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AJR 2005; 184:S28-S32
© American Roentgen Ray Society


Case Report

Demonstration of Complex Coronary-Pulmonary Artery Fistula by MDCT and Correlation with Coronary Angiography

Monica S. M. Chan1,2, Ivan Y. F. Chan1, K. H. Fung1, Gilbert Lee1, K. L. Tsui3 and T. C. Leung3

1 Department of Radiology, Pamela Youde Nethersole Eastern Hospital, Hong Kong SAR, China.
2 Present address: Department of Diagnostic Radiology and Organ Imaging, The Chinese University of Hong Kong, Prince of Wales Hospital, 30-32 Ngan Shing St., Shatin NT, Hong Kong SAR, China.
3 Department of Medicine, Pamela Youde Nethersole Eastern Hospital, Hong Kong SAR, China.

Received April 18, 2004; accepted after revision June 22, 2004.

 
Address correspondence to M. S. M. Chan (drmonicachan{at}hotmail.com).


Introduction
Top
Introduction
Case Report
Discussion
References
 
Coronary artery anomalies are rare, with an incidence of 0.2% to 1.2% [1], among which coronary-pulmonary artery fistula is usually detected in 0.1% to 0.2% of coronary angiograms [2-4]. Although not all coronary-pulmonary artery fistulas are clinically or hemodynamically significant, some can result in serious consequences including myocardial ischemia, myocardial infarction, or sudden death [5]. When complex anatomy or intervention is contemplated, coronary angiography may not be sufficient. An ideal investigation technique should be noninvasive and provide a quality anatomic description of the fistula.

We report a case of complex coronary-pulmonary artery fistula with two feeding vessels of separate origins: one from the left coronary artery via the left anterior descending artery and another arising from the right coronary sinus. The complex anatomy of the fistula was demonstrated in detail by an MDCT scanner using multiplanar reconstruction and different 3D reconstruction techniques.


Case Report
Top
Introduction
Case Report
Discussion
References
 
A 60-year-old woman was referred to our hospital for management of heart failure. She had a history of hypertension and heart murmur, the latter of which had not been investigated. Chest radiography showed moderate cardiomegaly. ECG showed persistent T wave inversion over V5 and V6 leads but the creatine kinase level was normal.

Transthoracic and transesophageal echocardiography were performed and showed findings suspicious for coronary-pulmonary artery fistula. A coronary angiogram was obtained that confirmed coronary fistula draining into pulmonary trunk (Figs. 1A and 1B). Despite various projections, the exact anatomic course of the suspected fistula could not be clearly shown by the coronary angiogram.



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Fig. 1A. 60-year-old woman presenting with heart failure and heart murmur. White arrowhead = origin of the anonymous vessel, black arrowhead = origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior vena cava, RA = right atrium, RCA = right coronary artery, MPA = main pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus of fine vessels along the surface of the root and proximal ascending aorta and along the left atrioventricular groove, 1 = C-shaped main draining vessel to the fistula, 2 = anonymous vessel arising from right coronary sinus. Left anteroposterior projection of left coronary angiogram shows an abnormal complex vascular structure composed of a plexus of fine tortuous vessels (open arrow) arising from the proximal left anterior descending artery (LAD) and a main draining vessel (1). Abnormal early opacification of main pulmonary artery (MPA) was present, suggestive of a coronary pulmonary artery fistula (F). Left main coronary artery (LCA) and LAD were dilated while the left circumflex artery (CFA) was of normal caliber.

 


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Fig. 1B. 60-year-old woman presenting with heart failure and heart murmur. White arrowhead = origin of the anonymous vessel, black arrowhead = origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior vena cava, RA = right atrium, RCA = right coronary artery, MPA = main pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus of fine vessels along the surface of the root and proximal ascending aorta and along the left atrioventricular groove, 1 = C-shaped main draining vessel to the fistula, 2 = anonymous vessel arising from right coronary sinus. Right anterolateral projection with caudal tilting of the left coronary angiogram has similar findings as in A but failed to resolve the complex anatomy. On this projection, an abnormally dilated branch of left anterior descending artery (asterisk) is seen.

 

Contrast-enhanced CT of the heart and great vessels, including coronary arteries, was performed in an attempt to demonstrate the course of the coronary-pulmonary artery fistula.

CT coronary angiography was performed using a 16-slice MDCT scanner (Aquilion TSX 101A M16, Toshiba). Imaging parameters of 120 kV, 250 mAs, and 0.5-mm slice collimation were preset for the scan. Given the patient's heart rate was between 65-70 beats per minute and was electrocardiogram gated (ECG gated), the CT scanner could automatically optimize the scanning parameters for the examination. In this case, gantry rotation speed at 0.4 sec per revolution and a helical pitch of 3.2 were applied. A multisegment reconstruction algorithm was also selected automatically, which effectively improved the temporal resolution to 116 msec from the 200 msec, if a half-reconstruction algorithm was to be used.

The examination was performed using a single breath-hold technique to cover 120 mm from the cardiac outflow tract to the apex of heart within a total scanning time of about 30 sec. A total of 120 mL nonionic water-soluble iodinated contrast medium at 370 mg I/mL concentration followed by 30 mL of normal saline IV was administered at a rate of 4 mL/sec. An automated contrast medium tracing program was applied to trigger the scan when the attenuation at the ascending aorta reached 180 H. Retrospective ECG-gated multisegment reconstruction was performed at a 0.4-mm interval (i.e., 20% overlapping), with a predefined temporal offset at 70% R-R wave interval (at diastole) of each cardiac cycle to demonstrate the anomaly, using multiplanar reconstruction and different 3D reconstruction techniques. Postexamination image processings were performed via a commercial workstation using 3D visualization software (Vitrea 2, version 3.4.5, Vital Images). Reconstruction at every 10% of R-R intervals of cardiac cycles allowed cardiac function analysis to be performed using the same set of image data.

The anatomy of the coronary artery-pulmonary artery fistula was complex but was well demonstrated after detailed analysis. The aortic and coronary sinuses were well shown with segmentation techniques and interactive sectioning on the 3D image to remove the overlying right and left atria. The left main coronary artery arising from the left coronary sinus was dilated, measuring up to 12 mm in caliber. The dilated left coronary artery immediately branched into the left anterior descending and left circumflex arteries. The left circumflex artery was of normal caliber and ran along the left atrioventricular groove. The left anterior descending artery passed posteroinferior to the left main pulmonary artery, running along the interventricular grove to emerge at the cardiac surface to the left of the main pulmonary artery, where it gave rise to a few branches (Figs. 1C and 1D). The continuation of the left anterior descending artery ran along the interventricular groove and became abruptly terminated at the mid interventricular groove (Figs. 1E and 1F). A plexus of fine tortuous vessels arose from the proximal left anterior descending artery and its side branches at the level of the atrioventricular groove. This plexus formed a network that encircled the left anterior descending artery and eventually coalesced to form a major tortuous dilated draining vessel that ran horizontally across the anterior aspect at the base of the main pulmonary artery (Figs. 1E and 1F). This large draining vessel in turn ran superiorly on the right side of the main pulmonary artery, where it formed an irregular C-shaped tubular structure, measuring 7.3 mm, on the surface of the root of the main pulmonary artery (Fig. 1E). Finally, it ended by joining to a 20-mm bulbous dilatation over the anterior aspect at the base of the main pulmonary artery before draining into the main pulmonary artery (Figs. 1E, 1F and 1G).



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Fig. 1C. 60-year-old woman presenting with heart failure and heart murmur. White arrowhead = origin of the anonymous vessel, black arrowhead = origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior vena cava, RA = right atrium, RCA = right coronary artery, MPA = main pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus of fine vessels along the surface of the root and proximal ascending aorta and along the left atrioventricular groove, 1 = C-shaped main draining vessel to the fistula, 2 = anonymous vessel arising from right coronary sinus. CT coronary angiogram by 2D normal rendering thick-slab oblique axial reconstruction shows the dilated left main coronary artery, which immediately branched into left anterior descending (LAD) and left circumflex arteries. A plexus of fine tortuous vessels (open arrow) arises from the proximal LAD and its side branches (asterisk) at the level of atrioventricular groove. A smaller anonymous vessel (2) arises from the left side of the right coronary sinus, another feeding vessel of the coronary-pulmonary artery fistula. AA = ascending aorta, DA = descending aorta, SVC = superior vena cava, LA = left atrium, RA = right atrium, RCA = right coronary artery, MPA = main pulmonary artery.

 


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Fig. 1D. 60-year-old woman presenting with heart failure and heart murmur. White arrowhead = origin of the anonymous vessel, black arrowhead = origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior vena cava, RA = right atrium, RCA = right coronary artery, MPA = main pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus of fine vessels along the surface of the root and proximal ascending aorta and along the left atrioventricular groove, 1 = C-shaped main draining vessel to the fistula, 2 = anonymous vessel arising from right coronary sinus. CT coronary angiogram by 2D normal rendering thick-slab oblique axial reconstruction at a more cranial level to C shows exact site of fistula draining into the main pulmonary artery (MPA).

 


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Fig. 1E. 60-year-old woman presenting with heart failure and heart murmur. White arrowhead = origin of the anonymous vessel, black arrowhead = origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior vena cava, RA = right atrium, RCA = right coronary artery, MPA = main pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus of fine vessels along the surface of the root and proximal ascending aorta and along the left atrioventricular groove, 1 = C-shaped main draining vessel to the fistula, 2 = anonymous vessel arising from right coronary sinus. Right anterolateral projection of the heart by 3D tissue tone volume rendering shows the surface anatomy of the complex coronary pulmonary artery fistula and its relationship with the adjacent structures. The left anterior descending artery (LAD) continues to run along the interventricular groove and is abruptly terminated at the mid interventricular groove. A plexus of fine tortuous vessels (open arrow) arises from the proximal LAD, and its side branches at the level of the atrioventricular groove form a network encircling the LAD and eventually coalesce to become a major tortuous dilated draining vasculature (1), running horizontally across the anterior aspect at base of the main pulmonary artery (MPA). Note the irregular C-shaped tubular structure formed by the draining vasculature (1) on the surface of the root of the main pulmonary artery (MPA). Similar plexus of fine tortuous vessels (open arrow) supplied by the anonymous vessel arising from the right coronary sinus (2) is found along the surface of the ascending aorta, arch of aorta, and MPA. The anonymous vessel arising from the right coronary sinus had separate origin from the right coronary artery (RCA). AA = ascending aorta, LA = left atrium, RV = right ventricle, LV = left ventricle, LPA = left pulmonary artery, F = coronary pulmonary artery fistula.

 


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Fig. 1F. 60-year-old woman presenting with heart failure and heart murmur. White arrowhead = origin of the anonymous vessel, black arrowhead = origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior vena cava, RA = right atrium, RCA = right coronary artery, MPA = main pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus of fine vessels along the surface of the root and proximal ascending aorta and along the left atrioventricular groove, 1 = C-shaped main draining vessel to the fistula, 2 = anonymous vessel arising from right coronary sinus. Left anteroposterior projection of the heart by 3D tissue tone volume rendering shows clearly the nondilated right coronary artery and the anonymous vessel (2) arising from the right coronary sinus. AA = ascending aorta, SVC = superior vena cava, RA = right atrium, RCA = right coronary artery, MPA = main pulmonary artery, F = coronary pulmonary artery fistula, arrow = plexus of fine vessels along the surface of the root and proximal ascending aorta, RV = right ventricle, LV = left ventricle.

 


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Fig. 1G. 60-year-old woman presenting with heart failure and heart murmur. White arrowhead = origin of the anonymous vessel, black arrowhead = origin of RCA, AA = ascending aorta, DA = descending aorta, SVC = superior vena cava, RA = right atrium, RCA = right coronary artery, MPA = main pulmonary artery, F = coronary pulmonary artery fistula, open arrows = plexus of fine vessels along the surface of the root and proximal ascending aorta and along the left atrioventricular groove, 1 = C-shaped main draining vessel to the fistula, 2 = anonymous vessel arising from right coronary sinus. CT coronary angiogram by 3D tissue tone volume rendering with oblique trimming shows clearly the exact drainage of fistula (F) into the main pulmonary artery (MPA). The anonymous vessel (2) is seen arising from the dilated right coronary sinus (RCS), with a separate origin from the right coronary artery (RCA).

 

Bulbous dilatation of the right coronary sinus was present (Fig. 1G). The right coronary artery arose from the right side of the coronary sinus and was of normal caliber (Figs. 1F and 1G). It was seen running along the right atrioventricular groove. Another smaller anonymous vessel was found arising from the left side of the right coronary sinus, which was another feeding vessel of the ultimate coronary-pulmonary artery fistula. It ran superiorly in a straight path for 15 mm, becoming a tortuous loop before forming another plexus of fine vessels along the surface of the ascending and arch of aorta and the main pulmonary artery. This plexus acted as a second supply to the fistula that eventually drained into the ultimate coronary-pulmonary artery fistula (Figs. 1F and 1G). This additional supply to the fistula was not appreciated in the coronary angiogram because of selective cannulation of left and right coronary arteries only.

Moderate dilatation of the left atrium and left ventricle was noted. CT of the thorax showed dilated pulmonary vasculatures consistent with congestive heart failure.

The ejection fraction was estimated to be 78.3% by cardiac functional analysis, comparable with that found on conventional coronary angiography (76%).


Discussion
Top
Introduction
Case Report
Discussion
References
 
Until recently, conventional coronary angiography was the diagnostic method of choice for detecting coronary anomalies. However, it is invasive and has a 0.15% mortality rate and 1.5% morbidity rate [6]. The diagnostic value of coronary angiography is limited by its planar imaging nature, restricted angle of angiographic projections, and concern for the contrast load. For complex pathology as presented in our case, conventional coronary angiography sometimes is inadequate for a clear demonstration of the exact anatomy.

Other noninvasive imaging techniques, including MRI and contrast-enhanced electron beam tomography (EBT), were introduced in the past few years and have proven to be reliable [7]. Their results are comparable to those obtained by conventional coronary angiography. However, both MRI and EBT have their limitations.

Although MRI can demonstrate a lesion in multiple planes, the temporal resolution only reaches 100-150 msec [8]. Hence, its use is limited, especially in patients with tachycardia or arrhythmia in whom significant motion artifacts occurred and only poor image quality of limited diagnostic value could be obtained. EBT has a temporal resolution of 100 msec [9] and is capable of demonstrating the lesion in multiple planes; however, its clinical application is limited by its availability and its limited image quality.

Because of its advancements, MDCT is an emerging practical method for the investigation of coronary artery disease. With the availability of 16-detector row MDCT, which improves both in its temporal and spatial resolutions, it is now possible and practical to have high-quality cardiac imaging. Although MDCT remains inferior in temporal resolution to conventional coronary angiography (40 msec), its spatial resolution is superior to that of MRI and EBT [9]. The high-contrast resolution of 16-detector row MDCT when using a 0.5-mm acquisition can achieve a spatial resolution of 0.35 ± 0.05 mm at all planes [10]. This spatial resolution is approaching that of conventional coronary angiography. The radiation dose from 16-detector row MDCT is similar to that of uncomplicated conventional coronary angiography [8]. Using MDCT, the data are collected after a single shot of contrast injection. As the spatial resolution of data collected is isotropic, images can be reviewed by multiplanar reconstruction and 3D reconstruction techniques including surface shaded display, volume rendering, and maximum intensity projection. The ultrafast rate of the machine allows data acquisition at different phases of the cardiac cycles, reducing the pulsation motion artifacts of normal cardiac cycles significantly.

The 3D reconstruction with viewing at an unlimited angle allows us to demonstrate a lesion such as a fistula at its best projection, without subjecting the patient to repeated radiation exposure and an additional contrast load, and makes assessment of the size and exact location of the lesion feasible. This could be helpful for planning future cardiovascular intervention.

Apart from the qualitative analysis of the complex cardiac pathology, similar to conventional coronary angiography, MDCT cardiac examination allows quantitative cardiac function analysis.

The short investigation time (total scanning time as short as 30 sec), relative noninvasiveness of the procedure, simple preparation, and minimal aftercare make MDCT coronary angiography advantageous over conventional coronary angiography.

We believe that with its increasing availability and advances in technology, including data acquisition and postprocessing, coronary angiogram and cardiac investigation using noninvasive MDCT could be more practical and become the main method of choice for investigation of coronary artery anomalies and diseases.


References
Top
Introduction
Case Report
Discussion
References
 

  1. Dodge-Khatami A, Mavroudis C, Backer CL, et al. Congenital Heart Surgery Nomenclature and Database Project: anomalies of the coronary arteries. Ann Thorac Surg2000; 69[suppl 4]:S270 -S297[Abstract/Free Full Text]
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  3. Kugelmass AD, Manning WJ, Piana RN, et al. Coronary arteriovenous fistula presenting as congestive heart failure. Cathet Cardiovasc Diagn 1992;26:19 -25[Medline]
  4. Angelini P. Normal and anomalous coronary arteries: definitions and classification. Am Heart J1989; 117:418 -434[Medline]
  5. Levin DC, Fellows KE, Abrams HL. Hemodynamically significant primary anomalies of the coronary arteries: angiographic aspects. Circulation1978; 58:25 -34[Abstract/Free Full Text]
  6. Achenbach S, Ulzheimer S, Baum U, et al. Noninvasive coronary angiography by retrospectively ECG-gated multislice spiral CT. Circulation2000; 102:2823 -2828[Abstract/Free Full Text]
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  8. Pannu HK, Flohr TG, Corl FM, Fishman EK. Current concepts in multi-detector row CT evaluation of the coronary arteries: principles, techniques, and anatomy. RadioGraphics2003; 23[spec no]:S111 -S125
  9. International Task Force Symposium. Ropers D. EBT/MSCT for risk assessment and detection of stenotic coronary artery disease: clinical experience. Available at: www.chd-taskforce.de/pdf/2003_scuol_ropers_dieter.pdf. Accessed May 20, 2004
  10. Toshiba Medical Corporation Systems 2003. Multislice helical CT scanner Aquilion 16 CFX edition. Product data No. MPDCT 0231EAA. Available at: www.toshiba-medical.co.jp/tmd/english/products/ct/aquilion/sixteen/index.html. Accessed May 20, 2004

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