CYANOTIC CONGENITAL

HEART DISEASE

Cyanosis
(form the Greek word meaning dark blue)

What is cyanosis?
Blue discolouration of lips / tongue / extremeties
Oxygenated Hb is bright red
Reduced Hb is blue / purple

Cyanosis is dependent upon the absolute

concentration of reduced Hb.
TcSaO2<85% OR > 3g deoxygenated Hb in
arterial blood
If present through the entire body= CENTRAL

Lees
1970

Consequences and complications

Polycythemia
Clubbing
CNS complications
Bleeding disorders
Hypoxic spells and squatting
Depressed I.Q
Scoliosis
Hyperuricemia and gout

Steps in the management of cyanotic

newborns:
1.

2.
3.

CHEST XRAY:

May reveal pulmonary cause

May hint to the presence or absence of cardiac lesion

ECG:

If cardiac disease suspected will give clue to diagnosis

ARTERIAL BLOOD GAS IN ROOM AIR:

Confirm or reject cyanosis

Elevated pCO2 suggests pulmonary or CNS cause
LOW pH in severe shock, sepsis, severe hypoxaemia

4.

HYPEROXIA TEST

5.

Umbilical artery line

6.

PROSTAGLANDIN E1:

If heart lesion suspected that is ductus dependent (eg pulmonary

atresia,Tetralogy of Fallot, TGA, Coarctation of the aorta)

The hyperoxia test

Cyanosis confirmed with arterial oxygen
measurement
to differentiate between respiratory and
cardiac causes of cyanosis
administer oxygen at the highest
concentration possible (head box)
blood gas from Right radial artery
Wait for 15 minutes repeat ABG
pO2 < 150 mm Hg (20 kPa) - cardiac
pO2 > 250 mm Hg (33 kPa) - respiratory

Failures of the Hyperoxia

test
Cyanotic heart defect with large
pulmonary blood flow (eg TAPVC)
pO2 may rise with O2 administration.
Massive intrapulmonary shunts but a
normal heart (eg PPHN, AVM) may
not raise the pO2 with oxygen pO2
wont rise with O2.
Response to oxygen inhalation must
be interpreted in the light of the
clinical picture

Cyanotic Congenital Heart Disease

1)Cyanosis with

PBF

a)TOF
b)Pulm. Atresia
c)Tricuspid Atresia
d)Critical PS
arteriosus

2) Cyanosis with

PBF

a) D-TGA
b) DORV
c) TAPVC
d) Truncus

Tetraology of Fallot

1. VSD
2. RVOT Obstruction
3. RVH
4. Overriding aorta

TETRALOGY OF FALLOT
Infundibular PS
Aorta
overrides
septum

LA

VSD

RA

LV
RV
RVH

Two Important Abnormalities

NonRestrictive VSD
Large enough to equalize pressures
in both ventricles

Degree of RVOT

Clinical Presentation of Cyanotic TOF

Cyanosis, clubbing, dyspnea on
exertion, squatting, hypoxic
spells.
Loud systolic ejection murmur,
systolic thrill at middle LSB
,RVH, no cardiomegaly.
Soft murmurs are associated
with less blood flow and more

TETRALOGY
OF FALLOT

Ejection systolic
systolic
Ejection
murmur
murmur
Single S2
S2
Single

RV
HYPERTROPHY

NO RV
RV heave
heave
NO
INFUNDIBULAR
STENOSIS
AORTIC OVERRIDE

VSD

NO
NO CYANOSIS
CYANOSIS
MILD
MILD CYANOSIS
CYANOSIS

CXR- boot shaped

Hypoxic Spell
(TET Spell)

Peak incidence of 2-4 months

Characterized by:
Hyperapnea (Rapid and deep
respirations)
Irritability and prolonged crying
Inc cyanosis
Decreased heart murmur

?
Spasm
of
RVOT
Decrease
d SVR

crying
Increased
R-L shunt

Hypoxic spell
Increased
systemic
venous return

Hyperapnea

Decreased
pO2
Increased
pCo2
Decreased pH

TETRALOGY
OF FALLOT
HYPERCYANOTIC
SPELL

POSITIVE
FEEDBACK

CYANOSIS
ACIDOSIS

Tachypnoea
Tachypnoea
NO ejection
ejection
NO
systolic murmur
murmur
systolic
Death
Death

Cyanotic Spells
Increasesystemicvascularresistance
Squat/Kneechestposition
Ketamine1-2mg/kgIV
Neosynephrine0.02mg/kgIV

TachycardiaPropranolol0.1mg/KgIV
Releaseofinfundibularspasm

Irritability
Morphine0.2mg/KgS.CorIM

HypoxiaOxygen
DehydrationVolume

Acidosis

NaHco3 1mEq/ Kg IV

PALLIATED TETRAOGY
Blalock-Taussig shunt

LA

RA

LV
RV

Other palliative surgeries:

Pott`s shunt ,waterton`s shunt

Treatment
Early surgical repair depending
on pts weight
VSD is closed and obstructing
ventricular muscle is removed

TRANSPOSITION OF THE GREAT

ARTERIES
Pulmonary Artery from LV

Aorta from RV
LA
RA
RV

LV

Complete Transposition of the Great

Arteries

Complete
separation
of the 2
circuits
Hypoxemic
blood
circulating in
the body
Hyperoxemic
blood
circulating in
the pulmonary
circuit

TGA
RA

RV

RA

RV

BOD
Y

PA

BOD
Y

PA

AOR
TA

LUN
GS

AOR
TA

LUN
GS

LV

LA

LV

LA

TGA
LA
ENLARGEMENT

RV

Aorta

SEVERE
CYANOSIS

LV

PA

CCF

LV
ENLARGEMENT

Two separate
separate
Two
parallel circuits
circuits
parallel
Incompatible with
with
Incompatible
life
life
No murmurs
murmurs
No

Clinical Symptoms
Depend on anatomy present
No mixing lesion and restrictive PFO
Profound hypoxia, deeply cyanotic baby
Rapid deterioration
Death in first hours of life
Absent respiratory symptoms or limited
to tachypnea
Single second heart sound (aortic),
cardiomegaly, no murmurs

Clinical Symptoms
Mixing lesion present (VSD or
large PDA)
Large vigorous infant
Cyanotic, a VSD murmer may be
audible
Little to no resp distress
Most likely to develop CHF(LHF) in
first few weeks of life
excessive sweating (a cold, clammy
sweat often noted during feeding);
poor feeding, slow weight gain,

CXR

Egg shaped
cardiac
silhouette
Narrow
superior
mediastinu
m

Management
Prostaglandin to
establish patency of the
ductus arteriosus
Increases shunting from aorta
into the pulmonary artery
Increases pulmonary venous
return distending the left
atrium
Facilitates shunting from the
left to the right atrium of fully
saturated blood across the
foramen ovale.

Management
Therapeutic balloon atrial
septostomy (Rashkind
Procedure) if surgery is not
going to be performed
immediately
Improves mixing and pulmonary
venous return at the atrial level

TGA

IVC

Rashkind
atrial
septostomy

PDA

Followed by
by ::
Followed
Arterial switch
switch or
or
Arterial
Mustard operation
operation
Mustard

Treatment

Surgery consists of switching the

right and left sided structures at
the atrial level, at the ventricular
level, or at the great artery level
(Jatene`s switch operation).

Total Anomalous Pulmonary Venous

Return
The pulmonary veins drain into
the RA or its venous tributaries
rather than the LA
A interatrial communication
(ASD or PFO) is necessary for
survival
Pulmonary venous return
reaches the RA
Systemic and pulmonary venous
blood are completely mixed

4 Types
1. Supracardiac
Common pulmonary vein drains
into the SVC via the left SVC and
left innominate vein.

2. Cardiac
The common PV drains into the
coronary sinus

3. Infracardiac
The common PV drains into the
portal vein, ductous venosus,
hepatic vein, or IVC.

Infracardiac-type TAPVC. Pulmonary

venous blood draining through the liver to
reach the IVC and right atrium.

4. Mixed
A combination of the other types

Clinical Signs for Unobstructed Veins

Mild cyanosis, signs of CHF in
infancy, history of pneumonia
Widely split S2, quadruple
rhythm, Grade 2-3/6 systolic
murmur heard at the ULSB
CXR- marked cardiomegaly

Like
ASD

Clinical Signs for Obstructed

Veins
Profound desaturation, severe cyanosis,
respiratory distress from pulmonary edema
P2 loud and single
Acidosis
Ground glass appearance on CXR
PGE1 administration does not improve oxygenation
because elevated pulmonary pressures in the right
side of the heart (due to obstructed pulmonary
outflow) will result in right to left shunting across
an open ductus further decreasing arterial
saturation.

Like

MS

Treatment
Digitalis and diuretics to treat
heart failure
Intubation and inc PEEP for
those with severe pulm over load
Corrective surgery

Tricuspid Atresia
Tricuspid valve is absent
RV and PA are hypoplastic
Associated defects- PFO/ASD/
VSD or PDA (necessary for
survival)
Dilation of LA and LV
Essentially single
ventricle physiology

TRICUSPID ATRESIA

LA
RA

VSD

LV

Atretic TV

Clinical Signs
Severe cyanosis, poor feeding,
tachypnea
Single S2, grade 3/6 systolic murmur
at LLSB if VSD is present, PS murmer
may present
Superior QRS axis, with RAH, LVH
CXR- boot shaped heart

Treatment
PGE IV infusion
1. Blalock-Taussig shunt in infancy
systemic to pulmonary arterial shunt
Provide stable blood flow to the lungs
A gortex tube is sewen between the
subclavian artery and the right
pulmonary artery

2. Bidirectional Glenn

(till age of

4-6yrs)

Superior vena cava is connected

to the right pulmonary arteries
IVC continues to be connected to
the heart

3. Fontan Procedure

(final procedure)

Redirects IVC to lungs

Truncus Arteriosus, Single

ventricle

A single trunk leaves the heart

Gives rise to pulm, systemic, and
coronary circulations
Large VSD is always present

TRUNCUS ARTERIOSUS
Aorta
Pulmonary
artery
Truncus
VSD
RA
LV
RV

Clinical Signs
Cyanosis immediately after birth
Early signs of CHF
2-4/6 systolic murmur at LSB suggestive of
VSD
In single ventricle there
is poor QRS progression

Treatment
Anticongestive medications (diuretics
and digitalis)
Corrective surgery
VSD is closed
Pulmonary artery is separated from the
truncus
Continuity is then established between the
right ventricle and the pulmonary artery
utilizing a valved homograft conduit

Thank you