DOI:10.2214/AJR.08.2192
AJR 2009; 193:388-396
© American Roentgen Ray Society
CT Evaluation of Congenital Heart Disease in Adults
Amanda Wiant1,
Eric Nyberg and
Robert C. Gilkeson
1 All authors: Heart Valve Center, Department of Radiology, Heart and Vascular
Institute, University Hospitals of Cleveland and Case Medical Center, 11100
Euclid Ave., Cleveland, OH 44106.
Received December 2, 2008;
accepted after revision February 21, 2009.
Address correspondence to R. C. Gilkeson.
R. C. Gilkeson receives research support from Siemens Healthcare.
Abstract
OBJECTIVE. The purpose of this article is to describe the spectrum
of imaging findings of congenital heart disease in adults.
CONCLUSION. Continued advances in CT have facilitated evaluation of
two important patient populations: adults with surgically palliated congenital
heart disease and adults with previously undiagnosed congenital heart
disease.
Keywords: cardiopulmonary imaging congenital heart disease CT
Introduction
The diagnosis and effective imaging of congenital heart disease (CHD) in
adults is a growing concern. A linearly increasing population of persons with
CHD are surviving to adulthood, creating demand for quality sub-specialized
care [1] and sophisticated
imaging [2]. With the increased
use of CT angiography in the emergency department, detection of structural
heart disease, especially CHD, is increasing
[3,
4]. It is important for
radiologists to be aware of the spectrum of CHD in adults, the contexts in
which the lesions occur, and the most effective methods of imaging.
Known CHD in Adults
CHD occurs in 4–10 of 1,000 live births
[5,
6]. As a result of effective
surgery and medical management, it is estimated that 85% of these children
will survive to adulthood [7],
a total population approaching 800,000 patients in the United States
[8,
9]. Since 1985, the prevalence
of severe CHD in surviving adults has increased to 85%, and currently more
adults than children have these malformations
[10]. It is estimated
[11] that as many as 50% of
children with CHD need specialist follow-up past the age of 16 years. The most
common diseases projected to be present among CHD patients needing close
observation include coarctation of the aorta, aortic stenosis, tetralogy of
Fallot, and ventricular septal defect (VSD)
[11]. Precise follow-up
imaging of these patients is important not only to characterize the lesions
and their functional status but also to plan for surgical reintervention.
Moreover, nearly one half of patients with CHD need two or more operations
during adulthood. Surgical alterations of native anatomic features, especially
of the coronary arteries, make preoperative imaging especially important in
the care of these patients [8,
9].
Technologic Advances in Imaging CHD in Adults
Angiography and echocardiography—Traditional imaging of CHD
included invasive conventional angiography
[12], but transthoracic
echocardiography has become the primary imaging technique in the assessment of
CHD [13]. In adults with CHD,
some anatomic areas are inadequately characterized with transthoracic
echocardiography, including the right ventricle, transverse and descending
aortic arch, and pulmonary vasculature
[14]. Although it often is
useful for assessing these structures, transesophageal echocardiography has
anatomic blind spots [15] and
can cause airway compromise if the pulmonary artery is enlarged
[16]. In patients who have
undergone palliative surgery for CHD, pulmonary artery enlargement is common,
a limitation of the diagnostic capabilities of transesophageal
echocardiography [17].
MRI—Advances in MRI have made it particularly useful for
evaluation of myocardial and valvular function
[18] and of the complex 3D
spatial relations in complex CHD
[19]. The noninvasive nature
and absence of radiation and iodinated contrast material often define MRI as
the preferred technique for assessing CHD in adults
[4,
20]. MRI does have limitations
that are particularly pertinent in the care of adults with CHD. Many of these
patients who have undergone surgical correction of CHD have pacemakers or
implantable cardioverter–defibrillators
[21], which preclude MRI. This
contraindication is being reconsidered owing to new implantable
cardioverter–defibrillator technology
[22], but it remains
substantial [23].
Claustrophobia and limited access continue to be recognized limitations of
MRI. MRI also is limited in the evaluation of the lungs and airways, important
considerations among adults with CHD
[24].
CT—Advances in CT of CHD first were made with electron-beam
CT. Faster imaging times with electron-beam CT enabled acquisition of spatial
and anatomic information better than that acquired with conventional
single-detector CT. ECG-gating technology yielded functional information not
obtainable with conventional CT technology. Although effective, electron-beam
CT has the disadvantages of limited availability and prohibitive cost, which
militate against its widespread clinical utility.
MDCT provides excellent 3D depiction of cardiovascular anatomic structures
in patients with CHD. Retrospective ECG gating facilitates functional
evaluation similar to that of conventional echocardiography. Although MDCT
requires ionizing radiation, the marked reduction in imaging time often
obviates the sedation needed for transesophageal echocardiography and
sometimes for MRI.
The limitations of ECG-gated MDCT include high heart rate and arrhythmia,
which are serious limitations in the evaluation of adults with CHD, who have a
high prevalence of arrhythmia
[2]. Use of dual-source CT
addresses these limitations. With this method, two x-ray tubes and the
corresponding detectors are placed 90° to each other on the rotating
gantry. The advantages of dual-source technology are marked improvement in
temporal resolution to 83 milliseconds
[25] and a reduction in
radiation dose to nonobese patients
[26]. The improved temporal
resolution of dual-source CT facilitates accurate diagnosis, usually without
β-blockade to reduce heart rate
[27]. Furthermore, dual-source
CT has established ability similar to that of transthoracic echocardiography
in assessment of cardiac function, volume, and mass
[28]. Prospective ECG gating
also is valuable for reducing radiation exposure but currently is limited by
the need for a low heart rate, which may be difficult to achieve in some CHD
patients [29].
Protocols and image analysis—At our institution, 64-MDCT is
performed as described by Brodoefel et al.
[30,
31]. After automated IV
injection of an 80-mL bolus of contrast material at 5 mL/s with a 60-mL saline
chaser, images are acquired with the following parameters: collimation, 32
x 0.6 mm; section acquisition, 64 x 0.6 mm; gantry rotation time,
330 milliseconds; pitch, 0.20–0.43; tube voltage, 120 kV; maximum tube
current, 400 mAs/rotation. To minimize radiation for varying heart rates
modulation of full current is as follows: heart rate 60 beats/min or less,
current runs during 60–70% of the cycle; heart rate 60–70
beats/min, 50–80% of the cycle; heart rate greater than 70 beats/min,
30–80% of the cycle.
Image reconstruction—In evaluation of patients with CHD,
primary review of axial images is important for accurate diagnosis. Most of
the imaging review is performed with multiplanar reconstruction, which
facilitates visualization of atrioventricular septal defects in imaging planes
analogous to those of echocardiography. Maximal intensity projection is
instrumental in evaluation of the great vessels. The flexibility achieved with
variable slab thickness in maximal intensity projection is particularly
important in the evaluation of anomalies of the aortic arch and pulmonary
artery. Volume rendering is used most often in presurgical evaluation.
Compared with the findings at catheter angiography, the 3D anatomic relations
of the blood vessels and chest wall are markedly enhanced with MDCT.
Optimization of CT window and level settings enables visualization of
intracardiac shunts.

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Fig. 1A —23-year-old man with history of ventricular septal defect and
pulmonary atresia. See also Figure S1C, cine loop, in supplemental data at
www.ajronline.org.
Short-axis maximum-intensity-projection image shows ventricular septal defect
(arrow), right ventricular hypertrophy (asterisk), and
overriding aorta.
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Fig. 1B —23-year-old man with history of ventricular septal defect and
pulmonary atresia. See also Figure S1C, cine loop, in supplemental data at
www.ajronline.org.
Image from cine CT loop obtained with optimized window and level settings
shows right to left flow across ventricular septal defect (arrow).
Asterisk indicates right ventricular hypertrophy.
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Examples of CHD in Adults
Ventricular septal defect—VSD is the most common congenital
heart defect in the pediatric population and is second to atrial septal defect
(ASD) in the adult population
[10]. There are two main types
of VSD, membranous and muscular. Membranous septal defects are subaortic (Fig.
1A,
1B). (Fig. S1C, the cine loop,
can be seen in the AJR electronic supplement to this article,
available at
www.ajronline.org.)
These defects account for 70% of VSDs. The muscular septum has several
divisions—inlet to the atrioventricular canal, outlet in the aorta, and
trabecular along the muscle—that account for the rest of the lesions.
These lesions can be observed, but if the defect is large, surgical closure is
indicated [32]. To more easily
detect a VSD at CT, it is helpful to use a saline bolus to optimize
visualization of the intracardiac shunts. These techniques have been
particularly helpful in the care of adults with symptomatic VSD
(Fig. 2).

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Fig. 2 —21-year-old woman with known ventricular septal defect and
increasing shortness of breath. Short-axis maximum-intensity-projection image
shows prolapse of dilated noncoronary aortic cusp (arrow) into
membranous ventricular septal defect (arrowhead) that resulted in
aortic valve regurgitation. Regurgitant aortic valve flow (asterisk)
is toward right ventricle.
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Tetralogy of Fallot—Tetralogy of Fallot accounts for
approximately 10% of cases of congenital heart defects and is the most common
cyanotic lesion. Tetralogy of Fallot is classically described as a collection
of four defects: infundibular or pulmonary stenosis, right ventricular
hypertrophy, overriding aorta, and VSD
[32]. In addition, coronary
artery anomalies, persistent left-sided superior vena cava, and a right aortic
arch may be present [24,
32,
33].
Most adults with tetralogy of Fallot have undergone either insertion of a
palliative shunt or definitive repair in infancy or early childhood
[34], but a small number of
patients survive to adulthood without surgery
[35]. The current preference
for treatment is early definitive repair
[34]. The reported
[20,
24] long-term effects of
surgical intervention include pulmonic valve regurgitation, pulmonary arterial
hypertension, right ventricular dilation, leak in the VSD patch, aortic
insufficiency, and electrophysiologic derangements. The improved temporal and
spatial resolution of dual-source CT improves definition of the defect in
older patients with symptomatic tetralogy of Fallot (Fig.
3A,
3B,
3C,
3D). (Fig. S3E, the cine loop,
can be seen in the AJR electronic supplement to this article,
available at
www.ajronline.org.)

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Fig. 3A —57-year-old man with history of tetralogy of Fallot repair.
Right-heart catheterization showed pulmonary artery hypertension and step-up
in oxygenation in pulmonary artery consistent with aortopulmonary shunt. See
also Figure S3E, cine loop, in supplemental data at
www.ajronline.org.
Oblique axial maximum-intensity-projection image shows calcified ventricular
septal defect patch (arrow).
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Fig. 3B —57-year-old man with history of tetralogy of Fallot repair.
Right-heart catheterization showed pulmonary artery hypertension and step-up
in oxygenation in pulmonary artery consistent with aortopulmonary shunt. See
also Figure S3E, cine loop, in supplemental data at
www.ajronline.org.
Coronal maximum-intensity-projection image shows thrombosed remnant of
Blalock-Taussig shunt (arrow).
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Fig. 3C —57-year-old man with history of tetralogy of Fallot repair.
Right-heart catheterization showed pulmonary artery hypertension and step-up
in oxygenation in pulmonary artery consistent with aortopulmonary shunt. See
also Figure S3E, cine loop, in supplemental data at
www.ajronline.org.
Sagittal oblique volume-rendered image shows patent Potts shunt
(arrow) from descending aorta (A) to pulmonary artery (P).
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Fig. 3D —57-year-old man with history of tetralogy of Fallot repair.
Right-heart catheterization showed pulmonary artery hypertension and step-up
in oxygenation in pulmonary artery consistent with aortopulmonary shunt. See
also Figure S3E, cine loop, in supplemental data at
www.ajronline.org.
Sagittal maximum-intensity-projection image from cine loop obtained with
optimized window and level settings shows shunt flow from aorta (A) to
pulmonary artery (P). Arrow indicates Potts shunt.
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Transposition of the great arteries—Transposition of the
great arteries has two main variants: dextrotransposition (complete) and
levotransposition (congenitally corrected). The more severe
dextrotransposition of the great arteries accounts for 5–7% of cases of
CHD. In this anomaly, the aorta connects to the anatomic right ventricle and
the pulmonary artery to the left ventricle. Anatomic anomalies, such as patent
ductus arteriosus, VSD, and ASD, allow mixing of blood.
Because of the poor prognosis without surgery, most adult patients with
transposition of the great arteries have undergone corrective procedures. More
than 80% of neonates treated surgically survive to adolescence, the best
results being associated with the arterial switch procedure
[36]. The most concerning
complications for adults surviving arterial switch are coronary artery
stenosis and neoaortic root dilation with valve regurgitation
[8,
9].
The long-term sequelae of the atrial switch procedures (Mustard, Senning)
are more severe than those of the arterial switch procedure, largely owing to
the prolonged effects of systemic pressure on the morphologic rightsided
ventricle. The right ventricle is prone to dilation and failure, and the
tricuspid valve becomes regurgitant. Important complications of atrial switch
procedures include obstruction and leakage of the atrial baffles and pulmonary
hypertension. MDCT facilitates dynamic evaluation of both the atrial baffles
and ventricular function in these patients. In patients with contraindications
to MRI, ECG-gated MDCT can be performed for diagnostic evaluation of the
affected right ventricle (Fig.
4A,
4B). (Fig. S4C, a cine loop,
can be seen in the AJR electronic supplement to this article,
available at
www.ajronline.org.)

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Fig. 4A —24-year-old man with progressive exercise intolerance and
history of Mustard procedure. MRI is contraindicated owing to presence of
epicardial pacemaker. See also Figure S4C, cine loop, in supplemental data at
www.ajronline.org.
Axial oblique maximum-intensity-projection image from cine loop shows intact
flow in superior vena caval–left atrial baffle (arrow) without
evidence of leak.
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Fig. 4B —24-year-old man with progressive exercise intolerance and
history of Mustard procedure. MRI is contraindicated owing to presence of
epicardial pacemaker. See also Figure S4C, cine loop, in supplemental data at
www.ajronline.org.
Short-axis image from cine loop shows marked right ventricular hypertrophy and
global hypokinesis of right ventricle. Septal dyskinesia consistent with
bundle branch block is evident. Arrow indicates superior vena caval–left
atrial baffle. R = right ventricle.
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Associated congenital heart lesions—CT is used for
noninvasive evaluation of adults with cyanotic heart disease who have
undergone palliative surgical treatment. Pulmonary hypertension is common in
adults with surgical aortopulmonary shunts. These patients can have marked
airway compromise, which is well characterized with volumetric CT (Fig.
5A,
5B,
5C,
5D). A large body of
literature describes the high incidence of coronary artery anomalies among
persons with CHD. Dual-source CT is used for noninvasive evaluation of the
coronary arteries of adults with symptomatic anomalies (Fig.
6A,
6B).

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Fig. 5A —43-year-old woman with pulmonary arterial hypertension, chest
pain, and dyspnea; dextrocardia and double-outlet right ventricle; and history
of Blalock-Taussig and Waterston shunts. A = aorta, P = pulmonary artery.
Coronal oblique volume-rendered image shows intact Blalock-Taussig shunt
(arrow).
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Fig. 5B —43-year-old woman with pulmonary arterial hypertension, chest
pain, and dyspnea; dextrocardia and double-outlet right ventricle; and history
of Blalock-Taussig and Waterston shunts. A = aorta, P = pulmonary artery.
Sagittal oblique volume-rendered image shows intact Waterston shunt from
ascending aorta to pulmonary artery (arrow).
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Fig. 5C —43-year-old woman with pulmonary arterial hypertension, chest
pain, and dyspnea; dextrocardia and double-outlet right ventricle; and history
of Blalock-Taussig and Waterston shunts. A = aorta, P = pulmonary artery.
Axial CT scan shows marked enlargement of pulmonary artery and calcification
(arrow) resulting in tracheal compression (arrowhead).
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Fig. 5D —43-year-old woman with pulmonary arterial hypertension, chest
pain, and dyspnea; dextrocardia and double-outlet right ventricle; and history
of Blalock-Taussig and Waterston shunts. A = aorta, P = pulmonary artery.
Three-dimensional reconstruction of tracheobronchial tree shows marked distal
tracheal narrowing (arrow).
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Fig. 6A —37-year-old man with exertional chest pain and history of
single ventricle and dextrotransposition. Axial maximum-intensity-projection
image shows anterior transposition of aorta (A) in relation to pulmonary
artery (P). White arrows indicate anomalous origin of right coronary artery
from left coronary cusp. Right coronary artery takes retrosternal course in
close apposition to inner table of sternum (black arrow).
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Fig. 6B —37-year-old man with exertional chest pain and history of
single ventricle and dextrotransposition. Oblique volume-rendered image shows
anomalous retrosternal course (arrowhead) of right coronary artery
(arrow), which shares common origin with left coronary artery.
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Undiagnosed CHD in Adults
The literature describes the suboptimal sensitivity, specificity, and
efficiency of traditional methods of diagnosis of the most concerning causes
of chest pain [37,
38]. Advances in MDCT have
resulted in its increased use in the evaluation of a variety of acute
cardiopulmonary disorders. The triple-rule-out protocol has become widely used
in evaluation of coronary artery disease, aortic dissection and aneurysm, and
pulmonary embolism [37,
39–41].
An estimated one in six patients undergoing MDCT for chest pain has a
nonischemic abnormality that accounts for the symptoms
[42]. It consequently is
important to be aware of other diagnoses, including congenital anomalies, that
may be clinically relevant in this population. Although the true prevalence of
unsuspected CHD in adults has not been described to our knowledge, literature
does exist on the presence of unsuspected CHD in adults undergoing MDCT for a
variety of cardiopulmonary symptoms
[3,
4]. The most common lesions
include aortic arch anomalies, septal defects, bicuspid aortic valve, and
coarctation of the aorta.

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Fig. 7A —84-year-old woman with recent transient ischemic attack and
chest radiographic finding of mediastinal mass. Axial CT scan shows marked
aneurysmal ulceration of aberrant right subclavian artery (arrow)
crossing behind trachea.
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Fig. 7B —84-year-old woman with recent transient ischemic attack and
chest radiographic finding of mediastinal mass. Coronal
maximum-intensity-projection image from cine loop shows mobile thrombus
(asterisk) within aneurysmal aberrant right subclavian artery
(arrow).
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Aortic Arch Anomalies
Important aortic arch anomalies include double aortic arch, right-sided
arch, and left aortic arch with aberrant right subclavian artery. Older adults
with aberrant right subclavian artery are prone to aneurysmal dilation and
atherosclerotic disease (Fig.
7A,
7B).
Congenital Venous Anomalies
The clinical consequences of congenital anomalous venous anomalies are most
commonly detected when an intrathoracic central venous catheter takes an
unexpected course [43].
Left-sided superior vena cava to the coronary sinus is otherwise asymptomatic.
When, however, the anomalous left superior vena cava drains into the left
atrium, the resulting cyanosis is often explained when the resultant
right-to-left shunt is visualized
[32] (Fig.
8A,
8B).

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Fig. 8B —74-year-old man with persistent hypoxia. Coronal
maximum-intensity-projection image shows unopacified left-sided superior vena
cava (arrow) with direct communication to enlarged left atrium
(arrowhead).
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Partial Anomalous Pulmonary Venous Return
Partial anomalous pulmonary venous return is rare, accounting for fewer
than 1% of cases of CHD, and is associated with ASD. The defect consists of a
pulmonary vein draining to the systemic venous circulation. The right atrium,
superior vena cava, inferior vena cava, coronary sinus, or brachiocephalic
vein can be the site of the anomalous draining vein, and left-sided partial
anomalous pulmonary venous return often drains through a vertical vein
(Fig. 9). Although this defect
often is asymptomatic, if the resultant pulmonary-to-systemic shunt is severe
enough, surgical repair involving rerouting of blood to the left atrium may be
indicated.

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Fig. 9 —78-year-old woman undergoing evaluation for pulmonary
embolism. Axial maximum-intensity-projection image shows anomalous right lower
lobe pulmonary vein draining into superior vena cava (arrows).
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Congenital Anomalies of the Aortic Valve and Valve Apparatus
The incidence of bicuspid and congenitally dysplastic aortic valve is
1–2% in the general population
[44]. Long-term complications
associated with bicuspid aortic valve include aortic stenosis (Figs.
10 and
11), regurgitation,
dissection, root dilation, and endocarditis
[21,
45]. Aortopathy associated
with a bicuspid valve may necessitate aortic root replacement in addition to
valve replacement. Quadricuspid aortic valve is a rare anomaly, frequently a
clinically silent lesion that manifests when patients are at a mean age of 49
years [46]. In patients who
have aortic regurgitation, it is important to accurately identify a
quadricuspid valve because of the faster functional decline associated with
asymmetric mechanical stress on the valve leaflets. Definitive diagnosis with
transesophageal echocardiography is suboptimal
[47]. The greater temporal
resolution of dual-source CT improves delineation of these congenital aortic
valve abnormalities (Fig.
12).

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Fig. 10 —74-year-old man with progressive dyspnea. Axial
maximum-intensity-projection image shows calcified stenotic bicuspid aortic
valve (arrow). Fusion of right and noncoronary cups
(arrowhead) is evident.
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Fig. 12 —44-year-old man with echocardiographic finding of aortic
regurgitation. Axial oblique maximum-intensity-projection image shows
quadricuspid aortic valve. Arrow indicates lack of coaptation of quadricuspid
valve leaflets in diastole.
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Subvalvular stenosis accounts for 23% of cases of left ventricular outflow
tract obstruction and usually consists of a discrete fibrous membrane that
forms as a result of turbulent flow (Fig.
13). Many patients have other cardiac malformations or have
undergone cardiac intervention. Approximately one third of patients have an
isolated lesion. A second type of web is tunneled stenosis, which is often
associated with other valve and left ventricular anomalies.

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Fig. 13 —57-year-old woman with exercise intolerance and
echocardiographic finding of subaortic flow acceleration. Coronal
maximum-intensity-projection image shows discrete subaortic web
(arrow).
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ASD
In adults with symptoms, advances in CT have improved detection of
undiagnosed ASD (Fig. 14).
ASDs include ostium primum, ostium secundum, and sinus venosus defects. Ostium
primum defects usually occur within the spectrum of endocardial cushion
defects and are usually diagnosed in the neonatal period. Ostium secundum ASD
is the most common ASD in adults, accounting for 50–70% of these
lesions. Ostium secundum ASD should always be considered in the evaluation of
adults with pulmonary hypertension (Fig.
15A,
15B). Sinus venosus ASD
involves the superior vena caval–right atrial junction. Its association
with anomalous pulmonary venous return should be considered when an adult has
right-heart enlargement and unexplained pulmonary hypertension
(Fig. 16).

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Fig. 14 —42-year-old man undergoing CT evaluation of coronary artery
disease. Axial CT image shows atrial septal aneurysm (arrow) with
associated patent foramen ovale. Saline flush with clearance of contrast
material from right-sided cardiac structures enables clear delineation of left
to right flow.
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Fig. 16 —36-year-old man with echocardiographic finding of right-heart
enlargement. Axial oblique maximum-intensity-projection image shows enlarged
right ventricle (R) and sinus venous atrial septal defect (asterisk).
Arrow indicates anomalous drainage of right middle lobe vein to right
atrium.
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Coronary Artery Anomalies
Although more common among persons with CHD than in the general population,
isolated coronary artery anomalies are rare. They have been found in
0.3–0.5% of cases in autopsy series and in 0.3–1.3% of patients
undergoing cardiac catheterization
[48]. Although most coronary
anomalies are benign incidental findings
[49], it is important to
accurately identify anomalies associated with ischemia and sudden cardiac
death. A 1956 autopsy study
[50] showed a 70–80%
incidence of sudden cardiac death among patients with isolated coronary artery
anomalies.
In the CT assessment of anomalous coronary arteries, it is important to
review the normal anatomic configuration of the coronary arteries and their
origins from the appropriate aortic sinuses. When the aortic valve is
visualized as a clock face, a normal right coronary artery origin exits in the
right aortic sinus between the 10- and 1-o'clock positions. The left coronary
artery exits in the left coronary sinus between the 2- and 5-o'clock
positions. An anomalous coronary artery is first suspected when the coronary
artery is not visualized in the expected coronary sinus. Although the origins
of the anomalous coronary arteries can be identified, echocardiographic and
angiographic evaluation of the distal course of these vessels is limited. In
the evaluation of adults with chest pain, accurate delineation of these
anomalous vessels in the chest is essential to future therapy, and MDCT is a
noninvasive diagnostic alternative.
A number of theories have been proposed to explain the cause of ischemia
and sudden cardiac death in patients with coronary artery anomalies. These
interarterial coronary arteries may have a substantial intramural component
before exiting the aorta. This intramural course results in a slitlike orifice
and compromised blood flow that may be particularly important in children and
young athletes [51]. The
interarterial coronary artery may take an acute angle as it courses to its
appropriate atrioventricular groove. This acute angle can compromise blood
flow and place myocardial tissue at risk. Dynamic compression of the
interarterial coronary artery between the aorta and pulmonary artery also is
hypothesized to be a source of compression, chest pain, and clinical signs of
coronary ischemia. It is also recognized that an interarterial anomalous
coronary artery can take an intramyocardial distal course, an additional risk
factor for ischemia and death.

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Fig. 17A —41-year-old man with exertional chest pain. Axial thin-slab
maximum-intensity-projection image shows common origin of left and right
coronary arteries from right coronary cusp (asterisk). Anomalous left
coronary artery (arrow) courses between aorta and pulmonary outflow
tract.
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Fig. 17B —41-year-old man with exertional chest pain. Coronal
maximum-intensity-projection image demonstrates common origin of right
coronary artery (arrowhead) and left main coronary artery
(arrow) from right coronary sinus (asterisk)
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Fig. 18A —62-year-old woman with chest pain. Axial
maximum-intensity-projection image shows origin of right coronary artery from
left anterior descending coronary artery (arrowhead). Arrow indicates
course of right coronary artery anterior to pulmonary artery.
|
|
The most important coronary arterial anomaly is an anomalous coronary
artery originating from the opposite sinus of Valsalva. The left coronary
artery originates from the right aortic sinus, and the right coronary artery
originates from the left aortic sinus. It is important to identify the
complete course of these anomalous vessels. An anomalous coronary artery that
courses between the aorta and pulmonary artery is associated with substantial
morbidity and mortality (Fig.
17A,
17B). Delineation of a benign
course also is important for avoiding unnecessary intervention (Fig.
18A,
18B).
Conclusion
The management of CHD in adults has improved as a result of the development
of successful surgical and medical therapies. Follow-up care of these patients
frequently entails detailed imaging of complex anatomic details. The
increasing use of MDCT in the evaluation of patients with chest pain has
improved detection of previously unsuspected CHD. These patients benefit from
advances in CT technology that enhance the diagnosis and management of these
complex anomalies.
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