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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3612_Библиотеки_им_академика_М_И_Перельмана
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Key Questions in CONGENITAL CARDIAC SURGERY
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Figure 34. Parasternal long-axis view demonstrating
double-outlet right ventricle, characterised by the aorta
overriding the interventricular septum (*) by >50% and
aorto-mitral discontinuity. RV = right ventricle; LA = left
atrium; Ao = aorta.
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Figure 35. Subcostal views demonstrating a: A) Taussig-Bing anomaly,
characterised by a double-outlet right ventricle with the aorta lying to the
right of the pulmonary artery and completely arising from the RV, and a
subpulmonary ventricular septal defect (*); and B) the pulmonary artery
with flow (red) from a patent ductus arteriosus, allowing it to be
distinguished from the aorta, on the corresponding colour flow Doppler
image. RV = right ventricle; LV = left ventricle; Ao = aorta; PA = pulmonary
artery.
4 Congenital echocardiography
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23 What are the principles of assessing vascular rings on
echocardiography?
Vascular rings are usually diagnosed with suprasternal
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echocardiographic views and often confirmed by CT or MRI (Figure
36).
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Figure 36. Suprasternal view colour flow Doppler images demonstrating
a: A) double aortic arch (*); and B) left pulmonary artery sling, with the left
pulmonary artery (LPA) originating (arrow) from the right pulmonary artery
(RPA), rather than its usual origin (*) at a symmetrical bifurcation with the
right pulmonary artery. Ao = aorta; PA = pulmonary artery.
Vascular rings can be diagnosed by foetal sonographers, such as a
•
right aortic arch with aberrant left subclavian artery and left ductus
arteriosus.
24 What are the principles of assessing isomerism on
echocardiography?
Isomerism refers to the atrial appendages and not the entire atria.
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As the appendages are not usually seen on echocardiography, the
•
situs is inferred from the arrangement of the aorta and IVC or azygos
and hemiazygos venous systems on the subcostal view (Figure 37).
On echocardiography, right isomerism is characterised by an absent
•
coronary sinus and totally anomalous pulmonary venous connection.
In addition, there is often an AVSD, poorly formed atrial septum,
DORV with pulmonary stenosis or atresia, bilateral vena cavae, rightsided heart and left-handed ventricular topology.
On echocardiography, left isomerism is characterised by an
•
interrupted IVC and azygos continuation. In addition, there is often an
AVSD and bilateral vena cavae.
161
Key Questions in CONGENITAL CARDIAC SURGERY
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Figure 37. Isomerism identification on echocardiography determined
by the relationship between the aorta (A) and the inferior vena cava (V),
or in the case of left atrial isomerism, the aorta and the hemiazygos (H)
vein: A) echocardiographic subcostal situs views demonstrating the usual
atrial arrangement with the aorta lying posterior and to the left of the
inferior vena cava. Schematic illustrations of: B) situs solitus; C) right atrial
isomerism; D) left atrial isomerism; and E) mirror image.
In addition, other malformations associated with isomerism include:
•
a) common atrioventricular junction — which are frequent with
both right and left isomerism;
b) double-inlet connection — which is more frequent with right
isomerism;
c) biventricular connections — which is more frequent with left
isomerism;
4 Congenital echocardiography
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d) concordant connections with coarctation — which is more
frequent with left isomerism;
e) discordant or double-outlet connections along with pulmonary
atresia or stenosis — which is expected with right isomerism.
25 What are the principles of assessing tricuspid atresia on
echocardiography?
Anatomically, the valve can be imperforate or there may be an absent
•
connection, which appears echocardiographically as a thick band of
the atrioventricular groove separating the atrium from the ventricle
(Figure 38).
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Figure 38. Apical four-chamber view demonstrating tricuspid atresia
characterised by a: A) thick band between the right atrium (RA) and the
rudimentary right ventricle (RV), consistent with an absent right connection;
and B) flow across the open mitral valve but no flow across the absent right
connection on the corresponding colour flow Doppler image. LV = left
ventricle; LA = left atrium.
It is associated with a rudimentary RV, and the left atrium connected
•
to a dominant LV. Ventriculo-arterial connections can be concordant
or discordant.
163
26 What are the principles of assessing double-inlet
ventricle on echocardiography?
Echocardiography uses the relationship of the rudimentary ventricle
•
to the main ventricle (not usually by the ventricular trabecular pattern
164
Key Questions in CONGENITAL CARDIAC SURGERY
that is much harder to distinguish) to distinguish the three types of
double-inlet ventricle:
a) double-inlet LV (DILV, commonest) — which is diagnosed by
finding a rudimentary anterosuperior RV either on the left or
right hand side of the main ventricle (Figure 39);
Figure 39. Apical view (in adult
orientation with the apex up)
demonstrating a double-inlet to a
dominant left ventricle (LV). TV =
tricuspid valve; MV= mitral valve.
b) double-inlet RV — which is associated with a rudimentary
posteroinferior LV;
c) double-inlet to a solitary, usually indeterminate, ventricle (rare).
The Holmes heart is a DILV with a rudimentary RV and ventriculo-
•
arterial concordance.
27 What are the principles of assessing hypoplastic left
heart syndrome on echocardiography?
Echocardiographic assessment of a patient with hypoplastic left
•
heart syndrome is used to identify a number of features (Figure 40),
including:
4 Congenital echocardiography
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Figure 40. Echocardiography images of hypoplastic left heart syndrome: A)
apical four-chamber view demonstrating a globular left ventricle (*), with
bulging of the interatrial septum into the right atrium (RA), suggestive of a
restrictive interatrial communication; B) apical four-chamber view
demonstrating a slit-like LV (*); and C) tricuspid regurgitation (blue jet) on the
corresponding colour flow Doppler image; D) parasternal short-axis view
demonstrating a very small aorta (arrow) relative to the enlarged pulmonary
artery (PA); E) suprasternal view demonstrating a small ascending aorta and
transverse arch (arrow) relative to the descending aorta (Desc Ao), which is
fed by the patent ductus arteriosus. RV = right ventricle; LA = left atrium.
165
a) hypoplastic left ventricle;
b) distinguishing aortic valve stenosis from aortic atresia by
assessing colour flow across the aortic valve;
c) distinguishing mitral valve stenosis from mitral atresia;
d) hypoplastic aortic arch and aortic coarctation;
e) patent ductus arteriosus size — which is essential for neonatal
survival;
f) restrictive secundum ASD — which worsens prognosis and
may require an urgent balloon atrial septostomy;
166
Key Questions in CONGENITAL CARDIAC SURGERY
g) tricuspid regurgitation;
h) coronary artery fistulae;
i) pulmonary venous stenoses or anomalous pulmonary venous
drainage (levoatrial cardinal vein);
j) RV function, using tricuspid annular plane systolic excursion
(TAPSE) and RV fractional area change (FAC).
28 What are the principles of assessing aortopulmonary
window on echocardiography?
An aortopulmonary (AP) window is best visualised in the parasternal
•
short-axis or suprasternal arch views, anywhere from just above the
semilunar valves to the more distal ascending aorta and main
pulmonary artery (Figure 41).
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Figure 41. Parasternal short-axis view
demonstrating an aortopulmonary window (*),
which inserts into the origin of the right pulmonary
artery (RPA). Ao = aorta; PA = pulmonary artery.
Left heart dilatation due to volume overload from left-to-right shunting
•
will also be present and is best seen in the apical and subcostal fourchamber views.
The most common lesions associated with AP window are
•
interrupted aortic arch (classically Type A) and coarctation.
4 Congenital echocardiography
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29 What are the principles of assessing anomalous origin
of the left coronary artery from the pulmonary artery on
echocardiography?
The anomalous left coronary artery typically arises from the left
•
inferolateral aspect of the main pulmonary artery just beyond the
pulmonary valve and is best seen in the parasternal short-axis view
(Figure 42). It then courses toward the interventricular groove and
branches into the left anterior descending (LAD) and left circumflex
(LCX) arteries.
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Figure 42. Parasternal short-axis view demonstrating: A) an anomalous
left coronary artery from the pulmonary artery (ALCAPA); with B) ALCAPA
originating from the pulmonary artery (PA) and dividing into the left anterior
descending (LAD) and left circumflex (LCX) coronary arteries, on the
corresponding colour flow Doppler image. Ao = aorta.
The retrograde flow from the left coronary artery to the main
•
pulmonary artery is well depicted on echocardiogram and is a
characteristic finding of the steal phenomenon.
The right coronary artery is often dilated.
•
An impression of endocardial fibroelastosis is also often seen with
•
brightness of the LV myocardium, especially the papillary muscles.
Left heart dilation occurs, often associated with functional mitral
•
regurgitation, and is best seen in the apical four-chamber view.
167
168
Key Questions in CONGENITAL CARDIAC SURGERY
30 What are the principles of assessing Ebstein’s anomaly
on echocardiography?
Ebstein’s anomaly is best seen on the apical (Figure 43) and
•
subcostal four-chamber views, which illustrate the key features,
including:
a) inferior displacement of the proximal attachments of the septal
and posterior leaflets of the tricuspid valve;
b) rightward and anterior rotational displacement of the valve —
which results in ‘atrialisation’ of the proximal portion of the right
ventricle, resulting in a reduction in the size of the functional
right ventricle and tricuspid regurgitation;
c) secundum ASD — which is a frequent association and may
show right-to-left shunting on echocardiography;
d) non-compaction of the left ventricle — which is a known
association.
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Figure 43. Apical four-chamber view of Ebstein’s anomaly demonstrating:
A) inferior displacement of the septal tricuspid valve leaflet (green arrow),
compared to the normally positioned anterior tricuspid valve leaflet origin
(red arrow) and septal mitral valve leaflet (blue arrow), thereby creating an
atrialised portion of the right ventricle (aRV), whilst the functional portion of
the right ventricle (fRV) is small; and B) severe tricuspid regurgitation, on the
corresponding colour flow Doppler image. RA = right atrium; LA = left atrium;
LV = left ventricle.
4 Congenital echocardiography
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The outflow echocardiographic views, namely the subcostal right
•
anterior oblique and parasternal short-axis views, are used to assess
the:
a) anterior tricuspid valve leaflet — which is frequently redundant
and curtain-like, and may cause right ventricular outflow
obstruction;
b) presence of any anatomical or functional pulmonary atresia.
An echocardiographic score to assess the severity of Ebstein’s
•
anomaly with prognostic implications has been used in neonates,
which involves calculating the ratio of the combined area of the right
atrium and atrialised right ventricle to that of the functional right
ventricle and left heart in a four-chamber view in end-diastole.
Recommended reading
1. Colan SD. The why and how of z scores.
40.
2. Silvestry FE, Cohen MS, Armsby LB, Burkule NJ, Fleishman CE, Hijazi ZM, Lang RM,
Rome JJ, Wang Y. Guidelines for the echocardiographic assessment of atrial septal
defect and patent foramen ovale: from the American Society of Echocardiography and
Society for Cardiac Angiography and Interventions.
28(8): 910-58.
3. Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C. Congenital heart surgery
nomenclature and database project: ventricular septal defect.
69(3): 25-35.
4. Rastelli GC, Ongley PA, Kirklin JW, McGoon DC. Surgical repair of the complete form
of persistent common atrioventricular canal.
299-308.
5. Need LR, Powell AJ, del Nido P, Geva T. Coronary echocardiography in tetralogy of
Fallot: diagnostic accuracy, resource utilization and surgical implications over 13
years.
g=^г=`здд=`~кЗбзд=
6. Hanley FL, Sade RM, Blackstone EH, Kirklin JW, Freedom RM, Nanda NC.
Outcomes in neonatal pulmonary atresia with intact ventricular septum. A
multiinstitutional study.
7. Craig JM, Darling RC, Rothney WB. Total pulmonary venous drainage into the right
side of the heart; report of 17 autopsied cases not associated with other major
cardiovascular anomalies.
8. Wernovsky G, Sanders SP. Coronary artery anatomy and transposition of the great
arteries.
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1993; 105(3): 406-23.
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1968; 55(3):
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169
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