AJR 2005; 184:241-247
© American Roentgen Ray Society
MDCT of Postoperative Anatomy and Complications in Adults with Cyanotic Heart Disease
Marilyn J. Siegel1,
Sanjeev Bhalla1,
Fernando R. Gutierrez1 and
Joseph B. Billadello2
1 Mallinckrodt Institute of Radiology, Washington University School of Medicine,
510 S Kingshighway Blvd., St. Louis, MO 63110.
2 Division of Cardiology, Department of Medicine, Washington University School
of Medicine, St. Louis, MO 63110.
Received March 18, 2004;
accepted after revision June 14, 2004.
Address correspondence to M. J. Siegel.
Introduction
Approximately 800,000 adults in the United States have congenital heart
disease, and their numbers are growing
[1]. Echocardiography is the
imaging examination of choice for the initial evaluation of these patients.
However, this technique does not allow adequate definition of intraatrial
baffles in patients who have undergone an atrial switch procedure for
transposition of the great vessels or conduits in patients who have undergone
a Fontan procedure for treatment of tricuspid atresia. MRI offers excellent
anatomic and functional information, but it is time-consuming, is
contraindicated in patients with pacemakers, and can be technically difficult
in uncooperative and seriously ill patients. Electron beam and 4-MDCT have
been described as examination techniques for a variety of congenital heart
diseases [2,
3], but little information has
been published on the use of MDCT in the evaluation of postoperative anatomy
and complications. We present a pictorial review on the use of MDCT in the
evaluation of adults with surgically treated cyanotic heart lesions.
Recognizing the postoperative appearance of these complicated lesions is
important because most adult patients with repaired cyanotic heart disease
require lifelong cardiac care.
Patients and MDCT Techniques
Patients in our study were referred from the center for adults with
congenital heart disease at our hospital. The most commonly treated cyanotic
lesions seen in our adult center are tetralogy of Fallot, transposition of the
great arteries, tricuspid atresia, and pulmonary atresia. Adult patients with
these diseases usually come to clinical attention because of recurrent or
increasing cyanosis, dyspnea on exertion, or fatigue or because of
echocardiographic findings suggesting postoperative complications that need
further characterization before therapy is instituted.
Nearly all MDCT scans presented in this essay were acquired with Sensation
16 scanners (Siemens) and a pulmonary embolism protocol using the following
parameters: 1.5-mm collimation, 24 mm per rotation table feed, 120–200
mA, and 120 kV. A few scans were acquired with a Plus 4 volume scanner
(Siemens) using 2.5-mm slice collimation and 20 mm per rotation table feed. In
all patients, unenhanced MDCT scans were obtained to identify subtle
dystrophic calcifications (Figs.
1A and
1B). We limited the unenhanced
scanning to the area to be interrogated to minimize the radiation dose.
Contrast-enhanced studies were obtained with 150 mL of nonionic contrast agent
(320 mg I/mL) that was administered with a power injector at a rate of
3–4 mL/sec. The scanning delay was determined with an automatic bolus
tracking system using a variable region of interest, depending on the
clinically suspected disease. The threshold level of 100 H triggered the
scanning. Scans were acquired in a craniocaudal direction during a single
breath-hold. Retrospective ECG gating was performed in patients with heart
rates under 90 beats per minute. Unenhanced scans were reconstructed at 5-mm
intervals, and contrast-enhanced scans were reconstructed at 2-mm intervals. A
standard reconstruction algorithm was used for both reconstructions.
Multiplanar and 3D reconstructions using volume-rendered technique were
performed on the scans of all patients.
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Fig. 1A. —37-year-old man with repaired tetralogy of Fallot and
cyanosis who had undergone complete repair in infancy and now presented with
dyspnea. MDCT was used to evaluate pulmonary outflow tract. Unenhanced MDCT
scan shows dystrophic calcification in right ventricular outflow patch
(arrow) and calcification at site of prior Waterston shunt
(arrowhead) that has been taken down.
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Fig. 1B. —37-year-old man with repaired tetralogy of Fallot and
cyanosis who had undergone complete repair in infancy and now presented with
dyspnea. MDCT was used to evaluate pulmonary outflow tract. Calcification at
site of Waterston takedown (arrowhead) is less apparent on
contrast-enhanced MDCT scan.
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Illustrative Cases
Tetralogy of Fallot
The classic components of tetralogy of Fallot are right ventricular
subpulmonic obstruction, a perimembranous ventricular septal defect,
overriding of the aorta, and right ventricular hypertrophy. Adults with
tetralogy of Fallot usually have undergone definitive repair, but occasionally
patients reach adulthood having had only a palliative procedure, usually a
Blalock-Taussig shunt (Figs.
2A,
2B,
3).
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Fig. 2A. —30-year-old woman with tetralogy of Fallot and increasing
cyanosis who had undergone palliative Blalock-Taussig shunt in infancy. MDCT
was used to evaluate shunt patency and intracardiac anatomy. Anatomic features
characteristic of tetralogy of Fallot can be seen in this patient who had had
only palliative shunt. Axial MDCT scan shows right aortic arch with
mirror-image branching. Also noted is patent left Blalock-Taussig shunt
(arrow).
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Fig. 2B. —30-year-old woman with tetralogy of Fallot and increasing
cyanosis who had undergone palliative Blalock-Taussig shunt in infancy. MDCT
was used to evaluate shunt patency and intracardiac anatomy. Anatomic features
characteristic of tetralogy of Fallot can be seen in this patient who had had
only palliative shunt. MDCT scan obtained caudad to A shows large
ventricular septal defect (arrow) and right ventricular hypertrophy
(RV). Incidentally noted is calcific aortic valve (arrowhead).
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Fig. 3. —In 43-year-old man who had undergone modified Blalock-Taussig
shunt, curved multiplanar reformatted image shows patent Blalock-Taussig shunt
(arrows) from left subclavian artery to pulmonary artery.
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Reparative surgery for tetralogy of Fallot involves closing the septal
defect and relieving the right ventricular outflow obstruction by placing a
patch across either the outflow tract or the pulmonary valve annulus (Figs.
4A and
4B). Many adults develop
significant pulmonary insufficiency after repair and require pulmonary valve
replacement. Aneurysmal dilatation or stenosis (Figs.
5A and
5B) of the right ventricular
outflow tract after repair is also seen. The aorta is often abnormal in these
patients and aortic enlargement with aortic insufficiency can occur
[4].
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Fig. 4A. —30-year-old woman with tetralogy of Fallot and cyanosis who
had undergone complete repair with placement of ventricular septal defect
patch. Echocardiography was of limited use in evaluation of pulmonary outflow
tract because of sternal deformity. MDCT was used to evaluate postoperative
anatomy. MDCT scan shows ventricular septal defect patch (arrow).
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Fig. 4B. —30-year-old woman with tetralogy of Fallot and cyanosis who
had undergone complete repair with placement of ventricular septal defect
patch. Echocardiography was of limited use in evaluation of pulmonary outflow
tract because of sternal deformity. MDCT was used to evaluate postoperative
anatomy. MDCT scan obtained cephalad to A shows calcified right ventricular
patch (arrowhead).
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Fig. 5A. —35-year-old woman with tetralogy of Fallot and dyspnea on
exertion who had undergone complete repair with placement of right ventricular
outflow tract patch. MDCT was used to evaluate outflow tract. Axial MDCT scan
shows thick right ventricle wall (RV) secondary to stenosed outflow tract.
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Fig. 5B. —35-year-old woman with tetralogy of Fallot and dyspnea on
exertion who had undergone complete repair with placement of right ventricular
outflow tract patch. MDCT was used to evaluate outflow tract. Sagittal
multiplanar image shows narrowed pulmonary outflow tract (straight
arrow) just distal to calcified Dacron ([polyethyene terephthalate],
Invista) patch. Moderately severe stenosis was confirmed at surgery.
Incidentally noted is persistent left superior vena cava (curved
arrow) as it drains into coronary sinus (C).
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Dextrotransposition of the Great Arteries
Dextrotransposition (D-transposition) of the great arteries is a cyanotic
lesion in which the aorta arises from the right ventricle and the pulmonary
artery arises from the left ventricle (atrioventricular concordance and
ventriculoarterial discordance). Although the arterial switch operation
(Jatene operation) is now the surgical treatment of choice, most adults with
D-transposition have undergone the atrial switch with either a Mustard or a
Senning operation in which an atrial baffle redirects systemic venous blood to
the anatomic left ventricle (pulmonary ventricle) and pulmonary venous blood
to the systemic (anatomic right) ventricle
(Fig. 6). The net effect is a
functional atrial switch. The left ventricle remains the pulmonary ventricle
and the right ventricle remains the systemic ventricle. The Mustard operation
uses pericardial tissue or Gor-tex (W. L. Gore & Associates) for the
baffle, whereas the Senning operation is based on reconstruction of the atrial
septum to form the intraatrial baffle (Figs.
7A and
7B). Complications after
atrial switch repairs include baffle leakage (Figs.
8A and
8B) and baffle obstruction
(Figs. 9A and
9B). Tricuspid insufficiency
(Figs. 10A and
10B), right ventricular
hypertrophy and enlargement, and right ventricular failure are common findings
in these patients.
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Fig. 6. —33-year-old woman with dextrotransposition (D-transposition)
of great arteries and acute chest pain had undergone Mustard operation in
infancy. MDCT was performed to evaluate for pulmonary embolus. Sagittal 3D
reformatted image shows pulmonary trunk arising from left ventricle (LV) and
aorta arising from right ventricle (RV), which is typical of
D-transposition.
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Fig. 7A. —27-year-old man with dextrotransposition of great arteries
who had undergone Mustard operation and presented with increasing cyanosis.
MDCT was performed to evaluate baffle patency. Multiplanar reformatted image
was obtained after antecubital vein injection of contrast material. Superior
limb (S) (superior vena cava to left ventricle) of systemic baffle is
opacified. Inferior limb (I) (inferior vena cava to left ventricle) is not
opacified.
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Fig. 7B. —27-year-old man with dextrotransposition of great arteries
who had undergone Mustard operation and presented with increasing cyanosis.
MDCT was performed to evaluate baffle patency. Axial MDCT scan shows
unobstructed pulmonary veins as they drain into right ventricle (RV). Leaflets
of tricuspid valve (arrows) can be clearly seen.
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Fig. 8A. —28-year-old woman with dextrotransposition of great arteries
and dyspnea on exertion who had undergone Senning operation in infancy.
Echocardiography findings suggested baffle leak. MDCT was performed to reveal
precise location of leakage. Axial MDCT scan shows small amount of contrast
medium (arrow) in pulmonary baffle, indicating communication with
superior limb (arrowhead) of systemic baffle. Normally, contrast
material would not be seen in superior limb because limb is no longer in
communication with superior vena cava.
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Fig. 8B. —28-year-old woman with dextrotransposition of great arteries
and dyspnea on exertion who had undergone Senning operation in infancy.
Echocardiography findings suggested baffle leak. MDCT was performed to reveal
precise location of leakage. Coronal multiplanar image shows retrograde
contrast leak (arrows) into pulmonary baffle. S = superior limb of
baffle.
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Fig. 9A. —35-year-old man with dextrotransposition of great arteries
and new onset of cyanosis who had undergone Mustard operation in infancy.
Findings on echocardiography were normal. MDCT was performed to evaluate
baffle patency. Axial MDCT scan shows stenotic superior baffle limb
(arrow). Note contrast material in pulmonary baffle
(arrowhead), consistent with baffle leak. Retrograde flow to azygous
venous system is also seen.
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Fig. 9B. —35-year-old man with dextrotransposition of great arteries
and new onset of cyanosis who had undergone Mustard operation in infancy.
Findings on echocardiography were normal. MDCT was performed to evaluate
baffle patency. Coronal maximum-intensity-projection image shows azygous and
pericardial collaterals that developed to divert blood away from stenosis.
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Fig. 10A. —26-year-old man with dextrotransposition of great arteries
and mild dyspnea on exertion who had undergone Mustard operation in infancy.
Echocardiography showed tricuspid insufficiency. MDCT was performed to
evaluate baffle status. Axial MDCT scan shows small communication
(arrow) between systemic (S) and pulmonary (P) baffles.
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Fig. 10B. —26-year-old man with dextrotransposition of great arteries
and mild dyspnea on exertion who had undergone Mustard operation in infancy.
Echocardiography showed tricuspid insufficiency. MDCT was performed to
evaluate baffle status. MDCT scan obtained at more cranial level than A
shows dilated inferior vena cava and dilated hepatic veins, consistent with
tricuspid insufficiency.
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Tricuspid Atresia
In tricuspid atresia, the tricuspid valve and the inflow portion of the
right ventricle are absent. There must be an obligatory interatrial
communication, either an atrial septal defect or patent foramen ovale, to
allow mixing of systemic and pulmonary venous blood. In most cases, a
rudimentary right ventricle and a ventricular septal defect are also found.
The left heart is normal.
Adult patients with repaired tricuspid atresia have undergone several
different operations. The classic Glenn shunt is an end-to-end anastomosis of
the right pulmonary artery to the superior vena cava, with ligation of the
superior vena cava at the right atrial junction and ligation of the right
pulmonary artery at its origin
[5]
(Fig. 11). This shunt results
in isolation of the right lung from hepatic blood flow and leads to the
development of pulmonary arteriovenous fistulas, and it is no longer performed
(Fig. 12).
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Fig. 11. —23-year-old man with classic Glenn shunt for tricuspid
atresia presented with increasing cyanosis. MDCT was performed to evaluate
shunt patency. Coronal multiplanar reformatted image shows Glenn shunt
(arrows), which extends from superior vena cava to right pulmonary
artery. Flow is also noted in large collateral vessels in left
mediastinum.
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Fig. 12. —34-year-old man with tricuspid atresia and recurrent cyanosis
had undergone classic Glenn shunt in childhood and returned with recurrent
cyanosis. MDCT was performed to evaluate shunt patency. Axial MDCT scan shows
arteriovenous malformation (arrow) in right lung.
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The classic Fontan procedure used a valved conduit between the right atrium
and the left pulmonary artery (Figs.
13A,
13B, and
13C) in patients who had a
classic Glenn shunt. Atrial to pulmonary artery conduits are no longer
performed because they result in a dilated right atrium and atrial
arrhythmias. Currently, a bidirectional Glenn shunt procedure is followed by
creation of an inferior vena cava to pulmonary conduit that can be either
intracardiac (lateral tunnel) or extracardiac. This is known as the total
cavopulmonary Fontan procedure (Fig.
14). Adult patients who have atrial to pulmonary artery conduits
may develop conduit stenosis and require replacement of the conduit or
conversion to the total cavopulmonary Fontan conduit
(Fig. 15).
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Fig. 13A. —25-year-old woman with tricuspid and pulmonary atresia who
had Fontan procedure and subsequently developed shunt stenosis requiring stent
placement. Follow-up echocardiography revealed enlarged right heart. MDCT was
performed to evaluate patency of Fontan shunt. Consecutive axial MDCT scans
show Fontan shunt (arrows, A and B) from right atrium
(A and B) to pulmonary artery (PA, C).
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Fig. 13B. —25-year-old woman with tricuspid and pulmonary atresia who
had Fontan procedure and subsequently developed shunt stenosis requiring stent
placement. Follow-up echocardiography revealed enlarged right heart. MDCT was
performed to evaluate patency of Fontan shunt. Consecutive axial MDCT scans
show Fontan shunt (arrows, A and B) from right atrium
(A and B) to pulmonary artery (PA, C).
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Fig. 13C. —25-year-old woman with tricuspid and pulmonary atresia who
had Fontan procedure and subsequently developed shunt stenosis requiring stent
placement. Follow-up echocardiography revealed enlarged right heart. MDCT was
performed to evaluate patency of Fontan shunt. Consecutive axial MDCT scans
show Fontan shunt (arrows, A and B) from right atrium
(A and B) to pulmonary artery (PA, C).
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Fig. 14. —20-year-old woman with tricuspid atresia and dyspnea on
exertion had undergone cavopulmonary repair and presented with dyspnea on
exertion. Coronal multiplanar reformatted image shows that conduit (C) is
between inferior vena cava (Inf) and anastomosis of right pulmonary artery
(arrow) and superior vena cava.
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Fig. 15. —25-year-old woman with tricuspid atresia had undergone Fontan
procedure and developed postoperative graft stenosis that was treated with
stent placement. MDCT was performed to evaluate graft patency. Axial MDCT scan
shows stent (arrow) between markedly dilated right atrium (RA) and
main pulmonary artery (PA).
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Conclusion
MDCT has an expanding role in the comprehensive evaluation of postoperative
congenital heart defects because of the complexity of such defects and
multisystem complications that can result from them. CT has several advantages
over MRI. First, CT is readily available, whereas MRI is not. Second, CT is
less time-consuming than MRI. CT requires less than 1 min to complete the
study, and MRI requires at least 1 hr. Third, CT allows a more complete
evaluation of lung parenchyma, mediastinum, and upper abdomen. Fourth, CT,
unlike MRI, is not hampered by postoperative metal artifacts. This difference
becomes crucial with the increasing use of vascular stents in this patient
population. Finally, with the newer generation CT scanners, the spatial
resolution of CT in some cases is superior to that of MRI. We recognize that
CT lacks the functional capabilities of MRI, but all patients with repaired
congenital heart disease have a complete echocardiographic evaluation that
provides the necessary functional data. In patients with inconclusive results
on echocardiography, MRI may need to be performed because of its superior
temporal resolution. As the temporal resolution of CT improves, CT may have a
wider role in assessing cardiac function.
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- Gilkeson RC, Ciancibello L, Zahka K. Multidetector CT evaluation of
congenital heart disease in pediatric and adult patients.
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- Goo HW, Park I-S, Ko J-K, et al. CT of congenital heart disease:
normal anatomy and typical pathologic conditions.
RadioGraphics2003; 23:S147
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- Gatzoulis MA. Tetralogy of Fallot. In: Gatzoulis MA, Webb GD,
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Introduction
Patients and MDCT Techniques
