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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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386
Diagnosis
The patient was diagnosed with severe pulmonary regurgitation several years post percutaneous pulmonary valvuloplasty with clinical symptoms of the right ventricular
failure.
Comment
Percutaneous pulmonary valve replacement was recommended for the patient.
Lesson
Moderate pulmonary insuffi ciency occurs in between 25 and
33 % of post pulmonary valvuloplasty cases. However, severe
pulmonary insuffi ciency is uncommon, and it requires intervention if it occurs in association with symptomatic right-sided
failure [ 116 ]. Pulmonary insuffi ciency is related to oversized
balloon even in the recommended range of 120–130 % of
annulus diameter. It seems better to accept mild degrees of
stenosis for avoiding pulmonary insuffi ciency [ 139 ].
a
b
c
Fig. 118.2 Severe pulmonary insuffi ciency ( arrow ) is seen in the para-
sternal short-axis view ( a ). Pulmonary insuffi ciency vena contracta is
8 mm by color Doppler study ( b ). Pulmonary deceleration time is
244 ms by continuous wave Doppler study ( c ). The patient’s right ven-
tricular systolic function was moderately impaired. PA pulmonary
artery, RVOT right ventricular outfl ow tract, AO aorta
Case 118 Severe Pulmonary Insuffi ciency Post Percutaneous Pulmonary Valvuloplasty
387
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_119, © Springer International Publishing Switzerland 2015
A 22-year-old woman presented with dyspnea (functional
class III) and cyanosis with clubbing. She was a known
case of complex cardiac diseases from childhood and had
undergone surgery at 6 months old. O 2 saturation was 44 %
in room air, which reached 77 % with oxygen.
Double-Outlet Right Ventricle:
“Taussig–Bing Complex and
Isenmenger Syndrome“
Case 119
a
b
c
Fig. 119.1 A ventricular septal defect (VSD) ( arrow ) ( a ) and right ventricular wall hypertrophy ( curved arrow ) are shown in the parasternal long-
axis view ( a ). The VSD measures 34 mm in this view ( b ), and the right ventricular free wall measures 13 mm ( c ). 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_119 ) contains supplementary
material, which is available to authorized users.
388
a
b
Fig. 119.2 A right-to-left shunt ( blue ) ( arrows ) is evident in the apical four-chamber ( a ) and parasternal long-axis ( b ) views, in favor of
Eisenmenger’s syndrome. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle, AO aorta
a
b
Fig. 119.3 The great artery, which is located posteriorly, is divided; it is, therefore, the pulmonary artery ( arrow ) ( a ). There is a conus below the
pulmonary valve ( curved arrow ) ( b ). PA pulmonary artery
Fig. 119.4 The aortic valve and the pulmonary valve are located side
by side. PV pulmonary valve, AOV aortic valve
Case 119 Double-Outlet Right Ventricle: “Taussig–Bing Complex and Isenmenger Syndrome“
389
a
b
c
d
Fig. 119.5 There is severe pulmonary regurgitation ( arrow ) ( a ). The mean pulmonary arterial pressure is 42 mmHg ( b ). The peak gradient across
the pulmonary valve is 10 mmHg ( c ). PA pulmonary artery
Fig. 119.6 The interventricular septum is fl at in systole, which is in
favor of pulmonary hypertension. RV right ventricle, LV left ventricle
Fig. 119.7 Severe right ventricular dilation is evident in the apical
four-chamber view. RV right ventricle, LV left ventricle
Case 119 Double-Outlet Right Ventricle: “Taussig–Bing Complex and Isenmenger Syndrome“
390
Diagnosis
The patient had a double-outlet right ventricle and a subpulmonic VSD. Accordingly, a diagnosis of Taussig–Bing
anomaly causing the Eisenmenger’s syndrome was
established.
Comment
The patient had undergone pulmonary artery banding at
6 months old, but it was ineffective. We recommended treatment with bosentan and sildenafi l for her. Our surgeons recommended catheterism; there were two possibilities: if the
pulmonary artery saturation was more than the aortic saturation, they would do atrial switch, and if the pulmonary artery
saturation was less than the aortic saturation, they would perform interatrial septectomy.
Lesson
In the Taussig–Bing anomaly, in which there is a subpulmonic VSD, the O2 saturation of the pulmonary artery is
more than that of the aorta. The pathology, therefore, is similar to the transposition of the great arteries [ 140 ].
Fig. 119.8 There is an overriding of the pulmonary artery on the
interventricular septum. More than 50 % of the pulmonary artery originates from the right ventricle. The VSD is under the pulmonary artery.
PA pulmonary artery
Case 119 Double-Outlet Right Ventricle: “Taussig–Bing Complex and Isenmenger Syndrome“
391
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_120, © Springer International Publishing Switzerland 2015
A boy with a history of cyanosis and dyspnea on exertion
(functional class III–IV) was referred to our echocardiography laboratory. Echocardiography revealed right ventricular
dilation with moderate atrioventricular valve regurgitation
and severe pulmonary arterial hypertension (peak systolic
arterial pressure of about 100 mmHg).
Double-Outlet Right Ventricle:
Taussig–Bing Syndrome
Case 120
Fig. 120.1 The subcostal visceral view shows the situs solitus of the
visceral organs and great vessels. (The ascending aorta is positioned
posteriorly and left sided to the inferior vena cava.) IVC inferior vena
cava, AO aorta
Fig. 120.2 The apical four-chamber view reveals that the pulmonary
veins drain into a left-sided atrium; this is indicative of the situs solitus
of the left atrium. Also, the moderator band is in the right ventricle
( arrowhead ), which is in favor of D-loop ventricles. PV pulmonary
vein, 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_120 ) contains supplementary
material, which is available to authorized users.
392
ab
Fig. 120.3 The apical four-chamber view, with further anterior angu-
lations of the probe (two-dimensional ( a ) and color-fl ow Doppler
study ( b )), shows that the two great arteries are parallel to each other
and both originate from the right ventricle. The two arrows point to the
bifurcation of the pulmonary artery, and the curved arrow shows a
conus below the aorta and pulmonary artery, indicative of a doubleoutlet right ventricle. PA pulmonary artery, AO aorta, LV left ventricle,
RV right ventricle
ab
Fig. 120.4 The peak gradient across the ascending aorta is 10 mmHg
( a ), the peak gradient across the pulmonary valve is approximately
29 mmHg ( b ), and the mean pulmonary arterial pressure is about
31 mmHg, which is nearly equal to the early diastolic gradient of pulmonary insuffi ciency
Case 120 Double-Outlet Right Ventricle: Taussig–Bing Syndrome
393
Diagnosis
A double-outlet right ventricle with subpulmonic VSD and
pulmonary arterial hypertension [ 140 ]
Comment
The patient was referred for catheterism.
Fig. 120.6 The ascending aorta is positioned anteriorly and is located
on the right side of the pulmonary artery, which denotes the transposition of the great arteries. AO aorta, PA pulmonary artery
ab
Fig. 120.5 The apical fi ve-chamber view demonstrates a subpulmonic
ventricular septal defect (VSD) ( arrowhead ) ( a ), in favor of a Taussig–
Bing double-outlet right ventricle. (The Taussig–Bing anomaly is a
double-outlet right ventricle plus a subpulmonic VSD.) This VSD has a
bidirectional shunt fl ow ( arrow ) ( b ), indicative of pulmonary arterial
hypertension. AO aorta, PA pulmonary artery, LV left ventricle, LA left
atrium, RA right atrium, RV right ventricle
Case 120 Double-Outlet Right Ventricle: Taussig–Bing Syndrome
395
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_121, © Springer International Publishing Switzerland 2015
A 22-year-old woman was referred to our clinic because of
multiple abortions. She had dyspnea on exertion (functional
class II). Physical examination revealed an early diastolic
murmur in the aortic area. The O
2
saturation of the patient
was 83 % by pulse oximetry in room air. Electrocardiography
showed normal sinus rhythm, right axis deviation, and equal
R and S in lead V1. Chest X-ray revealed hypovascularity of
the lungs and cardiomegaly. Transthoracic echocardiography
showed normal left ventricular size and mild systolic dysfunction, normal right ventricular size and function, and mild
mitral and tricuspid regurgitation with a tricuspid regurgitation gradient of about 70 mmHg.
Double-Outlet Right Ventricle,
Atretic Pulmonary Valve,
and Subaortic Ventricular
Septal Defect
Case 121
Fig. 121.1 In the standard parasternal long-axis view, the aorta is not visu-
alized. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle
Electronic supplementary material The online version of this chap-
ter (doi:
10.1007/978-3-319-12934-1_121 ) contains supplementary
material, which is available to authorized users.
396
ab
Fig. 121.2 A perimembranous ventricular septal defect (VSD) ( arrow ) and the overriding of the aorta are seen in this parasternal long-axis view,
with some angulation of the probe ( a ). The VSD measures 33 mm ( b ). LA left atrium, LV left ventricle, RV right ventricle, AO aorta
Fig. 121.3 The hypertrophy of the right ventricular outfl ow tract (up
to 15 mm) is seen in the parasternal long-axis view. LA left atrium,
LV left ventricle, RV right ventricle
ab
Fig. 121.4 The apical fi ve-chamber view shows the perimembranous VSD ( arrow ) and also demonstrates that the aorta overrides on the right
ventricle (more than 50 %) ( a ). A bidirectional fl ow is visualized across the VSD in this view ( b ). ( Arrows ) LA left atrium, LV left ventricle,
RV right ventricle, AO aorta
Case 121 Double-Outlet Right Ventricle, Atretic Pulmonary Valve, and Subaortic Ventricular Septal Defect
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