UNIT 5 ECHOCARDIOGRAPHY IN

CONGENITAL HEART
DISEASE
Structure
5.0 Objectives
5.1 Introduction
5.2 Views in Paediatric Echocardiography
5.2.1 Sub-costal/Sub-xiphoid View
5.2.2 Apical View
5.2.3 Para-sternal View
5.2.4 High Para-stemalhctal View
5.3 Common Congenital Heart Disease
5.3.1 Atrial Septal Defect
5.3.2 Ventricular Septal Defect (VSD)
5.3.3 Patient Ductus Arteriosus
5.4 Let Us Sum Up
5.5 Answers to Check Your Progress
5.6 Further Readings

5.0 OBJECTIVES
After going through this unit, you should be able to:
know salient differences from adult echocardiography;
which probes to be used;
various view used in paediatric cardiology;
f
salient morphological features of various chambers identified in
echocardiography; and
description of common congenital heart disease like ASD,VSD, PDA.

5.1 INTRODUCTION
Echocardiography in paediatric cardiology forms a different category from that
of adult echo with acquired heart disease. Some of views which provide great
deal of information in patients with congenital heart disease (CHD) are seldom
used in adult echocardiography.

I Some of salient features about paediatric cardiology are enlisted below:

1) Congenital heart disease deals with structural heart where identification of

t chamber or vessel is done by it's own morphological characteristics and not
i by its adjacent structures. Echocardiography in CHD consists of a systematic
I segmental approach towards identification of structures and subsequent

I generation of composite diagnosis based on these findings.

Fundamentals of 2) Usually upsida down images are used in subcostal and apical views so to
Echocardiography resemble the mrmal position of heart.

3) Multiple windbws are used to evaluate every structure. It is possible because

of excellent echo windows in this age group.

4) One has to usei different transducer for different patients and various views.
Transducer of high frequency and greater resolution are routinely used. For
example in neonate 7.5 and 5.5 rnHz transducer produces optimal images.

5) Sedation of child is part of the procedure and it is accomplished by using

chloral hydrate (50-100mg/kg) or nasal midazolam.

6) Source of oxygen and suction facilities should be available in echo lab.

Measurement of cuff blood pressure and saturation monitoring is mandatory for
proper decision-making.
Morpohological features of various chambers identified in echocardiography:

5.2 VIEWS IN PAEDIATRIC

ECHOCARDIOGRAPHY
Various vies in paedilatric echocardiography are as follows:
5.2.1 S u b - ~ ~ ~ t a l / S ~ b - ~ View
iph~id Echocardiography in'
Congenital Heart Disease
In children excellent images can be obtained by this window. For the subcostal
views, the transducer is placed in the abdomen just below the xyphoid process of
the sternum and is tilted caudally and cranially to obtain various views (sweep).

1) Subxiphoid Long Axis Sweep (Fig.5.1, 5.2 and 5.3)

This sweep begins with keeping the transducer at subxiphoid region, positioning
directly posterior with marker pointing towards left. This cut provide the
relationship between abdominal aorta and IVC (aorta towards left of the IVC and
spine in visceral situs solitus and towards right in situs inversus). This view
decides the situs of viscera as well atrial situs because

Fig. 5.1: Sub-xiphoid long axis

Transducer Position for Subxiphoid long axis and short axis visceral situs has
good correlation with atrial situs. Now transducer is moved cranially .The most
posterior plane demonstrates systemic veins, right and left upper pulmonary
veins entering into the heart together with the coronary sinus at posterior aspect
of interatrial septum. In the next view all four chambers can be evaluated. In this
view fossa ovalis area is seen. Next two views are obtained by angulating
transducer further cranially. These views provide structural details of semilunar
valve, origin of great vessels, defects of perimembranous and muscular
interventricular septum.
Sweep 1 Sweep 2

IVC

Fig. 5.2: Subcostal long axis view

Fundamentals of
Echocardiography

Pig.5.3: Subcostal long axis view

2 ) Subxiphoid Short Axis Sweep (Fig. 5.4, 5.5 and 5.6)

To obtain the subxiphoid short axis view transducer should be rotated by 90

degree clockwise, fi-om the previous position so that pointer points inferiorly.
Sweep should start from right to left gradually until apex of heart is seen. Atria
including its venou$ connections and interatrial septum are visualized in right
sided cuts. All type of atrial septa1 defects, right upper and right lower
pulmonary veins cauld be seen in this view. Atrial morphology can be decided in
this view.

Fig. 5.4: Ttansducer positions for subxiphoid short axis sweeps

When transducer is rotated further towards left, aortic valve along with proximal Echocardiography in
ascending aorta, mitral and tricuspid valves are seen. Further rotation of Congenital Heart Disease
transducer provide view of pulmonary valve and main pulmonary artery. One
may decide the location of VSD in this view, sub infundibular muscle bundle
and mal-alignment of outlet septum, relationship between semilunar valves and
AV valves can be seen in this view by slight rotation of the transducer. Hence
these view are important while making the more complex diagnosis. Ventricles
can be seen in short axis view (circular LV and crescent shape RV). Muscular
VSDs at various plane can be located by this sweep.
Sweep 1

Sweep 2

Fig. 5.5: Atrial septum in subxiphoid short axis view

Apical two, four and five chamber views, parasternal long axis, short axis views
are obtained in same way as in adults.

Sweep 5

Fig. 5.6: Interventricular septum as seen in subxiphoid short axis view

5.2.2 Apical View

For the patient with dextrocardia transducer is kept on right chest with marker
towards left side. Morphological parameters to identify the ventricles are seen in
this view. Gradient across both AV valves and semilunar valves can be taken.
Fundamentals of
Echocardiography

Fig+5.7: Apical View-Transducer Position taken

This view is also helpful in recognizing various types of VSDs, displacement of

tricuspid leaflet (~bs/tein'sanamoly of tricuspid valve) and AVSD. ASD is never
diagnosed in this vidw because false dropouts are common due to angle between
ultrasound beam and inter atrial septum. A good four chamber apical view is
very helpful in makibg structural as well as functional diagnosis of heart disease.
If symmetry of four chamber view is lost one should look for dilatation or
hypoplasia of chambers. Total anomalous pulmonary venous drainage is
suspected if left atria is bald i.e. no pulmonary venous opening seen and PFO/
ASD is shunting right to left along with dilated RARV. Partial anomalous
pulmonary venous cbnnections (PA PVC) of single or multiple pulmonary veins
should be looked foi if right sided chambers are enlarged in absence of ASD and
RVOT obstruction. Pulmonary veins are best seen in sub-costal, apical,
parasternal views. Tkicuspid atresia or Hypoplastic Tricuspid Valve are easily
recognized in this view. Dilated LV, presence of MR with glistening of papillary
muscles gives clue about ALCAPA. Hypertrophied LV may be seen when
coarctation of aorta Cs associated. If LA and LV are dilated one should suspect
PDA. In a new b o d having hypertrophied LV, if apex is formed by RV, critical
aortic stenosis is a Ejossibility. However, one has to decide about adequacy of LV
in such a situation.

5.2.3 Para-stern!al View

Various congenital anamolies can be detected in this view are perimembranous
VSD (transducer anbled towards right hip), doubly committed VSD (transducer
angulated towards lbft shoulder), aortic sinus prolapse, RSOV, abnormal origin of
great vessels and atdnormalities related to pulmonary valve and main pulmonary
artery.

This view provides unique opportunity to recognize bicuspid aortic valve, the
type of VSD, aortic!valve prolapse, pulmonary valve stenosis, evaluation of
branch pulmonary arteries, abnormal origin of coronary arteries and coronary AV
fistula. Inter atrial septum, pulmonary veins and left atrial appendage can also be
interrogated in this view. Angulation of the transducer towards left hip joint Echocardiography in
images mitral valve in short axis view and papillary muscles (4 and 8 O'Clock Congenital Heart Disease
position). By sweeping the transducer towards left hip inter ventricular septum
can be scanned for muscular VSDs.

5.2.4 High Para-sternal or Ductal View

This view is obtained by sliding transducer one or two spaces upward and
medially from PLAX view. This view is specifically used to visualize patent
ductus arteriosus and adjacent structures.

High Parasternal Short Axis Views (Fig. 5.8)

Fig. 5.8: Structures seen in apical 4 and 5 chamber views

These views are obtained form first and second intercostals spaces just left of the
sternum. The transducer orientation is similar to the conventional parasternal
short axis view. This view is helpful in demonstration pulmonary artery anatomy
in patients withy TOF-physiology/pulmonary atresia.
Suprasternal Views (Fig. 5.9 and 5.10)
To complete the echocardiography in the patients with CHD these views are
essential. For optimal windows patient should lie supine with the neck extended
by a pillow or a rolled-up shet placed underneath the shoulder. First view is
obtained by keeping transducer at 3 O'clock position (marker towards left
shonlder). By angulating the transducer about 45" posteriorly, aorta is seen in
oblique section and entire length of right pulmonary artery is seen just below the
aorta. SVC lies just right to the aorta innominate view seen at an anterior plane.
The LSVC if present, can be seen in this view. Side of arch can also be decided
by seing the direction of first branch and its bifurcation as well as by visualizing
the descending aorta in relation to the tracheal rings. Absence of bifurcation
should give rise suspicion about aberrant origin of subclvian artery. Suprasternal
long axis view shows the aortic arch and proximal descending aorta. This view
can be obtained by rotated the transducer 30" counter clockwise from the
previous position. By tilting the transducer towards left LPA can be seen.
Fundamentals of
Echocardiography
arte!riosus

LPA
/

Pig. 5.9: The high Parasternal or "Ductal" view

Fig. 5.10t Transducer position for suprasternal "short axis" view

Echocardiography in Neonates

In symptomatic neonates echocardiography is more challenging. Usually

babies are more sick and may require emergency intervention. Diagnosis of
CHD may be more complex in this group of patients. One should make the
mental ch,ecklist in such a case and go for quick and systemic scan to rule
out criticai PS, critical AS, transposition of great vessels, tricuspid atresia.,
mitral atresia, hypoplastic right and left heart syndrome, obstructed TAPVC,
coarctation of aorta and interrupted aortic arch. Recognizing restriction at
interatrial level and PDA dependency are of utmost importance in these
situations.
 Echocardiography in
Congenital Heart Disease

5.3 COMMON CONGENITAL HEART DISEASE

The description about common congenital Heart diseases are as follows:

5.3.1 Atrial Septa1 Defect

As mentioned above, ASD is best seen in subcostal views. These windows could
become suboptimal with increasing age, in that case one may have to
Fundamentals of
Echocardiography

RPA

Pig. 5.11: Transducer position for suprasternal "long axis" view

Pig. 5112: Fossa ovalis ASD (subcostal short axis view)

perfom transesopheeal echo. ASD should be defined under following heads:

ASD can be classified in four types:

i) Ostium primum ASDIpartial AVSD

ii) Ostium secbndum or fossa ovalis ASD

iii) Sinus venoSous ASD (SVCAVC type)

iv) Coronary shus defects.

In a patient of ASD one should look for associations like abnormally

draining systemic and pulmonary veins, mitral valve disease. Sometimes
ASDs are impdrtant and are necessary as they provide channel for
decompression 4s in tricuspidlmitral atresia or site for mixing as in TGA.
 PA pressures can calculated from TR jet Gradient: Echocardiography in
Congenital Heart Disease
TR Max PG + RA mean pressure = PA systolic pressure
In presence of RV failure in a relatively young patient with ASD one must
interrogate again for presence of PS, Pulmonary embolism, Pulmonary vein
stenosis, left sided obstructive and regurgitant lesions as well as LV
dysfunction.

Fossa Ovalis ASD

Commonest defect is fossa ovalis ASD. It can be closed by devices. ASD should
be sized in various views and their rims should also be assessed if device closure
is planned. Trans-esophageal echo is a must before and during the procedure.

Sinus Venosus Defect

Commonly found in relation to SVC or IVC. It is usually associated with

PAPVC of right pulmonary veins. It is best seen in sub-costal short axis view.

Ostium Primum ASD

It is caused by deficient lower part of atrial septum and is part of endocardia1

cushion defect. This is commonly associated with common AV valve or cleft
mitral valve. ASD can be evaluated in subcostal short and long axis views.
Associated findings like dilated RA, RV, abnormal septa1 motion should be
recorded. TR and TR velocity are helpful in measuring the PA systolic pressure.

5.3.2 Ventricular Septa1 Defect (VSD)

VSD can be found in various part of ventricular septum hence all possible views
except suprasternal views are used to detect and define the VSD. VSDs are
classified in two ways:

A) According to their location

B) According to change they bring in PA pressures

Fig. 5.13: Ostium primum ASD. subcostal 4 C view

Fundamentals of Classification According to Location
Echocardiography
1) Perimembranous VSD
VSD is present in perimembranous area hence it causes TV-AV continuity.
2) Doubly Committed VSD

These are anterior dtfects roofed by both semilunar valves. Majority of cases in
this variety develop aortic valve prolapse and subsequently AR.

Fig. 5.14: Subcostal short axis view: inlet VSD

3) Inlet VSD
Found in between Wo AV valves. It is a posterior defect. AVSD (endocardia1
cushion defect) is usbally associated with VSD in this area when it is called as
AV canal type VSD. It is usually associated with abnormal chordal attachment of
TV, also known as straddling of TV.
4) Muscular VSD
Found in any part o septum isolated or in association with other VSDs. They
have high rate of ntaneous closure.
Perimembranous and muscular VSDs can be closed by device.

Classification Accoitding to Change They Bring in PA Pressures

1) Restrictive VSDi
VSD is restrictive when VSD pressure gradient is more than 60mmHg (systolic
cuff pressure-VSD lpressure gradient = PA systolic pressure). PA systolic
pressure can also be estimated Erom TR gradient. Tybulent jet is noticed in
colour Doppler. VSOs are restrictive due to its smaller size or sometimes
surrounding structur6s like tricuspid valve or aortic valve cover it partially.
Whenever VSD is getting smaller due to aortic valve prolapse, aortic valve
should be evaluated for incompetence and surgery should be advised at the right
time so that valve cduld be preserved.
2) Non-restrictive VSD Echocardiography in
Congenital Heart Disease
In non-restrictive VSD PA pressures are elevated hence VSD gradient is low.
Non-restrictive VSD is larger in size and has laminar flow in colour Doppler
study. Serial echocardiographic studies are done in infants with VSD to assess
the VSD size, VSD jet velocity, LV dimension (indirect assessment of level of
shunt) and surrounding structures to decide the exact time of intervention.
Smaller VSDs are hemodynamically insignificant but they are more prone for
bacterial endocarditis due to turbulence created by VSD jet. Associated
conditions in a case of VSD makes the major shift in clinical course and
management like association of severe RVOT obstruction (TOF). RVOT
obstruction changes the direction of flow across the VSD and patient needs early
intervention. VSD may be only outlet for LV when both great vessels align with
RV. In that case finding of restrictive VSD is an ominous sign and requires
urgent intervention. VSD in inlet area may be associated with abnormal
attachment of tricuspid valve i.e. chordae to opposite side of septum. Shunt
calculation across the VSD can be done utilizing the RVOT and LVOT
dimensions and RVOT VTI but such a calculation are not very correct and they
are not done routinely. Best echocardiographic parameter of increased shunt is
LVIDd (LV end diastolic dimension) Z-Score.

5.3.3 Patient Ductus Arteriosus

Best view to visualize a patient ductus arteriosus is high parasternal or ductal
view. A patient ductus arteriosus in this view is seen as a 3rd channel beside
RPA and LPA producing a dinner fork appearance. Descending aorta can also

Fig. 5.15: Apical 5 chamber view

be imagined in this view when it curves down to meet ductus in its anterior
aspect and left subclavian artery; Opening of PDA in pulmonary artery is clearly
seen and can be measured. A PDA size beyond 4-5 mm can be send for surgical
ligation. A smaller sized PDA can be closed by interventional procedure like
coiling or device closure. In a patient with PDA one should further interrogate
for VSD, bicuspid aortic valve and coarctation of aorta. Some times PDA can be
sole source of pulmonary blood flow (like in pulmonary atresia) or systemic
Fundamentals of blood flow (aortic &-esia/HLHS).CW Doppler velocity of PDA can be used to
Echocardiography
assess PA pressure. PDA flow signals are continuous, maximal in late systole and
are above the baseline. Like the VSD, end diastolic LV dimension is an
important echocarddographic indicator of the level of shunt.

5.4 LET U$ SUM UP

~ c h o c a r d i o ~ r a pfor
h ~accurate diagnosis of congenital heart disease requires a
long checklist of points to be looked for. As far as possible one should
endeavour to complete all the views mentioned above. The expertise required for
accurate diagnosis1 could be achieved by thorough familiarity of
echocardiographic anatomy and consistent use of a well defined strategy for
examination.

5.5 ANSWERS TO CHECK YOUR PROGRESS

Check Your Progress 1

1) Answer is (b]. As name suggests tricuspid valve has three leaflets-anterior,

posterior and septal. Mitral valve has only two leaflet-anterior and posteior.
2) Answer is (d). LV has no subaortic inhndibulum and has mitral aortic Echocardiography in
continuity, While RV has infundibulum below the semilunar valve. Congenital Heart Disease

3) Answer is (c). Great vessels are identified by their branching pattern. PA

bifurcate early to give rise LPA & RPA. Aorta continues till it arches over
one of bronchus to give rise to its branches then it continues as descending
aorta.

4) Answer is (d). First thing decided in a patient of CHD is visceral

situs,which has good correlation with atrial situs, It is done in subcostal
coronal view when aorta is seen as rounded pulsatile left sided
retro-peritoneal structure. IVC lies inside the liver and has venous flow on
Doppler examination. If dominant venous channel is posterior to aorta, TVC
interruption must be suspected.

5) Answer is (d). A perimebranous VSD is surrounded by aortic valve

superiorly, tricuspid valve posteriorly and inter ventricular septum inferiorly.
It can get restricted by any of these structure.
Check Your Progress 2

1 ) Answer is (c). This VSD is found just below the two semilunar valves
hence it is named as double committed VSD.

2) Answer is (a). Devics closure requires good margins around the ASD so
that device would hold the margins without encroaching the surrounding
structures. This can be done when ASD is situated in fossa ovalis area.

3) Answer is (c). High parasternal view is also known as 'Ductal View'. In

this view PDA is seen as third channel besides PA branches giving rise to
'dinner fork' sign

4) Answer is (a). TR gradient is calculated then assumed RA pressure are

added in it to get the PA systolic pressure. PR jet is used to estimate PA
mean pressure and PA end diastolic pressures.

5.6 FURTHER READINGS

Braunwald, E., Zipes, D.P., Libby, P., Heart Disease: A Text Book of
Cardiovascular Medicine, 6th edn., W.B. Saunders Company, 2003.
Edwards, W.D., Tajik, A.J., Seward, J.B., "Standard nomenclature and anatomic
basis for regional tomographic analysis of the heart". Mayo Clin Proc 1981;
56: 479-497.
Feigenbaum, H., Echocardiography, 5th edn. Philadelphia, Lea and Febiger,
1993, 1-59.
Marso, P., Griffin, B.P., Topol, E.J., Manual of Cardiovascular Medicine, 2nd
edn., Lippincott Williams and Wilkins, 2000: 744-757.
Nishimura, R.A., Tajik, A.J., Quantitative hemodynamics by Doppler
echocardiography a noninvasive alternative to cardiac catheterization, Prog
Cardiovasc Dis 1994; 36:309-42.
Oh, J.K., Seward, J.B., Tajik, A.J., l%e Echo Manual. 2nd edn., Little Brown and
Company, 1994: 7-22.
Fundamentals of Otto, C.M., Pearlman, A.S., Otto and Pearlman b Textbook o f Clinical
Echocardiography Echocardiography, Philadelphia: WB Saunders, 1995.
Weyman, A., Principles and Practice of Echocardiopphy; 2nd edn.,
Philadelphia: Lda and Febiger, 1994.

Acknowledgemients
We thank Dr. Smita Mishra MD, DNB (Paed. Cardiology) for contributing this
unit on Echocardiography in Congenital Heart Disease and Mr. Sanjeev Kumar
for secretarial assisance in typing of this unit.