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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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Fig. 121.5 Moderately severe aortic regurgitation ( arrow ) is also vis-
ible in this apical fi ve-chamber view. LA left atrium, LV left ventricle,
RV right ventricle, AO aorta
Fig. 121.6 The parasternal short-axis view shows that there is no
pulmonary valve ( arrow ). AO aorta, RVOT right ventricular outfl ow
trcat
ab
Fig. 121.7 The suprasternal long-axis view illustrates the continuous fl ow of the major aortopulmonary collaterals (MAPCA) by the color-fl ow
study ( a ) and the continuous wave Doppler ( b ). PA pulmonary artery, AO aorta
Fig. 121.8 An atretic pulmonary valve ( arrow ) and the proximal part
of the main pulmonary artery are visible on transesophageal echocardiography (TEE) (short-axis view) ( curved arrow ). LA left atrium,
RA right atrium, AO aorta
Fig. 121.9 TEE (long-axis) view shows the perimembranous VSD
( arrow ) and also reveals that in the overriding of the aorta, more than
50 % of the aorta arises from the right ventricle. Thus, the diagnosis is
a double-outlet right ventricle. LA left atrium, LV left ventricle,
AO aorta, RV right ventricle
Case 121 Double-Outlet Right Ventricle, Atretic Pulmonary Valve, and Subaortic Ventricular Septal Defect
398
Diagnosis
The patient was diagnosed with a double-outlet right ventricle,
pulmonary atresia, and subaortic VSD with a bidirectional
fl ow across it. The MAPCA fi lled the pulmonary branches.
Diagnosis
Double-outlet right ventricle, perimembranous VSD, atretic
pulmonary valve and main pulmonary artery, MAPCA, moderately severe aortic regurgitation, and dilation of ascending aorta
Comment
The patient was referred for cardiac catheterism. With
respect to cyanosis, the fl ow of the MAPCA is not suffi cient;
consequently, a systemic-to-pulmonary shunt seems logical
for increasing the pulmonary blood fl ow.
Lesson
The rule of 50 %: if more than 50 % of the aorta arises from
the left ventricle, the diagnosis is the tetralogy of Fallot with
pulmonary atresia, but if more than 50 % of the aorta arises
from the right ventricle, the diagnosis is a double-outlet
right ventricle with pulmonary atresia and a subaortic VSD
[ 140 , 141 ].
ab
Fig. 121.10 The vena contracta of the aortic regurgitation is about 6 mm ( a ), and the aortic annulus, sinus of Valsalva, sinotubular junction, and
ascending aorta are 38, 53, 49, and 54 mm in size, respectively, ( b ) on TEE (long-axis view). AO aorta
Fig. 121.11 TEE (0°) at the base of the heart, with a clockwise rota-
tion, reveals the right pulmonary artery, which measures 25 mm. LA left
atrium. RPA right pulmonary artery
Fig. 121.12 TEE (short-axis view) shows the distal portion of the
main pulmonary artery, which measures about 20 mm. AO aorta
Case 121 Double-Outlet Right Ventricle, Atretic Pulmonary Valve, and Subaortic Ventricular Septal Defect
399
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_122, © Springer International Publishing Switzerland 2015
A 22-year-old man presented with fever of 1-month dura-
tion. He was a known case of dextrocardia and situs inversus
from childhood. Physical examination revealed a systolic
ejection murmur at the right sternal border and right precordium, cyanosis and clubbing, an ejection systolic murmur at
the second right intercostal space, and a holosystolic murmur
at the right apex. On transthoracic echocardiography, the left
atrium was located on the right side of the right atrium. All
the images were obtained from the right precordium, with
the patient lying in the right lateral decubitus position.
Situs Inversus, Dextrocardia,
Corrected Transposition
of Great Arteries, Subpulmonic
Ventricular Septal Defect,
and Pulmonary Stenosis
Case 122
a
b
Fig. 122.1 The right apical four-chamber view shows the left atrium,
which receives the pulmonary veins ( arrows ). The left atrial appendage
is inserted into the tricuspid valve, which has a lower insertion ( arrow-
head ) than the mitral valve, and connects to the right ventricle, whose
papillary muscles are attached to the interventricular septum ( curved
arrows ) ( a ). This is a schematic illustration of the ventricular inversion
in the corrected transposition of the great arteries and situs inversus in
the right apical four-chamber view ( b ). LA left atrium, RV right ventri-
cle, RA right atrium, LV left ventricle, LAA left atrial appendage, PV
pulmonary vein
Electronic supplementary material The online version of this chap-
ter (doi:
10.1007/978-3-319-12934-1_122 ) contains supplementary
material, which is available to authorized users.
400
a
b
c
Fig. 122.2 The right parasternal short-axis view shows the pulmonary
artery and the aorta side by side. The aorta is located anteriorly to the
pulmonary valve ( a ). The great artery, which is divided, is located pos-
teriorly; it is, therefore, the pulmonary artery ( arrows ) ( b ). This is a
schematic illustration of the position of the great arteries in this anomaly in the parasternal short-axis view ( c ). PA pulmonary artery, AO aorta,
LV left ventricle, RV right ventricle
a
b
Fig. 122.3 The right parasternal short-axis view by color Doppler
echocardiography shows that the great artery, which is located posteriorly, is divided ( arrows ) ( a ) and is, thus, the pulmonary artery. There is
systolic and diastolic turbulency within the pulmonary artery and its
branches ( arrows ) ( b ). AO aorta, PA pulmonary artery
Case 122 Situs Inversus, Dextrocardia, Corrected Transposition of Great Arteries
401
ab
Fig. 122.4 The right parasternal long-axis view reveals that there is
discontinuity between the systemic atrioventricular valve and the pulmonary valve. Indeed the systemic ventricle does not connect to this
great artery ( arrow ) (diastolic and systolic ( a , b )). LA left atrium, RV
right ventricle, PA pulmonary artery
Fig. 122.5 The right parasternal long-axis view shows moderately
severe systemic atrioventricular valve regurgitation ( arrow ). There is
also systolic ( curved arrow ) in the great artery, which is the pulmonary
artery ( arrow ). The systolic turbulency begins below the pulmonary
valve. LA left atrium, RV right ventricle, PA pulmonary artery
Case 122 Situs Inversus, Dextrocardia, Corrected Transposition of Great Arteries
402
ab
c
Fig. 122.6 Two-dimensional echocardiography ( a ) and the color-fl ow
Doppler study ( b ) in the right apical four-chamber view demonstrate a
ventricular septal defect ( VSD ) ( arrow ). A schematic illustration of the
VSD in the corrected transposition of the great arteries is shown in the
right apical four-chamber view ( c ). LA left atrium, RV right ventricle,
RA right atrium, LV left ventricle, LAA left atrium
Case 122 Situs Inversus, Dextrocardia, Corrected Transposition of Great Arteries
403
a
b
c
Fig. 122.7 The right apical fi ve-chamber view shows that the right
atrium, which is located on the left side of the left atrium, connects
through the mitral valve to the left ventricle, which connects to a great
artery (the pulmonary artery), which has a relationship with the VSD
( arrow ) ( a ). Thus, the VSD is subpulmonic. There is also systolic
turbulency ( arrow ) ( b ) across the pulmonary valve with a gradient
of about 82 mmHg ( c ). RA right atrium, LV left ventricle, RV right
ventricle, PA pulmonary artery
Case 122 Situs Inversus, Dextrocardia, Corrected Transposition of Great Arteries
404
Fig. 122.8 The right parasternal long-axis outlet view reveals that
the right ventricle, which is the systemic ventricle, connects to the
aorta. RV right ventricle, AO aorta
a
b
Fig. 122.9 Transesophageal echocardiography (TEE) (0°) depicts the
VSD ( curved arrow ) ( a ). The left atrium, which is located posteriorly,
connects to an atrioventricular valve, which has a lower insertion than
the mitral valve ( arrow ) and is, therefore, the tricuspid valve ( double
arrow ). A schematic illustration of the VSD and the ventricular inversion is shown in this view ( b ). LA left atrium, RV right ventricle, RA
right atrium, LV left ventricle
Fig. 122.10 TEE (0°) view reveals moderately severe systemic
atrioventricular valve regurgitation ( arrow ). LA left atrium,
RV right ventricle
Case 122 Situs Inversus, Dextrocardia, Corrected Transposition of Great Arteries
405
a b
Fig. 122.11 TEE (0°) view shows that the VSD is 16 mm in size ( a ). A turbulent fl ow across the VSD in TEE long-axis view ( arrow ) via the
color-fl ow study ( b ). LA left atrium, RA right atrium, RV right ventricle, LV left ventricle
a
b
Fig. 122.12 A patent foramen ovale (PFO) ( arrow ) is evident on TEE
(short-axis view) by two-dimensional echocardiography ( a ). This is a
schematic illustration of the PFO in this view ( b ). LA left atrium, RA
right atrium, LV left ventricle, RAA right atrial appendage, PFO patent
foramen ovale
Case 122 Situs Inversus, Dextrocardia, Corrected Transposition of Great Arteries
406
a
c
b
d
Fig. 122.13 The aorta and the pulmonary valve are located side by
side on TEE (short-axis view) ( a ). The aorta is located anteriorly, and
there is a conus beneath the aortic valve ( arrow ). This is a schematic
illustration of the great arteries in this view ( b ). The position of the
great arteries is shown in 96° ( c ) and 120° ( d ) views. LA left atrium, RV
right ventricle, PA pulmonary artery, AO aorta
Fig. 122.14 There is no fl ow from the systemic ventricle toward the
pulmonary artery ( arrow ). LA left atrium, RV right ventricle, PA pulmo-
nary artery, AO aorta
Case 122 Situs Inversus, Dextrocardia, Corrected Transposition of Great Arteries
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