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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5795_Библиотеки_им_академика_М_И_Перельмана.pdf
218
M. Köstenberger et al.
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.
11.8.1.4 Patent Ductus Arteriosus ofBotalli (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.11.13).
• 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 ow.
Fig. 11.13 Persistent ductus arteriosus (PDA)—parasternal short axis at heart base: (a) Left-to-
right shunt demonstrated by CDS. (b) Shunt conrmed/further assessed by CW-Doppler spectral
analysis (right image). CW-Doppler measurements: gradient of 129 mmHg indicating normal pulmonary artery (PA) pressure (peak ow velocity 568cm/s). RPA right pulmonary artery, LPA left
pulmonary artery
11 Basics ofPaediatric Echocardiography
219
CDS and CW-Doppler demonstrate direction of shunt:
• Normal systolic pulmonary pressure—left-to-right shunt, continuous ow from
aorta to PA (toward transducer).
• Pulmonary hypertension—bidirectional shunt.
• Severe left heart obstructions—right-to-left shunt.
Systolic (peak) PA pressure calculated by Bernoulli equation (systolic blood
pressure minus peak Doppler pressure gradient = systolic pulmonary artery pressure) when simultaneously measuring blood pressure.
11.8.1.5 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.
11.8.2 Obstructions ofLeft Ventricular Outflow
11.8.2.1 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.11.14).
Stenotic aortic valve often consists only of two cusps (occasional only one cusp)
creating “sh-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 can be detected.
CDS in parasternal long axis, subcostal and suprasternal views demonstrate
high-velocity jet into ascending aorta—occasionally associated with aortic
regurgitation.
Fig. 11.14 Valvular aortic stenosis—parasternal
long axis view. Aortic valve leaets thickened
(→), hypertrophy of left ventricle (LV). Ao aorta
220
M. Köstenberger et al.
• 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 broelastosis.
If LV dilated and contractility reduced: low-pressure gradient measured—low
cardiac output.
11.8.2.2 Subaortic Stenosis (Sub-AS)
Parasternal long axis, apical and subcostal views: brous membrane just below aortic valve or tunnel-like narrowing of left ventricular outow tract detected (DDx
asymmetric hypertrophic cardiomyopathy).
Aortic valve regurgitation often seen.
• Sub-AS may be associated with multiple left heart obstructions (“Shone
complex”).
11.8.2.3 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.
11.8.2.4 Aortic Coarctation (CoA)
CoA best evaluated from suprasternal notch in supine position with hyperextended
neck. Suprasternal long axis view (Fig.11.15): entire aortic arch must be imaged,
because occasionally other parts of aortic arch may be hypoplastic—particularly in
neonates.
Short stenosis or longer hypoplastic segment demonstrated near origin of left
subclavian artery.
Fig. 11.15 Coarctation of aorta (CoA)—suprasternal view. (a) Hypoplastic isthmus with turbu-
lent blood ow (→) on CDS. (b) CW-Doppler measurement: increased ow velocity with characteristic ow pattern extended into diastole
11 Basics ofPaediatric Echocardiography
221
CDS shows high-velocity jet distal to CoA.
CW-Doppler measurements reveal increased ow 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.
11.8.2.5 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).
11.8.3 Obstructions oftheRight Ventricular Outflow
11.8.3.1 Isolated Pulmonary Valve Stenosis (PS)
Pulmonary valve best evaluated in parasternal short axis (Fig.11.16), parasternal
long axis through right ventricular outow 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 leaets.
• 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.
Fig. 11.16 Pulmonary valve stenosis—
parasternal short axis view. High-velocity mosaic
jet on CDS distal to pulmonary valve. Ao Aorta,
PA main pulmonary artery
222
M. Köstenberger et al.
11.8.3.2 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.
11.8.3.3 Supravalvular Pulmonary Stenosis
Circumscribed stenosis or longer hypoplastic segments in main PA or pulmonary
branches.
Best visualised on parasternal short axis and subcostal views.
• Occasionally postoperatively or associated with other congenital heart defects,
rubella or Williams–Beuren syndrome.
11.8.3.4 Tetralogy ofFallot (TOF) andPulmonary Atresia (PA)
withVSD
• TOF—most common cyanotic congenital heart defect.
• Characterised by large VSD with overriding aorta, RV outow tract obstruction,
RV hypertrophy. Pulmonary valve annulus, main PA and PA branches usually
hypoplastic to different degrees (Fig.11.17). Pulmonary valve leaets 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 aortic
branches.
• Large subaortic VSD with overriding aorta—best imaged in parasternal long
axis view.
• Parasternal and subcostal short axis views, long axis view through RV outow
tract: evaluate degree of subpulmonic stenosis, morphology, size of pulmonary
valve and 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 outow tract—usually beginning below pulmonary
valve (infundibulum), predominant right-to-left shunt via VSD.
• CW-Doppler: estimates pressure gradient across RV outow tract.
– PA with VSD: no pulmonary valve seen, blind end of RV outow tract, no
antegrade ow into main PA.
Fig. 11.17 Tetralogy of fallot—parasternal long
axis view. Large ventricular septal defect (→) with
overriding aorta (Ao). RV right ventricle, LV left
ventricle
11 Basics ofPaediatric Echocardiography
223
11.8.4 Miscellaneous Congenital Heart Defects
11.8.4.1 Transposition ofGreat 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.11.18).
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 (identied 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.11.19).
Fig. 11.18 Transposition of great arteries
(TGA)—suprasternal view. Parallel alignment of
great arteries with aorta (Ao) anteriorly and main
pulmonary artery (PA) posteriorly
Fig. 11.19 Transposition of great arteries
(TGA)—subcostal view. Main pulmonary artery
(PA) with its bifurcation arising from left ventricle
(LV). RV right ventricle
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M. Köstenberger et al.
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 outow obstruction (i.e. pulmonary stenosis).
11.8.4.2 Total Anomalous Pulmonary Venous Return (TAPVR)
Different Forms:
• Cardiac type: all PVs drain directly (or via coronary sinus) into RA.
• Supracardiac type: all PVs form pulmonary venous conuence draining into
SVC via left innominate vein; pulmonary venous conuence may be visualised
posteriorly 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 ow
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 PV drainage: dilated coronary sinus, innominate vein/SVC or IVC.
CDS: exclude obstruction of PV return.
11.8.4.3 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 connections.
• Assess presence of obstructions of ventricular outow.
• 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).
11 Basics ofPaediatric Echocardiography
225
Tricuspid Atresia:
• No tricuspid valve.
• Systemic venous blood ows 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 ow into hypoplastic
ascending aorta.
11.8.4.4 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.
11.8.4.5 Ebstein Anomaly
Inferior displacement of proximal attachments of septal and posterior tricuspid
valve leaets.
• Best demonstrated in four-chamber views.
• Depending on degree of inferior displacement: large RA, small functioning RV
(“atrialisation of RV”).
CDS, CW/PW-Doppler: evaluate severity of tricuspid valve regurgitation, rightto- left shunt via ASD or PFO nearly always present.
11.8.4.6 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 receiving PV return, membrane
bulges toward mitral valve during diastole.
226
M. Köstenberger et al.
11.9 Acquired Paediatric Heart Diseases
11.9.1 Cardiomyopathies (CMP)
11.9.1.1 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.
11.9.1.2 Hypertrophic Obstructive CMP (HOCMP)
Subaortic obstruction caused by hypertrophy of IVS and systolic anterior motion of
anterior mitral leaet (Fig.11.20).
11.9.1.3 Dilated (Congestive) CMP
LV and LA dilated—with signicantly decreased systolic LV function.
Mitral regurgitation common—due to dilatation of mitral valve annulus.
Occasionally thrombus visible in LA and/or LV.
Sometimes endocardial broelastosis—particularly in infants.
11.9.1.4 Restrictive CMP
Rare; ventricles usually show normal size with normal systolic function.
Atria excessively dilated due to severely impaired diastolic ventricular lling.
11.9.2 Acute Myocarditis
US Finding
Ventricles and atria may be enlarged, with reduced contractility particularly of LV.
11.9.3 Acute (Infective) Endocarditis
Intracardiac vegetations may be demonstrated (Fig.11.21).
Fig. 11.20 Hypertrophic obstructive
cardiomyopathy—parasternal long axis view.
Massive hypertrophy (↔) of interventricular
septum (IVS) and left ventricular posterior wall
(LVPW). LA left atrium, Ao aorta
11 Basics ofPaediatric Echocardiography
Fig. 11.21 Endocarditis—apical four-chamber
view. Endocarditic vegetation attached to mitral
valve (→). LA left atrium, LV left ventricle
• TEE increases sensitivity in detecting vegetations not seen transthoracically.
Occasionally paravalvular abscess can be detected.
Valve disruption may lead to valve insufciency.
11.9.4 Pericarditis/Pericardial Effusion
227
US Findings
Anechoic space surrounding (and possibly compressing) heart (Fig.11.22).
Compression of RA and RV wall indicates beginning tamponade.
11.9.5 Kawasaki Disease
Associated with coronary artery aneurysms or ectatic coronary arteries:
• From second week of illness.
• Best seen in parasternal short axis view at base of heart.
• Pericardial effusion, valve regurgitations and LV dysfunction common.
• Follow-up studies mandatory—particularly in patients with coronary artery
involvement.
11.9.6 Intracardiac Thrombi
In children, intracardiac thrombi mainly associated with intravascular catheters,
dilated CMP, atrial dilatation (atrial utter or brillation) and prosthetic valves.
DDx: thrombus like impression of (mostly atrial) walls, tumours.