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35
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_12, © Springer International Publishing Switzerland 2015
A 31-year-old woman presented with dyspnea on exertion
(functional class III). O2 saturation was 83 % in the room air
and reached 91 % with O2. Physical examination revealed a
loud S2 and clubbing. Electrocardiography showed rightaxis deviation and R > S in the right precordial leads.
Atrial Septal Defect
(Ostium Secundum Type)
with Eisenmenger’s Syndrome
Case 12
Fig. 12.1 The parasternal long-axis view shows severe right ventricu-
lar dilation and right ventricular wall hypertrophy ( arrow ). 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_12 ) contains supplementary
material, which is available to authorized users.
36
ab
c
Fig. 12.2 There is no evidence of pulmonary stenosis in the paraster-
nal short-axis view. Also, there is no turbulent fl ow in the pulmonary
artery in systole ( a ), and there is mild pulmonary regurgitation ( arrow )
( b ). The pulmonary arterial diastolic pressure ( PAPd ) is equal to the
end-diastolic gradient of the pulmonary regurgitation fl ow by continuous wave Doppler study plus the right atrial pressure (25 + 10 = 35 mmHg)
( c ). LA left atrium, RA right atrium, RVOT right ventricular outfl ow
tract, PA pulmonary artery
Fig. 12.3 The main pulmonary artery is severely dilated and measures
approximately 46 mm in the parasternal short-axis view. PA pulmonary
artery
Case 12 Atrial Septal Defect (Ostium Secundum Type) with Eisenmenger’s Syndrome
37
a
b
Fig. 12.5 Severe right ventricular dilation is seen in the apical four-
chamber view ( a ). The apex is found by the right ventricle ( arrow ) ( a ).
There is a large atrial septal defect ( ASD ) (ostium secundum type) with
a bidirectional red and blue fl ow across it ( arrows ) ( b ). LA left atrium,
LV left ventricle, RA right atrium, RV right ventricle
a
b
Fig. 12.4 Moderate tricuspid regurgitation ( arrow ) is seen in the apical
four-chamber view ( a ). The tricuspid regurgitation gradient is 94 mmHg
( b ). RA right atrium, RV right ventricle
Case 12 Atrial Septal Defect (Ostium Secundum Type) with Eisenmenger’s Syndrome
38
The shunt was measured as follows:
Pulmonary annulus = 2.8 cm, pulmonary velocity–time
integral (VTI) =11 cm, HR =90
Aortic annulus =1.8 cm, aortic VTI = 23 cm
Qp l= ××××=0 785 2 8 2 8 11 90 6 01... ./min
Qs l= ××××=0 785 1 8 1 8 23 90 5 26... ./min
The Qp/Qs was 1.14, which is relatively small compared to
the size of the ASD.
Pulmonary vascular resistance (PVR) = mean pulmonary
arterial pressure (PAP)/Qp.
PAP mean PAPd PAPs mmHg=+ =++==2 3 35 35 104 3 174 3 58///
Pulmonary vascular resistance PVR PAP mean Qp un
()
===//..58 6 01 9 65 iits
The patient’s blood pressure was 110/80 mmHg.
Systemic vascular resistance units=+ = =110 80 2 3 90 5 26 17 11*/ /. .
Nasal O2 was commenced for the patient for 10 min.
Subsequently, the measurements of the shunts and PVR were
repeated: no signifi cant change had occurred. Accordingly,
treatment with sildenafi l and bosentan was started, and she
was referred for catheterism
Diagnosis
Our patient was diagnosed with an ASD with Eisenmenger’s
syndrome.
Comment
Medical treatment with bosentan and sildenafi l was recommended for the patient and follow-up echocardiography
[ 24 – 28 ].
Lesson
1. When there is right ventricular free wall hypertrophy,
there are two possible diagnoses: pulmonary stenosis and
pulmonary hypertension.
2. On transthoracic echocardiography, the ASD fl ow is
always red when there is a left-to-right shunt. The color
blue in the ASD is in favor of the passage of blood from
right to left and an increased PVR.
3. It is said that patients with ASDs go toward Eisenmenger’s
syndrome with lesser degrees of PAP. If in other left-toright shunts such as ventricular septal defects a PVR of
less than 7 units is considered suitable for intervention, in
the ASD the fi gure is 5 units.
4. In case of a PVR ≥ 5 units in an ASD, the reactivity of the
pulmonary vascular bed to vasodilators like oxygen
should be checked [ 7 ]. A reduction in the PVR ≥33 % [ 2 ]
or reduction of mean pulmonary arterial pressure
≥10 mmHg to reach an absolute value of less than
40 mmHg is considered as a good response [ 29 , 30 ].
5. The defi nite diagnosis of pulmonary arterial hypertension is made by catheterism. A mean PAP greater than
25 mmHg is considered as pulmonary hypertension
[ 29 ].
6. In Eisenmenger’s syndrome, the shunt is right to left or
bidirectional, and the PAP is near systemic.
7. In patients with ASD and Eisenmenger’s syndrome,
genetic mutation has been suggested [ 31 ].
Case 12 Atrial Septal Defect (Ostium Secundum Type) with Eisenmenger’s Syndrome
39
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_13, © Springer International Publishing Switzerland 2015
A 57-year-old woman, who had undergone device closure
for an atrial septal defect (ASD) (ostium secundum type)
2 months previously, was referred for transthoracic echocardiography. She had no symptoms.
Follow-up Echocardiography
of Atrial Septal Defect
Device Closure
Case 13
ab
Fig. 13.1 The apical four-chamber view shows the good position of
the Amplatzer ( arrow ) ( a ). There is no evidence of turbulency and
obstructive effects on the other organs, and there is no residual shunt
fl ow across the interatrial septum ( arrow ) ( b ). LA left atrium, LV left
ventricle, RA right atrium, RV right ventricle
40
Diagnosis
The ASD device closure yielded excellent results without
any residual shunt and obstructive effect on the other organs.
Lesson
Some of the possible complications of ASD device closure
include partial obstruction of left ventricular outfl ow tract
due to device embolization [ 32 ] and aorto-right atrial
fi stula [ 33 ].
LA
RA
Fig. 13.2 The Amplatzer ( arrow ) is also evident in the parasternal
short-axis view without turbulency and shunt fl ow by color Doppler
study. LA left atrium, RA right atrium
Case 13 Follow-up Echocardiography of Atrial Septal Defect Device Closure
41
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_14, © Springer International Publishing Switzerland 2015
A 40-year-old woman presented with dyspnea on exertion
(functional class II) of recent duration.
Atrial Septal Defect (Ostium
Secundum Type) with a Prominent
Chiari Network
Case 14
ab
Fig. 14.1 There is severe dilation of the right atrium and right ventri-
cle. A defect ( arrow ) can also be detected in the interatrial septum,
which is in favor of an atrial septal defect (ostium secundum type).
There is a fi lamentous mobile mass in the right atrium ( curved arrow ),
suggestive of the Chiari network ( a ). The passage of the fl ow ( arrow )
from the atrial septal defect (ostium secundum type) is demonstrated
( b ). 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_14 ) contains supplementary
material, which is available to authorized users.
42
Diagnosis
ASD ostium secundum with adequate rims and prominent
Chiari network.
Comment
The patient was referred for ASD device closure.
Lesson
1. For differentiation of the eustachian valve from the Chiari
network, there are two essential points: (a) the eustachian
valve originates from the posterior border of the inferior
vena cava and continues up to the fossa ovalis, while the
Chiari network originated near the orifi ce of the inferior vena
cava and serves as the valve of the coronary sinus, and (b)
usually the Chiari network is highly mobile. Both the eustachian valve and Chiari network are normal variation [ 34 ].
2. When ASD produces a remarkable shunt, the right ventricle becomes dilated.
(a) Severe right ventricular dilation usually represents
signifi cant left-to-right shunt (Qp/Qs>2) [ 2 ], diagnosis of
severe right ventricular dilation is made by the apex
which is formed by the right ventricle, and the ratio of the
right ventricular to left ventricular diameter is more than
1 in the apical four-chamber view [ 35 ]. (b) Moderate
right ventricular dilation is diagnosed by the apex equally
made from the right and left ventricles, and the ratio of
the right ventricular to left ventricular diameter is equal
to 1. (c) Mild right ventricular dilation is present when
the ratio of the right ventricular to left ventricular diameter is more than 2/3, but the right ventricle does not
reach to the apex [ 35 ].
a
b
Fig. 14.2 Transesophageal echocardiography (bicaval view) shows
that this fi lamentous mobile mass ( arrow ) originates near the inferior
vena cava; this is in favor of the Chiari network ( a ). This mass extends
up to the tricuspid valve ( arrow ) ( b ). LA left atrium, RA right atrium,
SVC superior vena cava, IVC inferior vena cava
Case 14 Atrial Septal Defect (Ostium Secundum Type) with a Prominent Chiari Network
43
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_15, © Springer International Publishing Switzerland 2015
A 24-year-old woman was referred to our echocardiography
laboratory for the evaluation of the feasibility of the
Amplatzer device closure of an atrial septal defect (ASD).
Transthoracic echocardiography revealed severe right ventricular dilation and a large ASD (ostium secundum type).
Extremely Large Atrial Septal
Defect (Ostium Secundum Type)
with Severe Tricuspid Regurgitation
and Severe Annulus Dilation
Case 15
RA
LA
LVRV
a
b
c
Fig. 15.1 Severe tricuspid regurgitation (TR) is shown in the apical four-chamber view ( arrow ) ( a ). The TR gradient is 45 mmHg ( b ). The tricus-
pid annulus is 43 mm in this view ( c ). 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_15 ) contains supplementary
material, which is available to authorized users.
44
Diagnosis
The patient was diagnosed with an extremely large ASD
(ostium secondum type) (42 mm) with severe TR and signifi cant annulus dilation. The tricuspid annulus was 43 mm in size.
Comment
Considering the large ASD and severe TR with annulus dilation, surgery (ASD closure and tricuspid annuloplasty) was
recommended.
Lesson
1. Large ASDs (ostium secundum type) are not suitable for
device closure. ASDs larger than 35 mm may need special techniques [ 17 ].
2. Even mild TR with signifi cant annulus dilation (≥40 mm
or >21 mm/m
2
) at the time of cardiac surgery should be
repaired [ 36 ].
RA
LA
a
RA
LA
b
Fig. 15.3 TEE (bicaval view) shows the large ASD ( arrow ). The ASD/IAS is 42/66 mm. LA left atrium, RA right atrium
RA
a
AO
LA
RA
LA
AO
b
Fig. 15.2 The large ASD is evident on transesophageal echocardiography (TEE) (short-axis view) by color Doppler study ( arrow ) ( a ). The
ASD/interatrial septum (IAS) is 36/45 mm ( b ). LA left atrium, RA right atrium, AO aorta
Case 15 Extremely Large ASD (Ostium Secundum Type) with Severe Tricuspid Regurgitation
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