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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5761_Библиотеки_им_академика_М_И_Перельмана
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58
ab
Fig. 19.7 The intermediate form of the AVSD with only one annulus and two orifi ces of the common AV valves ( arrow ) is patently visualized on
this TEE (0°) in diastole and systole ( a , b ). LA left atrium, LV left ventricle, RA right atrium, RV right ventricle
ab
Fig. 19.6 The common
atrioventricular valve
( arrowheads ) an the ASD
(ostium primum type) ( arrow )
are visualized on transesophageal
echocardiography ( TEE ) (0°) by
two-dimensional
echocardiography ( a ) and
color-fl ow Doppler study ( b ). LA
left atrium, LV left ventricle, RA
right atrium, RV right ventricle
Case 19 Atrioventricular Septal Defect (Intermediate Form)
59
Diagnosis
ASD ostium primum type in addition to inlet VSD and common atrioventricular valve, moderately severe left-sided
atrioventricular valve regurgitation due to cleft, intermediate
form of AVSD or complete form of AVSD.
Comment
On our recommendation, the patient underwent surgery
for the AVSD closure in tandem with mitral valve
repair.
Lesson
The intermediate form of the AVSD is a subtype of the
AVSD and is characterized by the fact that the common
atrioventricular valve has one annulus and two orifi ces. It
has the same physiology as the complete form of the AVSD,
but it has nearly the same anatomy as the partial AVSD. In
both forms of the intermediate and partial AVSDs, a tongue
tissue separates the common AV valve into two separate
right and left components [ 52 , 53 ].
There is another categorization for atrioventricular septal
defect in which AVSD is divided into complete and partial forms:
(a) In partial form, there is only ASD ostium primum, fusion
of the anterior and posterior bridging leafl et creating two
separate atrioventricular valves (left and right), and cleft
of the left atrioventricular valve and no VSD.
(b) In complete form, there is no fusion of the central part of
bridging leafl ets, so there is only one orifi ce and a defect
in crux of interventricular septum extending to both the
interventricular and interatrial septum [ 7 ].
In this case and other cases of AVSD, whenever the term
mitral or tricuspid valve is used, it is only for easier understanding of the echocardiographic images, and indeed, they
represent left and right atrioventricular valves.
ab
Fig. 19.8 This is a schematic illustration of the AVSD (intermediate
form) in TEE (0°) view in systole ( a ) and diastole ( b ). The single annu-
lus and the two orifi ces of the common atrioventricular valves as well as
the ASD (ostium primum type) and the ventricular septal defect are
visualized. LA left atrium, LV left ventricle, RA right atrium, RV right
ventricle
19 Atrioventricular Septal Defect (Intermediate Form)
61
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_20, © Springer International Publishing Switzerland 2015
A 26-year-old woman presented with dyspnea on exertion
(functional class II) of 1-year duration. Physical examination
revealed a harsh systolic murmur at the apex and the left
sternal border.
Atrioventricular Septal Defect
(Transitional Form)
Case 20
a b
Fig. 20.1 The parasternal short-axis view shows the anterior mitral leafl et (AML) cleft ( arrow ) ( a ). This is a schematic illustration of the AML
cleft in this view ( b ). LV left ventricle, RVOT right ventricular outfl ow tract
Fig. 20.2 The apical four-chamber view demonstrates the mitral and tri-
cuspid valves at one level, mitral regurgitation originating from the AML
cleft ( arrow ), and ventricular septal defect (VSD) fl ow ( curved arrow ). LA
left atrium, LV left ventricle, RA right atrium, RV right ventricle
Fig. 20.3 Transesophageal echocardiography (TEE) (0°) reveals an
atrial septal defect (ASD) (ostium primum type), measuring 17 mm in
size. LA left atrium, RA right atrium, RV right ventricle
62
Diagnosis
ASD ostium primum type in addition to inlet VSD and common atrioventricular valve, moderate left-sided atrioventricular valve regurgitation due to cleft, transitional form of
AVSD or complete form of AVSD.
Comment
The patient was referred for the surgical closure of the ASD
(ostium primum type) and the VSD as well as the repair of
the mitral cleft.
Lesson
In the transitional form of the AVSD, there are two annuluses and two orifi ces of the atrioventricular valve, and a
connective tissue separates the left and right atrioventricular valves. Consequently, there are two atrioventricular
valves, although these valves are at the same level. The
difference between the transitional and partial AVSDs is
that in the partial form, there is no VSD [ 52 , 53 ].
Fig. 20.4 This is a schematic illustration of the transitional form of the
atrioventricular septal defect (AVSD) on TEE (0°). LA left atrium, LV
left ventricle, RA right atrium, RV right ventricle
Case 20 Atrioventricular Septal Defect (Transitional Form)
63
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_21, © Springer International Publishing Switzerland 2015
A 35-year-old man presented with dyspnea on exertion
(functional class II) of recent duration. Physical examination
revealed a systolic murmur at the lower left sternal border
and the apex.
Atrioventricular Septal Defect
(Partial Form)
Case 21
Fig. 21.1 The right ventricular dilation and cleft of the anterior mitral
leafl et (AML) is evident in this parasternal long-axis view ( arrow ). LA
left atrium, LV left ventricle, RV right ventricle, AO aorta
Electronic supplementary material The online version of this
chapter (doi:
10.1007/978-3-319-12934-1_21 ) contains supplementary
material, which is available to authorized users.
64
ab
Fig. 21.3 An atrioventricular septal defect (AVSD) is shown in this
apical four-chamber view ( arrow ) ( a ) and right ventricular infl ow view
( arrow ) ( b ). The atrioventricular (AV) valves are at one level, and
moderate right ventricular dilation is also evident in these views. LA left
atrium, LV left ventricle, RA right atrium, RV right ventricle
Fig. 21.4 The apical four-chamber view illustrates a turbulent mitral
regurgitation fl ow, originating from the cleft of the AML ( arrow ). Also,
the fl ow through an atrial septal defect (ASD) (ostium primum type)
( curved arrow ) is evident. LA left atrium, LV left ventricle, RA right
atrium, RV right ventricle
ab
Fig. 21.2 The cleft of the AML is illustrated in the parasternal short-axis view ( arrow ) ( a ). The cleft is about 14 mm in size ( b ). LV left ventricle,
RV right ventricle
Case 21 Atrioventricular Septal Defect (Partial Form)
65
a
cd
b
Fig. 21.5 Transesophageal echocardiography (TEE) (0°) shows the
ASD (ostium primum type) ( arrow ) and the two atrioventricular valves,
which are at one level, in diastole ( a ) and systole ( b ). A schematic illus-
tration of the partial form of the AVSD in this view is shown in diastole
and systole ( c , d ). LA left atrium, LV left ventricle, RA right atrium, RV
right ventricle
Case 21 Atrioventricular Septal Defect (Partial Form)
66
Diagnosis
Partial form of atrioventricular septal defect including ASD
ostium primum, anterior mitral leafl et cleft, and moderate
mitral regurgitation.
Comment
The patient had a Qp/Qs of 2.7 and a systolic pulmonary
arterial pressure of 42 mmHg and moderate right ventricular
dilation. The patient was referred for surgery.
Lesson
In the partial form of the AVSD, there is an ASD (ostium primum
type) together with the cleft of the AML without a ventricular
septal defect [ 7 , 52 , 53 ]. Not all partial atrioventricular septal
defects are candidates for surgery, and surgery is indicated when
there is signifi cant volume overload of right ventricle or symptomatic patient with moderate to severe atrioventricular valve
regurgitation or asymptomatic patients with moderate or severe
left-sided atrioventricular valve regurgitation with left ventricular end-systolic diameter more than 45 mm and/or impaired left
ventricular systolic dysfunction [ 7 ].
ab
Fig. 21.7 There is no ventricular septal defect on TEE (short-axis view) ( a ) and (long-axis view) ( b )
Fig. 21.6 The fl ow of the moderate mitral regurgitation ( arrow ) and
the ASD (ostium primum type) ( curved arrow ) is shown on TEE (0°) in
systole. LA left atrium, LV left ventricle, RA right atrium, RV right
ventricle
Case 21 Atrioventricular Septal Defect (Partial Form)
67
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_22, © Springer International Publishing Switzerland 2015
A 57-year-old woman presented with dyspnea on exertion
(functional class II) of 1-year duration. She had a history of
breast ductal invasive carcinoma 2 years previously and
had undergone mastectomy and chemotherapy. She was
nulliparous. Physical examination revealed a holosystolic
murmur at the apex and the lower left sternal border.
Electrocardiography showed normal sinus rhythm and left
axis deviation.
Atrioventricular Septal Defect
(Transitional Form)
Case 22
ab
Fig. 22.1 The parasternal long-axis view shows the mild thickening
and doming of the mitral valve by two-dimensional echocardiography
( arrows ) ( a ). There is also moderate mitral regurgitation by color-fl ow
study ( arrow ) ( b ). LA left atrium, LV left ventricle, AO aorta, RV right
ventricle
Electronic supplementary material The online version of this
chapter (doi:
10.1007/978-3-319-12934-1_22 ) contains supplementary
material, which is available to authorized users.
68
ab
Fig. 22.3 Severe right ventricular dilation and an atrial septal defect
(ASD) (ostium primum type) ( arrow ) are evident in the apical four-
chamber view. The mitral and tricuspid valves are at the same level by
two-dimensional echocardiography ( a ). The left-to-right shunt through
the ASD (ostium primum type) is visualized in this view by color-fl ow
study ( arrow ) ( b ). LA left atrium, LV left ventricle, RA right atrium, RV
right ventricle
ab
Fig. 22.4 A small ventricular septal defect (VSD) ( arrow ) is evident in the apical four-chamber view ( a ) and on transesophageal echocardiogra-
phy ( TEE ) (long-axis view) ( b ). LA left atrium, LV left ventricle, RA right atrium, RV right ventricle, AO aorta
ab
Fig. 22.2 The parasternal long- and short-axis ( a , b ) views demonstrate the cleft of the mitral valve ( arrow ). LA left atrium, LV left ventricle, AO
aorta, RV right ventricle
Case 22 Atrioventricular Septal Defect (Transitional Form)
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