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Approach to a Neonate with

Cyanosis
Dr. Sunil Agrawal
1st yr MD pediatrics

Contents

Introduction
Central, Peripheral and Differential cyanosis
Mechanism
Etiology
Approach
Principles of Treatment
Conclusion
References

Introduction
Bluish discoloration of the tissues that
results when the absolute level of reduced
hemoglobin in the capillary bed exceeds 3
g/dL
Depends upon the total amount of
reduced hemoglobin rather than the ratio
of reduced to oxygenated hemoglobin.

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.

Peripheral cyanosis
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

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 .

Differential cyanosis
Upper half of the body is pink and the
lower half cyanotic, or vice versa
Requires pulmonary vascular resistance
elevated to a systemic level and a patent
ductus arteriosus.

Mechanism of cyanosis

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

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.

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. Reproduced with permission from:
Lees, MH. Cyanosis of the newborn infant. J Pediatr 1970;
77:484.

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.

Factors affecting the detection of


cyanosis in the newborn
Other physiologic factors common in sick
newborns affect the oxygen dissociation
curve.

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). Reproduced with permission from: Levin, AR.
Management of the cyanotic newborn. Ped Ann 1981; 10:127. Copyriht 1981
SLACK, Inc.

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.

Etiology

Non- cardiac causes


Alveolar hypoventilation
Central nervous system depression: asphyxia,
maternal sedation, intraventricular
hemorrhage, seizure, meningitis, encephalitis
Neuromuscular disease: Werdnig-Hoffman
disease, neonatal myasthenia gravis, phrenic
nerve injury
Airway obstruction: choanal atresia,
laryngotracheomalacia, macroglossia, Pierre
Robin syndrome

Non- cardiac causes


Ventilation/perfusion mismatch
Airway disease: pneumonia, aspiration, cystic
adenomatoid malformation, diaphragmatic hernia,
pulmonary hypoplasia, labor emphysema, atelectasis,
pulmonary hemorrhage, hyaline membrane disease,
transient tachypnea of the newborn
Extrinsic compression of lungs: pneumothorax,
pleural effusion, chylothorax, hemothorax, thoracic
dystrophy

Non-cardiac causes
Hemoglobinopathy
Methemoglobinemia: congenital or secondary to toxic
exposure
Other hemoglobinopathies

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

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

Cardiac causes
Severe heart failure Hypoplastic left heart syndrome
Coarctation of the aorta
Interrupted aortic arch
Critical valvular aortic steanosis

Mnemonic
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).

Differential cyanosis
With normally related great arteries, oxygen
saturation may be higher in the upper than lower
extremity in patients if there is right-to-left
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

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.

Approach

Aim
Differentiate physiologic from pathologic
cyanosis
Differentiate cardiac from non- cardiac
cause of cyanosis
Find cause which needs urgent treatment
or referral

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

Perinatal history
Drug intake
Causing neonatal depression
Lithium- Ebstein anomaly
Phenytoin- PS and AS

Maternal diabetes TGA, ventricular septal defect (VSD), and


hypertrophic cardiomyopathy

Connective tissue disorder- Heart blocks


Congenital intrauterine infections
Antenatal fetal echocardiography

History
Methemoglobinemia may be acquired
following exposure to aniline dyes,
nitrobenzene, nitrites, and nitrates.

Onset of cyanosis in cardiac lesions Depends on Nature 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

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

History- Risk factors


Pneumonia/ sepsis

PROM
Foul smelling liquor
Maternal pyrexia
Maternal GBS

TTN
Birth by cesarean section
with or without labor
Male sex
Family history of asthma
(especially in mother)
Macrosomia
Maternal diabetes

Polycythemia small-for-gestational age

MAS Post maturity


Small for gestational age
Placental dysfunction
Fetal distress
Meconium stained liquor
Pneumothorax Aggressive resucitation
IPPV
Meconiun aspiration
HMD
Hypoplastic lung
Staph pneumonia

Hyaline membrane
disease Premature infant
Infant of diabetic mother

History
Choanal atresia Cyanosis decreases during crying
Confirmed by failure to pass a soft No. 5F to
8F catheter through each nostril

Physical Examination
Vital signs Hypothermia 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.

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.

Physical Examination
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 on an emergent basis

Physical Examination
P/A Scaphoid abdomen
Congenital diaphragmatic hernia

Hepatosplenomegaly congestive heart failure, maternal diabetes,


or congenital infection.

Physical Examination
Central nervous depression Causes shallow, irregular respirations and
periods of apnea.
Affected infants typically appear hypotonic
and lethargic.

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 varies

Criteria used for abnormal test


Timing of screening
Probe site
Quality of the equipment
Signal quality and neonate behaviour
Health care workers expertise

Pulse Oximetry
Oxygen saturation should be performed
initially on room air to serve as a baseline.
Subsequently can be served to
differentiate between cardiogenic and noncardiogenic causes

Limitations of pulse oximetry

effects of ambient light


skin pigmentation
dyshemoglobinemia
low peripheral perfusion states
motion artifact

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
It is not recommended in preterm infants.
Useful in distinguishing cardiac from
pulmonary causes of cyanosis.

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).
Pulse oximetry cannot be used- in neonate given
100% inspired O2 a value of 100% saturation
may be obtained with an arterial PO2 ranging
from 80 torr( abnormal) to 680 torr (normal)

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

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

Investigation

Hematocrit or hemoglobin
Sepsis screening
Blood glucose concentration
Arterial blood gases (Pao2, Paco2, pH)
Blood cultures
Electrocardiography
Echocardiography, cardiac catheterisation,
angiocardiography
Hemoglobin electrophoresis- Hb M

Chest X-Ray
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

Chest X- Ray
Pulmonary vascular markings Decreased 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.

Total Anomalous Pulmonary


Venous Return
Snowman

Tetralogy of Fallot
Boot
shape

Transposition of Great Arteries


Egg on a
string

Investigation
If central cause appropriate scan and drug levels

Methemoglobinemia Place few drops of pt blood on filter paper


Appear chocolate brown

Treatment
Goals Provide adequate tissue oxygen and CO2
removal

Principles

Establish airway
Ensure oxygenation
Ensure adequate ventilation
Correct metabolic abnormalities
Alleviate the cause of respiratory distress

Treatment- Buy time


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 0.050.1mcg/kg/min intravenously to maintain
patency

Treatment- buy time


Prostaglandin E1 S/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.

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

References
Nelson textbook of pediatrics
Cloherty manual of neonatal care
Approach To Cyanotic Heart Disease In The First Month
Of Life , Harry J. D'Agostino, Jr., M.D. and Eric L.
Ceithaml, M.D.
Pediatrics in Review. 1999;20:350-352.)
1999, Consultation with the Specialist, Nonrespiratory
Cyanosis, Jon Tingelstad, MD
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