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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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244
Diagnosis
The patient was diagnosed with tetralogy of Fallot, accompanied by pulmonary atresia and an abnormal course of the
coronary arteries.
Comment
Cardiac catheterism for a precise detection of the main aortopulmonary collateral artery was recommended.
Lesson
1. The MAPCA acts like a shunt for the patient. More than
90 % of these arteries originate from the descending
aorta. The diagnosis is crucial before surgery because it
does alter the surgical strategy. If the MAPCA is the sole
source of blood supply to a pulmonary segment, an initial
procedure will be needed to bring the vessels to an area;
and if there is dual supply to a pulmonary segment, the
vessel should be coiled before surgery [ 103 ].
2. The presence of the MAPCA is more likely when there is
pulmonary valve atresia.
Fig. 75.9 The origin of the MAPCA ( arrows ) from the descending
aorta is evident on TEE (0°). DAO descending aorta
Case 75 Tetralogy of Fallot and Pulmonary Atresia
245
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_76, © Springer International Publishing Switzerland 2015
A 30-year-old symptomatic woman, who had undergone
total correction for the tetralogy of Fallot at childhood, was
referred to our center for an evaluation of the need for
reoperation. Physical examination revealed edema at the
lower extremities. Cardiac auscultation revealed a systolic
ejection murmur at the left sternal border and an early
diastolic murmur in the pulmonic area. The QRS duration
was 200 ms.
Postsurgery of Tetralogy of Fallot,
Aneurysm of the Right Ventricular
Outflow Tract, and Branch
Pulmonary Insufficiency
Case 76
Fig. 76.1 A severe right ventricular dilation and a ventricular septal
defect (VSD) repair patch ( arrow ) can be seen in this parasternal long-
axis view. LA left atrium, LV left ventricle, AO aorta, RV right
ventricle
Fig. 76.2 The severe right ventricular dilation is patent: the right ven-
tricle/left ventricle is 55/33 in the apical four-chamber view. 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_76 ) contains supplementary
material, which is available to authorized users.
246
Fig. 76.3 This is severe pulmonary insuffi ciency. The pulmonary
insuffi ciency fl ow fi lls most of the right ventricular outfl ow tract with a
wide vena contracta in the short-axis view. RVOT right ventricular out-
fl ow tract, PA pulmonary artery
ab
Fig. 76.4 The short-axis view illustrates branched pulmonary regurgitation from the right and left pulmonary arteries ( arrows ) ( a ) and ( b ). AO
aorta, RVOT right ventricular outfl ow tract, RPA right pulmonary artery, LPA left pulmonary artery, PA pulmonary artery
Case 76 Postsurgery of Tetralogy of Fallot, Aneurysm of the Right Ventricular Outfl ow Tract, and Branch Pulmonary Insuffi ciency
247
Fig. 76.6 The multiplane modality of the four-dimensional probe of
the right ventricle in the apical view shows that the right ventricular
ejection fraction is 46 % (mild right ventricular systolic dysfunction),
right ventricular end-diastolic volume is 184 cc, and right ventricular
end-systolic volume is 99 cc
Fig. 76.5 The short-axis view shows the aneurysm of the right ven-
tricular outfl ow tract (up to 57.7 mm). RVOT right ventricular outfl ow
tract
Case 76 Postsurgery of Tetralogy of Fallot, Aneurysm of the Right Ventricular Outfl ow Tract, and Branch Pulmonary Insuffi ciency
248
Diagnosis
The patient was symptomatic following surgery for the
tetralogy of Fallot. She was diagnosed with severe pulmonary insuffi ciency, right ventricular outfl ow tract aneurysm,
and mild right ventricular systolic dysfunction.
Comment
The patient was referred for surgery in light of the severe
pulmonary insuffi ciency and the right ventricular outfl ow
tract aneurysm [ 103 ].
Lesson
Because of the transannular patch during surgery for the
tetralogy of Fallot, the pulmonary valve is damaged, and so
pulmonary insuffi ciency is common in the follow-up of
patients post total correction of the tetralogy of Fallot. An
underdeveloped pulmonary vascular bed and relatively small
pulmonary branches, especially the left pulmonary artery,
may give rise to branch pulmonary insuffi ciency. Severe
pulmonary insuffi ciency alone is not an indication for redo
surgery. Nevertheless, not all cases of severe pulmonary
insuffi ciency are candidates for redo surgery. The indications
for reoperation include:
1. Right ventricular systolic dysfunction (right ventricular
ejection fraction <45 %)
2. Severe right ventricular dilation, e.g., right ventricular
volume index greater than 150–170 CC/m 2
3. Aneurysm formation of the right ventricular outfl ow tract
with rapid enlargement
4. Substantial reduction in the left ventricular systolic
function
5. Exercise intolerance
6. Sustained arrhythmia
7. QRS duration ≥180 ms
Surgery or percutaneous pulmonary valve replacement is
indicated. Some degree of gradient across the pulmonary
valve is acceptable after surgery for the tetralogy of Fallot [ 2 ,
7 , 103 , 104 ].
Case 76 Postsurgery of Tetralogy of Fallot, Aneurysm of the Right Ventricular Outfl ow Tract, and Branch Pulmonary Insuffi ciency
249
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_77, © Springer International Publishing Switzerland 2015
A 28-year-old man, who had undergone total correction for
the tetralogy of Fallot at childhood, was referred to our center for follow-up. He was asymptomatic.
Post Surgery of Tetralogy of Fallot:
Severe Pulmonary Insufficiency with
Normal Right Ventricular Systolic
Function
Case 77
Fig. 77.1 A ventricular septal defect patch repair can be seen in this
parasternal long-axis view ( arrow ). LA left atrium, LV left ventricle, RV
right ventricle, AO aorta
Fig. 77.2 This apical four-chamber view demonstrates severe right
ventricular dilation: the right ventricle/left ventricle is 47/35. 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_77 ) contains supplementary
material, which is available to authorized users.
250
Diagnosis
The patient, postsurgery for the tetralogy of Fallot, was diagnosed with severe right ventricular dilation, normal right
ventricular systolic function, severe pulmonary insuffi ciency,
branch pulmonary regurgitation, and mild pulmonary
stenosis.
Comment
Because the patient was asymptomatic and had a normal
right ventricular systolic function, only follow-up was recommended [ 105 ].
Fig. 77.4 The right ventricular outfl ow tract is dilated and is 34.5 mm
in the short-axis view. RVOT right ventricular outfl ow tract
ab
Fig. 77.3 The short-axis view demonstrates severe pulmonary insuffi -
ciency. The pulmonary insuffi ciency fi lls most of the right ventricular
outfl ow tract width with a wide vena contracta ( arrow ) ( a ). There is also
branched pulmonary regurgitation ( arrows ) ( b ). RVOT right ventricular
outfl ow tract, PA pulmonary artery
Case 77 Post Surgery of Tetralogy of Fallot: Severe Pulmonary Insuffi ciency
251
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_78, © Springer International Publishing Switzerland 2015
A 42-year-old man, who had undergone total correction for
the tetralogy of Fallot at childhood, was referred to our echocardiography laboratory. His complaint was lower extremity
edema.
Post Surgery of Tetralogy of Fallot:
Severe Pulmonary Insufficiency with
Moderate Right Ventricular Systolic
Dysfunction
Case 78
Fig. 78.1 The parasternal long-axis view shows the ventricular septal
defect patch repair ( arrow ). 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_78 ) contains supplementary
material, which is available to authorized users.
252
Fig. 78.3 The right and left pulmonary arteries are 17 and 13 mm in
size, respectively. RVOT right ventricular outfl ow tract, AO aorta, PA
pulmonary artery
a
b
c
Fig. 78.2 The parasternal short-axis view reveals mild valvular pulmo-
nary stenosis, peak gradient of 38 mmHg ( a ), and severe valvular pulmo-
nary insuffi ciency ( b ). There is also branch pulmonary insuffi ciency
( arrow ) ( c ). PA pulmonary artery, LPA left pulmonary artery, RVOT
right ventricular outfl ow tract, AO aorta
Diagnosis
The patient, postsurgery for the tetralogy of Fallot, was diagnosed with mild pulmonary stenosis, severe pulmonary insuffi ciency, and moderate right ventricular systolic dysfunction.
Comment
Percutaneous pulmonary valve replacement was recommended
for the patient. Since Bonhoeffer introduced percutaneous pulmonary valve replacement for pulmonary regurgitation, it
becomes an alternative way for surgery, but when native outfl ow
tract is more than 22 mm, it is contraindicated [ 7 , 106 , 107 ].
Lesson
Right ventricular outfl ow tract obstruction with right ventricular pressure gradient more than 80 mmHg needs intervention postsurgery of tetralogy of Fallot [ 7 ].
Case 78 Post Surgery of Tetralogy of Fallot: Severe Pulmonary Insuffi ciency
253
H. Sadeghian, Z. Savand-Roomi, Echocardiographic Atlas of Adult Congenital Heart Disease,
DOI 10.1007/978-3-319-12934-1_79, © Springer International Publishing Switzerland 2015
A 12-year-old boy, who had a history of dyspnea on exertion
(functional class II), cyanosis, and a palliative surgical operation
several years before, was referred to our center. Because his
family refused corrective surgery, the patient was referred to our
echocardiography laboratory for further cardiac evaluation.
Diagnosis
The patient was diagnosed with situs solitus, dextrocardia,
common atrioventricular valve, ventricular and atrial septal
defects, transposition of the great arteries, and valvular and
subvalvular pulmonary stenosis.
The patient had undergone a palliative surgical opera-
tion (Blalock–Taussig shunt) with a view to enabling the
pulmonary artery branches to develop several years before
he referred to our center. However, his family did not consent to a second corrective operation (Figs. 79.10 and
79.11 ).
Modified Blalock–Taussig Shunt
Case 79
a
b
Fig. 79.1 The subcostal view shows that the liver leans to the right.
The yellow arrowhead indicates the spine. The inferior vena cava and
the aorta are on the right and left sides of the spine, respectively ( a ). The
red fl ow in the color-fl ow Doppler study depicts the position of the aorta
on the left side of the spine ( b ). Therefore, the situs of the visceral and
the great arteries is solitus (S). AO aorta, IVC inferior vena cava
Electronic supplementary material The online version of this chapter
(doi:
10.1007/978-3-319-12934-1_79 ) contains supplementary material,
which is available to authorized users.
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