Congenital Heart Disease

Abbreviations

VSD ventricular septal defect

ASD atrial septal defect

PS pulmonary stenosis

AS aortic stenosis

HLHS hypoplastic left heart syndrome

TAPVC/D totally anomalous pulmonary venous connection/drainage

TA transposed great arteries

P!" patent foramen ovale

PDA patent ductus arteriosus

CAVC# complete Atrioventricular canal defect $PAPVC# partial%

ostium primum ASD&

AV valve# atrioventricular valve $usually mitral or tricuspid&

Congenital Heart Disease

Commonest group of life threatening

anomalies

'/())) live births

*
VSD +)#,)-. PDA ()-. ASD /-0
*
PS /-
*
Coarctation 1-. AS ,-
*
Tetralogy ,-. TA ,-
*
AV canal defects +-
!etal Physiology

2ight#to#left shunting at atrial level $P!"&

and at arterial level $ductus arteriosus&

High pulmonary vascular resistance

Little pulmonary blood flo3

Ventricles 3or4 in parallel

Presentation During !etal Life

Valve regurgitation $especially T2.

also CAVC. Truncus&

Arrhythmia
#
Slo3 $complete heart bloc4&
#
!ast $usually atrial arrhythmias&

5ay cause fetal loss or hydrops

Transition !rom the !etal
Circulation

Pulmonary vascular resistance falls

Ductus venosus and ductus arteriosus close

2ight#to#left shunting through foramen ovale

ceases
Timing of these events determines the
timing of presentation of congenital heart
defects
Ho3 3ill this
baby loo4 at
(6 hours of
age7
Presentation !irst 68 Hours

Critically ill - like

asphyxia

Cyanosis (may be mild)

Pure heart failure is

uncommon

5urmur
Wall to wall heart
Ebsteins anomaly
9bstein:s
anomaly

The tricuspid valve is

abnormal and inserts 3ell
do3n into the 2V0 There is
often severe trisuspid
regurgitation. 3hich can
lead to death in the fetus
or infant0 ;sually also 3ith
ASD so right#to#left flo3
results in cyanosis0
Totally anomalous pulmonary
venous drainage
$infradiaphragmatic&

All four pulmonary veins

drain to the right side0
<elo3 the diaphragm they
are al3ays obstructed0
=nfant presents in first
days 3ith cyanosis.
circulatory and respiratory
failure and collapse0
Supracardiac
TAPVD
Presentation !irst 68 Hours
Critically ill>

Valve regurgitation especially 9bstein?s.

absent pulmonary valve syndrome

"bstructed TAPVD

@9arlyA duct dependent presentation

Respiratory distress is usually not of

cardiac cause at this age
68 Hours to 6 3ee4s %
3 types of @Ductal DependentA Lesions

Depend on patent duct for either

*
pulmonary blood flo!
*
systemic blood flo! or
*
mixing of systemic and pulmonary blood
!allots 3ith
pulmonary atresia

<lue0 Bill need a

systemic# pulmonary
shunt0
Transposed reat
Arteries

<lue0 Presents 3ith

cyanosis 3hen the duct
closes0
=nterrupted aortic
arch/coarctation

Heart
failure/collapse
68 Hours to 6 Bee4s
Cyanotic @Ductal#DependentA Lesions

C"R helps in diagnosis

#
oligaemic lungfields
PS. pulmonary atresia etc
# plethoric lungfields
TA
# congestion
TAPVD
# massive cardiomegaly
9bstein:s
Bhat is the difference bet3een these t3o films7#
both are infants 3ith congenital heart disease0
Cyanotic infant# a lung
problem or a heart problem7

=nvestigations# suggests heart if>

#
CC2# clear lung fields and
#
9C# normal and
#
HyperoDia test normal $ breathing ())-
oDygen. if p"
6
E(,)mmHg very unli4ely to
be cyanotic heart disease&
#
$9chocardiogram is the definitive test&
68 Hours to 6 Bee4s
Heart failure @Ductal#DependentA Lesions

Heart failure is due to left heart

obstruction F"T L#2 shunts at this ageG
eg0
*
Coarctation
*
Critical Aortic stenosis
*
Hypoplastic left heart
*
5itral stenosis $rare&
Symptoms of cardiac failure in
the infant

Poor feeding

!ailure to thrive

!atigue
Signs of cardiac failure in the
infant

Tachypnoea

Tachycardia

Hepatomegally

S3eating
6 # 1 Bee4s
Decreasing Pulmonary Vascular 2esistance

Congestive heart failure due to L#2

shunt> eg0
*
Large VSD. PDA. CAVC
*
Truncus. single ventricle 3ith no PS
*
9arlier presentation if combined 3ith
left heart obstruction eg coarctation
Complete AV canal
defect

<ig L#2 shunt 3hen

the PV2 falls
Truncus arteriosus

There is a common
arterial trun40 The
trun4al valve is often
very abnormal0
Presents 3ith
cyanosis and heart
failure in the first fe3
3ee4s. as pulmonary
vascular resistance
falls0
Look for cyanosis and dysmorphology
Examination of CVS in the newborn
Diagnosis?
!acial features# of 3hat7
Bhat syndrome is associated 3ith
Truncus arteriosus/
tetralogy/interrupted aortic arch7
Dieorge syndrome

Thymic aplasia $T#cell. immune problems&

Hypocalcaemia $hypothyroid gland aplasia.

seiHures&

Dysmorphism $unusual facial appearance&

"utflo3 tract abnormalities in the heart

Feel the pulses
especially brachial and femoral
Look at the respiratory pattern and for evidence of recession
Feel the precordium for
hyperactivity
and for thrills
Locate the
apex beat
Feel for
hepatomegally
Listen carefully
Do what is necessary to
calm the baby down!
Listen at the back for radiation of murmurs
3 months old- severe failure to thrive
missed
!"D
"pecial tests-
echocardiography
Pulmonary veins # colour Doppler
Subcostal view
Apical view
TAPVC
<ig 2VG
!allots Tetralogy

A Case 3ith very small

pulmonary arteries. a
duct $and retroaortic right
subclavian artery&0

Duct dependant
To!

"verriding aorta

Anterior deviation of conal septum

!ind and define the PAs. and

nature of 2V"T/PV obstruction
Arrow indicates
anterior deviation
of conal septum
Br PAs
PA velocity
Fe3born 3ith heart failure#
heart rate I6))bpm# miles
from help# do 3hat m7
<lue ne3born# no respiratory
distress# miles from help# do
3hat7
Learn your #$!
2astelli operation

An eDample of a repair of
compleD CHD 3ith t3o
ventricles0 This one had a big
VSD. TA and PS0 The
severe sub#pulmonary
stenosis precluded an arterial
s3itch $it 3ould become the
neo#aortic valve&. so a
conduit Joins 2V to PA. and
the VSD is patched0
Trisupid atresia 3ith
a small VSD

The small VSD leads to

little flo3 into 2V in the
fetus. so the 2V is small0
After birth the infant is
blue $and needs a shunt&0
=f the VSD 3ere large. the
infant presents later 3ith
cyanosis and heart failure.
and 2V is normal siHed0
!ontan operation

The right atrium is

connected to the pulmonary
artery directly0 ;sed 3hen it
is not possible to have a t3o
ventricle repair $this case
has tricuspid atresia&0
Pulmonary vascular
resistance must be lo30 =t is
done at age 8 years or so0