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Elektronischer Sonderdruck für A.Y. 5 Andreou Ein Service von Springer Medizin Herz 2012 · 37:10–14 · DOI 10.1007/s00059-011-3519-3 © Urban & Vogel 2011
A.Y. Andreou · I. Iakovou · A.K. Dimopoulos · G. Karatasakis · P. Anastasiou · I. Vasiliadis · G. Pavlides
Komplexe Koronararterienanatomie bei einem Patienten mit linksventrikulärem Myxomprolaps
Organ des Bundesverbandes Niedergelassener Kardiologen (BNK) www.bnk.de
Organ der Arbeitsgemeinschaft Leitende Kardiologische Krankenhausärzte e.V. www. alkk.de
Case study Herz 2012 · 37:342–346 DOI 10.1007/s00059-011-3519-3 Received: 13 April 2011 Accepted: 29 June 2011 Published online: 28 September 2011 © Urban & Vogel 2011
e-Herz
A 40-year-old asymptomatic man, cigarette smoker with a family history of premature coronary artery disease was found on echocardiography to have a 7.2×3.5 cm pedunculated left atrial mass suggestive of a myxoma (. Fig. 1). The mass was nonhomogeneous in appearance with lucent areas and lobulated. It was attached to the interatrial septum and protruded into the
A.Y. Andreou · I. Iakovou · A.K. Dimopoulos · G. Karatasakis · P. Anastasiou · I. Vasiliadis · G. Pavlides A’ Department of Cardiology, Onassis Cardiac Surgery Center, Athens
Complex coronary artery anatomy in a patient with prolapsing left atrial myxoma
left ventricle during diastole, resulting in mild obstruction of left ventricular filling. Preoperative coronary angiography using the right radial approach revealed absence of obstructive coronary artery disease and neovascularization of the mass from the right coronary artery (. Fig. 2a). Selective left coronary angiography revealed a left anterior de-
scending (LAD) artery that terminated high in the anterior interventricular sulcus (AIVS) after supplying septal branches to the proximal septum and two diagonal branches (. Fig. 2b, c). Neither the continuation of the LAD artery beyond the proximal AIVS nor the left circumflex (LCx) artery was visualized. The aortogram (. Fig. 2d) obtained in the left an-
Fig. 1 9 Tranthoracic echocardiography in the parasternal long-axis view (a), parasternal short-axis view at the level of the mitral valve (b), apical four-chamber view (c), and subxiphoid view (d) displaying a large left atrial (LA) myxoma (m) protruding into the left ventricle (LV) through the mitral valve orifice during diastole. The myxoma shows nonhomogeneous echotexture and contains lucent areas (arrows) corresponding to necrosis; it is attached to the interatrial septum (dashed arrow)
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Abstract · Zusammenfassung terior oblique projection to locate the ectopic vessels revealed only the LCx artery; it arose from the right aortic sinus adjacent to the ostium of the RCA and had a limited area of distribution for which reason no further attempts were made to selectively demonstrate it. It formed a caudal posterior loop to the left indicating a retroaortic course. Subsequently, with the use of a multipurpose 1 catheter, a second LAD artery was selectively demonstrated to arise from the right aortic sinus (. Fig. 2e). In a left anterior oblique projection, it traveled to the left forming a caudal anterior loop and after reaching the AIVS it coursed towards the apex; it supplied a septal branch as its first branch. These two angiographic features indicated a subpulmonary or intraseptal course (i.e., through the superior aspect of the crista supraventricularis in a subendocardial position and then intramyocardially inside the upper interventricular septum); systolic external muscular compression of the intraseptal segment was not observed. Subsequently, the patient underwent successful surgical excision of the mass. The clinical diagnosis of cardiac myxoma was confirmed by histology.
Discussion Congenital coronary artery anomalies (CAAs) are increasingly being discovered since the widespread use of coronary angiography. In the largest angiographic series conducted in the field of CAAs, the incidence of an anomalous LCx artery originating from either the right aortic sinus or the RCA has been reported to be 0.37%; it was the second most common anomaly after separate origin of the LAD and LCx arteries from the left aortic sinus and in all cases the anomalous LCx artery followed a retroaortic course [1].
Herz 2012 · 37:342–346 DOI 10.1007/s00059-011-3519-3 © Urban & Vogel 2011 A.Y. Andreou · I. Iakovou · A.K. Dimopoulos · G. Karatasakis · P. Anastasiou · I. Vasiliadis · G. Pavlides
Complex coronary artery anatomy in a patient with prolapsing left atrial myxoma Abstract The case of an asymptomatic patient with prolapsing left atrial myxoma, in whom preoperative coronary angiography revealed a rare coronary artery anatomy in the absence of atherosclerotic obstructive disease, is presented. There was a type IV dual left anterior descending (LAD) artery with intraseptal course of the right aortic sinus-connected (long) LAD artery and an ectopic left circumflex artery originating from the right aortic sinus and having a retroaortic course. The patient underwent successful surgical excision of the mass which was confirmed by histology to be cardiac myxoma. This particular coronary artery anatomy has only been
described once, and this is the first reported case of its combination with cardiac myxoma. This report highlights the importance of differentiating between the possible courses of such ectopic coronary arteries. The angiographic signs which enabled differentiation of the intraseptal course of the long LAD artery from the malignant interarterial course with which it is frequently confused are presented. Keywords Myxoma · Coronary angiography · Coronary anomaly · Dual LAD artery · Cardiovascular abnormalities
Komplexe Koronararterienanatomie bei einem Patienten mit linksventrikulärem Myxomprolaps Zusammenfassung Vorgestellt wird der Fall eines asymptomatischen Patienten mit prolabierendem linksventrikulärem Myxom, bei dem sich in der präoperativen Koronarangiographie eine seltene Variante der Koronararterien bei Fehlen obstruierender atherosklerotischer Veränderungen zeigte. Es lag ein Ramus interventricularis anterior (RIVA) in 2-facher Ausführung vor („dual LAD“, Typ IV) mit intraseptalem Verlauf des rechten, mit dem Aortensinus verbundenen (langen) RIVA und mit einem ektopen linken R. circumflexus, der aus dem rechten Aortensinus entsprang und einem retroaortalen Verlauf folgte. Bei dem Patienten wurde der Tumor erfolgreich chirurgisch exzidiert, histologisch wurde das Vorliegen eines kardialen Myxoms bestätigt. Diese spezielle
Variante der Koronararterienanatomie ist bisher nur einmal beschrieben worden, und hier liegt nun der erste Fallbericht ihrer Kombination mit einem kardialen Myxom vor. Diese Arbeit betont die Bedeutung der Differenzierung zwischen den möglichen Verläufen solcher ektoper Koronararterien. Dargestellt werden die angiographischen Hinweise, die es ermöglichten, den intraseptalen Verlauf der langen RIVA von dem malignen interarteriellen Verlauf, mit dem er oft verwechselt wird, zu unterscheiden. Schlüsselwörter Myxom · Koronarangiographie · Koronararterienanomalie · Doppelter RIVA · Kardiovaskuläre Anomalien
E The dual LAD artery variant has been reported with an incidence of 0.13–1% and consists of four types [2, 4]. In the first three types, the proximal LAD artery bifurcates early into a short branch that terminates in the proximal AIVS and a long branch that courses on the left (type I) or right (type II) of the AIVS, Herz 3 · 2012
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Fig. 2 8 a Right coronary artery (RCA) angiogram displaying myxoma neovascularization by a plexus of multiple tiny and tortuous vessels (small arrows) which arise from a left atrial branch. Right anterior oblique cranial (b) and left anterior oblique (LAO) caudal (c) views displaying the left aortic sinus-connected left anterior descending (LAD I) artery; it terminates high in the anterior interventricular sulcus after supplying two diagonal (D1 and D2) and some septal branches (arrowheads). d Aortogram obtained in the LAO projection displaying a nondominant left circumflex (LCx) artery arising abnormally from the right aortic sinus. It forms a caudal posterior loop to the left indicating a retroaortic course. e A 30° LAO view displaying the right aortic sinus-connected LAD (LAD II) artery; it forms a caudal anterior loop during its course towards the anterior interventricular sulcus and supplies a septal branch (arrowhead) as its first branch indicating an intraseptal course
or intramurally in the ventricular septum (type III) before reentering the distal AIVS to course toward the apex. In type IV dual LAD artery, the short branch originates from the left main stem or left aortic sinus [2, 5]. The long branch originates from the proximal RCA, right aortic sinus, or conus artery and more frequently follows a prepulmonic course to the left [2, 6]. In the original description of this variant, Spindola-Franco et al. [3] found only 2 cases of type IV dual LAD artery among 2,140 angiographic studies (0.09%); in both cases the long branch followed a prepulmonic course. In another angiographic series comprising 70,850 patients, Tuncer et al. [7] found only 3 cases of type IV vari-
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ant (0.004%) with a prepulmonic course of the long branch in 2 cases and an intraseptal course in 1 case. Type IV dual LAD artery with a prepulmonic long branch has been reported in combination with a retroaortic right-sided LCx artery in several case reports [8, 9, 10]. However, to the best of our knowledge such a combination comprising an intraseptal long LAD artery has only been described by Agarwal and Kazerooni et al. [11] who revealed it on computed tomographic angiography. While most CAAs are considered benign, some have been associated with adverse clinical ramifications [1, 12]. An ectopic, coronary artery arising from the opposite (improper) aortic sinus and coursing to the left may take one of sev-
eral courses to reach its dependent area, including the prepulmonic, interarterial, intraseptal, or retroaortic course. However, only the interarterial course that is between the aortic root and pulmonary trunk has been consistently associated with ischemic sequelae [12]. Intramural proximal intussusception at the aortic root wall, coronary hypoplasia, and lateral compression constitute invariable features of this course revealed by intravascular ultrasound. As a result, the proximal interarterial segment is rendered stenotic at rest and ischemia ensues due to intermittent worsening of the stenosis driven by phasic systolic accentuation of the compression under conditions entailing increased stroke volume and/or aor-
tic pressure, typically during exercise. Accordingly, an interarterial right aortic sinus-connected LAD artery mandates surgical management in case of documented ischemia associated with the intramural segment or vascular wall hypoplasia, coronary compression, or obstruction to coronary flow irrespective of inability to document ischemia [13]. E Discrimination between the possible courses of such an ectopic coronary artery is, therefore, important for patient management. Not infrequently, differentiation between the interarterial and intraseptal courses becomes a perplexing task. Indeed, misinterpretation of an intraseptal course as interarterial and subsequent unnecessary surgery has been reported [14, 15]. The intraseptal course is recognized in the right (preferred) or left anterior oblique projections because of the formation of a caudal anterior loop to the left, a myocardial bridge behavior and proximal branching into septals; the latter is a frequent though not invariable feature of this course [2, 4, 15, 16]. The myocardial bridge behavior is due to muscular compression of the intramyocardial ectopic segment during tachycardia which may occasionally lead to myocardial ischemia. In contrast, the interarterial course forms a cranial posterior loop to the left in these views and is devoid of proximal septal branches. The case presented herein is peculiar not only because of the illustration of a rare coronary artery anatomy but also because this anatomy was found in a patient undergoing surgery for cardiac myxoma. We are aware of a similar case in which a cardiac myxoma coexisted with an ectopic, right aortic sinus-connected LCx artery [17]. Our patient had no history of myocardial infarction, while echocardiography showed normal left ventricular systolic function in the absence of collateral circulation to the area of the “missing” arteries. These pieces of evidence argued against total occlusion of these vessels and favored an ectopic origin of the LCx and second LAD arteries. Yet, selective angiography of the RCA did not provide any clues to the presence of these vessels arising from its proximal segment, while the
fact that the RCA supplied a large distribution to the posterolateral wall via prominent posterolateral branches and the apex (superdominant RCA) let us consider that the LCx artery must be small. These considerations enabled us to depict the ectopic, right aortic sinus-connected LCx and long LAD arteries; however, their combination made interpretation of coronary angiography a challenge. By virtue of its typical angiographic presentation, their proximal course was recognized as retroaortic and intraseptal, respectively, and because neither of these two courses is usually associated with adverse outcomes, surgical intervention to these vessels was not indicated. This case also prompted a discussion regarding whether our patient should undergo concomitant surgical revascularization if he had an interarterial long LAD artery. Given that he denied symptoms of ischemia and the LAD artery had a binary distribution, one could defer such an intervention because of the relatively small long LAD artery-dependent area in which case major clinical manifestations are unlikely to take place [18]. However, concomitant surgical revascularization would have likely been a wise approach in case of an interarterial long LAD artery with sufficient area of distribution, because such defects are known to lead to subclinical episodes of ischemia and consequent myocardial necrosis and fibrosis which create the substrate for the emergence of malignant ventricular arrhythmias and sudden cardiac death [19].
Conclusion This is only the second reported case of the combination of a type IV dual LAD artery with intraseptal course of the right aortic sinus-connected (long) LAD artery and an ectopic LCx artery with origin from the right aortic sinus and retroaortic course. Furthermore, this is the first reported case of the combination of this particular coronary anatomy with cardiac myxoma. Patients undergoing noncoronary cardiac surgery, who are found on preoperative coronary angiography to have an ectopic improper aortic sinusconnected coronary artery with interarterial course, face a unique opportunity for
concomitant correction of this malignant anomaly. In the setting of “missing” coronary arteries during coronary angiography, a thoughtful approach taking into account information from patient’s history and precatheterization evaluation as well as anatomical information revealed during angiography aids in differentiating between ectopic and occluded arteries. Depiction of ectopic coronary arteries and interpretation of coronary angiography may be challenging particularly in the presence of combined coronary artery anomalies. Accurate recognition of the ectopic coronary artery course is important for patient management.
Corresponding address A.Y. Andreou A’ Department of Cardiology, Onassis Cardiac Surgery Center 356 Syngrou Avenue, 17674 Athens Greece
[email protected] Conflict of interest. The corresponding author states that there are no conflicts of interest.
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