Hanan Fathy

Pediatric Nephrology Unit

Gas exchange across the alveolar-capillary barrier
can be described as either perfusion-limited
or diffusion-limited:
• 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
Increased Hemoglobin Affinity for
Oxygen--a left
shift of the curve...easy to load
oxygen...difficult to unload

Decreased Hemoglobin Affinity for

Oxygen-- a right shift
of the curve...difficult to load...easy to
unload
 Causes of shifts to the right

↑PCO2, ↓pH, ↑ temperature,

↑[2,3-DPG]

Causes of shifts to the left

↓PCO2, ↑pH, ↓
temperature, ↓[2,3-DPG]
• Bluish discoloration of skin and mucous
membranes

• Noticeable when the concentration of

deoxy-hemoglobin is at least 5g/dl
(O2 sat < 85%)
• 5g/dL of reduced Hb  clinical cyanosis

• Hb 15  cyanosis at 75-80%
• Hb 20  cyanosis at 80-85%
• Hb 6  cyanosis at 45-50%
• patient whose hemoglobin content is 15 g/dL
(hematocrit approximately 45%) would not
generate 5 g/dL of reduced (ie, deoxygenated)
hemoglobin in the capillaries until his/her
SaO2 level reached about 85% (PaO2 50 mm Hg).

• When hemoglobin content is 9 g/dL (hematocrit

approximately 27%), the threshold SaO2 level for
manifesting cyanosis is lowered to about 73%
(PaO2 38 mm Hg). At this level of hypoxemia, the
patient would certainly have other manifestations
of hypoxemia (eg, respiratory symptoms, mental
status changes) apart from cyanosis.

• With a hemoglobin content of less than 9 g/dL,

the patient would likely succumb from hypoxemia
before cyanosis became evident.
Cyanosis has one of two causes,
which may act together:
• Reduction of the arterial saturation
(low S aO2)
• Or increase in the arterio-venous
oxygen content difference. 
(A large oxygen extraction is seen in
disorders with low bloodflow).

• Central cyanosis may be simulated

by methaemoglobulinaemia and
sulphaemoglobulinaemia.
 Central Cyanosis

Impaired pulmonary Right-to-left shunting

function of blood
1. Airway obstruction
2. Pulmonary diseases
Cyanosis (central, in children)

• This is cyanosis that is caused by

reduced oxygen saturation of the
systemic arterial blood.
Etiology
• Possible causes include:
• cardiac shunt - where the venous
blood enters the left side of the heart
without passing through the lungs, ie.
a right to left shunt
• pulmonary shunt - where there is
inadequate oxygenation of the blood
as it passes through unventilated
alveoli
Cyanosis (central, in children)

• Parenchymal lung disease

• Right to left cardiac shunt - congenital
cyanotic heart disease
• Decreased PO2 of inspired air - high altitude
• Hypoventilation
Parenchymal lung disease

• Airway obstruction,pneumonia,massive
pulmonary embolism, pulmonary
edema,chronic airflow obstruction
emphysema

• Seriously impaired pulmonary function,

through perfusion of unventilated or
poorly ventilated areas of the lung or
alveolar hypoventilation, resulting in
decreased alveolar PO2 and SaO2
Shunting of systemic venous blood into the
arterial circuit

• Congenital cardiac lesion :

tetralogy of Fallot (the combination of ventricular
septal defect and pulmonary outflow tract
obstruction ).

• Pulmonary arteriovenous fistulae :

congenital or acquired, solitary or multiple,
microscopic or massive.
Associated features
• Possible clinical features include:

• The warm mucous membranes are blue, for

example the tongue, the inside of the lips

• Central cyanosis increases immediately on

exercise which is not the case for peripheral
cyanosis

• Often there is polycythaemia with an

abnormally high haemoglobin and haematocrit;
this must not be confused with neonatal
polycythaemia which may mimic cyanosis

• Clubbing is often seen in patients with central

cyanosis

• Note that the absolute discriminating feature

between central and peripheral cyanosis is
obtained from testing the oxygen saturation of
arterial blood.
Chronic cyanosis causes clubbing of the digits
Cyanosis (peripheral)

• This is due to poor peripheral circulation.

• Possible causes:
• All causes of central cyanosis cause
peripheral cyanosis
• Low cardiac output e.g. heart failure
• Vasoconstriction e.g. due to low ambient
temperature, Raynaud's phenomenon
• Arterial obstruction e.g. atheroma
• Venous obstruction
peripheral vs central

peripheral central

• Sluggish blow flow in • Abnl of lungs or heart

capillaries that interferes w/ O2
• transport
Involves extremities
(acrocyanosis) • Involves trunk &
• mucous membranes
Spares trunk &
mucous membranes
Possible causes of mixed cyanosis

• all causes of central

cyanosis may lead to
peripheral cyanosis

• low cardiac output e.g.

heart failure
Cyanosis due to abnormal Hb derivatives

Central cyanosis may be

simulated by
methaemoglobulinaemia and
sulphaemoglobulinaemia.
 Evaluation of cyanosis: 100% O2 test
measure PaO2 in room air and 100% O2

Lung disease: Cardiac disease:

1. Room air PaO2 30 mmHg O2
1. Room air PaO2 30 mmHg O2
sat=60%
sat=60%
2. 100% O2 PaO2 40 mmHg O2
2. 100% O2 PaO2 110 mmHg O2
sat=75%
sat=100%

PaO2 >100 mmHg suggests lung disease

Little or no change in PaO2 O2 suggests
cyanotic heart disease
• 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
• History (age, gender, family disease history)

• Clinical differentiation of central as opposed to

peripheral cyanosis

• The presence or absence of clubbing of the digits

• Determination of PaO2 tension and SaO2

• Spectroscopic and other examinations of the

blood for abnormal types of hemoglobin (critical
in the differential diagnosis of cyanosis)
• particularly the onset (cyanosis
present since birth is usually due to
congenital heart disease)

• possible exposure to drugs or

chemicals that may produce
abnormal types of hemoglobin
• Determination of arterial
oxygen saturation
• Oximetric studies
• physical or radiographic
examination ,
echocardiography, right heart
catherization and
angiocardiography
• Spectroscope
• Cyanosis + Dyspnea
Disorders of respiratory or cardiovascular
system

• Cyanosis with mild or no dyspnea

Methemoglobinemia
Sulfhemoglobinemia: Spectroscopy helpful

• Cyanosis + clubbing
Severe, long duration
• Classification of CHD:
Acyanotic versus cyanotic

Acyanotic Cyanotic

Pulmonary Obstruction to Mixed

Pulmonary
Blood flow Blood flow blood flow
blood flow
from ventricles
•Atrial •Coarctation •Tetrology •Transposition
septal of Aorta of Fallot Of great
defect •Aortic •Tricuspid vessels
(ASD) stenosis atresia •Truncus
•Ventricul
• Placenta is the source of oxygen for the
fetus, it has 2 arteries and 1 vein.

• Fetal lungs receive less than 10% of the

blood volume ; lung don’t exchange gas.

• Right atrium of fetal heart is the

chamber with the highest oxygen
concentration.
The three openings that close at birth
are:
• Ductus Arteriosus connects the pulmonary artery
to the aorta, bypassing the lungs

• Ductus Venosus connects the umbilical vein and

the inferior vena cava bypassing the liver.

• Foramen Ovale is the opening between right and

left atrias of the heart , bypassing the lungs.
 C Y A N O T I C

R V L V

O l i g e P m l ei at h O o lr i a g e P m l ei at h

T O F T G A T+ VA St r De A s V i a S
P A t r E e i s s i ea n
•Severe Cyanosis PaO2 ≤ 40 mmHg
• Decreased pulmonary blood flow
• Tricuspid atresia, intact
ventricular septum
• Pulmonary atresia (with or
without other lesion)
• Critical pulmonary stenosis
• Ebstein’s anomaly
• Tetralogy of Fallot*
• Chest X-Ray
Decreased pulmonary vascular markings
Huge cardiac silhouette in Ebstein’s
anomaly
“Boot-shaped heart” in Tetralogy of
Fallot
•MIXING LESIONS PaO2 > 40 and ≤ 100 mmHg
•Clinical Characteristics

• Transposition/ VSD
• Total anomalous
pulmonary venous return
• Truncus arteriosus

•Arterial oxygen content depends upon the relative amounts

of pulmonary and systemic blood flow
•Left sided obstruction
• Clinical Characteristics
• Can present during newborn
period when ductus arteriosus
closes if severe
• If left-sided obstruction coexists
with a left-to-right shunt or
mixing lesion, can cause earlier
symptoms
• Can present due to murmur,
pulse abnormality, blood
pressure abnormality, or shock
• Too much oxygen can hurt
steals blood flow from systemic to pulmonary
High sats – low blood pressure
Lower sats – better blood pressure
• A polycythemic newborn with a hemoglobin of 20 g/dL
requires an arterial oxygen saturation below 75–85% (a P
O2 of 40–45 mm Hg) before appearing cyanotic.
This arterial desaturation may be a result of
• (1) structural defects, such as transposition of the great
arteries, tetralogy of Fallot, total anomalous pulmonary
venous return, tricuspid atresia, or truncus arteriosus;
• (2) intracardiac right-to-left shunting, which occurs at the
foramen ovale and ductus arteriosus in newborns with
persistence of the fetal circulation due to high pulmonary
vascular resistance, but an otherwise structurally and
functionally normal heart; or
• (3) left heart obstructive lesions, such as critical aortic
stenosis or coarctation, due to associated pulmonary
edema and pulmonary venous oxygen desaturation.
• primary lung disease, including parenchymal and airway or
diaphragmatic abnormalities, must be differentiated from
cardiovascular etiologies as a cause of cyanosis.
 PPHN

Normal Arterial Number Decreased Arteries e.g. CDH

Normal muscularization Increased muscularization

Maladaptation due
to acute injury
Developmental Chronic injury Malform-
(commonest) e.g.
immaturity with vascular ation
Sepsis, Meconium
aspiration synd., remodeling
asphyxia
Primary lung disease
• RDS, pneumonia, hypoplasia, CCAM, lymphangectasia,
lobar
• Emphysema

• Mechanical interference with lung function

• Congenital diaphragmatic hernia, pneumothorax,
pneumomediastinum, chylothorax, choanal atresia,
vascular ring, tracheal esophageal atresia, anomalies of
the airway/larynx, Pierre-Robin/ micrognathia

• Pulmonary hypertension
• Meconium aspiration, pneumothorax, RDS, sepsis,
pneumonia
CENTRAL NERVOUS SYSTEM
Seizure, meningitis, encephalitis, severe
IVH, subdural or subarachnoid
hemorrhage,
• Severe hypermagnesemia
• Shock
• Sepsis, hypovolemia, adrenal crisis
• Severe hypoglycemia (IDM, SGA babies)
• Congenital neuromuscular condition
(Werdnig-Hoffman)
• - METHEMOGLOBINEMIA
Newborn Presentation of CHD

• Cyanosis
- Usually minimal symptoms
- First 24-72 hours of life
- Duct-dependent pulmonary
blood flow
• CHF/Circulatory Collapse/Shock
- First 2 weeks of life
- Ductal-dependent systemic
blood flow
• Secondary end-organ dysfunction
- Heart, Brain, Kidneys, GI
Evaluation of the Cyanotic Neonate

• Detecting cyanosis:
• ambient lighting
• skin color
• hemoglobin
• if Hg is 20 gm/dl; 4 gm desaturated-visible cyanosis
• if Hg is 10 gm/dl; 2 gm desaturated-not cyanotic
• Check Saturation

• Hyperoxia Test to Determine Intrapulmonary vs.

Intracardiac Shunt
Hyperoxia Test

1. DANGERS: Pulmonary
overcirculation
Closing the PDA
Death
 Pulse Oximetry

•easy to use, harmless when

done correctly
•accuracy of 2% in the range of
70 to 100%
•consider cyanotic when Sat
<94% at 24 hours of age
•should be obtained prior to
discharge from nursery
CHD in the Neonate -- Cyanosis

Chest Radiograph may be helpful:

• Decreased pulmonary vascular

markings with intracardiac right to
left shunting

• Normal/increased pulmonary
vascular markings with
intracardiac mixing of pulmonary
and systemic venous return
d-TGA CXR: “egg on a string”

• Narrow mediastinum
due to anterior-
posterior orientation
of great arteries and
small thymus

• Cardiomegaly is
present w/ increased
pulmonary vascular
markings
Tetralogy of Fallot - CXR

• Typical “boot-
shaped” heart
secondary to RVH
and small main
pulmonary artery
segment

•Pulmonary
vascular markings
are decreased
Supracardiac TAPVC - CXR

“Snowman”
appearance
secondary to
dilated vertical
vein, innominate
vein and right
superior vena
cava draining all
the pulmonary
venous blood
TAPVR - CXR
Infracardiac = Obstructed = Surgical
Emergency
Ebstein’s malformation of TV

• Massive RA dilation due

to severe tricuspid
regurgitation
• R to L shunt at atrial
level causes cyanosis
• Degree of cyanosis
related to size and
compliance of functional
RV
• Cyanosis usually
decreases as PVR falls
shortly after birth
 Cyanosis CHF/Shock

Rt to Lt shunting: Lt Ventricular Outflow

Tract Obstruction:
 Tricuspid atresia
 HLHS
 TOF/ Pulm
 Coarctation of Aorta/ AS
atresia
 Truncus arteriosus
 Ebstein’s
 TGA with VSD
anomaly
 TAPVR
Critical CHD: Initial Evaluation and
Management

• ABC’s
• Oxygen (judicial) to saturations of 80-85%
• Place umbilical lines
• PGE (0.025-0.1 micrograms/Kg/min)
• History
• Complete physical with four extremity BP’s
• Pre and post-ductal oxygen saturations
• Hyperoxia test
• CXR
• EKG
• Echocardiogram
Suspected CHD: Initial Evaluation and
Management
• Pre and post-ductal oxygen saturations
• If pre-ductal sat higher than post-ductal sat
(differential cyanosis)
• Left heart abnormalities (such as aortic arch
hypoplasia, critical aortic stenosis,
interrupted aortic arch)
• Persistent pulmonary hypertension
• If post-ductal sat higher than pre-ductal
(reverse differential cyanosis)
• TGA with CoA or TGA with IAA
• TGA with supersystemic pulmonary vascular
resistance
THANK

YOU