3. Introduction
Cyanosis is derived from the colour ‘cyan’, which
comes from ‘kyanous’, the Greek word for blue
It is defined as the bluish discoloration of the skin
and the mucous membranes, resulting from an
increase in the reduced Haemoglobin or of
haemoglobin derivatives in the small vessels of
those areas.
Bluish discoloration of the tissues that results when the
absolute level of reduced hemoglobin in the capillary
bed exceeds 3- 4 g/dL
Depends upon the total amount of reduced
hemoglobin rather than the ratio of reduced to
oxygenated hemoglobin.
4. Sites to detect cyanosis
Lips
Nail beds
Ears
Malar Prominences
Palms and Soles
Tongue
Mucous membranes of gum,soft palate,cheeks
5. Types of Cyanosis
Central Cyanosis
Peripheral Cyanosis
Mixed Cyanosis
Other Types
Enterogenous/Pigment Cyanosis
Differential Cyanosis
Acrocyanosis
Orthocyanosis
6. Central cyanosis
Pathologic condition
caused by reduced arterial
oxygen saturation.
Involves highly vascularized
tissues, such as the lips
and mucous membranes,
through which blood flow is
brisk and the arteriovenous
difference is minimal.
Cardiac output typically is
normal, and patients have
warm extremities.
7. Mechanism
Decreased arterial oxygen saturation due to
marked decrease in oxygen tension in the
arterial blood(arterial PaO2 is reduced)
Sites-
Tongue (margins & undersurface)
Inner aspect of lips
Mucous membranes of gums,soft palate,cheeks
8. Peripheral cyanosis
Causes○ vasomotor instability,
vasoconstriction caused by
exposure to cold, venous
obstruction, elevated venous
pressure, polycythemia, and
low cardiac output,
Affects the distal
extremities and circumoral
or periorbital areas .
9. Mechanism
Normal systemic arterial oxygen saturation and
increased oxygen extraction, resulting in a wide
systemic arteriovenous oxygen difference
The increased extraction of oxygen results from
sluggish movement of blood through the
capillary circulation
•
Sites
Tip of nose
Ear lobules
Outer aspect of lips,chin,cheek
Tips and nailbeds of fingers,toes
Palms,soles
•
•
•
•
•
10. Mixed Cyanosis
Cardiogenic shock+ pulmonary oedema
CCF due to lt.sided heart failure
Polycthemia (rare)
Orthocyanosis
Present in upright position due to hypoxia
occuring in erect posture in Pulmonary
Arteriovenous Malformation
11. Enterogenous/pigment cyanosis
Due to presence of excessive –
sulphaemoglobin(>0.5g/dl),methaemoglobin(
>1.5g/dl
Causes
Hereditary haemoglobin M disease
Poisoning by aniline dyes
Drugsnitratres,nitrites,phenacetin,sulphonamides
Carboxyhaemoglobinaemia
12. Differential Cyanosis
Hands red (less blue) and feet blue seen in PDA with
reversal of shunt (Differential Cyanosis) Requires
pulmonary vascular resistance elevated to a systemic
level and a patent ductus arteriosus
L
R shunt
Pulmonary
hypertension
Reversal
of shunt
R to L
Desaturated blood from the ductus
enters the aorta distal to the left
subclavian artery, sparing the
brachiocephalic circulation.
13. Reverse Differential Cyanosis
Hands blue and feet red seen in
Coarctation of Aorta with TGA(Reverse
Differential Cyanosis
Intermittent Cyanosis seen in Ebstein’s
Anomaly
14. Central Vs Peripheral Cyanosis
SITES
TONGUE,ORAL CAVITY
TONGUE UNAFFECTED
HANDSHAKE
FEELS WARM
FEELS COLD
APPLICATION OF
WARMTH,COLD
NO CHANGE
WARMTH-CYANOSIS
INCR,COLDDECREASES
APPLICATION PURE O2
MAY IMPROVE
NO RESPONSE
CLUBBING,POLYCYTHA
EMIA
USUALLY PRESENT
ABSENT
PULSE VOLUME
MAYBE HIGH
LOW VOL
DYSPNOEA
PT BREATHLESS
NO RESPIRATORY
PROBLEM
15. Acrocyanosis
Condition in which there is arterial
vasoconstriction,and secondary dilation
of capillaries and venules with resulting
persistant cyanosis of the hands and
less fequently the feet.
part of normal transition
may last 72hr
beware APGAR of 10
○ hypoperfused
○ severe anemia
16. Psuedocyanosis
Bluish tinge to the skin and or mucous
membranes that is not associated with
either Hyoxemia or Peripheral
Vasoconstriction
Metals
Drugs
17. Factors altering cyanosis
Colour of the cutaneous pigment
Thickness of the skin
State of cutaneous capillaries
Cyanosis becomes apparent when the
concenteration of the reduced haemoglobin in
capillary blood vessels exceeds 40 g/l or 4g/dl
18. Factors affecting the detection of
cyanosis in the newborn
Hemoglobin concentration Detected at higher levels of saturation in
polycythemic than in anemic patients.
Significant oxygen desaturation can be present
in an anemic patient without clinically detectable
cyanosis.
As an example, 3 g/dL of reduced hemoglobin is
associated with an oxygen saturation of 67
percent when the total hemoglobin
concentration is 9 g/dL, and 85 percent when
the hemoglobin concentration is 20 g/dL.
19. 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.
20. Factors affecting the detection of
cyanosis in the newborn
Fetal hemoglobin —
Binds oxygen more avidly than adult hemoglobin.
The oxygen dissociation curve is shifted to the left, so
that for a given level of oxygen tension (PO2), the
oxygen saturation (SO2) is higher in the newborn than
older infants or adults
It also follows that for a given level of oxygen
saturation, the PO2 is lower in newborns.
As a result, cyanosis is detected at a lower PO2 in
newborns compared with older patients. Thus, in
evaluating a cyanotic newborn, PO2 should be
measured in addition to SO2 to provide more
complete data.
21. Factors affecting the detection of
cyanosis in the newborn
Other physiologic factors common in
sick newborns affect the oxygen
dissociation curve.
Those that increase the affinity of
hemoglobin for oxygen (shifting the
oxygen dissociation curve to the left),
decrease the concentration of reduced
hemoglobin at a given arterial P02, and
lower the PO2 at which cyanosis first
appears.
23. Cyanosis…
Cyanosis is
dependent on HCT
and % Sat
Florescent light
makes cyanosis
hard to see.
Except in the
extreme, cyanosis
is not obvious
Any question,
check a pulse ox
100
90
80
70
% Saturation
Cyanosis
60
50
40
30
20
10
0
0
20
40
HCT
60
80
24.
In contrast, acidosis,
fever, or increased
adult hemoglobin shift
the curve to the right.
As a result, at a given
arterial PO2, there is
increased oxygen
delivery to the tissues
resulting in a greater
concentration of
reduced hemoglobin,
and cyanosis appears
more readily.
For fetal hemoglobin, the normal curve
(a) is shifted to the left (b)
25. Factors affecting the detection of
cyanosis in the newborn
Skin pigmentation Less apparent in the skin of patients with
darker pigmentation.
Examination should include the nail beds,
tongue, and mucous membranes, which are
less affected by pigmentation.
26.
27. Etiology Can be divided in to,,
Site of cynosis
Central causes
Peripheral causes
Mecanism of
cynosis
Alveolar hypoventilation
Diffusion impairment
Ventilation-perfusion
mismatch
Right-to-left shunting at the
intracardiac, great vessel,
or intrapulmonary level
Hemoglobinopathy
(including
methemoglobinemia) that
limits oxygen transport
34. Cardiac causes
Severe heart failureHypoplastic left heart syndrome
Coarctation of the aorta
Interrupted aortic arch
Critical valvular aortic steanosis
35. The 6 T’s
Total Anomalous Pulmonary Veins
Tetrology of Fallot
Tricuspid Atresia
Transposition
Truncus Arteriosus
Total Acardia
36. Mnemonic
A 7T" 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
37. Differential cyanosis
With normally related great arteries, oxygen
saturation may be higher in the upper than
lower extremity in patients if there is right-toleft shunting through the ductus arteriosus.
Seen with severe coarctation or interrupted
aortic arch.
May also occur in patients persistent
pulmonary hypertension of the newborn
The differential effect is reduced if there is also
right-to-left shunting at the level of the foramen
ovale, or if there is left-to-right shunting across
a coexisting ventricular septal defect
38. Differential cyanosis
Reversed differential cyanosis is a rare
finding that may occur in patients with
transposition of the great arteries
associated with either coarctation or
pulmonary hypertension.
In these infants, oxygen saturation is
higher in the lower than upper extremity.
39.
40. Aim
Differentiate physiologic from pathologic
cyanosis
Differentiate cardiac from non- cardiac
cause of cyanosis
Find cause which needs urgent
treatment or referral
41. Not so serious
Acrocyanosis
Bluish color in the hands and feet and around
the mouth (circumoral cyanosis). The mucus
membranes generally remain pink.
Reflects benign vasomotor changes in the
diffuse venous structures in the affected areas.
Does not indicate pathology unless cardiac
output is extremely low, resulting in cutaneous
vasoconstriction
Cyanosis soon after birth- transition from
intrauterine to extrauterine life
Hand or leg prolapse
42. Perinatal 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
43. History
Methemoglobinemia may be acquired
following exposure to aniline dyes,
nitrobenzene, nitrites, and nitrates.
Advanced maternal age Trisomy 21
associated with many congenital heart
defects (cyanotic and acyanotic)
Oligohydramnios …..Pulmonary
hypoplasia
44. Onset of cyanosis in cardiac lesions
Depends onNature and severity of the anatomic defect
In utero effects of the structural lesion
Alterations in cardiovascular physiology secondary
to the effects of transitional circulation like closure of
ductus arteriosus and the fall in pulmonary vascular
resistance
A ductal dependent lesion is one that depends on the ductus to get
adequate blood flow to the pulmonary and systemic circuits, or provide
mixing
○ PS
○ CoA
○ TGA
46. Onset of cyanosis in cardiac lesions
Age on admission
In order of frequency
0-6 days
D- transposition of great arteries
Hypoplastic left ventricles
Tetralogy of fallot
7-13 days
Coarctation of aorta
Hypoplastic left ventricle
D-transposition of great arteries
Tetralogy of fallot
14-28 days
Coarctation of aorta
Tetralogy of fallot
D- transposition of great arteries
Neonatology- Pathophysiology and management of newborn, 5th edition ed.
1999. Philadelphia; Lippincott Williams and Wilkins
47. History- Risk factors
Pneumonia/ sepsis-
PROM
Foul smelling liquor
Maternal pyrexia
Maternal GBS
TTN –
Post maturity
Small for gestational age
Placental dysfunction
Fetal distress
Meconium stained liquor
Birth by cesarean section
Polycythemia-
with or without labor
Male sex
Family history of asthma
(especially in mother)
Macrosomia
Maternal diabetes
small-for-gestational age
MAS-
Pneumothorax Aggressive resucitation
IPPV
Meconiun aspiration
HMD
Hypoplastic lung
Staph pneumonia
Hyaline membrane
disease-
Premature infant
Infant of diabetic mother
49. Physical Examination
Vital signsHypothermia or hyperthermia- infection.
Tachycardia-hypovolemia.
Weak pulses- Hypoplastic left heart
syndrome or hypovolemia.
Pulses or blood pressures stronger in
the upper than in the lower extremitiescoarctation of the aorta.
50. Physical Examination
Congenital heart disease-
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.
CVS-
Presence or absence of a heart murmur is of little
assistance. Loud S2 suggests pulmonary or
systemic hypertension or malposition of the aorta.
several of the most serious anatomic
abnormalities, such as transposition of the
great arteries, produce only a very soft
murmur or no murmur at all
51. Physical Examination
Inspiratory stridorupper airway obstruction
ChestAsymmetric chest movement combined with
severe distress○ alarming sign for tension pneumothorax,
diaphragmatic hernia
Transillumination of the chest○ Pneumothorax on an emergent basis
53. Physical Examination
Central nervous depressionCauses shallow, irregular respirations and
periods of apnea.
Affected infants typically appear hypotonic
and lethargic.
54. Investigations
CBC & diff :
Increase or decrease 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 Increase fetal Hb affinity for O2
(left-shift)
Increase PaCO2: may indicate pulmonary or CNS
disorders, heart failure
Decrease pH: sepsis, circulatory shock, severe hypoxemia
Methemoglobinemia: Decrease SaO2, normal PaO2,
chocolate-brown blood
55.
If central cause- appropriate scan and
drug levels
Hb electropheresis…..Hb M
Decrease pH: sepsis, circulatory shock,
severe hypoxemia
Decrease pH: sepsis, circulatory shock,
severe hypoxemia
Sepsis screening
56. Pulse oximetry screening
Difficulty in visual detection of cyanosis
Potentially severe consequences of
missing an early sign of CHD
“5th vital sign”
Sensitivity and specificity variesCriteria used for abnormal test
Timing of screening
Probe site
Quality of the equipment
Signal quality and neonate behaviour
Health care workers expertise
Signal quality and infant behavior — Measurements should not be
performed when the infant is crying or moving as it reduces the quality of the
signal and the accuracy of the test.
57.
Oxygen saturation should be performed
initially on room air to serve as a
baseline.
Subsequently can be served to
differentiate between cardiogenic and
non-cardiogenic causes
58. Terms
PaO2
Arterial Oxygen Pressure
Measured on an ABG machine
Oxygen dissolved in plasma
○ 0.003 ml O2/mmHg/dl plasma
SaO2
Percent Oxygen Saturation
Measured by saturation monitor (pulse-Ox)
~1.34ml O2/g Hb
59. Hyperoxia test
If a low-pulse oximeter reading persists, it
may be appropriate to proceed to a
hyperoxia test. It is indicated if the pulse
oximeter reading is less than 85% in both
room air and 100% oxygen
Useful in distinguishing cardiac from
pulmonary causes of cyanosis.
60. Hyperoxia test
Arterial oxygen tension is measured in the
right radial artery (preductal) and in a lower
extremity artery while the patient breathes
100 percent oxygen (postductal).
61. Hyperoxia Test
Infant on Room Air, get ABG
Infant on 100% oxygen, get ABG
PaO2 unchanged = fixed shunt = CCHD
Max PaO2 <100 = CCHD
Max PaO2 >200 = No CCHD
62. Hyperoxia test
Disease
Lung disease is more likely than
CHD
Result- Increase
in PaO2
>150 mmHg
TGA or severe pulmonary outflow
obstruction
<50 to 60 mmHg
In lesions with intracardiac mixing
and increased pulmonary blood
flow such as truncus arteriosus-
>75 to 150
mmHg
63. Differential cyanosis
To detect differential cyanosis, oxygen
saturation should be measured in sites
that receive blood flow from both
preductal (right hand) and postductal
(foot) vessels. It is preferable to use the
right (rather than left) upper extremity,
since the left subclavian artery arises
close to the ductus arteriosus, and some
of its flow may come from the ductus
and thus not reflect preductal values
64. Chest X-Ray
To identify pulmonary causes of cyanosis:
pneumothorax, pulmonary hypoplasia, diaphragmatic
hernia, pulmonary edema, pleural effusion, etc.
Useful in evaluating congenital heart disease: e.g.,
cardiomegaly & vascular congestion: heart failure
Aberrancy of the cardiothymic silhouette Suggest the presence of structural heart disease,
and
Abnormalities of the lung fields may be helpful in
distinguishing a primary pulmonary problem such as
meconium aspiration
65. Chest X- Ray
Pulmonary vascular markingsDecreased in CHD with obstructed
pulmonary blood flow such as tetralogy of
Fallot, severe pulmonary stenosis or atresia,
and tricuspid atresia.
Increased in admixture lesions like
transposition of the great arteries, total
anomalous pulmonary venous connection,
and truncus arteriosus.
69. Echocardiography
Indicated if abnormal cardiac
examination suggestive of congenital
heart defect, failed hyperoxia test
(cardiac disease suspected) or has
unclear diagnosis
70. Treatment
GoalsProvide adequate tissue oxygen and CO2
removal
PrinciplesEstablish airway
Ensure oxygenation
Ensure adequate ventilation
Correct metabolic abnormalities
Alleviate the cause of respiratory distress
71.
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 meds (if needed): umbilical vessels
convenient for placement of intravenous & intraarterial
catheters
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.
.
72.
An infant who fails the hyperoxia test & does
not have PPHN or a CXR showing pulmonary
disease likely has a congenital heart defect
that’s ductus-dependent. If cardiac disease is
suspected, immediately start PGE1 infusion.
Complications of PGE1 infusion include
hypotension, tachycardia, apnea. Secure a
separate intravenous catheter to provide fluids
for resuscitation and ensure accessibility of
intubation equipment should they be required
73. Treatment
Prostaglandin E1
For ductal dependant CHD/ reduced
pulmonary blood flow- Fail hyperoxia
test( An arterial PO2 of less than 100 torr in
the absence of clear- cut lung disease)
Infusion of prostaglandin E1 at a dose of
0.05- 0.1mcg/kg/min intravenously to
maintain patency
74. Treatment
Prostaglandin E1S/E- hypoventilation, apnea, edema and low
grade fever
Benefits- Can be stabilized more easily,
allowing for safe transport to a tertiary care
center. More time is also available for
thorough diagnostic evaluation and patients
can be brought to surgery in a more stable
condition.
75. Conclusion
Identify those that are life-threatening.
complete maternal and newborn history
perform a thorough physical examination
recognize the common respiratory and
cardiac disorders
differentiate among various diagnostic
entities
For ductal dependent lesion, start
prostaglandin E1 and early referral
Methemoglobinemia- An oxidized form of hemoglobin, cannot carry oxygen and, when present in significant quantities, will cause cyanosis. Acquired or congenital disorder due to nicotinamide adenine dinucleotide (NADH) cytochrome b5 reductase deficiency and hemoglobin M disorder.
Lithium- Ebstein anomaly
Fetal hydantoin synd- PS, AS
Fetal alcohol- VSD,ASD
Connective tissue disorder- congenital complete heart block associated with anti-Ro/SSA and anti-La/SSB antibodies.
Congenital infections- cytomegalovirus, herpesvirus, rubella, or coxsackie virus can lead to cardiac structural abnormalities or functional impairment.
several of the most serious anatomic abnormalities, such as transposition of the great arteries, produce only a very soft murmur or no murmur at all.
Probe site — Postductal probe placement is the optimal site because defects with right-to-left shunting of desaturated blood through the ductus arteriosus will not be detected with preductal placement.
Signal quality and infant behavior [28] — Measurements should not be performed when the infant is crying or moving as it reduces the quality of the signal and the accuracy of the test.