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H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_40, © Springer International Publishing Switzerland 2015
A 22-year-old man referred to our echocardiography labora-
tory because of dyspnea on exertion (functional class II).
Physical examination revealed pectus excavatum and a systolic ejection murmur (grade III/VI) at the second left intercostal space. Electrocardiography showed right axis
deviation and right ventricular hypertrophy.
Atrial Septal Defect (Ostium
Secundum Type) with Valvular
Pulmonary Stenosis
Case 40
Fig. 40.1 The parasternal long-axis view shows right ventricular wall
hypertrophy ( arrow ). LA left atrium, LV left ventricle, AO aorta
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_40 ) contains supplementary mate-
rial, which is available to authorized users.
124
a
c
b
Fig. 40.2 The parasternal short-axis view demonstrates a turbulent
fl ow in the pulmonary artery ( a ), accompanied by a thick and dome-
shaped pulmonary valve ( arrow ) ( b ) with a peak gradient of 68 mmHg
( c ), in favor of severe valvular pulmonary stenosis. RVOT right ven-
tricular outfl ow tract, PA pulmonary artery, AO aorta
Fig. 40.3 The apical four-chamber view shows that despite right atrial
dilation and right ventricular free wall hypertrophy, there is no right
ventricular dilation. LA left atrium, LV left ventricle, RA right atrium,
RV right ventricle
Fig. 40.4 Transesophageal echocardiography (TEE) (0°) reveals an
atrial septal defect (ASD) (ostium secundum type) ( arrow ). LA left
atrium, RA right atrium, AO aorta
Case 40 Atrial Septal Defect (Ostium Secundum Type) with Valvular Pulmonary Stenosis
125
a
c
b
Fig. 40.5 TEE (bicaval view) demonstrates the malalignment of the
interatrial septum (IAS) (4 a ). The degree of the malalignment is 17 mm
( b ). This is a schematic illustration of the IAS malalignment ( c ). LA left
atrium, RA right atrium, IAS interatrial septum, SVC superior vena cava,
IVC inferior vena cava
a b
Fig. 40.6 Another ASD (ostium secundum type) can be seen above the fi rst defect ( arrow ) ( a ). This is a schematic presentation of these defects
( b ). LA left atrium, RA right atrium, AO aorta, ASD atrial septal defect, ASDOS atrial septal defect ostium secundum type
Case 40 Atrial Septal Defect (Ostium Secundum Type) with Valvular Pulmonary Stenosis
126
Diagnosis
The patient was diagnosed with two ASDs (ostium secundum type), malalignment [ 17 ] of the IAS, and severe valvular pulmonary stenosis.
Comment
Given the patient’s two ASDs and especially the malalignment of the IAS as well as severe pulmonary valve stenosis,
we recommended surgery. Surgery consisted of the repair of
the ASDs and the valvotomy of the pulmonary valve.
a
b
Fig. 40.7 The doming of the pulmonary valve and the hypertrophy of
the right ventricular outfl ow tract are patent on TEE (short-axis view)
( arrow ) ( a ). A schematic depiction of these abnormalities is presented
in this view ( b ). LA left atrium, RA right atrium, AO aorta, RV right
ventricle, PV pulmonary valve, RVOT right ventricular outfl ow tract,
PA pulmonary artery
Fig. 40.8 The dilation of the sinus of Valsalva is demonstrated on TEE
(long-axis view). LA left atrium, LV left ventricle, AO aorta
Case 40 Atrial Septal Defect (Ostium Secundum Type) with Valvular Pulmonary Stenosis
127
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_41, © Springer International Publishing Switzerland 2015
A 37-year-old man presented with dyspnea on exertion
(functional class II). Transthoracic echocardiography showed
normal left ventricular size and systolic function, severe
right atrial and ventricular dilation, mild tricuspid regurgitation, and systolic pulmonary arterial pressure of about
42 mmHg.
Abnormal Left Pulmonary Venous
Return to the Coronary Sinus
Case 41
a
b
Fig. 41.1 The parasternal long-axis view illustrates severe right
ventricular dilation by two-dimensional echocardiography ( a ). A para-
doxical septal motion is evident on M-mode echocardiography ( arrow )
( b ). 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_41 ) contains supplementary
material, which is available to authorized users.
128
a
b
Fig. 41.2 The parasternal short-axis view demonstrates no atrial septal defect (ASD) by two-dimensional echocardiography ( a ) and color Doppler
study ( b ) ( arrows ). LA left atrium, RA right atrium, RVOT right ventricular outfl ow tract, AO aorta, PA pulmonary artery
ab
Fig. 41.3 Severe right ventricular dilation is evident in the apical four-chamber view ( a ). The interatrial septum (IAS) seems intact by two-
dimensional echocardiography ( a ) and color Doppler study ( b ). LA left atrium, LV left ventricle, RA right atrium, RV right ventricle
Case 41 Abnormal Left Pulmonary Venous Return to the Coronary Sinus
129
Fig. 41.4 The apical four-chamber view with some posterior angula-
tion of the probe for the evaluation of the ASD (sinus venosus type)
reveals no defect and a normal connection between the right upper pulmonary vein and the left atrium ( arrow )
Fig. 41.5 Right ventricular
systolic function is about 37 %
by multiplane modality of a
four-dimensional probe
Fig. 41.6 A precise evaluation of the left atrium shows an echo-free
space ( arrow ) behind the left atrium in short-axis view. LA left atrium
Case 41 Abnormal Left Pulmonary Venous Return to the Coronary Sinus
130
Fig. 41.7 The injection of agitated saline in the patient’s left arm with
a slight posterior angulation of the probe in the apical four-chamber
view for visualizing the coronary sinus ( arrow ) shows no bubbles in the
coronary sinus. This rules out a left persistent superior vena cava
(SVC). CS coronary sinus
ab
Fig. 41.8 The modifi ed apical four-chamber view demonstrates the intact course of the coronary sinus ( arrow ) by two-dimensional echocardiog-
raphy ( a ) and color Doppler study ( b ). LV left ventricle, RA right atrium, RV right ventricle, CS coronary sinus
Case 41 Abnormal Left Pulmonary Venous Return to the Coronary Sinus
131
Fig. 41.9 Transesophageal echocardiography (TEE) (0°) shows a
dilated coronary sinus, up to 16 mm, at the insertion site to the right
atrium. LV left ventricle, RA right atrium, RV right ventricle, CS coro-
nary sinus
a b
Fig. 41.10 TEE (0°) reveals an intact interatrial septum (IAS) and a space ( arrow ) behind the left atrium, which is separated from it by a mem-
brane in systole ( a ) and diastole ( b ). This space is the venous confl uence. LA left atrium, LV left ventricle, VC venous confl uence
Case 41 Abnormal Left Pulmonary Venous Return to the Coronary Sinus
132
Fig. 41.11 The fl ow of the venous confl uence is evident in red ( arrow )
on TEE (0°). LA left atrium, LV left ventricle, VC venous confl uence
ab
Fig. 41.12 The coronary sinus is intact in its course and at its entrance
into the right atrium on TEE (0°) by two-dimensional echocardiography ( a ) and color Doppler study ( b ) ( arrows ). These fi ndings rule out an
unroofed coronary sinus. LV left ventricle, RV right ventricle, RA right
atrium, CS coronary sinus
Case 41 Abnormal Left Pulmonary Venous Return to the Coronary Sinus
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