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Fig. 36.2 The dilated coronary sinus is also evident in the apical four-chamber view ( arrow ) ( a ). A schematic illustration of the dilated coronary
sinus in this view is presented ( b ). LA left atrium, LV left ventricle, RA right atrium, RV right ventricle
ba
Fig. 36.3 The modifi ed apical four-chamber view with some posterior
angulation shows that the coronary sinus is dilated and intact throughout its course ( arrow ) ( a ). This is a schematic illustration of the dilated
coronary sinus in this view ( b ). LV left ventricle, RA right atrium, RV
right ventricle, CS coronary sinus
Case 36 Left Persistent Superior Vena Cava and Cerebral Stroke

113
Diagnosis
The patient was diagnosed with ischemic cerebral stroke, left
persistent SVC, and left ventricular hypertrophy.
Recommendation
Treatment and work-up was recommended for the patient’s
hypertension, and he was followed up for his left persistent
SVC.
Lesson
The normal range for coronary sinus diameter is between 4
and 10 mm [ 65 ].
Fig. 36.5 Transesophageal echocardiography (0°) in the lower esoph-
ageal level demonstrates that the coronary sinus is dilated and intact
throughout its course ( arrow ). CS coronary sinus, LV left ventricle
ba
Fig. 36.4 Following a contrast injection in the patient’s left arm,
bubbles appear fi rst in the coronary sinus, which is in favor of a left
persistent superior vena cava (SVC). Also, no bubbles appear in the left
atrium and the left ventricle in the parasternal long-axis view ( a ). This
is a schematic depiction of the contrast appearance, fi rst in the coronary
sinus ( b ). LV left ventricle, AO aorta, DAO descending aorta, RV right
ventricle, CS coronary sinus
Case 36 Left Persistent Superior Vena Cava and Cerebral Stroke

115
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_37, © Springer International Publishing Switzerland 2015
A young woman with a history of palpitation was referred to our
echocardiography laboratory. Physical examination was nor-
mal, and echocardiography revealed normal left and right ventricular sizes and functions as well as a dilated coronary sinus.
Left Persistent Superior Vena Cava
Case 37
a
b
Fig. 37.1 The suprasternal long-axis view with mild displacement and
tiling of the probe to the left reveals a laminar blue color fl ow, which
drains into the coronary sinus ( a ). Continuous fl ow Doppler study
shows a continuous fl ow, in favor of a left persistent superior vena cava
(SVC) fl ow ( b ). LPSVC left persistent superior vena cava
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_37 ) contains supplementary mate-
rial, which is available to authorized users.

116
Diagnosis
The patient was diagnosed with a left persistent SVC.
Comment
Because there was no evidence of an unroofed coronary
sinus, no appearance of the agitated saline in the left atrium,
and no associated anomaly, only follow-up echocardiography was recommended for the patient.
Fig. 37.3 After the injection of agitated saline into the patient’s left
arm, the agitated saline fi rst appears in the coronary sinus and then in
the right side: this is indicative of a left persistent SVC. The left persistent SVC is connected to the coronary sinus, which in turn is connected
to the right atrium. The curved arrow points at the coronary sinus. LAA
left atrial appandage, RA right atrium
Fig. 37.2 Transesophageal echocardiography (TEE) (0°) at the lower
esophageal level visualizes an intact and dilated coronary sinus, which
drains into the right atrium. The arrow points to the coronary sinus, and
the curved arrow shows the tricuspid valve. RA right atrium, LV left
ventricle
Case 37 Left Persistent Superior Vena Cava

117
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_38, © Springer International Publishing Switzerland 2015
An 11-year-old girl presented with dyspnea on exertion
(functional class II). Physical examination showed a fi xed S2
splitting and an ejection systolic murmur at the upper left
sternal border. Electrocardiography revealed right axis deviation and right ventricular hypertrophy.
Unroofed Coronary Sinus
Case 38
Fig. 38.1 The parasternal long-axis view illustrates an unroofed coro-
nary sinus at its origin ( arrow ). LA left atrium, LV left ventricle, RV
right ventricle, AO aorta, CS coronary sinus
Fig. 38.2 Dilated coronary sinus as well as the defect of the wall of the
coronary sinus in its course ( arrow ). LA left atrium, LV left ventricle,
RA right atrium, RV right ventricle, CS coronary sinus
Electronic supplementary material The online version of this
chapter (doi:
10.1007/978-3-319-12934-1_38 ) contains supplementary
material, which is available to authorized users.

118
Diagnosis
The patient was diagnosed with an unroofed coronary sinus
complete form.
Comment
The patient was referred for surgery after cardiac
catheterism.
Lesson
An unroofed coronary sinus is a type of an atrial septal
defect. It is usually associated with a left persistent superior
vena cava and produces a right-to-left shunt. This defect may
create some degree of cyanosis [ 7 , 60 ].
There is a classifi cation by Kirklin and Barratt-Boyes for
unroofed coronary sinus:
Type I: Completely unroofed with left persistent superior
vena cava
Type II: Completely unroofed without left persistent supe-
rior vena cava
Type III: Partially unroofed midportion
Type IV: Partially unroofed terminal portion [ 66 ]
According to this classifi cation, this patient is type I.
ab
Fig. 38.3 The apical four-chamber view with some posterior angula-
tion reveals the defect of the coronary sinus wall to the left atrium in its
course ( arrow ) by two-dimensional echocardiography ( a ) and the pas-
sage of the fl ow from the coronary sinus to the left atrium ( arrow )
( b ). LA left atrium, LV left ventricle, RA right atrium, RV right ventricle,
CS coronary sinus
Case 38 Unroofed Coronary Sinus

119
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_39, © Springer International Publishing Switzerland 2015
A young woman with a history of palpitation and dyspnea on
exertion (functional class II–III) and a history of an atrial
septal defect (ASD) (ostium secundum type) was admitted
for surgery. She had a systolic ejection murmur (grade III/
IV) in the pulmonic area and a fi xed splitting S2.
Echocardiography revealed a normal left ventricular size
and systolic function and a severely dilated right ventricle
with a good systolic function.
Ostium Secundum Accompanied
by Sinus Venosus Atrial Septal
Defects and Valvular Pulmonary
Stenosis
Case 39
Fig. 39.1 The apical four-chamber view depicts the severe dilation of
the right ventricle as well as a large ASD (ostium secundum type)
( arrow ). LA left atrium, LV left ventricle, RA right atrium, RV right
ventricle
Electronic supplementary material The online version of this
chapter (doi:
10.1007/978-3-319-12934-1_39 ) contains supplemen-
tary material, which is available to authorized users.

120
ba
Fig. 39.2 Transesophageal
echocardiography ( TEE )
(four-chamber view) confi rms the
presence of the ASD (ostium
secundum type) ( arrow ) by
two-dimensional
echocardiography ( a , left ) and
color Doppler study ( b , right ).
LA left atrium, RA right atrium,
RV right ventricle
ab
Fig. 39.3 Another ASD in the posterior part of the interatrial septum is visualized on TEE (18°). The fl ow across the ASD is bidirectional, red ( a )
and blue ( b ). LA left atrium, RA right atrium, RV right ventricle
Case 39 Ostium Secundum Accompanied by Sinus Venosus Atrial Septal Defects and Valvular Pulmonary Stenosis

121
ba
Fig. 39.5 A thick and dome-shaped pulmonary valve ( a ) with a maxi-
mum peak gradient of about 68 mmHg ( b ) can be seen on transthoracic
echocardiography (short-axis view) and continuous wave Doppler
study, indicative of a severe valvular pulmonary stenosis. PA pulmo-
nary artery, PV pulmonary valve, AOV aortic valve
ba
Fig. 39.4 TEE (bicaval view)
depicts the ASD (sinus venosus
type) ( arrow ) below the superior
vena cava ( SVC ). SVC superior
vena cava, RA right atrium, LA
left atrium, RPA right pulmonary
artery. ( a ) left , two-dimensional
echocardiography; ( b ) right ,
color Doppler study
Case 39 Ostium Secundum Accompanied by Sinus Venosus Atrial Septal Defects and Valvular Pulmonary Stenosis

122
Diagnosis
The patient was diagnosed with a combination of the ostium
secundum and sinus venosus types of the ASD with a concomitant severe valvular pulmonary stenosis.
Comment
The patient was referred for surgery after cardiac catheterism.
Lesson
1. The association between two ASD types, e.g., secundum
type and sinus venosus type, is not common.
2. The incidence rate of the combination of an ASD and a
valvular pulmonary stenosis is about 5 % [ 67 ]. Usually
in a large ASD, there is an overfl ow gradient across the
pulmonary valve (up to 30 mmHg peak gradient [ 61 ]).
In the overfl ow gradient, the pulmonary valve is not
severely thick and dome shaped, although some thickening of the pulmonary valve may occur due to the
overfl o w .
Case 39 Ostium Secundum Accompanied by Sinus Venosus Atrial Septal Defects and Valvular Pulmonary Stenosis
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