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133
Fig. 41.13 The right pulmonary veins connect normally to the left
atrium on TEE (short-axis view) with a clockwise rotation ( arrows ). LA left atrium
a
b
Fig. 41.14 The IAS is intact on TEE both in 0° ( a ) and short-axis ( arrows ) ( b ) views. LA left atrium, RA right atrium, AO aorta
Case 41 Abnormal Left Pulmonary Venous Return to the Coronary Sinus
134
ab
Fig. 41.15 There is no evidence of an ASD (sinus venosus type) on TEE both in 104° ( a ) and 133° ( b ) views. LA left atrium, RA right atrium
a b
Fig. 41.16 There is a longitudinal membrane ( arrow ) in the left
atrium, which has divided the left atrium into two parts on TEE (long­axis view) ( a ). The posterior part, which receives the fl ow of the left
pulmonary veins ( red, arrow ), acts as a reservoir ( b ) or a venous confl u- ence. LA left atrium, VC venous confl uence, AO aorta, RV right ventri- cle, LV left ventricle
Case 41 Abnormal Left Pulmonary Venous Return to the Coronary Sinus
135
Fig. 41.17 A turbulent red fl ow ( arrow ) in the coronary sinus indicates
that it receives the fl ow of the left pulmonary veins through the CPC on TEE (0°)
a b
Fig. 41.18 The three left pulmonary veins (upper, middle, and lower) connect to the venous confl uence behind the left atrium on TEE (long-axis
view) ( a , b ). 1 left upper pulmonary vein, 2 left middle pulmonary vein, 3 left lower pulmonary vein, VC venous confl uence
Case 41 Abnormal Left Pulmonary Venous Return to the Coronary Sinus
136
Diagnosis
Abnormal left pulmonary venous return to coronary sinus
Recommendation
The patient was referred for cardiac catheterism and then surgery. Cardiac catheterism confi rmed the diagnosis of an abnormal connection between the left pulmonary veins and the coronary sinus.
Lesson
When confronting right atrial and ventricular dilation, in the absence of an ASD, one possible diagnosis is an abnormal left pulmonary venous return. An abnormal pulmonary venous return may be supracardiac, cardiac, or infracardiac. In the infracardiac return, the pulmonary veins connect to the inferior vena cava; in the supracardiac return, they connect to the innominate vein or the SVC; and in the cardiac type, they connect to the coronary sinus [ 68 – 71 ].
a b
Fig. 41.19 The insertion of the left pulmonary vein into the venous
confl uence and then into the coronary sinus behind the left atrium is evident on TEE (short-axis view) by two-dimensional echocardiogra-
phy ( a ) and color Doppler study ( b ). The coronary sinus dilation is also visible in this view. LUPV left upper pulmonary vein, VC venous con- fl uence, CS coronary sinus
Fig. 41.20 There is a turbulent fl ow in the venous confl uence behind
the left atrium ( arrow ). VC venous confl uence, LA left atrium, LV left ventricle
Case 41 Abnormal Left Pulmonary Venous Return to the Coronary Sinus
137
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease, DOI 10.1007/978-3-319-12934-1_42, © Springer International Publishing Switzerland 2015
A 17-year-old girl presented with dyspnea on exertion (func- tional class I) of 1-year duration. She was a known case of ventricular septal defect (VSD) since childhood. Physical
examination revealed a holosystolic murmur at the apex and the left sternal border.
Perimembranous Ventricular Septal Defect with No Rim to the Aortic Valve
Case 42
a
b
Fig. 42.1 The VSD is evident in the parasternal long-axis view by
color-fl ow study ( arrow ) ( a ). The peak gradient across the VSD is 90 mmHg in this view ( b ). LA left atrium, LV left ventricle, RV right ventricle, AO aorta
Fig. 42.2 A perimembranous VSD can be seen below the aortic valve
in the apical fi ve-chamber view ( arrow ). LA left atrium, LV left ventri- cle, RA right atrium, RV right ventricle, AO aorta
Electronic supplementary material The online version of this chapter (doi:
10.1007/978-3-319-12934-1_42 ) contains supplementary
material, which is available to authorized users.
138
Comment
The patient’s Qp/Qs was 1.3, but she was symptomatic. Consequently, she was subjected to transesophageal echo­cardiography (TEE).
ab
Fig. 42.3 This is a four-dimensional reconstruction of the VSD in the parasternal long-axis ( a ) and apical fi ve-chamber ( b ) views ( arrows ).
LA left atrium, LV left ventricle, RA right atrium, RV right ventricle, AO aorta
Fig. 42.4 TEE (short-axis view) shows the turbulent fl ow of the VSD
just below the right coronary cusp ( arrow ) at nearly 7 o’clock. LA left atrium, RA right atrium, RV right ventricle, AO aorta, PA pulmonary artery, RVOT right ventricular outfl ow tract
Case 42 Perimembranous Ventricular Septal Defect with No Rim to the Aortic Valve
139
a
c
b
Fig. 42.5 TEE (long-axis views) demonstrates the VSD ( arrow ) ( a ). The defect is 5 mm in size ( b ), and there is no rim to the aortic valve ( arrow )
( c ). LA left atrium, LV left ventricle, RV right ventricle, AO aorta
Diagnosis
The symptomatic patient was diagnosed with a perimembra­nous VSD. She had a Qp/Qs of 1.3, systolic pulmonary arte­rial pressure within normal limits, and no rim of the VSD to the aortic valve.
Comment
Because there was no rim to the aortic valve, the patient was not a suitable candidate for VSD device closure. Prophylaxis
for infective endocarditis and follow-up were, therefore, recommended.
Lesson
The VSD device closure is possible in perimembranous and muscular VSDs, and the rim to the aortic valve should be >2 mm [ 72 , 73 ].
Case 42 Perimembranous Ventricular Septal Defect with No Rim to the Aortic Valve
141
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease, DOI 10.1007/978-3-319-12934-1_43, © Springer International Publishing Switzerland 2015
An 11-year-old boy (41 kg) referred to our clinic because of a systolic murmur. He had no symptoms. Physical examina­tion showed a harsh systolic murmur at the left sternal border
and apex. Electrocardiography was normal, and chest X-ray revealed cardiomegaly due to left ventricular enlargement.
Perimembranous Ventricular Septal Defect
Case 43
RV
AO
LV
LA
Fig. 43.1 The parasternal long-axis view shows a mild left ventricular
dilation and no fl ow across the interventricular septum. LA left atrium, LV left ventricle, AO aorta, RV right ventricle
RV
AO
RA
LA
Fig. 43.2 The parasternal short-axis view demonstrates a turbulent
systolic fl ow ( arrow ) across the interventricular septum, in favor of a perimembranous ventricular septal defect (VSD). LA left atrium, RA right atrium, RV right ventricle, AO aorta
Electronic supplementary material The online version of this chapter (doi:
10.1007/978-3-319-12934-1_43 ) contains supplementary
material, which is available to authorized users.
142
Diagnosis
Perimembranous VSD with mild left ventricular dilation and Qp/Qs = 2 and elevated pulmonary arterial systolic pressure
Comment
The peak gradient across the VSD was about 80 mmHg, but the pulmonary arterial pressure was 55 mmHg and Qp/Qs was 2. Moreover, the patient had a mild left ventricular dila­tion. He was, therefore, subjected to transesophageal echo­cardiography for the evaluation of the possibility of VSD device closure.
Lesson
Perimembranous VSDs may be visualized in the parasternal long-axis or short-axis views or both. Apical fi ve-chamber view can also visualize this type of VSD. Perimembranous VSDs are the most common form of VSD and comprising about 80 % of VSDs.
The spontaneous closure of muscular and perimembra-
nous VSDs occurs by the time the patient has reached puberty. Closure is more frequent in the muscular type. The criteria for intervention in VSDs include:
1. Left ventricular and atrial dilation or left ventricular sys­tolic dysfunction.
2. Qp/Qs greater than 1.5/1.
3. Systolic pulmonary arterial pressure greater than 50 mmHg in the absence of pulmonary arterial hypertension. If there is pulmonary arterial hypertension, the pulmonary vascular resistance should be measured. In the presence of a pulmo­nary vascular resistance greater than 7 units, if the Qp/Qs is greater than 1.5 and there is pulmonary reactivity by nitric oxide or oxygen, the VSD can be closed [ 2 ]. More than mild aortic regurgitation is a relative indication
for intervention in perimembranous and double outlet VSDs [ 2 ], although some authors have suggested progressive aortic regurgitation as an indication for intervention [ 7 ].
Different types of left-to-right shunts have various effects
on the ventricular chambers. The atrial septal defect (ASD) produces right atrial and ventricular dilation, while the ven­tricular septal defect (VSD) and the patent ductus arteriosus (PDA) cause left atrial and ventricular dilation.
It is because for the VSD, blood enters from the left ven-
tricle into the right ventricle. However, because the passage of the fl ow through the VSD is a systolic phenomenon, blood enters the pulmonary artery immediately, followed by the pulmonary veins, left atrium, and left ventricle. Consequently, there is a volume overload on the left atrium and ventricle.
For the PDA, blood passes from the descending aorta
toward the pulmonary artery through the ductus arteriosus and then enters the pulmonary veins, left atrium, and left ventricle. Consequently, the left atrium and ventricle become dilated [ 2 ].
In the ASD, the two atria are fi lled with the same pres-
sure. (In the normal heart, the left atrial pressure is 5 mmHg more than the right atrial pressure.) The right atrium and ventricle, which have more compliance for volume, accept more fl ow and become dilated [ 74 ].
RV
LV
RA
LA
Fig. 43.3 The size of the defect is about 9 mm in the apical four-
chamber view. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle
Case 43 Perimembranous Ventricular Septal Defect
143
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease, DOI 10.1007/978-3-319-12934-1_44, © Springer International Publishing Switzerland 2015
A 40-year-old woman was referred to our echocardiography laboratory for decision-making about a ventricular septal defect (VSD) and rule out of Gerbode defect (VSD between
the left ventricle and right atrium). She was asymptomatic and had a holosystolic murmur (grade II/VI) at the lower left sternal border.
Perimembranous Ventricular Septal Defect Partially Closed with the Septal Leaflet of the Tricuspid Valve
Case 44
RV
LV
LA
RA
b
a
Fig. 44.2 The apical four-chamber view demonstrates that the VSD is
partially closed by the septal leafl et of the tricuspid valve ( arrow ) ( a ). The VSD is about 2 mm in size ( b ). The tricuspid regurgitation fl ow is also evident in this view ( curved arrow ) ( b ). LA left atrium, LV left ventricle, RA right atrium, RV right ventricle
RV
LV
LA
AO
Fig. 44.1 The parasternal long-axis view demonstrates a turbulent
fl ow across the interventricular septum below the aortic valve, in favor of a perimembranous VSD ( 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_44 ) contains supplementary mate-
rial, which is available to authorized users.