on ECG
 Ebstein’s anomaly
 LVH on ECG
 Pulmonary atresia
 Tricuspid atresia
 BVH on ECG
 TGA and PS
 Persistent truncus arteriosus
 Single ventricle and PS

35. Total Anomalous Pulmonary Venous
Return
 Snowman

36. Tetralogy of Fallot
 Boot shape

37. Transposition of Great
Arteries
 Egg on a string

38. Arterial PaO2 in preductal and
postductal arteries
 Right upper body-radial,brachial,temporal
 Umbilical artery line
 PaO2 should be compared
 Right radial-umbilical artery=>10-15 mm Hg

39. Differential cyanosis
 In severe R-L ductal shunt
 Pink-upper and cyanosed-lower
 Causes
 PPHN
 Severe AS
 Interrupted aortic arch
 Coarctation of aorta

40. Initial management
 Monitor Airway, breathing, circulation (ABCs)
 with respiratory compromise, establish an airway &
provide supportive therapy (e.g., oxygen, mechanical
ventilation)
 Monitor Vital signs
 Establish vascular access for sampling blood &
administering medicatons(if needed)
 umbilical vessels convenient for placement of intravenous
& intra-arterial catheters

41.  If sepsis is suspected or another specific cause is not
identified, start on broad spectrum antibiotics (e.g.,
ampicillin and gentamycin) after obtaining a CBC,
urinalysis, blood & urine cultures (if possible). Left
untreated, sepsis may lead to pulmonary disease & left
ventricular dysfunction.
 Secure a separate intravenous catheter to provide
fluids for resuscitation and ensure accessibility of
intubation equipment should they be required.

42. Prostaglandin E1 infusion
 Prostaglandin E1
 For cyanotic CHD/duct dependent cardiac defect
 Infusion of prostaglandin E1 at a dose of 0.05-
0.1mcg/kg/min intravenously
 Increase PaO2,increase systemic blood
pressure,improved pH-tapered 0.01mcg/kg/min
 No effect-increased upto 0.4mcg/kg/min
 Side effects-apnea(12%),fever(14%),flushing(10%)
 Less common side effects-
tachy/bradycardia,hypotension,cardiac arrest

43. Cyanosis
Pulmonary
Central CNS
depression
Local
Ventilation-
perfusion
mismatch
Alveolar
hypoventilation
Diffuse
impairment
Cardiac
Increased
pulmonary
vascularity
Decreased
pulmonary
vascularity
Hemoglobi-
nopathies

44. System Causes Clinical findings
CNS depression Perinatal asphyxia
Heavy maternal sedation
Intra uterine fetal distress
• Shallow irregular respiration
• Poor muscle tone
• Cyanosis disappears when
patient is stimulated or O2
given
Pulmonary disease Parenchyma
Pneumothorax or pleural
effusion
Diaphragmatic hernia
PPHN
• Tachypnea, respiratory
distress with retraction and
expiratory grunt
• Crackles or decreased
breath sounds
• X-ray findings
• Improve/abolish with oxygen
inhalation
Cardiac disease Cyanotic CHD with R-L shunt • Tachypnea without
retractions
• lack of crackles/abnormal
breath sounds
• Continuous murmur(PDA)
• X-ray findings
• Little/no increase with O2

45. Conclusion
 Central cyanosis in a newborn is an abnormal finding and
one must consider all of the possible etiologies with a
complete history, physical examination and relevant
investigations.
 Remember to think about the various mechanisms causing
cyanosis and go through each systematically until you have
your diagnosis.
 Prompt management should be undertaken while you are
trying to figure out your diagnosis.
 For ductal dependent lesion, start prostaglandin E1 and
early referral

46. Thank You