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(Circulation. 1995;92:3163-3171.)
© 1995 American Heart Association, Inc.

Articles

Magnetic Resonance Angiography of Anomalous Coronary Arteries

A New Gold Standard for Delineating the Proximal Course?

Johannes C. Post, MD; Albert C. van Rossum, MD, PhD; Jean G.F. Bronzwaer, MD; Carel C. de Cock, MD, PhD; Mark B.M. Hofman, PhD; Jacob Valk, MD, PhD; Cees A. Visser, MD, PhD

From the Departments of Cardiology (J.C.P., A.C.v.R., J.G.F.B., C.C.d.C., C.A.V.), Clinical Physics and Engineering (M.B.M.H.), and Radiology (J.V.), Free University Hospital and the Institute for Cardiovascular Research of the Free University, Amsterdam/Interuniversity Cardiology Institute of the Netherlands, Utrecht.

	Abstract

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Background The clinical significance of anomalously originating coronary arteries depends on their proximal course. Diagnosis of this course by conventional x-ray coronary angiography alone may be equivocal. We postulated that with fast magnetic resonance (MR) angiography, accurate detection of anomalous coronary arteries and unambiguous delineation of their proximal course is feasible.

Methods and Results In a selected group of 38 patients, 19 of them having an anomalously originating coronary artery, a fast MR angiographic technique was used to study the proximal coronary anatomy. Blinded analysis of randomly ordered MR studies was performed independently by two observers. Both origin and proximal course of the coronary arteries were defined. Two cardiologists reviewed all x-ray coronary angiograms. After the separate analyses, a final consensus result was defined for each patient. In 37 patients, successful MR coronary angiography could be performed. Interobserver agreement for determining both origin and proximal course was 100%. An x-ray coronary angiogram was available in 36 patients. In 3 patients (all with an anomalous left main coronary artery originating from the right aortic sinus), there was disagreement about the proximal course between the results of MR and x-ray coronary angiography. Review of these cases demonstrated that MR angiography had unambiguously visualized the proximal coronary artery course, whereas the results of x-ray angiography had been equivocal. Thus, sensitivity and specificity for detecting anomalous coronary arteries and delineating their proximal course were 100%.

Conclusions These data suggest that fast MR angiography is highly accurate in determining the origin and delineating the proximal course of anomalous coronary arteries, even in those cases in which x-ray coronary angiographic diagnosis is difficult or even erroneous.

Key Words: angiography • magnetic resonance imaging • arteries • coronary disease • heart defects, congenital

	Introduction

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An anomalous origin and proximal course of the coronary arteries may be associated with myocardial infarction and sudden death. Major anomalies are found in 0.3% to 0.8% of the population.^{1 2 3 4} Not all anomalies will have sequelae that serious, however. In particular, the pattern with proximal coronary artery segments crossing between the aorta and the pulmonary trunk has been reported to be potentially lethal.^{5 6 7 8 9 10 11} With the current standard technique for the assessment of coronary artery anatomy, ie, conventional x-ray coronary angiography, the exact proximal course of anomalous coronary arteries may sometimes be difficult to determine.^{7 8 12 13 14 15} Misdiagnosis has been reported to occur in up to 50% of patients.¹³ In particular, differentiation between an increased-risk interarterial and a low-risk septal course of an anomalous artery has been reported to be often incorrect.^{13 14}

MR coronary angiography is a novel noninvasive technique that has proved to be accurate in the imaging of proximal coronary anatomy in patients.^{16 17 18} The free choice of the imaging plane is an advantage over the limited possibilities of angulation in conventional coronary angiography. Also, MR angiography is a tomographic technique that is potentially better in elucidating three-dimensional anatomy than a projection technique. Both features might be of particular value in the imaging of anomalous proximal coronary anatomy. Its ability to demonstrate anomalously arising coronary arteries has been reported in only a few cases.^{19 20 21 22} In all but one, conventional "black-blood" spin-echo techniques were used, in contrast to newer "bright-blood" gradient-echo angiographic techniques, which are superior for coronary imaging. We performed a blinded study in which the results of a fast gradient-echo technique, breathhold two-dimensional MR angiography, were compared with those of conventional x-ray contrast angiography in the demonstration of anomalous coronary anatomy. Given the earlier reported angiographic capabilities of the technique and the particular advantages of MR imaging techniques mentioned above, we postulated that MR angiography would be capable of depicting anomalous coronary anatomy and delineating precisely the proximal course of anomalous arteries.

	Methods

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Patients
Patients to be included in the study were recruited from the files of outpatient clinics within the area of Amsterdam. Attending cardiologists were requested to refer patients known by a previous x-ray coronary angiographic study to have an anomalously originating coronary artery to our department for an MR angiographic study, without disclosing the anomaly in the referral form. Furthermore, for each patient with anomalous anatomy, another patient with known normal coronary anatomy was included. An anomalously originating artery was defined as an artery arising from another or from above another than its normal aortic sinus of origin. A separate origin of the LAD and LCx from the left aortic sinus (absence of an LMCA) was not labeled as an anomaly in this study. Exclusion criteria were other congenital cardiac anomalies, atrial fibrillation or marked ventricular ectopy, indwelling pacemaker, presence of intracranial vascular clips, and severe claustrophobia. The investigators performing and analyzing the MR studies were blinded to the results of conventional angiography. Thus, a total of 38 patients were studied, 30 men and 8 women. Mean age was 57±10 years (range, 34 to 74 years). Of this group, 19 patients (50%) had been judged by previous x-ray coronary angiography to have anomalous coronary anatomy. All studies were performed in accordance with guidelines of the hospital Committee on Medical Ethics and Clinical Investigations. Informed consent was obtained from all patients participating in the study.

Two-dimensional MR Angiography
Imaging was performed on a whole-body imaging system (Magnetom SP, Siemens Medical Systems) operating at 1.5 T. A standard circularly polarized radiofrequency receiver coil was used. Patients were positioned prone with their chests against the surface coil. Signal acquisition was ECG triggered and gated to mid diastole. A flow-compensated gradient-echo sequence with incrementing excitation flip angle (=22° to 90°) and k-space segmentation was used, with an echo time of 7.0 ms and a repetition time of 12.6 ms. The principles of this technique have been described.²³ The field of view was 260x260 mm², and matrix size was 144x256, resulting in an in-plane spatial resolution of 1.8x1.0 mm². Slice thickness was 4 mm. Nine phase-encoding steps per cardiac cycle were obtained, resulting in a scan time of 16 cardiac cycles per image. This was performed during a breathhold in end expiration. One fat-selective saturation pulse per cardiac cycle was applied before the imaging pulse train to suppress the strong signal from epicardial fat surrounding the coronary arteries.

Imaging Protocol
In all patients, a standard imaging protocol was used. First, a series of 1-mm overlapping parallel transverse images was obtained at the level of the aortic root, followed by a series of oblique images perpendicular to the transverse plane and oriented through the left and right atrioventricular grooves. We have previously shown that in this set of images, the proximal epicardial coronary arteries are accurately imaged.²⁴ Total imaging time was up to 45 minutes on average.

Image Analysis
All images were stored without patient data on optical disk. Studies were marked with a seven-digit number that was also assigned to the x-ray contrast angiography. After imaging of all patients had been completed, images were magnified, mounted in oscillating cine loops to optimize the apprehension of the continuity of coronary arteries, and recorded in random order on VHS videotape. Analysis of the MR studies from videotape was performed independently by two investigators. These observers were blinded to the results of conventional x-ray contrast angiography. Proximal coronary anatomy was classified as normal or anomalous. In case of anomalous anatomy, the anomaly was specified, describing (1) the aortic sinus from which the anomalous artery originated and (2) the proximal course of the artery in relation to the aorta and pulmonary trunk. After completion of independent analysis of all patients, a comparison of the results from both observers was made to discuss potential differences in opinion until consensus was reached.

X-ray Contrast Coronary Angiography
Conventional x-ray contrast coronary angiographic studies from all patients, performed by the Judkins or the Sones technique, were reviewed independently by two cardiologists from the cardiac catheterization laboratory who were blinded to the MR angiography findings. They described the origin and proximal course of all coronary arteries. After independent review, the results of both reviewers were compared, and any difference in opinion was discussed until a consensus about origin and proximal course of all coronary arteries was reached.

Comparison of MR Angiography and X-ray Contrast Angiography
After completion of the analyses, the consensus results of the different techniques were compared for the individual patients. In a final meeting of the Catheterization Laboratory cardiologists and the MR angiographers, incongruent assessments of coronary artery origin and proximal course by both techniques were reevaluated, and a final consensus result for each patient was defined.

	Results

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MR Angiography
One of the patients was not capable of holding her breath long enough for image data acquisition. For this reason, no successful MR angiographic study could be performed in this patient. In 37 patients, images of sufficient quality to be analyzed were obtained. The differentiation between anomalously originating and normally originating coronary arteries could be made independently by both observers in all studies. Both observers judged 18 of the patients to have normal coronary anatomy and 19 to have anomalous coronary anatomy. Interobserver agreement for differentiation of anomalous from normal anatomy was 100%.

For the 19 patients judged to have an anomalous origin of at least one of their coronary arteries, no difference in opinion existed between the two observers concerning either the origin or the proximal course of these anomalous arteries. Four patients were judged to have an anomalous origin of the LMCA from the right coronary sinus (either a common ostium with the RCA or a separate ostium), 2 taking a retroaortic course to the left (Fig 1), 1 an interarterial course between the aorta and pulmonary trunk (Fig 2), and 1 an anterior free wall course over the right ventricular outflow tract. In 11 patients, an anomalous origin of the LCx from the right aortic sinus was detected, with a retroaortic course of the anomalous artery in all patients. An LAD arising anomalously from the right aortic sinus, taking a septal course to the interventricular groove, was found in 1 patient (Fig 3). In this patient, the LCx also arose anomalously from the right aortic sinus (taking a retroaortic course). In 4 patients, an anomalous RCA with an origin adjacent to the commissure between the right and left coronary cusps was found, all taking an interarterial course to the right atrioventricular groove (Fig 4). The results for the 19 patients judged by MR angiography to have anomalous coronary anatomy are summarized in the Table.

View larger version (163K): [in this window] [in a new window]   Figure 1. A, Conventional x-ray coronary angiography showing an LMCA (arrow) anomalously originating from the right aortic sinus. B and C are from the oblique series of MR angiographic images. B shows the origin of the anomalous LMCA (arrow) from the proximal right coronary artery (arrowhead). In C, which is posterior to B, the LMCA (arrow) is seen to pass caudally and posteriorly from the aortic root to the left. A middle segment of the RCA (arrowhead) is also seen in this section. Transverse image D is pathognomonic for all left coronary arteries originating from the right aortic sinus and taking a retroaortic course to the left: just caudal and posterior to the origin of the aorta from the left ventricle, a coronary segment is seen to cross over. Ao indicates aorta; c, catheter; LA, left atrium; LV, left ventricle; RA, right atrium; and RV, right ventricle.

View larger version (222K): [in this window] [in a new window]   Figure 2. A, Conventional x-ray coronary angiography showing an anomalous LMCA originating from the right aortic sinus. B, Transverse MR image depicting the LMCA (arrows) arising together with the RCA (arrowhead) from the right aortic sinus. The LMCA runs between the aorta and pulmonary trunk to the left, where it bifurcates into an LAD and LCx. C is perpendicular to B, along the main axis of the interarterial segment, and depicts this interarterial segment (arrows) in an oblique orientation. Ao indicates aorta; c, catheter; LV, left ventricle; PT, pulmonary trunk; and RV, right ventricle.

View larger version (116K): [in this window] [in a new window]   Figure 3. Facing page. Image A, from the conventional x-ray coronary angiography, shows an anomalous LAD, originating from the right aortic sinus. B through F are from a series of contiguous MR sections visualizing the anomalous LAD (arrow) arising with the RCA (arrowhead) from the right aortic sinus (B) and subsequently descending and entering the interventricular myocardial septum (D), where it runs through the septum (E) to reappear in the interventricular groove (F). Apprehension of the contiguity was optimized by mounting the images in a cine loop. Ao indicates aorta; c, catheter; LV, left ventricle; RV, right ventricle; and RVOT, right ventricular outflow tract.

View larger version (153K): [in this window] [in a new window]   Figure 4. Image A, from the conventional x-ray coronary angiography, shows an RCA (arrow) anomalously originating from (above) the left aortic sinus. B through D illustrate the proximal RCA (arrow) originating from above the left aortic sinus adjacent to the commissure and running between the aorta and pulmonary trunk to the right atrioventricular groove. Ao indicates aorta; c, catheter; and PT, pulmonary trunk.

View this table: [in this window] [in a new window]   Table 1. Angiographic Characteristics of the 19 Patients Judged to Have Anomalous Coronary Anatomy

X-ray Angiography
From one of the patients included, the original x-ray coronary angiogram could not be traced by the referring cardiologist. Furthermore, the two independent observers reviewing all x-ray coronary angiograms both judged that the coronary angiogram of 1 patient could not be analyzed because it was an incomplete study. Of the remaining 36 films, observer A judged 19 angiograms to show normal coronary anatomy and 17 to show anomalously arising coronary arteries, whereas observer B judged 20 to show normal and 16 to show anomalous anatomy. Interobserver agreement for differentiating anomalous from normal coronary anatomy was 97%.

In 5 of the patients, there was difference in opinion about the site of origin of an (anomalous) artery. In 4 of these 5 patients, the disagreement consisted of one of the observers having labeled the origin of an anomalous artery as "unclear," whereas the other had specified an anomalous origin (in 3 patients, an anomalous origin of the RCA from or from above the left sinus and in 1 patient, an anomalous origin of the LCx from the right aortic sinus). In the remaining patient, observer B judged the proximal coronary anatomy to be normal, whereas observer A judged an anomalous origin of the RCA from or from above the left sinus to be present (patient 31).

In 6 patients, a difference in opinion existed about the proximal course of the anomalous artery. Again, 3 of these 6 differences consisted of one of the observers labeling the course as "unclear." In 1, the incongruence was caused by one observer labeling the coronary artery as normal while the other did not (patient 31). In the remaining 2 patients, there was a true difference in opinion concerning the proximal course of the anomalous artery: In patient 5, observer A defined the course of an anomalous LMCA from the right aortic sinus as interarterial, whereas observer B judged the artery to take an anterior free wall course over the right ventricular outflow tract to the left. In patient 28, observer A judged an anomalous LMCA from the right sinus to take a proximal course through the septum, whereas observer B described the course as retroaortic.

In a second joint session, the angiograms that had been classified differently were revised by both observers together, and a consensus could be reached in all cases. The consensus results for the individual patients judged to have anomalous coronary anatomy are listed in the Table.

Comparison Between X-ray Angiography and MR Angiography
To reach consensus about the definite origin and course of all coronary arteries, a final joint session of catheterization laboratory cardiologists and MR angiographers was organized. A consensus was obtained for all patients.

There was 100% agreement between x-ray angiography and MR angiography in differentiating patients with anomalous coronary anatomy from patients with normal anatomy. Also, no differences existed between the consensus results of MR angiography and x-ray angiography concerning the origin of the anomalous coronary arteries.

In 3 patients, however, differences of opinion existed about the proximal course of an anomalous artery. In patient 5, the consensus result of x-ray angiography was an anomalous LMCA from the right aortic sinus, taking an interarterial course between the aorta and pulmonary trunk. MR angiography demonstrated the anomalous LMCA to cross over the pulmonary trunk to the left, subsequently bifurcating into an LAD and an LCx. In patient 25, the proximal course of the anomalous LAD was defined as interarterial by x-ray angiography, whereas a septal course of this artery to the interventricular groove was demonstrated by MR angiography (Fig 3). A similar disagreement existed in patient 28, in whom the course of the anomalous LMCA was judged to be septal by x-ray angiography, whereas an interarterial course between the aorta and pulmonary trunk was depicted by MR angiography (Fig 2). After a joint review of these cases, it was unanimously decided that MR angiography had unambiguously delineated the proximal course of these anomalous arteries, whereas on conventional x-ray angiograms, interpretation of this course had been difficult and erroneous.

In the patient from whom the x-ray angiography could not be traced by the referring cardiologist (patient 11), MR angiography showed an anomalous origin of the LCx from the right aortic sinus, taking a retroaortic course. In patient 24, whose x-ray angiography was judged to be not analyzable because only the LAD had been visualized and no selective injection of the RCA had been performed, MR angiography showed an anomalous LCx arising together with the RCA from a common ostium posteriorly in the right aortic sinus. In the patient in whom no MR angiography could be performed because she was unable to hold her breath, x-ray angiography showed normal coronary anatomy.

	Discussion

Top Abstract Introduction Methods Results Discussion References

 
In this study in a selected patient group with high prevalence of anomalous coronary arteries, the value of a fast MR angiographic technique for the detection of anomalously originating coronary arteries and its accuracy in delineating their proximal course in relation to the great arteries were demonstrated. Transverse and oblique sections depicted the proximal coronary artery anatomy in nearly all patients. Especially on the transverse images, the precise origin of an anomalous artery and its relation to the aorta and the pulmonary trunk were clearly visualized. The high interobserver agreement illustrates the unambiguity of the images obtained. The results in this patient group suggest that MR coronary angiography is capable of delineating the proximal course of anomalous arteries even in cases in which x-ray coronary angiographic diagnosis is difficult or even erroneous.

Previous Work
The few previous reports on the capability of MR imaging techniques to demonstrate anomalously originating coronary arteries have documented only incidental cases as yet. In all but one, conventional "black-blood" spin-echo techniques were used.^{19 20 21 22} Other authors have reported the imaging of coronary artery aneurysms^{25 26} and fistulas^{26 27 28} with MR techniques.

After the introduction of gradient-echo techniques for coronary artery imaging in 1991, a rapid further development of these "bright-blood" MR coronary angiographic techniques occurred.^{16 23 29 30 31 32 33 34 35 36} In this study, an MR coronary angiographic technique that was shown earlier to be successful in patients¹⁶ was used to demonstrate aberrant coronary arteries.

Clinical Value
The proximal course of an aberrant coronary artery is of major importance in defining the associated risk of myocardial infarction or sudden cardiac death.^{5 6 7 8 9 10 11 12} Thus, unambiguous visualization of this course is important. The increased-risk interarterial course must be discriminated from the rather innocent septal, retroaortic, and anterior free wall courses in any patient with a coronary anomaly. However, delineating the proximal course of an anomalous coronary artery from a conventional x-ray coronary angiogram may be difficult,^{7 8 12 13 14 15} and misdiagnosis has been reported to occur in up to 50% of patients.¹³ In the present study, this was illustrated again: delineation of the proximal course of an anomalous artery was erroneous in 3 of 19 patients (16%).

MR angiography seems to be a highly accurate, noninvasive diagnostic tool in patients with or suspected of having anomalous coronary anatomy. Its indications may include (1) patients who have undergone conventional x-ray contrast coronary angiography and in whom uncertainty exists concerning the precise delineation of the course of an aberrant coronary artery; (2) investigation of patients in whom total proximal occlusion or congenital absence of a major epicardial coronary artery is suspected but anomalous origin of the artery cannot be excluded; (3) a primary investigation in adolescent and young patients who present with angina, arrhythmias, or syncope on severe exercise; (4) workup before cardiac surgery of patients with an uncertain course of an anomalous coronary artery to avoid the risk of trauma to the aberrant artery^{37 38} ; and (5) the screening of certain subgroups of individuals who, because of the presence of an interarterially coursing aberrant coronary artery, run an especially increased mortality risk, eg, highly competitive athletes.³⁹

Comparison With Other Noninvasive Imaging Techniques
Other noninvasive or semi-invasive techniques reported to be capable of imaging anomalous coronary arteries are echocardiography and ultrafast computed tomography.

The use of transthoracic two-dimensional echocardiography seems to be limited,^{15 40 41 42 43} although in pediatric populations the results have been better.^{44 45 46} Transesophageal echocardiography seems much more sensitive in diagnosing anomalous coronary arteries and delineating their course,^{15 42 43} but it is semi-invasive.

A single case of anomalous left coronary artery demonstrated by electron-beam computed tomography has recently been reported.⁴⁷ Drawbacks of this technique include the still necessary intravenous infusion of contrast agent and exposure to ionizing radiation. Only transverse images can be obtained.

Limitations
Study design. Because the vast majority of coronary artery anomalies are found accidentally during coronary angiography and because the incidence of coronary anomalies is low, we intended to design a study protocol that guaranteed the inclusion of sufficient coronary anomalies and at the same time complied with the requirements of a blinded study. Although the pretest likelihood of encountering anomalous anatomy in this patient group was of course far higher than in normal clinical practice, we believe that this fact has not weakened the main findings of this study.

In the diagnosis of anomalous coronary anatomy, the ultimate gold standard is pathology. This, of course, was not attainable. Instead, the consensus of both catheterization laboratory cardiologists and MR angiographers, after review and comparison of a patient's conventional x-ray angiography and MR angiography, was considered to be the standard in this study.

Rhythm disturbances. One of the limitations of MR angiography is the dependence on a regular heart rhythm. All patients in our study had sinus rhythm. It can be expected that the ability of MR angiography to delineate an anomalous course in patients with atrial fibrillation will be worse because of the degrading of image quality that will occur.

Breathholding. The success of fast gradient-echo MR coronary angiography in depicting the coronary arteries depends on the capability of the patient to hold his or her breath for the period of 16 cardiac cycles (with the technique we used). Only 1 patient was not able to hold her breath, which resulted in severe respiratory motion artifacts that precluded visualization of the coronary arteries. A coarser matrix or more phase-encoding steps per RR interval may be used to shorten the period of breathholding.

Contraindications. MR imaging is contraindicated in patients with pacemakers, intracranial surgical clips, or intraocular metal debris. A few patients experience claustrophobic reactions inside the MR system, which may preclude MR imaging.

Conclusions
Delineating the proximal course of anomalous coronary arteries from a conventional x-ray angiogram may sometimes be difficult or even erroneous. There is a need for a noninvasive diagnostic technique capable of unequivocally defining this proximal course. In this study, fast gradient-echo MR angiography proved to be very accurate in detecting the anomalies and delineating the proximal course. MR angiography appears to be a very useful adjunct to conventional coronary angiography and, with confirmation of these results in larger series, might even be considered a new gold standard in the imaging of the proximal course of anomalous coronary arteries.

	Selected Abbreviations and Acronyms

 

LAD = left anterior descending coronary artery LCx = left circumflex coronary artery LMCA = left main coronary artery MR = magnetic resonance RCA = right coronary artery

	Acknowledgments

 
The authors thank the following colleagues for referring patients to be included in this study: S.C. Baldew, Spijkenisse; G.J. van Beek, Spijkenisse; M. Bijl, Assen; J.J.M. Kainama, Ymuiden; B.L.E. Lutterman, Amsterdam; J.J. Piek, Amsterdam; T.B. Tan, Amsterdam; J.A. Verheul, Almere; P.M.J. Verhorst, Amstelveen; A. Vermeulen, Amsterdam; and J. Visser, Amsterdam.

	Footnotes

 
Reprint requests to Johannes C. Post, MD, Department of Cardiology, Free University Hospital, De Boelelaan 1117, 1081 HV Amsterdam, Netherlands.

Received July 31, 1995; revision received September 28, 1995; accepted October 1, 1995.

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