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5.8 Pathologic Findings
Fig. 5.9 Atrioventricular
(AV) septal defect – apical
four-chamber view. Defects
at ventricular and atrial level
(→), common AV-valve. LV
left ventricle, LA left atrium,
RA right atrium, RV right
ventricle
RV
LV
RA
LA
175
Because IVS visible not in single plane entirely, different views necessary to
provide detailed information about localisation and size of defect and its relation to
adjacent structures.
CDS improves detection of small or multiple VSDs, shows direction of shunt
across defect.
CW-Doppler allows indirect estimation of RV systolic pressure using Bernoulli
equation (systolic blood pressure minus peak pressure gradient between LV and RV =
systolic RV pressure) when blood pressure measured simultaneously; the larger the
defect, the lower the gradient between ventricles. Hemodynamic relevance of leftto-right shunt demonstrated by degree of enlargement of LA, LV and RV and dilatation of PA due to increased pulmonary blood flow and pulmonary venous return.
Perimembranous VSD (Fig. 5.10):
• Parasternal long and short axis views, in modified four-chamber view: defect
beneath aortic valve
• Sometimes associated with ventricular septal pseudoaneurysm
Muscular VSD:
• Parasternal long axis view: in middle third of IVS (mid muscular) or inferiorly
(apical)
• Parasternal short axis views: at different levels
• Apical four-chamber view: inlet VSD in upper third, mid muscular VSD in mid-
dle of IVS and apical VSD in most distal part of IVS
Subarterial (infundibular) VSD:
• Standard long axis view: beneath aortic valve
• Parasternal short axis view: beneath pulmonary valve
• Often associated with aortic valve regurgitation due to prolapse of aortic valve
176
Fig. 5.10 Perimembranous
ventricular septal defect
(VSD) – parasternal long axis
view. CDS demonstrates
left-to-right shunt beneath
aortic valve through VSD
(→). LV left ventricle, LA left
atrium, Ao Aorta, RV right
ventricle
5 Basics of Paediatric Echocardiography
RV
LV Ao
LA
ab
PA
PDA
RPA
Fig. 5.11 Persistent ductus arteriosus (PDA) – parasternal short axis at heart base: (a) Left-to-
right shunt demonstrated by CDS. (b) Shunt confirmed/further assessed by CW-Doppler spectral
analysis (right image). CW-Doppler measurements: gradient of 129 mmHg indicating normal pulmonary artery (PA) pressure (peak flow velocity 568 cm/s). RPA right pulmonary artery, LPA left
pulmonary artery
LPA
5.8.1.4 Patent Ductus Arteriosus of Botalli (PDA)
Persistent patency of ductus arteriosus occurs in preterm infants as an isolated
defect or associated with other congenital heart defects:
• PDA best visualised in parasternal short axis or high left parasternal views (“duc-
tus view”) – three vessels arise from main PA: right and left PA and PDA con-
necting to descending aorta (Fig. 5.11).
5.8 Pathologic Findings
177
• In pulmonary atresia: atypical course of PDA arising from aortic arch, best
detected on suprasternal long axis view.
• Large PDA: LA and LV enlarged due to increased pulmonary blood flow.
CDS and CW-Doppler demonstrate direction of shunt:
• Normal systolic pulmonary pressure – left-to-right shunt, continuous flow from
aorta to PA (towards transducer).
• Pulmonary hypertension – bidirectional shunt.
• Severe left heart obstructions – right-to-left shunt.
Systolic PA pressure calculated by Bernoulli equation (systolic blood pressure
minus peak Doppler pressure gradient = systolic pulmonary artery pressure) when
simultaneously measuring blood pressure.
5.8.1.5 Persistent Truncus Arteriosus (Truncus Arteriosus Communis)
Parasternal long axis view: single artery (truncus) overriding a large VSD.
The single truncal valve (occasionally with four cusps) and type of origin of
PA from truncus best demonstrated in parasternal short axis view at base of
the heart.
5.8.2 Obstructions of Left Ventricular Outflow
5.8.2.1 Aortic Valve Stenosis (AS)
Morphology of aortic valve including number of cusps best evaluated in parasternal
short axis view - at base of the heart (Fig. 5.12).
Stenotic aortic valve often consists only of two cusps (occasional only one cusp)
creating “fish-mouth shape” of valve in systole.
Parasternal long axis view enables measurement of valve diameter, shows
restricted opening of thickened cusps resulting in dome shape of valve in systole.
Poststenotic dilatation of ascending aorta frequently seen.
Depending on severity of AS hypertrophy of LV demonstrated.
CDS in parasternal long axis, subcostal and suprasternal views demonstrate highvelocity jet into ascending aorta – occasionally associated with aortic regurgitation.
• Measurements of peak and mean Doppler pressure gradients using different
views (suprasternal, subcostal) allow estimation of severity.
– Critical AS of newborn: LV cavity and aortic annulus small with echogenic
thickened endocardium presenting endocardial fibroelastosis.
If LV dilated and contractility reduced: low-pressure gradient measured – low
cardiac output.
5.8.2.2 Subaortic Stenosis (Sub AS)
Parasternal long axis, apical and subcostal views: fibrous membrane just below aortic valve or tunnel-like narrowing of left ventricular outflow tract detected (DDx
asymmetric hypertrophic cardiomyopathy).
Aortic valve regurgitation often seen.
• Sub AS may be associated with multiple left heart obstructions (“Shone complex”).
178
Fig. 5.12 Valvular aortic
stenosis – parasternal long
axis view. Aortic valve
leaflets thickened (→),
hypertrophy of left ventricle
(LV). Ao aorta
5 Basics of Paediatric Echocardiography
Ao
LV
5.8.2.3 Supravalvular Aortic Stenosis
Circumscribed stenosis or long hypoplastic segment of ascending aorta visualised
in parasternal, apical and subcostal long axis and suprasternal views.
Rare anomaly, associated frequently with Williams-Beuren syndrome.
5.8.2.4 Aortic Coarctation (CoA)
CoA best evaluated from suprasternal notch in supine position with hyperextended neck. Suprasternal long axis view (Fig. 5.13): entire aortic arch must be
imaged, because occasionally aortic arch may be hypoplastic – particularly in
neonates.
Short stenosis or longer hypoplastic segment demonstrated near origin of left
subclavian artery.
CDS shows high-velocity jet distal to CoA.
CW-Doppler measurements reveal increased flow extended into diastole and distal
to CoA allows calculation of pressure gradient. LV may be hypertrophied.
• In neonates LV function may be severely depressed – PDA-dependent systemic
perfusion.
5.8.2.5 Interrupted Aortic Arch
Exact site of interruption, origin of aortic arch vessels from proximal and distal segments of aorta - best demonstrated in suprasternal long axis view.
PDA with right-to-left shunt provides systemic perfusion distal to interruption.
Additional congenital heart defects common (e.g. VSD or persistent truncus
arteriosus).
ab
5.8 Pathologic Findings
CoA
Fig. 5.13 Coarctation of aorta (CoA) – suprasternal view. (a) Hypoplastic isthmus with turbulent
blood flow (→) on CDS. (b) CW-Doppler measurement: increased flow velocity with characteristic flow pattern extended into diastole
179
5.8.3 Obstructions of the Right Ventricular Outflow
5.8.3.1 Isolated Pulmonary Valve Stenosis (PS)
Pulmonary valve best evaluated in parasternal short axis (Fig. 5.14), parasternal
long axis through right ventricular outflow tract, and subcostal views.
Commonly pulmonary valve diameter normal, valvular cusps thickened with
restricted opening and systolic doming, usually poststenotic dilatation of main PA.
RV usually normal, may be hypertrophic – in particular at infundibulum.
• Patients with Noonan syndrome and PS: pulmonary valve often dysplastic, with
myxomatous changes of leaflets.
• Neonates with critical PS: pulmonary valve annulus hypoplastic with reduced
RV cavity; pulmonary blood supply may be dependent on PDA.
– CDS: high-velocity jet distal to pulmonary valve.
– CW-Doppler: estimates pressure gradient.
5.8.3.2 Subvalvular Pulmonary Stenosis
Fibromuscular stenosis at infundibulum or anomalous muscle band below infundibulum dividing RV into two chambers (“double-chambered right ventricle”).
Seen on subcostal four-chamber view or subcostal long axis view of RV.
Associated congenital heart defects common.
5.8.3.3 Supravalvular Pulmonary Stenosis
Circumscribed stenosis or longer hypoplastic segments in main PA or pulmonary
branches.
Best visualised on parasternal short axis and subcostal views.
• Associated with other congenital heart defects, rubella or Williams-Beuren
syndrome.
180
Fig. 5.14 Pulmonary valve
stenosis – parasternal short
axis view. High-velocity
mosaic jet on CDS distal to
pulmonary valve. Ao Aorta,
PA main pulmonary artery
5 Basics of Paediatric Echocardiography
PA
Ao
5.8.3.4 Tetralogy of Fallot (TOF) and Pulmonary
Atresia (PA) with VSD
• TOF – most common cyanotic congenital heart defect.
• Characterised by large VSD with overriding aorta, RV outflow tract obstruction,
RV hypertrophy. Pulmonary valve annulus, main PA and PA branches usually
hypoplastic to different degrees (Fig. 5.15). Pulmonary valve leaflets may be
thickened with systolic doming.
• PA with VSD – most extreme type of TOF. Pulmonary blood supply provided via PDA
or multiple aortopulmonary collateral arteries arising from aorta or arch arteries.
• Large subaortic VSD with overriding aorta – best imaged in parasternal long axis
view.
• Parasternal and subcostal short axis views, long axis view through RV outflow
tract: evaluate degree of subpulmonic stenosis, morphology, and size of pulmo-
nary valve, size of the main PA.
• Parasternal short axis and suprasternal views: demonstrate PA bifurcation, size
of PA branches and PDA, occasionally right aortic arch.
• CDS: high-velocity jet in RV outflow tract – usually beginning below pulmonary
valve (infundibulum), predominant right-to-left shunt via VSD.
• CW-Doppler: estimates pressure gradient across RV outflow tract.
– PA with VSD: no pulmonary valve seen, blind end of RV outflow tract, no
antegrade flow into main PA.
5.8 Pathologic Findings
Fig. 5.15 Tetralogy of
fallot – parasternal long axis
view. Large ventricular septal
defect (→) with overriding
aorta (Ao). RV right ventricle,
LV left ventricle
181
RV
LV
Ao
5.8.4 Miscellaneous Congenital Heart Defects
5.8.4.1 Transposition of Great Arteries (TGA)
Simple TGA - ventriculoarterial discordance: aorta arises anteriorly from RV, PA
posteriorly from LV. In contrast to normal heart (crossing of great arteries) great
arteries exit from heart in parallel course.
Systemic and pulmonary circulation connected in parallel (instead of normal
serial connection); survival depends on communications between circulations (commonly PDA, PFO/ASD in patients with an intact IVS or VSD).
Parallel course of the great arteries demonstrated in parasternal short and long
axis and in suprasternal long axis views (Fig. 5.16).
Parasternal long axis and suprasternal views: both arteries seen in longitudinal
section. The aorta and aortic arch branches lay anteriorly to PA. In parasternal short
axis view, great arteries seen in cross section as double circles; posterior artery –
pulmonary artery (identified by demonstrating bifurcation into left and right PA).
Subcostal four-chamber view: connection of LV to PA with its bifurcation seen,
whereas aorta with aortic arch arises from RV (Fig. 5.17).
Coronary artery origin from aortic sinuses best evaluated in parasternal short
axis view.
CDS reveals information about amount of mixing via PDA, PFO/ASD or both.
Associated anomalies common: VSD, CoA, LV outflow obstruction (i.e. pulmonary stenosis).
5.8.4.2 Total Anomalous Pulmonary Venous Return (TAPVR)
Different Forms:
• Cardiac type: all PV drain directly (or via coronary sinus) into RA.
182
Fig. 5.16 Transposition of great arteries
(TGA) – suprasternal view. Parallel
alignment of great arteries with Aorta (Ao)
anteriorly and main pulmonary artery (PA)
posteriorly
Fig. 5.17 Transposition
of great arteries (TGA) – subcostal view.
Main pulmonary artery (PA) with its
bifurcation arising from left ventricle (LV).
RV right ventricle
5 Basics of Paediatric Echocardiography
Aa
PA
RV
LV
PA
• Supracardiac type: all PV form pulmonary venous confluence draining into SVC
via left innominate vein; pulmonary venous confluence may be visualised poste-
riorly to LA without direct communication.
• Infracardiac or subdiaphragmatic type: all PV drain into IVC, portal vein, hepatic
vein or ductus venosus – large vessel passing below diaphragm with venous flow
away from heart/chest.
ASD or PFO with right-to-left shunt mandatory for survival. RV, RA and main PA
enlarged, whereas LA and LV normal or small.
• In cases with small PFO: IAS bulged to left.
Dependent on site of pulmonary venous drainage: dilated coronary sinus, innominate vein/SVC, or IVC.
CDS: exclude obstruction of pulmonary venous return.
5.8 Pathologic Findings
183
5.8.4.3 Univentricular Heart (UVH)
UVH summarises wide spectrum of complex congenital heart defects with one large
dominant ventricle and a small rudimentary ventricular chamber.
Various congenital heart defects often associated (e.g. VSD, ASD, PDA, TGA,
PS or PA, CoA, systemic and pulmonary venous anomalies, situs anomalies).
Echocardiographic evaluation of UVH:
• Include morphology and site of dominant and rudimentary ventricle/ventricular
chamber.
• Assess type and size of communication between atria and ventricles, interven-
tricular and interatrial communications and ventriculoarterial connecxions.
• Assess presence of obstructions of ventricular outflow.
• Assess other associated anomalies.
Many Different forms – Most Common Examples:
Double Inlet Left Ventricle:
• Both AV-valves drain into large morphological LV.
• Rudimentary (right) chamber communicates with LV via VSD.
• Usually PA arises from LV, aorta from rudimentary ventricle (TGA).
Tricuspid Atresia:
• No tricuspid valve.
• Systemic venous blood flows from RA via ASD/PFO to LA which drains
into large LV.
• Hypoplastic RV communicates via VSD with LV.
• Great arteries may be transposed – aorta arising from hypoplastic RV, PA
from LV.
Hypoplastic Left Heart Syndrome:
• LA and LV hypoplastic – with small or even atretic mitral and aortic valves.
• Hypoplastic ascending aorta.
• Large RV acts as systemic ventricle.
• LA drains into RA via ASD or PFO.
• Systemic perfusion dependent on PDA – retrograde flow into hypoplastic
ascending aorta.
5.8.4.4 Double Outlet Right Ventricle (DORV)
Aorta and PA arise completely or predominantly from RV.
The usually normal sized LV empties via VSD into RV.
Wide anatomic variability of DORV regarding site of VSD and relationship of
great arteries (normal or side-by-side position, TGA), PS frequently associated.
5.8.4.5 Ebstein Anomaly
Inferior displacement of proximal attachments of septal and posterior tricuspid
valve leaflets.
• Best demonstrated in four-chamber views.
• Depending on degree of inferior displacement: large RA, small functioning RV
(“atrialisation of RV”).
184
CDS, CW/PW-Doppler: evaluate severity of tricuspid valve regurgitation, rightto-left shunt via ASD or PFO nearly always present.
5 Basics of Paediatric Echocardiography
5.8.4.6 Cor Triatriatum
Fibromuscular membrane with small hole divides LA in posterior and anterior chamber.
Imaged in apical and subcostal four-chamber view and parasternal long axis views.
Due to higher pressure in posterior chamber which receives pulmonary venous
return, membrane bulged toward mitral valve during diastole.
5.9 Acquired Paediatric Heart Diseases
5.9.1 Cardiomyopathies (CMP)
5.9.1.1 Hypertrophic CMP
US feature: commonly impressive thickening in particular of IVS and – to a lesser
degree – of LV posterior wall.
End-diastolic LV diameter usually normal, but end-systolic LV diameter reduced.
Shortening and ejection fraction of LV usually normal or increased.
5.9.1.2 Hypertrophic Obstructive CMP (HOCMP)
Subaortic obstruction caused by hypertrophy of IVS and systolic anterior motion of
anterior mitral leaflet (Fig. 5.18).
5.9.1.3 Dilated (Congestive) CMP
LV and LA dilated – with significantly decreased systolic LV function.
Mitral regurgitation common – due to dilatation of mitral valve annulus.
Occasionally thrombus visible in LV and/or LA.
Sometimes endocardial fibroelastosis – particularly in infants.
5.9.1.4 Restrictive CMP
Rare; ventricles usually show normal size with normal systolic function.
Atria excessively dilated due to severely impaired diastolic ventricular filling.
5.9.2 Acute Myocarditis
US findings: ventricles and atria may be enlarged, with reduced contractility particularly of LV.
5.9.3 Acute (Infective) Endocarditis
Intracardiac vegetations may be demonstrated (Fig. 5.19).
• TEE increases sensitivity in detecting vegetations not seen transthoracically.
Occasionally paravalvular abscess can be detected.
Valve disruption may lead to valve insufficiency.