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DOI:10.2214/AJR.08.2192
AJR 2009; 193:388-396
© American Roentgen Ray Society


Review

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
Top
Abstract
Introduction
Known CHD in Adults
Undiagnosed CHD in Adults
Conclusion
References
 
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
Top
Abstract
Introduction
Known CHD in Adults
Undiagnosed CHD in Adults
Conclusion
References
 
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
Top
Abstract
Introduction
Known CHD in Adults
Undiagnosed CHD in Adults
Conclusion
References
 
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.


Figure 1
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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.

 


Figure 2
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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.

 
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).


Figure 3
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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.

 
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.)


Figure 4
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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).

 

Figure 5
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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).

 

Figure 6
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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).

 

Figure 7
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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.

 

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.)


Figure 8
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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.

 

Figure 9
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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.

 
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).


Figure 10
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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).

 

Figure 11
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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).

 

Figure 12
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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).

 

Figure 13
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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).

 

Figure 14
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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).

 

Figure 15
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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.

 


Undiagnosed CHD in Adults
Top
Abstract
Introduction
Known CHD in Adults
Undiagnosed CHD in Adults
Conclusion
References
 
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, 3941]. 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.


Figure 16
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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.

 


Figure 17
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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).

 
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).


Figure 18
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Fig. 8A 74-year-old man with persistent hypoxia. Axial maximum-intensity-projection image shows marked heterogeneity in contrast enhancement within enlarged left atrium (arrow).

 

Figure 19
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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).

 
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.


Figure 20
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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).

 
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).


Figure 21
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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.

 

Figure 22
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Fig. 11 34-year-old female long distance runner with decreasing exercise tolerance. Axial maximum-intensity-projection image shows markedly dysplastic and thickened valve leaflets (arrow).

 

Figure 23
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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.

 

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.


Figure 24
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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).

 

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).


Figure 25
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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.

 

Figure 26
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Fig. 15A 46-year-old man undergoing evaluation for pulmonary embolism. Axial CT image shows marked enlargement of pulmonary artery (arrow) consistent with pulmonary arterial hypertension.

 

Figure 27
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Fig. 15B 46-year-old man undergoing evaluation for pulmonary embolism. Axial maximum-intensity-projection image shows large ostium secundum atrial septal defect (arrow).

 

Figure 28
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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.

 

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.


Figure 29
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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.

 


Figure 30
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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)

 


Figure 31
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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.

 


Figure 32
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Fig. 18B 62-year-old woman with chest pain. Volume-rendered image shows nonmalignant course of anomalous right coronary artery (arrows) 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
Top
Abstract
Introduction
Known CHD in Adults
Undiagnosed CHD in Adults
Conclusion
References
 
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.


References
Top
Abstract
Introduction
Known CHD in Adults
Undiagnosed CHD in Adults
Conclusion
References
 

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