DR BIVIN WILSON

DNB REGISTRAR CARDIOLOGY

 Cyanosis -- Greek word meaning "dark blue")
 Cyanosis is dependent upon the absolute
concentration of the reduced hemoglobin
and not on the ratio of reduced hemoglobin
to oxyhemoglobin.
 Central cyanosis is one, which is present
throughout the body, including the mucous
membranes and tongue. If cyanosis is limited
to the extremities, it is called peripheral
cyanosis
 Differential cyanosis - PDA with reversal of
shunt or severe left sided obstructive lesions

 In Reverse differential cyanosis -TGA with

PHT&PDA and TAPVC above the diaphragm
with PDA. 
 General causes of Cyanosis

Cyanosis

Pulmonary Cardiac Others

Intrapulmonary Intracardiac
Airway disease
shunting shunting
 CAUSES OF CENTRAL CYANOSIS

A. Congenital Heart Disease

 
    1) Cyanosis with PBF 2) Cyanosis with PBF

a)      TOF a) D-TGA

 b)      Pulm. Atresia b) DORV
 c)      Tricuspid Atresia c) TAPVC
 d)      Critical PS d) Truncus arteriosus

 
 CAUSES OF CENTRAL CYANOSIS
 B) LUNG DISEASE D) CNS DEPRESSION
 
 a)      RDS a) IVH
 b)      Pneumonia b) Perinatal asphyxia
 c)      Pneumothorax c) Heavy maternal sedation
 d)      Pleural effusion
 e)      Diaphragmatic hernia
 f)       T.E.Fistula


  C) PERSISTENT PULMONARY E) MISCELLANOUS
HYPERTENSION
a) shock & sepsis
b) Hypoglycemia
c) Methemoglobinemia
d) Neuromuscular conditions
( Werdnig – Hoffman)
STEPS IN THE MANAGEMENT OF
CYANOTIC NEWBORNS
 ABG in room air: confirm or reject central
cyanosis.
An elevated PCO 2 suggests pulmonary or
central nervous system problems.
A low pH may be seen in sepsis, circulatory
shock, or severe hypoxemia
 Hyperoxia test:
 To differentiate between Cardiac from Pulmonary cause.
 Oxygen should be administered through a plastic hood for at least
10 minutes to replace the alveolar air completely with oxygen.
 With pulmonary disease, arterial PO 2 usually rises to> 150 mm
Hg.
 When there is a significant intracardiac right-to-left shunt, the
arterial PO 2 does not exceed 100 mm Hg, and the rise is not more
than 10 to 30 mm Hg.
 However in Persistent Pulmonary Hypertension of newborn with a
normal heart) PaO 2 may not have a rise in arterial PO 2 to 100
mm Hg.
 The purpose of positive hyperoxia test helps in ruling out
significant cyanotic congenital heart defect but when negative
does not differentiate between cyanotic heart and PPHN. 
 ECG may be helpful in cardiac origin of
cyanosis
 Chest x-ray films: may reveal pulmonary
causes of cyanosis. They can also hint at the
presence or absence of cardiac defects and
the type of defect

 2-D Echocardiography and a Doppler

examination
 RADIOLOGICAL FEATURES
 CXR may exclude non cardiac causes of cyanosis e.g. RDS. .
Meconium aspiration, Diaphgramatic hernia, Pneumothorax
Pulmonary Vascular Markings

Decreased Increased

Heart Size Heart Size

Normal Increased Increased

( “Boot shaped”) (“ Wall-to-Wall”)
TOF Ebstein (“ egg-on-end”)
D-TGA
Aortic Arch \ Mediastinum
Abdominal Situs
CLUES TO DIAGNOSIS
 TRANSIENT CYANOSIS – ASD,Ebsteins anomaly,
DORV with subaortic VSD without PS & Truncus
arteriosus

 INTERMITTENT CYANOSIS --Ebsteins anomaly,

TAPVC unobstructed,Complete A-V canal defect &
Eisenmengers with bidirectional shunt
 PERSISTENT AND PROGRESSIVE
CYANOSIS –TGA, Single ventricle,Hypoplastic
left heart Syndrome ,Tricuspid atresia
,Pulmonary atresia &Ebsteins anomaly

 DEFERRED CYANOSIS –TOF,TGA with VSD

with PS, Corrected TGA with VSD with PS,
DORV with VSD with PS & Single ventricle with
VSD with PS
CYANOSIS WITH FAILURE

Congestive cardiac failure in a patient with cyanosis

denotes cyanotic heart disease with increased
pulmonary blood flow physiology.

 1) Transposition of great vessels

 2) Taussing Bing anomaly
 3) Truncus arteriosus
 4) Total anomalous Pulmonary venous connection
 5) Single ventricle with low PVR and no pulmonary
stenosis
 6) Common atrium
CYANOSIS WITH SQUATTING

 1) Tetralogy of Fallot
 2) Tricuspid atresia
 3) Pulmonary atresia
 4) Double outlet right ventricle
 5) Single ventricle
 7) Eissenmengers syndrome
 CYANOSIS WITH COLLAPSING PULSE --TOF
with AR ,Truncus arteriosus TOF/PA with increased
aorto-pulmonary collaterals , Following shunt
surgeries & Cyanotic CHD with PDA

 CYANOSIS WITH a WAVE IN JVP -Tricuspid

atresia, Pulmonary atresia with intact IVS,TGA with
intact IVS, Eissenmengers ASD &TOF
 CYANOSIS WITH CONTINUOUS MURMUR
- Pulmonary atresia with VSD ,Truncus
arteriosus ,TOF with peripheral artery
stenosis ,Post shunt surgery,& Hypoplastic
left heart

 CYANOSIS WITHOUT MURMUR--PAH /

Eisenmenger,
CYANOSIS WITH LEFT AXIS
DEVIATION IN ECG

 Cyanotic heart disease with LAD in ECG are

 1) Tricuspid atresia
 2) Complete AV canal defect
 3) Single ventricle –LV type
 4) Large Pulmonary AV fistula
INTERVENTIONS
Ductus dependent lesions

 Those with severe RVOT obstruction:

Pulmonary atresia, TOF, Severe
pulmonary stenosis.
 Transposition of great arteries with
intact ventricular septum.
 Tricuspid atresia with pulmonary
stenosis.
 Hypoplastic left heart syndrome.
Interventions to maintain
ductal patency
 Prostaglandin infusion to maintain ductal
patency.

 Transcatheter intervention

 Endo vascular stents

PROSTAGLANDINS :

 PG E1 : 0.01 – 0.1 mcg / kg / min

 PG E2 : 0.003 – 0.01 mcg / kg / min

 Start low dose and increase at 15 – 30

mins. interval, if no response.
 > 3 fold increase in dose – rarely needed.

 Oral PG E 2 – 25 – 40 mcg / kg / h . go
stepwise to 4 – 6 hourly for c/h use.
Balloon atrial septostomy

 Introduced by Rashkind

 Improves mixing of oxygenated and

deoxygenated blood in pts with transposition
physiology or in those requiring venting of an
atrium with restricted outflow
 Better inter - atrial mixing.
 Transposition of great arteries

 Augmentation of L-R shunt

 Mitral atresia
 DORV with restrictive left A-V valve,
 Univentricular heart with restrictive left A-V valve,

 Augmentation of R-L shunt

 TAPVC
 Pulmonary atresia, tricuspid atresia
 Ebstein's anomaly
 Blalock – Taussig shunt
 Allows partially desaturated systemic blood to enter
the pulmonary artery, thus increasing pulmonary
blood flow, and hence, oxygenation.
 To avoid the problems of growth retardation of the
limb associated with the ligation of the subclavian
artery, modification was introduced
 Modification of the Blalock - Taussig shunt in which a
Teflon prosthetic tube is anastomosed end to side to
the undivided subclavian artery and end to side to the
pulmonary artery was first described by Klinner and
colleagues.
 Cyanotic Heart Disease
Tetralogy of
Fallot

Transposition
Of Great Arteries

Truncus
Arteriosus

Tricuspid
Atresia

Total Anomalous
Pulmonary venous Return
Tetralogy of Fallot (TOF)

1. RVOT
obstruction
2. VSD
3. Overriding
aorta
4. RV
hypertrophy
Tetralogy of Fallot (TOF)

1. RVOT
obstruction
2. VSD
3. Overriding
aorta
4. RV
hypertrophy
Hypercyanotic spell
 Primary problem is decreased pulmonary
blood flow with increased R->L shunting
 Typically occurs in morning of after sleep
(SVR is low, and blood volume is low)
 May be precipitated by activity or fright, but
may also be spontaneous
Hypercyanotic spell
 Cyanosis will be accompanied by
 Hyperpnea
 Increased rate and depth of respirations
 Increased fussiness progressing to decreased level
of consciousness
 Increasing acidosis, can be fatal
Hypercyanotic spell
 Theories
 Primary infundibular spasm (unlikely)
 Hyperpnea as a primary cause
 Circulating catecholamines
 Reduced SVR

 Spells are an indication for need of surgical

intervention
 Hypercyanotic Spells ( TET spells)
Morphine Hypovolemia Fluid

Syncope  Agitation Tachycardia

Oxygen
Impaired RV
 Cyanosis filling
Knee-chest

Rt Lt RVOT
shunt obstruction

 PVR Phenylepherine  Age

 Cyanotic Spells
 Increase systemic vascular resistance
Squat/Knee chest position
Ketamine 1-2mg/kg IV
Phenylephrine 0.02mg/kg IV

 Tachycardia Propranolol 0.1mg/ Kg IV

Release of infundibular spasm
 
 Irritability Morphine 0.2mg/ Kg   S.C or IM

 Hypoxia Oxygen
 
 Dehydration Volume

 Acidosis NaHco3 1mEq/ Kg IV

Surgical Palliation
Surgical Management
 VSD closure
 transatrial access if possible
 Infundibular resection for visualization
 Patch closure
 Relief of RVOT obstruction
 Infundibular resection vs. transannular patch
Transposition of Great
Areries (TGA)
 Aorta originating
from the right
ventricle, and
pulmonary artery
originating from the
left ventricle
 Accounts for 5-7%
of all congenital
heart disease
TGA .. Acute Management

 PGE-1 with no supplemental O2

Maintain ductus arteriosus patency, this will
increase the effective pulmonary blood flow, and
thence increase the left atrial pressure, therefore
inhance the left to right shunt at the atrial level
 Balloon atrial septostomy
Life saving procedure in the presence of inadequate
atrial septal defect
 TGA .. Surgical Management

 Arterial switch
 with re-implantation of the coronary artery to the new
aortic site.
 Atrial switch :
 the old style surgery
 Redirecting the pulmonary and systemic venous
return to result in a physiologically normal state
 The right ventricle remains the systemic ventricle
 Rarely needed
Truncus Arteriosus
 The presence of a
common trunk that
supply the systemic,
pulmonary and
coronary circulation
 Almost always
associated with VSD
 1.2-2.5% of all
congenital heart
disease
 Managment
 Acute management
 Diuretics
 Afterload reduction to enhance systemic blood flow

•Surgical management: complete

repair with VSD closure and
conduit placement between the
right ventricle and pulmonary
arteries
•Long term problems :
–truncal valve dysfunction
–RV conduit obstruction
Trcuspid Atresia
 Complete absence of
communication
between the right
atrium and right
ventricle
 About 3 % of
congenital heart
disease
Management
PBF

Decreased Increased

PGE-1, and minimal Afterload reduction

supplemental O2 to Diuretics
maintain ductal patency
Surgical Management
Single ventricle paliation
 First stage : to establish a reliable source of PBF
 Aorta to pulmonary artery shunt ( BT shunt)
 Pulmonary arterial banding in cases of increased PBF
 Second stage: Glenn Anastomosis ( superior
vena cava to pulmonary artery
 Third stage : Fontan anastomosis ( Inferior vena
cava to pulmonary artery
 TAPVC Treatment

 Correct acidosis

 Antifailure

 Surgery:
Anastomosis of
Common Pulmonry Vein to the left atrium
 Cyanotic Heart Disease
Tetralogy of
Fallot

Transposition
Of Great Arteries

Truncus
Arteriosus

Tricuspid

THANK
Atresia

Total Anomalous
Pulmonary venous Return

YOU