Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / @xirurgi_2025 / @xirurgi_2025 - 467 - файл
.pdf
158
https://t.me/medicina_free
2. The most common age to close asymptomatic atrial septal defects (ASDs) is:
A. In the immediate newborn period.
B. After the child reaches 10 kg in weight.
C. Age 4–5 years.
D. During puberty.
CHAPTER 20
Congenital Heart Disease
3. Which of the following is not acceptable treatment for
aortic valve stenosis with a hypoplastic left ventricle?
A. Balloon valvotomy
B. Intubation and initiation of prostaglandin
C. Surgical valvotomy
D. Norwood procedure
Answer: C
In general, ASDs are closed when patients are between 4 and
5 years of age. Children of this size can usually be operated on
without the use of blood transfusion and have excellent outcomes. Patients who are symptomatic may require repair earlier, even in infancy. Some surgeons advocate routine repair
in infants and children especially in cases where prematurityrelated lung disease may accelerate damage to the pulmonary
vascular bed, though this philosophy may not be widespread.
In a review by Reddy and colleagues, 116 neonates weighing
less than 2500 g who underwent repair of simple and complex
cardiac defects with the use of cardiopulmonary bypass were
found to have no intracerebral hemorrhages, no long-term
neurologic sequelae, and a low operative mortality rate (10%).
These results correlated with the length of cardiopulmonary
bypass and the complexity of repair. These investigators also
found an 80% actuarial survival at 1 year and, more importantly, that growth following complete repair was equivalent
to weight-matched neonates free from cardiac defects. (See
Schwartz 11th ed., p. 754.)
Answer: A
The first decision that must be made in the neonate with
critical left ventricular outflow tract (LVOT) obstruction is
whether the patient is a candidate for biventricular or univentricular repair. Central to this decision is assessment of the
degree of hypoplasia of the LV and other left-sided structures.
Alsoufi and colleagues have described a rational approach to
the neonate with critical LVOT obstruction. The options vary
depending on whether the infant follows a single or a biventricular pathway. The options for a single ventricle include the
Norwood operation, a hybrid strategy (initial ductal stent and
bilateral pulmonary artery bands followed by later completion of the Norwood operation) or heart transplantation. The
options for a biventricular heart include balloon valvuloplasty,
surgical valvotomy, neonatal Ross operation, or a Yasui operation. Often valvotomy is accompanied by LV rehabilitation
techniques, including endocardial fibroelastosis (EFE) resection and mitral valve interventions. Fetal aortic valvotomy,
which is now offered at specialized centers, is another promising strategy to decompress the LV in fetal life and potentially
allow growth of the left-sided structures sufficient to permit
a biventricular circulation. Regardless of whether the baby is
triaged to a single or biventricular strategy, any infant with
severe aortic stenosis (AS) requires urgent intervention. Preoperative stabilization, however, has dramatically altered the
clinical algorithm and outcomes for this patient population.
The preoperative strategy begins with endotracheal intubation and inotropic support. Prostaglandin infusion is initiated to maintain ductal patency, and confirmatory studies are
performed prior to operative intervention. Therapy is generally indicated in the presence of a transvalvular gradient of
50 mm Hg with associated symptoms including syncope, congestive heart failure (CHF), or angina, or if a gradient of 50 to
75 mm Hg exists with concomitant electrocardiography
(ECG) evidence of LV strain or ischemia. In the critically ill
neonate, a gradient across the aortic valve may not be present
because of poor LV function. However, the decision regarding
treatment options must be based on a complete understanding
of associated defects. For example, in the presence of a hypo-
https://t.me/medicina_free
plastic LV (left ventricular end-diastolic volume < 20 mL/m2)
or a markedly abnormal mitral valve, isolated aortic valvotomy should not be performed because studies have demonstrated high mortality in this population following isolated
valvotomy.
Patients who have an LV capable of providing systemic
output are candidates for intervention to relieve AS, generally
through balloon valvotomy. (See Schwartz 11th ed., p. 757.)
159
CHAPTER 20
4. The most common location for a coarctation of the aorta
is:
A. Aortic arch.
B. Distal to the left subclavian artery.
C. At the diaphragm.
D. At the level of the renal arteries.
5. Which of the following is a TRUE surgical emergency in
a newborn?
A. Tetralogy of Fallot
B. Truncus arteriosus
C. Total anomalous pulmonary venous connection
D. Coarctation of the aorta
Answer: B
Coarctation of the aorta (COA) is defined as a luminal narrowing in the aorta that causes an obstruction to blood flow.
This narrowing is most commonly located distal to the left
subclavian artery. The embryologic origin of COA is a subject of some controversy. One theory holds that the obstructing shelf, which is largely composed of tissue found within
the ductus, forms as the ductus involutes. The other theory
holds that a diminished aortic isthmus develops secondary to
decreased aortic flow in infants with enhanced ductal circulation. (See Schwartz 11th ed., pp. 761– 762.)
Answer: C
Total anomalous pulmonary venous connection (TAPVC)
occurs in 1% to 2% of all cardiac malformations and is characterized by abnormal drainage of the pulmonary veins into the
right heart, whether through connections into the right atrium
or into its tributaries. Accordingly, the only mechanism by
which oxygenated blood can return to the left heart is through
an ASD, which is almost uniformly present with TAPVC.
Unique to this lesion is the absence of a definitive form
of palliation. Thus, TAPVC with concomitant obstruction
(Fig. 20-2) represents one of the only true surgical emergencies across the entire spectrum of congenital heart surgery.
(See Schwartz 11th ed., pp. 765– 768.)
Congenital Heart Disease
FIG. 20-2. Infracardiac type of TAPVR. Note the stenosis (`*’) of
the descending vertical vein as it drains into the portal system.
160
https://t.me/medicina_free
6. The Bidirectional Glenn procedure is used to correct:
A. Tricuspid atresia.
B. Patent ductus arteriosus.
C. Transposition of the great arteries.
D. Total anomalous pulmonary venous connection.
CHAPTER 20
Congenital Heart Disease
Answer: A
Recognizing the inadequacies of the initial repairs, Glenn
described the first successful cavopulmonary anastomosis, an end-to-side right pulmonary artery-to-superior vena
cava shunt in 1958, and later modified this to allow flow to
both pulmonary arteries. This end-to-side right pulmonary
artery-to-superior vena cava anastomosis was known as the
bidirectional Glenn, and it is the first stage to final Fontan repair in widespread use today (Fig. 20-3). The Fontan
repair was a major advancement in the treatment of congenital heart defect (CHD), as it essentially bypassed the
right heart and allowed separation of the pulmonary and
systemic circulations. It was first performed by Fontan in
1971 and consisted of a classic Glenn anastomosis, ASD closure, and direct connection of the right atrium to the proximal
end of the left pulmonary artery using an aortic homograft.
The main pulmonary artery was ligated, and a homograft
valve was inserted into the orifice of the inferior vena cava.
(See Schwartz 11th ed., pp. 770– 773.)
FIG. 20-3. Angiogram showing a widely patent Glenn. The SVC
(`*’) is seen draining into the central pulmonary artery.
7. Hypoplastic left heart syndrome (HLHS) is surgically
treated with:
A. Bilateral pulmonary artery banding and stent place-
ment in the patent ductus arteriosus.
B. Norwood procedure with a Blalock-Taussig (B-T)
shunt.
C. Norwood procedure with a right ventricle to pulmo-
nary artery conduit (Sano shunt).
D. All of the above.
Answer: D
In 1983, Norwood and colleagues described a two-stage palliative surgical procedure for relief of HLHS that was later
modified to the currently used three-stage method of palliation. Stage 1 palliation, also known as the modified Norwood
procedure, bypasses the LV by creating a single outflow vessel, the neoaorta, which arises from the RV.
The current technique of arch reconstruction involves
completion of a connection between the pulmonary root,
the native ascending aorta, and a piece of pulmonary homograft used to augment the diminutive native aorta. There are
several modifications of this anastomosis, most notably the
Damus-Kaye-Stansel (DKS) anastomosis, which involves
dividing both the aorta and the pulmonary artery at the sinotubular junction. The proximal aorta is anastomosed to the
proximal pulmonary artery, creating a “double-barreled” outlet from the heart. This outlet is anastomosed to the distal
aorta, which can be augmented with homograft material if
there is an associated coarctation. At the completion of arch
reconstruction, a 3.5- or 4-mm shunt is placed from the
innominate artery to the right pulmonary artery. The inter-
https://t.me/medicina_free
atrial septum is then widely excised, thereby creating a large
interatrial communication and preventing pulmonary venous
hypertension. (See Schwartz 11th ed., pp. 773–775.)
161
8. The arterial switch operation for transposition of the
great vessels is best performed:
A. Within 2 weeks of birth.
B. At 1 year of age.
C. At 10 kg of weight.
D. In adolescence.
9. Which of the following is NOT one of the components of
the tetralogy of Fallot (TOF)?
A. Atrial septal defect
B. Ventricular septal defect (VSD)
C. Right ventricular hypertrophy
D. Right ventricular outflow obstruction
Answer: A
The most important consideration is the timing of surgical
repair because arterial switch should be performed within
2 weeks after birth, before the left ventricle (LV) loses its ability to pump against systemic afterload. In patients presenting
later than 2 weeks, the LV can be retrained with preliminary
pulmonary artery banding and aortopulmonary shunt followed by definitive repair. Alternatively, the unprepared LV
can be supported following arterial switch with a mechanical
assist device for a few days while it recovers ability to manage
systemic pressures. Echocardiography can be used to assess
left ventricular performance and guide operative planning in
these circumstances. (See Schwartz 11th ed., pp. 780–781.)
Answer: A
The original description of tetralogy of Fallot (TOF) by
Ettienne Louis Fallot, as the name implies, included four
abnormalities: a large perimembranous VSD adjacent to the
tricuspid valve; an overriding aorta; a variable degree of right
ventricular outflow tract (RVOT) obstruction, which might
include hypoplasia and dysplasia of the pulmonary valve as
well as obstruction at the subvalvar and pulmonary artery
level; and right ventricular hypertrophy. More recently, the
Van Praagh et al pointed out that TOF could be more correctly termed monology of Fallot, since the four components
are explained by the malposition of the infundibular septum.
When the infundibular septum is displaced anteriorly and
leftward, the RVOT is narrowed and its anterior displacement
results in failure of fusion of the ventricular septum between
the arms of the trabeculo-septo-marginalis. (See Schwartz
11th ed., p. 784.)
CHAPTER 20
Congenital Heart Disease
10. What is the best predictor of spontaneous closure of a
ventricular septal defect (VSD)?
A. Size
B. Age at diagnosis
C. Gestational age
D. Lack of electrocardiogram changes
Answer: B
VSDs may close or narrow spontaneously, and the probability
of closure is inversely related to the age at which the defect is
observed. Thus, infants at 1 month of age have an 80% incidence of spontaneous closure, whereas a child at 12 months
of age has only a 25% chance of closure. This has an important impact on operative decision-making because a small or
moderate-size VSD may be observed for a period of time in
the absence of symptoms. Large defects and those in severely
symptomatic neonates should be repaired during infancy to
relieve symptoms and because irreversible changes in pulmonary vascular resistance may develop during the first year of
life. (See Schwartz 11th ed., pp. 786–787.)
162
https://t.me/medicina_free
11. Flow across a ventricular septal defect (VSD) is dependent upon:
A. Size of defect.
B. Left and right ventricular pressure and size of the
defect.
C. Pulmonary and systemic vascular resistance and
defect size.
D. Pulmonary and systemic vascular resistance.
CHAPTER 20
Congenital Heart Disease
Answer: C
The size of the VSD determines the initial pathophysiology
of the disease. Large VSDs are classified as nonrestrictive and
are at least equal in diameter to the aortic annulus. These
defects allow free flow of blood from the left ventricle (LV) to
the right ventricle (RV), elevating right ventricular pressures
to the same level as systemic pressure.
Consequently, the pulmonary-to-systemic flow ratio (Qp to
Qs) is inversely dependent on the ratio of pulmonary vascular
resistance to systemic vascular resistance. Nonrestrictive VSDs
produce a large increase in pulmonary blood flow, and the
afflicted infant will present with symptoms of congestive heart
failure. However, if untreated, these defects will cause pulmonary hypertension with a corresponding increase in pulmonary
vascular resistance. This will lead to a reversal of flow (a rightto-left shunt), which is known as Eisenmenger syndrome.
Small restrictive VSDs offer significant resistance to the
passage of blood across the defect, and therefore right ventricular pressure is either normal or only minimally elevated
and the ratio of Qp to Qs rarely exceeds 1.5. These defects
are generally asymptomatic because there are few physiologic
consequences. However, there is a long-term risk of endocarditis because endocardial damage from the jet of blood
through the defect may serve as a possible nidus for colonization. (See Schwartz 11th ed., pp. 786–787.)
12. Beyond early childhood, high pulmonary blood flow is
most apt to produce:
A. Cyanosis on exercise.
B. Diminished exercise tolerance.
C. Periodic episodes of hemoptysis.
D. Right ventricular hypertrophy.
13. During left thoracotomy for repair of patent ductus arteriosus the blood pressure is 70/22. Immediately after
placement of a clip across the duct the blood pressure is:
A. 70/22.
B. 70/40.
C. 90/22.
D. 90/40.
Answer: B
High pulmonary blood flow beyond infancy may produce surprisingly little disability for a period of time, and
the diminished exercise tolerance may be subtle. Cyanosis,
hemoptysis, and pneumonia are not anticipated. With the
volume overloading in the right ventricle, ventricular dilatation is more common than ventricular hypertrophy. (See
Schwartz 11th ed., p. 751.)
Answer: B
The hemodynamic consequences of an unrestrictive ductal
shunt are left ventricular volume overload with increased left
atrial and pulmonary artery pressures and right ventricular
strain from the augmented afterload. These changes result
in increased sympathetic discharge, tachycardia, tachypnea,
and ventricular hypertrophy. The diastolic shunt results in
lower aortic diastolic pressure and increases the potential for
myocardial ischemia and underperfusion of other systemic
organs, while the increased pulmonary flow leads to increased
work of breathing and decreased gas exchange. Unrestrictive
ductal flow may lead to pulmonary hypertension within the
first year of life (See Schwartz 11th ed., pp. 759–760.)
163
https://t.me/medicina_free
14. A 5-day-old man undergoes echocardiography during
preoperative work-up of a tracheoesophageal fistula.
The patient is found to have subvalvular aortic stenosis
with a left ventricular (LV) gradient of 20 mm Hg but is
otherwise asymptomatic and without aortic valve insufficiency. What is the next best step in management of his
congenital heart defect (CHD)?
A. Balloon valvotomy
B. Observation with follow-up
C. Aortic 1-patch repair (Doty procedure)
D. Aortoventriculoplasty
Answer: A
Subvalvular aortic stenosis (AS) occurs beneath the aortic
valve and may be classified as discrete or tunnel-like (diffuse).
A thin, fibromuscular diaphragm immediately proximal to
the aortic valve characterizes discrete subaortic stenosis. This
diaphragm typically extends for 180° or more in a crescentic or circular fashion, often attaching to the mitral valve as
well as the interventricular septum. The aortic valve itself is
usually normal in this condition, although the turbulence
imparted by the subvalvular stenosis may affect leaflet morphology and valve competence.
Diffuse subvalvular AS results in a long, tunnel-like obstruction that may extend to the left ventricular apex. In some
individuals, there may be difficulty in distinguishing between
hypertrophic cardiomyopathy and diffuse subaortic stenosis.
Operation for subvalvular AS is indicated with a gradient
exceeding 30 mm Hg, in the presence of aortic valve insufficiency, or when symptoms indicating left ventricular outflow
tract (LVOT) obstruction are present. Given that repair of isolated discrete subaortic stenosis can be done with low rates of
morbidity and mortality, some surgeons advocate repair in all
cases of discrete AS to avoid progression of the stenosis and the
development of aortic insufficiency, although more recent data
demonstrate that subaortic resection should be delayed until the
LV gradient exceeds 30 mm Hg because most children with an
initial LV gradient less than 30 mm Hg have quiescent disease.
Diffuse AS is a more complex lesion and often requires aortoventriculoplasty. Results are generally excellent, with operative
mortality less than 5%. (See Schwartz 11th ed., pp. 755–758.)
CHAPTER 20
Congenital Heart Disease
15. Which structure must be identified prior to ligation and/
or division of a patent ductus arteriosus?
A. Recurrent laryngeal nerve
B. Left superior pulmonary vein
C. Left bronchial artery
D. Phrenic nerve
16. Which of the following treatment paradigms most aptly
describes the routine management of aortic coarctation
in a 4-month-old child?
A. Balloon dilation followed by stent placement for
recoarctation
B. Endovascular stent deployment with serial balloon
dilations
C. Observation
D. Surgical repair followed by catheter based interven-
tions for recoarctation
Answer: A
Surgical closure can be achieved via either open or videoassisted approaches. The open approach employs a musclesparing posterior lateral thoracotomy in the third or fourth
intercostal space on the side of the aorta (generally the left).
The lung is then retracted anteriorly. In the neonate, the patent
ductus arteriosus (PDA) is singly ligated with a surgical clip or
permanent suture. Care must be taken to avoid the recurrent
laryngeal nerve, which courses around the PDA. The PDA can
also be ligated via a median sternotomy; however, this approach
is generally reserved for patients who have additional cardiac or
great vessel lesions requiring repair. Occasionally, a short, broad
ductus, in which the dimension of its width approaches that of
its length, will be encountered. In this case, division between
vascular clamps with oversewing of both ends is advisable. In
extreme cases, the use of cardiopulmonary bypass (CPB) to
decompress the large ductus during ligation is an option. (See
Schwartz 11th ed., pp. 759–760.)
Answer: D
Although operative repair is still the gold standard, treatment
of coarctation of the aorta (COA) by catheter-based intervention has become more widespread for older children and
adults. Both balloon dilatation and primary stent implantation have been used successfully. The most extensive study of
the results of balloon angioplasty reported on 970 procedures:
422 native and 548 recurrent COAs. Mean gradient reduction was 74% ± 24% for native and 70% ± 31% for recurrent
COA. This demonstrated that catheter-based therapy could
164
https://t.me/medicina_free
CHAPTER 20
Congenital Heart Disease
17. Which type of aortic coarctation repair is most associated with aneurysm formation at the repair site?
A. Resection with end-to-end anastomosis
B. Resection with extended end-to-end anastomosis
C. Endovascular stenting
D. Dacron patch aortoplasty
produce equally effective results both in recurrent and in primary COA, a finding with far-reaching implications in the
new paradigm of multidisciplinary treatment algorithms
for congenital heart defect (CHD). In the valvuloplasty and
angioplasty of congenital anomalies (VACA) report, higher
preangioplasty gradient, earlier procedure date, older patient
age, and the presence of recurrent COA were independent
risk factors for suboptimal procedural outcome. In summary, children younger than age 6 months with native COA
should be treated with surgical repair, while those requiring
intervention at later ages may be ideal candidates for balloon dilatation or primary stent implantation. Additionally,
catheter-based therapy should be employed for those cases of
restenosis following either surgical or primary endovascular
management. (See Schwartz 11th ed., pp. 761–763.)
Answer: D
The most common complications after coarctation of the
aorta (COA) repair are late restenosis (Fig. 20-4) and aneurysm formation at the repair site. Aneurysm formation is particularly common after patch aortoplasty when using Dacron
material. In a large series of 891 patients, aneurysms occurred
in 5.4% of the total, with 89% occurring in the group who
received Dacron-patch aortoplasty and only 8% occurring
in those who received resection with primary end-to-end
anastomosis. A further complication, although uncommon,
is lower-body paralysis resulting from ischemic spinal cord
injury during the repair. This dreaded outcome complicates
0.5% of all surgical repairs, but its incidence can be lessened
with the use of some form of distal perfusion, preferably left
heart bypass with the use of femoral arterial or distal thoracic
aorta for arterial inflow and the femoral vein or left atrium
for venous return. These techniques are generally reserved
for older patients with complex coarctations that may need
prolonged aortic cross clamp times for repair, often in the
setting of large collateral vessels and/or previous surgery.
(See Schwartz 11th ed., pp. 762–763.)
FIG. 20-4. Reformatted images obtained from a CT angiogram
after recurrent coarctation repaired by an extra anatomic bypass
(`*’ points to the bypass graft).
165
https://t.me/medicina_free
18. In a patient with truncus arteriosus, which of the following choices best describes definitive repair?
A. Pulmonary artery banding
B. Division of the main pulmonary artery (PA), aortic
arch reconstruction with PA homograft, and creation
of an innominate artery to right pulmonary artery
shunt
C. Mobilization of the coronary arteries, excision and
transposition of the pulmonary valve into aortic position, reimplantation of the coronary arteries, and right ventricular outflow tract (RVOT)
reconstruction
D. Mobilization of the pulmonary arteries, patch repair
of the aorta and ventricular septal defect (VSD), and
extracardiac reconstruction of the RVOT
Answer: D
Truncus arteriosus was first managed with pulmonary artery
banding as described by Armer and colleagues in 1961.
However, this technique led to only marginal improvements
in 1-year survival rates because ventricular failure inevitably
occurred. In 1967, however, complete repair was accomplished
by McGoon and his associates based on the experimental
work of Rastelli, who introduced the idea that an extracardiac valved conduit could be used to restore ventricular-topulmonary artery continuity. Over the next 20 years, improved
survival rates led to uniform adoption of complete repair even
in the youngest and smallest infants. Surgical correction entails
the use of cardiopulmonary bypass (CPB). Repair is completed by separation of the pulmonary arteries from the aorta,
closure of the aortic defect (occasionally with a patch) to
minimize coronary flow complications, placement of a valved
cryopreserved allograft or jugular venous valved conduit
(Contegra) to reconstruct the RVOT, and VSD closure. Important branch pulmonary arterial stenosis should be repaired at
the time of complete repair and can usually be accomplished
with longitudinal allograft patch arterioplasty. Severe truncal
valve insufficiency occasionally requires truncal valve repair
or even replacement, which can be accomplished with a cryopreserved allograft. (See Schwartz 11th ed., p. 764.)
CHAPTER 20
Congenital Heart Disease
19. Which type of anomalous venous connection (according
to the Darling classification) is most commonly associated with pulmonary venous obstruction?
A. Supracardiac connection
B. Cardiac connection
C. Infracardiac connection
D. Connection at multiple levels
20. Which of the following factors confers the highest postoperative mortality after total anomalous pulmonary
venous connection (TAPVC) repair?
A. Postoperative atrial arrythmias
B. Patient age at operation
C. Pulmonary venous sclerosis
D. Postoperative left ventricular (LV) dysfunction
Answer: C
Darling and colleagues classified total anomalous pulmonary
venous connection (TAPVC) according to the site or level of
connection of the pulmonary veins to the systemic venous system: type I (45%), anomalous connection at the supracardiac
level; type II (25%), anomalous connection at the cardiac level;
type III (25%), anomalous connection at the infracardiac level;
and type IV (5%), anomalous connection at multiple levels.
Within each category, further subdivisions can be implemented, depending on whether pulmonary venous obstruction
exists. Obstruction to pulmonary venous drainage is a powerful predictor of adverse natural outcome and occurs most frequently with the infracardiac type, especially when the pattern
of infracardiac connection prevents the ductus venosus from
bypassing the liver. (See Schwartz 11th ed., pp. 765–767.)
Answer: C
The most significant postoperative complication of TAPVC
repair is pulmonary venous obstruction (Fig. 20-5), which
occurs 9% to 11% of the time, regardless of the surgical technique employed. Mortality varies between 30% and 45%,
and alternative catheter interventions do not offer definitive
solutions. Recurrent pulmonary venous obstruction can be
localized at the site of the pulmonary venous anastomosis
(extrinsic), which usually can be cured with patch enlargement or balloon dilatation, or it may be secondary to endocardial thickening of the pulmonary venous ostia frequently
resulting in diffuse pulmonary venous sclerosis (intrinsic),
which carries a 66% mortality rate because few good solutions
exist. More commonly, post repair left ventricular dysfunction can occur as the noncompliant LV suddenly is required
to handle an increased volume load from redirected pulmonary venous return. This can manifest as an increase in pulmonary artery pressure but is distinguishable from primary
166
https://t.me/medicina_free
CHAPTER 20
Congenital Heart Disease
FIG. 20-5. Angiogram showing the discrete stenosis (`*’) of
the right-sided pulmonary veins after conventional repair for
supracardiac-type TAPVC.
pulmonary hypertension (another possible postoperative
complication following repair of TAPVC) from the elevated
left atrial pressure and LV dysfunction along with echocardiographic evidence of poor LV contractility. In pulmonary
hypertension, the left atrial pressure may be low, the LV may
appear “underfilled” (by echocardiography), and the RV may
appear dilated. In either case, postoperative support for a few
days with extracorporeal membrane oxygenation may be lifesaving, and TAPVC should be repaired in centers that have
this capacity. (See Schwartz 11th ed., pp. 765–767.)
21. An 8-month-old female infant is admitted for her third
respiratory infection. She is noted by her parents to otherwise be exhibiting age-appropriate behavior, normal
stooling, and normal feeding. A chest XRAY is performed
which demonstrates unilateral, left lung hyperinflation.
Ultimately the patient undergoes a computed tomography angiography (CTA) and echocardiography confirming the presence of a pulmonary artery sling. Which of
the following is the next best step after confirming the
diagnosis?
A. Bronchoscopy
B. Cardiac catheterization
C. Intubation and mechanical ventilation
D. Barium swallow
Answer: A
The symptoms associated with vascular rings include respiratory distress, barking cough, stridor, apnea, dysphagia, and
recurrent respiratory tract infections. The diagnosis often
requires a high index of suspicion. Minor respiratory tract
infections may precipitate serious respiratory distress. The
work-up includes chest X-rays, echocardiography, bronchoscopy, CT scan (Fig. 20-6), magnetic resonance imaging
(MRI) (Fig. 20-7), and, rarely, cardiac catheterization. Chest
X-rays show the relationship of the aortic arch to the trachea.
Tracheal compression can be better evaluated using lateral
films. Unilateral hyperinflation of the lung is sometimes
seen and is often associated with a pulmonary artery sling
(Fig. 20-8). Pulmonary artery (PA) slings (Fig. 20-9) are often
associated with complete tracheal rings necessitating a bronchoscopy when this diagnosis is made (Fig. 20-10). Patients
with dysphagia require a barium esophagogram as a part of
their work-up (Fig. 20-11). (See Schwartz 11th ed., p. 769.)
FIG. 20-6. CT angiogram showing the four artery sign classic of
https://t.me/medicina_free
double aortic arch.
167
CHAPTER 20
Congenital Heart Disease
FIG. 20-7. MRI showing a double aortic arch.
FIG. 20-8. Unilateral hyperinflation of the left lung associated with
a rare vascular ring: left ascending aorta and right-sided descending
aorta.
FIG. 20-10. Rigid bronchoscopy showing complete
tracheal rings in a the patient with pulmonary artery sling.
FIG. 20-9. CT angiogram showing a PA sling. Note the LPA
wrapping around behind the trachea.
Соседние файлы в папке @xirurgi_2025