Coronary arteries that course between the pulmonary

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1 Surgical Treatment of Right Coronary Arteries With Anomalous Origin and Slit Ostium Raúl García-Rinaldi, MD, Javier Sosa, MD, Samuel Olmeda, ORT, Hernán Cruz, MD, Jorge Carballido, MD, and Cyd Quintana, MD Division of Cardiovascular Surgery, Advanced Cardiology Center and the Puerto Rico and the Caribbean Cardiovascular Center, Mayagüez and San Juan, Puerto Rico Background. Right coronary arteries arising in the left sinus or ectopically in the anterior coronary sinus with slit ostium course inside the aorta. They are subject to variable systolic compression and can cause myocardial ischemia with its sequelae or death. Methods. From May 1991 to March 2003, we treated 16 patients with anomalous origin of the right coronary artery from the left sinus and 4 whose right coronary artery arose ectopically in the anterior sinus. All patients had a slit ostium and underwent transaortic unroofing of the trunk to modify the proximal portion of the anomalous artery. Results. All patients survived operation, although 1 patient died of unrelated causes. Nineteen patients were followed for a period from 0.2 to 11.8 years (median age, 53 years). One experienced angina 1 year after surgery and underwent percutaneous transluminal coronary angioplasty of a left internal thoracic to left anterior descending coronary artery anastomosis. All patients are New York Heart Association class I, without angina; none has sustained a myocardial infarction or required reoperation. Conclusions. Right coronary arteries that arise in anomalous fashion with a slit ostium can cause myocardial ischemia or death. Transaortic modification of the anomalous trunk addresses the anatomic and pathophysiologic features of the malformation that cause myocardial ischemia. Excellent results can be achieved with this surgical approach. (Ann Thorac Surg 2004;77:1525 9) 2004 by The Society of Thoracic Surgeons Coronary arteries that course between the pulmonary artery and the aorta can cause myocardial ischemia and sudden death [1 3]. This applies both to left coronary arteries that arise from the right (anterior) sinus as well as right coronary arteries (RCAs) that arise from the left (posterior) sinus [1 3]. In both situations, the anomalous coronary trunk shares a common wall with the aorta, courses within the aorta, and is subject to lateral, variable, systolic compression, an important pathophysiologic feature of these malformations [1 4]. The anomalous artery usually arises as a slit instead of an ostium and at a very acute angle from the aorta [1, 2]. Coincidentally, RCAs, which arise ectopically in the right sinus, behave in a similar manner to those that arise anomalously from the left sinus. Interestingly, RCAs that arise ectopically in the right sinus course within and share a common wall with the aorta [4]. Likewise, the origin at the aorta is a slit and the trunk arises at a very acute angle from the aorta [1, 2]. Whether lateral systolic compression of the trunk occurs in this malformation is not known. It is, however, probable that this is a mechanism of ischemia in this malformation. In a recent report, Yip and coworkers [5] discussed 8 patients who presented with acute inferior wall myocardial infarction and had anomalous origin of the RCA. Accepted for publication Aug 28, Address reprint requests to Dr García-Rinaldi, PO Box 6684, Marina Station, Mayagüez, PR ; garciarinald@prtc.net. Interestingly, of this group of 8 patients, 6 had ectopic origin of the RCA, anterior and above the sinotubular junction of the aorta. Two had anomalous origin of the RCA in the left sinus. Their report confirms our impression that these anomalies functionally behave the same way, all causing myocardial ischemia or death. Historically, several mechanisms have been proposed to explain the restriction of coronary flow in the ectopic or anomalous right coronary trunk; these include the following: Acute angulation at the coronary takeoff, which may increase or become kinked during exertion [1, 6]. Presence of the ostial ridge, which may function as a valvelike mechanism, restricting flow during exertion [1]. Slitlike orifice, which becomes compressed by exercise-induced aortic dilation [7]. Stretching and compression of the intramural segment by the aortic valve commissure, which would apply only to some RCAs that arise in the left sinus [8]. Most recently, and with the use of intravascular ultrasound, Angelini has demonstrated variable lateral luminal compression of the intramural trunk that worsens during systole [4]. The most interesting part of this observation is that individual variations in the degree of lateral compression could serve to 2004 by The Society of Thoracic Surgeons /04/$30.00 Published by Elsevier Inc doi: /j.athoracsur

2 1526 GARCÍA-RINALDI ET AL Ann Thorac Surg RCA WITH ANOMALOUS ORIGIN AND SLIT OSTIUM 2004;77: explain the different clinical behaviors observed in different patients [4]. We have found an unusually large number of patients in Puerto Rico whose RCAs arise from the left sinus or ectopically in the right sinus. Whether these malformations are more common in Puerto Rico or whether there is a greater index of suspicion by our cardiologists is not known. At any rate, the number of cases referred for treatment has fostered our interest in these malformations of the RCA. Historically, the treatment for RCAs that arise from the left sinus has varied. Approaches have included the following: Medical (expectant) treatment [9], Coronary bypass grafting with the internal thoracic artery or autogenous saphenous vein [8 10], Implantation into the correct sinus from outside the aorta [9, 11, 12], Translocation of the pulmonary artery [13], The use of primary angioplasty in patients with myocardial infarcts in evolution [5]. The use of stents to prevent compression of the intraluminal segment area [14]. The one we prefer, transaortic modification of the origin and proximal portion of the ectopic or anomalous RCA because it addresses all the mechanisms postulated to cause ischemia, including systolic compression of the trunk [15, 16]. We previously reported the treatment of these anomalies by this technique [15]. Our experience now totals 20 patients without operative mortality and excellent long-term follow-up. An analysis of these cases constitutes the basis of this report. Patients and Methods Patient Characteristics The patient population consists of 20 patients referred for surgical treatment and constitutes our entire experience with these malformations. The patients (16 men) were seen during a 142-month period from May 1991 to March 31, The median age was 53 years (range 28 to 79 years). The patients were either seen in our office or contacted by telephone during March 2003 to find out their most recent status. In most cases the diagnosis of the specific type of anomaly was difficult to establish by coronary angiography. Several times the first clue to the diagnosis of either of these malformations was the inability of the catheterizing cardiologist to easily locate and inject the RCA. In fact in 6 cases, a second catheterization by another cardiologist was required for the diagnosis. The coronary angiography may or may not establish exactly whether the RCA arises in ectopic fashion in the region of the right sinus of Valsalva or anterior in the aorta or in the left sinus. Origin of the RCA anteriorly, in the ascending aorta, above the sinotubular junction as described by Yip and associates [5] is highly suggestive of ectopic origin rather than an anomalous origin from the Table 1. Kragel and Roberts [7] Classification of Anomalous Origin of Right Coronary Artery From the Left Sinus Type 2A 2B 2C 2D Origin of Right Coronary Artery Posteriorly in the left sinus Above the left sinus Above the right-left (anterior) commissure Common ostium with left main coronary and straddling the left sinus and rightleft (anterior) commissure left sinus. Likewise, simultaneous opacification of the right and left coronary arteries on injection of the left coronary artery is highly suggestive of anomalous origin of the RCA from the left sinus [5]. However, because of the multiple types of anomalous trunks, most times it is not possible to determine from the angiogram the specific type of the anomalous origin of the RCA from the left sinus. Thus, confirmation of the anomaly is performed during operation and therapy tailored to the specific patient and his or her type of malformation. The number of patients with RCAs that arose ectopically from the right sinus was 4. Of these, 1 patient had occlusive disease in other coronary arteries. None of these 4 patients had occlusive disease in the proximal portion of the artery in complete opposition to the findings of Yip and colleagues [5]. Sixteen patients had anomalous origin of the RCA from the left sinus. Five patients had type 2A, 1 patient had type 2B, 8 patients had type 2C, and 1 patient had type 2D according to the Kragel and Roberts [6] classification (Table 1). The specific type of anomalous origin of 1 of the patients is unknown because he underwent an internal thoracic artery bypass graft for an 80% stenosis of the anomalous trunk. Modification of the trunk and its origin were not necessary because the internal thoracic artery provided sufficient flow. Twelve of the 16 patients whose RCA arose from the left sinus did not have associated coronary occlusive disease. The average age of patients without associated coronary disease was 46 years (range, 28 to 65 years). The average age of patients with associated coronary diseases was years (range, 53 to 79 years). All patients had angina. Some patients were unstable with angina even without having associated occlusive coronary artery disease. Eleven patients (55%) had a history of a previous inferior wall myocardial infarction. Of these, only 6 had associated occlusive coronary artery disease. None of the patients had sustained a syncopal episode or a treated cardiac arrest. Eighteen patients underwent stress testing that was positive in 16 (88%). One patient had an associated septum secundum atrial septal defect that was corrected with a polyethylene terephthalate fiber (Dacron) patch. The average ejection fraction for all patients was 0.50 (range, 0.30 to 0.80). Surgical Technique For patients with ectopic origin of the RCA from the right sinus with slit ostium, we first ascertained the direction

3 Ann Thorac Surg GARCÍA-RINALDI ET AL 2004;77: RCA WITH ANOMALOUS ORIGIN AND SLIT OSTIUM 1527 Fig 1. (A, B) The direction of the anomalous trunk is ascertained. (C) The anomalous trunk is incised, and the incision lengthened 1 cm. (D) The intimal surfaces of the aorta and right coronary artery are approximated with 7-0 polypropylene suture. (E) Completed repair. (intramural course) of the coronary artery (Fig 1A). A portion of the common wall (septum) is excised, and the incision is lengthened to at least 1 cm (Figs 1B, 1C). The intimal surfaces of the aorta and RCA are approximated with interrupted sutures of 7-0 polypropylene suture. (Figs 1D, 1E). Deroofing of the entire intramural portion is possible with this technique. We have found that 1 cm is the approximate length of the intramural portion. More laterally (toward the acute margin of the heart) the RCA starts separating from the aorta to course in the usual anatomic location. For some patients who have anomalous origin of the RCA from the true left sinus (Fig 2), the aortic valve must be detached at the right-left (anterior) commissure because the trunk courses below this commissure. The slit ostium is identified and incised. With a coronary artery probe the direction of the intramural coronary trunk is ascertained. The left-right (anterior) commissure is detached (Fig 2B). A portion of the common wall (septum) between the aorta and RCA is excised (Fig 2C). The incision must be at least 1 cm to modify both the orifice and the proximal portion of the intramural seg- Fig 2. (A) The right coronary arises in the left sinus and courses under the right-left commissure. (B) The aortic valve is detached at the level of the anterior commissure. (C) The slit ostium is incised and lengthened 1.5 cm. (D) The intimal surfaces of the aorta and the anomalous right coronary artery are approximated with 7-0 polypropylene suture. (E) The aortic valve is resuspended with a pledgeted 6-0 polypropylene suture.

4 1528 GARCÍA-RINALDI ET AL Ann Thorac Surg RCA WITH ANOMALOUS ORIGIN AND SLIT OSTIUM 2004;77: ment. Deroofing is continued laterally (toward the acute margin of the heart) until the point at which the trunk and aorta begin to separate. RCA then courses in the usual anatomic location. After completing the incision, the intimal surfaces of the aorta and RCA are approximated with interrupted sutures of 7-0 polypropylene (Fig 2D). The aortic valve is resuspended with a 6-0 polypropylene suture over a polytetrafluoroethylene (Teflon) pledget (Fig 2E), avoiding narrowing of the newly created orifice. A 4-mm dilator can be easily introduced into the modified orifice. Results Four patients had RCAs that arose ectopically from the right sinus. All had modification of the proximal portion of the ectopic trunk (Fig 1) to prevent angulation and provide ample, unrestricted flow to the RCA. One patient had occlusive disease in other coronary arteries and underwent concomitant aortocoronary bypass. All survived operation and are asymptomatic (New York Heart Association class I). None of the patients has required reoperation. Of the remaining 16 patients with RCAs that arose from the left sinus, the technique illustrated in Figure 2 was used when the trunk passed below the aortic commissure. Six patients (38%) had occlusive disease in the other coronary arteries and underwent concomitant coronary artery bypass grafting (average, 2.8 bypasses). None of the patients who underwent temporary detachment of the anterior commissure has developed aortic valve insufficiency. One patient with anomalous origin of the RCA from the left sinus had an 80% stenosis of the anomalous trunk. He underwent a successful internal thoracic artery bypass and is asymptomatic. One patient bled from one of the associated grafts, and 1 patient experienced a trapped lung that required decortication. One patient had angina 1 year after the operation. Angiography revealed a widely patent right coronary orifice and stenosis of the left internal thoracic artery to left anterior descending coronary artery anastomosis. He underwent successful dilatation (by percutaneous transluminal coronary angioplasty) of the anastomosis. None of the patients has required reoperation. The hospital mortality for the series of patients was 0. One patient (age, 79 years) died of multiple organ failure unrelated to his cardiac operation in another institution 2 months after surgery. The follow-up period ranged from 0.2 to 11.8 years (mean, 6.8 years). All patients are asymptomatic (New York Heart Association class I). None of the patients has had a stress test. Only 1 patient has undergone a postoperative coronary angiogram (see above). Comment Right coronary arteries that arise from the left sinus or ectopically from the right sinus can cause myocardial ischemia, infarction, arrhythmias, syncope, or sudden death [1, 2, 3, 5]. Several mechanisms have been implicated as the cause of ischemia: Increased acuteness of the angle of origin on exercise, The ostial ridge, invariably present, can function like a restraining valve mechanism, Stretch of the intramural segment, Compression of the anomalous artery by the rightleft (anterior) commissure of the aortic valve. Lateral, systolic compression of the intramural trunk [1 4]. Of the techniques that have been recommended to treat these anomalies, our only other experience is the reimplantation from outside the aorta. We no longer use this technique because one cannot adequately inspect or modify the abnormal ostium [9, 10, 12]. Our experience with 2 patients treated in this manner was discouraging. Both patients arrested on discontinuation of bypass. Cardiopulmonary bypass was reinstituted and an aorta-to-right coronary saphenous vein graft was performed. Both survived operation and have done well [15]. Recently, translocation of the pulmonary artery was recommended as a method to reduce intramural compression of coronary arteries that course between the aorta and pulmonary artery [13]. We believe that an operation of such magnitude is not necessary to resolve the problem, particularly because it does not address the issue of the acute takeoff and slit ostium. Hariharan and colleagues [14] have suggested the treatment of coronary trunks that course between the aorta and pulmonary artery by stent implantation. A stent addresses the issue of lateral systolic compression within the trunk but leaves untouched the problem of the ostial ridge and acute takeoff angle of the coronary orifice. Furthermore, stent implantation is ill-suited for patients with anomalous origin of the left coronary artery from the right sinus. The intramural path that this trunk courses is long, curved, and tortuous. The two reported cases in which they used stents to treat these malformations have not done well and remain with chest pain [14]. Long-term follow-up of stented patients will determine the exact role of this therapy. Yip and associates [5] reported the use of primary angioplasty in 8 patients with anomalous origin of the RCA presenting with inferior wall myocardial infarction. Although they misclassified the malformations (6 patients had ectopic rather than anomalous origin of the RCA from the left sinus), their contribution is of great importance [5]. These authors demonstrated occlusion of the RCA in all cases and a high prevalence of atherosclerotic occlusive disease in the trunk in the absence of significant obstructive coronary disease in the other arteries. They treated these patients with primary angioplasty. Two of 8 patients died (25% mortality). Of the 6

5 Ann Thorac Surg GARCÍA-RINALDI ET AL 2004;77: RCA WITH ANOMALOUS ORIGIN AND SLIT OSTIUM who survived, 5 underwent repeat cardiac catheterization. Two (40%) had RCA restenosis and 1 required a repeat percutaneous transluminal coronary angioplasty. Thus, it seems that this method of treatment at the present time is far from optimal. We previously reported our initial experience with modification of the proximal portion of the anomalous trunk from inside the aorta [15]. This technique enlarges the ostium and effectively reduces lateral compression of the intramural segment of the coronary. All patients treated in this manner survived the operation and continue to be asymptomatic. We disagree with the opinion of Hariharan and coworkers [14] that this technique is not possible in patients whose anomalous trunk passes below the right-left (anterior) aortic commissure. We have temporarily detached the right-left (anterior) commissure, which allows us to modify the trunk and eliminate compression without sequelae of aortic valve insufficiency [15]. Reul and associates [11] advocate the use of internal thoracic artery bypass to the RCA in patients with anomalous RCAs that arise in the left sinus. We used the internal thoracic artery in 1 patient who had occlusive disease in the anomalous trunk. We do not advocate the use of internal thoracic artery bypass in the absence of occlusive disease to avoid competitive flow that can occlude the bypass [15, 17]. Right coronary arteries that arise from the left sinus, ectopically from the right sinus, or anteriorly in the aorta above the sinotubular junction should be considered significant and dangerous. If the diagnosis is made, we suggest that transaortic modification of the ostium and trunk be carried out. The long-term effect of this modification on the RCA is not known, although clinically, the patients have done well in long-term follow-up. References 1. Virmani R, Chun PKC, Goldstein RE, Rabinowitz M, McAllister HA. Acute take-off of the coronary arteries along the aortic wall and congenital coronary ostial ridges; association with sudden death. J Am Coll Cardiol 1984;3: Isner JM, Shen EM, Martin ET. Sudden unexpected death as 1529 a result of anomalous origin of the right coronary artery from the left sinus of Valsalva. Am J Med 1984;76: Ness MS, McManus BM. Anomalous right coronary artery origin in otherwise unexplained infant death. Arch Pathol Lab Med 1988;112: Angelini P. Coronary artery anomalies current clinical issues: definitions, classification, incidence, clinical relevance and treatment guidelines. Tex Heart Inst J 2002;29: Yip H, Chen MC, Wu CJ, et al. Primary angioplasty in acute inferior myocardial infarction with anomalous-origin right coronary arteries as infarct-related arteries: focus on anatomic and clinical features, outcomes, selection of guiding catheters and management. J Invasive Cardiol 2001;13: Cheitlin MD, De Castro CM, McAllister HA. Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva. Circulation 1974;50: Kragel AH, Roberts WC. Anomalous origin of either the right or left main coronary artery from the aorta with subsequent coursing between aorta and pulmonary trunk: analysis of 32 necropsy cases. Am J Cardiol 1988;62: Liberthon RR. Case records of the Massachusetts General Hospital. N Engl J Med 1989;720: Fernández ED, Kadivar H, Hallman GL, Reul GS, Ott DA, Cooley DA. Congenital malformations of the coronary arteries: the Texas Heart Institute experience. Ann Thorac Surg 1992;54: Bett JHN, O Brien MF, Murray PJ. Surgery for anomalous origin of the right coronary artery. Br Heart J 1985;53: Reul RM, Cooley DA, Hallman GL, et al. Surgical treatment of coronary artery anomalies. Tex Heart Inst J 2002;29: Di Lello F, Munk JF, Flemma RJ, Mullen DC. Successful coronary reimplantation for anomalous origin of the right coronary artery from the left sinus of Valsalva. J Thorac Cardiovasc Surg 1991;102: Rodefel MD, Culbertson CB, Rosenfeld HM, Hanley FL, Thompson LD. Pulmonary artery translocation: a surgical option for complex anomalous coronary artery anatomy. Ann Thorac Surg 2001;72: Hariharan R, Kacere RD, Angelini P. Can stent-angioplasty be a valid alternative to surgery when revascularization is indicated for anomalous origination of a coronary artery from the opposite sinus? Tex Heart Inst J 2002;29: García-Rinaldi R, Carballido J, Giles R, et al. Right coronary artery with anomalous origin and slit ostium. Ann Thorac Surg 1994;58: Nelson-Piercy C, Rickand AF, Yacoub NH. Aberrant origin of the right coronary artery as a potential cause of sudden death: successful anatomical correction. Br Heart J 1990;64: Tector AS, Schmahl TM, Janson B, Kallies JR, Johnson G. The internal mammary artery graft: its longevity after coronary bypass. JAMA 1981;246: