Surgical Treatment of Anomalous Aortic Origin of Coronary Arteries: The Reimplantation Technique and Its Modifications

Abstract

Although anomalous aortic origin of a coronary artery has been described as a benign anomaly in the past, several reports suggest that such an anomaly may be the cause of sudden death in children and younger adults. Increased cardiac output during exercise may result in compression of the anomalous coronary artery when it runs between the aorta and the pulmonary artery or when its aortic origin is narrowed and its proximal course is intramural. It is unlikely that this rare anomaly would be identified during infancy, but once diagnosis has been made, indication to proceed with surgery is controversial. Nevertheless, if surgery is recommended, several operative techniques may be used, depending on the specific anatomy; unroofing, direct reimplantation, patch enlargement, and even coronary artery bypass grafting have been proposed. In this short report, we focus on different techniques of coronary reimplantation. As we already know from the arterial switch operation in neonates and from aortic root repair or replacement in adults, sufficient mobilization is necessary to allow direct reimplantation in the correct sinus to avoid kinking and other distortion. Although anomalous aortic origin of a coronary artery has been described as a benign anomaly in the past, several reports suggest that such an anomaly may be the cause of sudden death in children and younger adults. Increased cardiac output during exercise may result in compression of the anomalous coronary artery when it runs between the aorta and the pulmonary artery or when its aortic origin is narrowed and its proximal course is intramural. It is unlikely that this rare anomaly would be identified during infancy, but once diagnosis has been made, indication to proceed with surgery is controversial. Nevertheless, if surgery is recommended, several operative techniques may be used, depending on the specific anatomy; unroofing, direct reimplantation, patch enlargement, and even coronary artery bypass grafting have been proposed. In this short report, we focus on different techniques of coronary reimplantation. As we already know from the arterial switch operation in neonates and from aortic root repair or replacement in adults, sufficient mobilization is necessary to allow direct reimplantation in the correct sinus to avoid kinking and other distortion. Anomalous aortic origin of a coronary artery (AAOCA) occurs when the right coronary artery arises from the posterior (left) sinus of Valsalva or when the left main coronary artery (exceptionally the left anterior descending artery only) arises from the anterior (right) sinus of Valsalva. The anomalous artery may have a common ostium with the anatomically correct coronary artery or arise from a separate ostium in the same sinus of Valsalva (Fig. 1). An anomalous aortic origin is almost always followed by an anomalous course between the great vessels in either an intramural fashion or an extramural fashion. When 2 ostia originate from the same sinus, the origin of the anomalous coronary artery is often abnormally small or slit-like. In exceptional instances, there is only a common ostium (Fig. 2).Figure 1Different types of anomalous aortic origin of the coronary arteries: right coronary artery from the left posterior sinus of Valsalva (A); Left main coronary artery from the right anterior sinus of Valsalva (B).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 2Common and separate ostium in anomalous aortic origin of the coronary arteries: right coronary artery and left main coronary artery from a common ostium (A); small separate and narrowed ostium in a case of anomalous right coronary artery (B).View Large Image Figure ViewerDownload Hi-res image Download (PPT) Anomalous origin of coronary arteries is a rare malformation (prevalence 0.1-1.0%) that remains asymptomatic until adult age in the majority of patients. Increased cardiac output during exercise may result in compression of the anomalous coronary artery when it runs between the aorta and the pulmonary artery or when its aortic origin is narrowed and its proximal course is intramural (because of increased intramural aortic pressure) or transcommissural. Several reports demonstrate that such an anomaly may cause sudden death in children and young adults.1Benson P.A. Lack A.R. Anomalous aortic origin of left coronary artery.Arch Pathol. 1968; 86: 214-216PubMed Google Scholar, 2Cohen L.S. Shaw L.D. Fatal myocardial infarction in a 11 year old boy associated with a unique coronary artery anomaly.Am J Cardiol. 1967; 19: 420-423Abstract Full Text PDF PubMed Scopus (76) Google Scholar, 3Cheitlin M.D. De Castro C.M. McAllister H.A. Sudden death as a complication of anomalous left coronary origin from the anterior sinus of Valsalva: A not so-minor congenital anomaly.Circulation. 1974; 50: 780-787Crossref PubMed Scopus (649) Google Scholar, 4Liberthson R.R. Dinsmore R.E. Bharati S. et al.Aberrant coronary artery origin from the aorta: Diagnosis and clinical significance.Circulation. 1974; 50: 774-779Crossref PubMed Google Scholar Different mechanisms to explain sudden death because of myocardial ischemia have been proposed (Fig. 3).5Bartoli C.R. Wead W.B. Giridharan G.A. et al.Mechanism of myocardial ischemia with an anomalous left coronary artery from the right sinus of Valsalva.J Thorac Cardiovasc Surg. 2012; 144: 402-408Abstract Full Text Full Text PDF PubMed Scopus (25) Google Scholar, 6Hoffman J.L.E. Abnormal origins of the coronary arteries from the aortic root.Cardiol Young. 2014; 24: 774-791Crossref PubMed Scopus (12) Google Scholar, 7Mosca R.S. Phoon C.K.L. Anomalous aortic origin of a coronary artery is not always a surgical disease.Pediatr Cardiac Surg Annu. 2016; 19: 30-36Scopus (21) Google Scholar They include compression between the great vessels, small or stenotic ostium, acute take-off angle with potential for kinking at the exit of the aortic wall, compression of the intramural segment within the aortic wall, and compression of the intramural segment by the commissure of the aortic valve. Often, diagnosis is made during a systematic cardiac evaluation and established by echocardiography, computed tomography, magnetic resonance imaging (MRI), or cine-angiography. Interventional treatment is not indicated because neither balloon dilatation nor stent implantation can resolve any of the compression mechanisms. Indication to proceed with surgery remains controversial. However, surgical treatment seems reasonable in patients with angina, syncope, ventricular arrhythmias, and in survivors of sudden death episodes. Indication for surgery in asymptomatic patients has not been clearly defined, but reversible ischemia in the distribution of the anomalous coronary artery may be a wise reason to correct this anomaly. Several operative techniques have been described: unroofing, direct reimplantation, patch enlargement (ostioplasty) with or without pulmonary translocation, and coronary artery bypass grafting. As with any coronary artery transfer, known from the arterial switch operation in neonates and from aortic root repair (David) or replacement (Bentall) in adults, sufficient but not excessive mobilization of the coronary button is necessary to allow direct reimplantation in the correct sinus and to avoid kinking and other distortion. In the case of separate orifices in the sinus of Valsalva without intramural course, reimplantation is most often suitable, although not for other types because there is no sufficient aortic tissue around the orifice to create a coronary button that can be easily reimplanted. Surgical approach is performed through median sternotomy. An alternative approach may be a limited upper sternotomy. The aortic purse-string suture is placed cranially in the ascending aorta, near the origin of the innominate artery. Venous return is achieved through right atrial cannulation. Cardiopulmonary bypass is conducted in moderate hypothermia (32°C-34°C). A left ventricular vent is placed via the right superior pulmonary vein to empty the left ventricle and give optimal visualization. Before aortic cross-clamping, extensive dissection of the space between the ascending aorta and the main pulmonary artery is performed. The aortic clamp is placed as cranial as possible, and single-shot low-volume crystalloid cardioplegia (100 mL Cardioplexol; Bichsel AG, Interlaken, Switzerland) is administered. Cold blood cardioplegia using Buckberg solution was applied if cross-clamping was expected to be longer than 45 minutes. Transverse aortotomy is performed well above the sinotubular junction to avoid any injury to the anomalous coronary artery. This simple step should be performed with care because the more distal site of the anomalous coronary origin is often underestimated. The operative technique is described on the following pages (Figure 4, Figure 5, Figure 6, Figure 7, Figure 8, Figure 9, Figure 10).Figure 5Anomalous left coronary artery from the right sinus. In patients with a truly extramural type of a left anomalous coronary artery (A), excision of a generous aortic button including the anomalous coronary artery (similar to coronary artery excision performed during aortic root repair [David procedure] or replacement [modified Bentall procedure]) (B) and direct reimplantation into the anatomically appropriate sinus of Valsalva is a good option. Adequate mobilization of the proximal portion of the coronary artery may be necessary to avoid tension or any degree of distortion. Adequate orientation of the button (sometimes a 90-degree rotation is necessary to match the most ideal footpoint of the button together with that of the neo-ostium). The site of button excision is filled with a small piece of xenopericardium (C-E).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 5Anomalous left coronary artery from the right sinus. In patients with a truly extramural type of a left anomalous coronary artery (A), excision of a generous aortic button including the anomalous coronary artery (similar to coronary artery excision performed during aortic root repair [David procedure] or replacement [modified Bentall procedure]) (B) and direct reimplantation into the anatomically appropriate sinus of Valsalva is a good option. Adequate mobilization of the proximal portion of the coronary artery may be necessary to avoid tension or any degree of distortion. Adequate orientation of the button (sometimes a 90-degree rotation is necessary to match the most ideal footpoint of the button together with that of the neo-ostium). The site of button excision is filled with a small piece of xenopericardium (C-E).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 5Anomalous left coronary artery from the right sinus. In patients with a truly extramural type of a left anomalous coronary artery (A), excision of a generous aortic button including the anomalous coronary artery (similar to coronary artery excision performed during aortic root repair [David procedure] or replacement [modified Bentall procedure]) (B) and direct reimplantation into the anatomically appropriate sinus of Valsalva is a good option. Adequate mobilization of the proximal portion of the coronary artery may be necessary to avoid tension or any degree of distortion. Adequate orientation of the button (sometimes a 90-degree rotation is necessary to match the most ideal footpoint of the button together with that of the neo-ostium). The site of button excision is filled with a small piece of xenopericardium (C-E).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 6Anomalous right coronary artery from the left sinus. The direct reimplantation technique for a right coronary artery originating from the left sinus (A) is summarized in the following steps: excision of the aortic button with the coronary artery (B); creation of a neo-ostium in the corresponding sinus using a punch (C); the reimplantation itself using a 6.0 polypropylene running suture and closure of the button procurement site using a small patch of autologous or xenopericardium (D).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 6Anomalous right coronary artery from the left sinus. The direct reimplantation technique for a right coronary artery originating from the left sinus (A) is summarized in the following steps: excision of the aortic button with the coronary artery (B); creation of a neo-ostium in the corresponding sinus using a punch (C); the reimplantation itself using a 6.0 polypropylene running suture and closure of the button procurement site using a small patch of autologous or xenopericardium (D).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 6Anomalous right coronary artery from the left sinus. The direct reimplantation technique for a right coronary artery originating from the left sinus (A) is summarized in the following steps: excision of the aortic button with the coronary artery (B); creation of a neo-ostium in the corresponding sinus using a punch (C); the reimplantation itself using a 6.0 polypropylene running suture and closure of the button procurement site using a small patch of autologous or xenopericardium (D).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 6Anomalous right coronary artery from the left sinus. The direct reimplantation technique for a right coronary artery originating from the left sinus (A) is summarized in the following steps: excision of the aortic button with the coronary artery (B); creation of a neo-ostium in the corresponding sinus using a punch (C); the reimplantation itself using a 6.0 polypropylene running suture and closure of the button procurement site using a small patch of autologous or xenopericardium (D).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 7View from outside during the running suture into the punched hole. Closure of the aortotomy has been performed using 4.0 monofilament running suture.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 8Modified reimplantation of the right coronary artery using an enlargement technique of the ostium and refixation of the aortic valve commissure: because the anomalous coronary artery usually arises from the opposite sinus and is very close to the commissure, detachment of the aortic valve commissure may be necessary to remodel and enlarge the ostium or to excise it with a sufficient button of aortic wall tissue for further reimplantation. The commissure is then resuspended with a 6.0 polypropylene suture, pledgeted with autologous pericardium. Small anomalous right coronary artery within or close to the commissure (A). Detachment of the aortic valve commissure between the left and right aortic cusps and excision of the aortic button (B). Translocation of the right coronary artery to the right (anterior) sinus together with enlargement of the ostium (C). Refixation of the commissure (D). In case of a slit-like ostium, direct reimplantation may be possible only if cranial enlargement using a xenopericardial patch is performed (E).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 8Modified reimplantation of the right coronary artery using an enlargement technique of the ostium and refixation of the aortic valve commissure: because the anomalous coronary artery usually arises from the opposite sinus and is very close to the commissure, detachment of the aortic valve commissure may be necessary to remodel and enlarge the ostium or to excise it with a sufficient button of aortic wall tissue for further reimplantation. The commissure is then resuspended with a 6.0 polypropylene suture, pledgeted with autologous pericardium. Small anomalous right coronary artery within or close to the commissure (A). Detachment of the aortic valve commissure between the left and right aortic cusps and excision of the aortic button (B). Translocation of the right coronary artery to the right (anterior) sinus together with enlargement of the ostium (C). Refixation of the commissure (D). In case of a slit-like ostium, direct reimplantation may be possible only if cranial enlargement using a xenopericardial patch is performed (E).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 9Modified reimplantation technique after Karl et al8Karl T. Coronary artery from the wrong sinus of Valsalva: A physiologic repair strategy.Oper Tech Thorac Cardiovasc Surg. 2008; 13: 35-39Abstract Full Text Full Text PDF Scopus (6) Google Scholar, 9Karl T. Provenzano M.C. Nunn G.R. Anomalous aortic origin of a coronary artery: An universally surgical strategy.Cardiol Young. 2010; 20: 44-49Crossref PubMed Scopus (12) Google Scholar; this represents an ideal approach for an LMCA originating from the right sinus (A). The aorta is transected. An incision is made into the ostium of the anomalous coronary artery beginning from the cut edge of the aorta. The incision extends close on the bifurcation of the left main coronary artery. The pericardial patch is sutured into this incision to enlarge the proximal segment of the anomalous coronary and create, thereby, an ostium of 5 mm or more. Thereafter, the ascending aorta is re-anastomosed, incorporating the base of the pericardial patch into the anastomotic suture line (B). This procedure is almost physiological because it allows enlargement of a slit-like ostium, augmentation of the diameter of the proximal coronary segment, and improvement of the acute angulation at the take-off site. In case the anomalous coronary artery runs between the great arteries, the pulmonary artery is transected just under the bifurcation. The incision is continued into the left branch, and thereafter pulmonary translocation (the main pulmonary artery is anastomosed to the left branch, whereas the right pulmonary artery is closed with a pericardial patch to avoid stenosis) helps to resolve compression of the coronary artery between the great vessels.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 9Modified reimplantation technique after Karl et al8Karl T. Coronary artery from the wrong sinus of Valsalva: A physiologic repair strategy.Oper Tech Thorac Cardiovasc Surg. 2008; 13: 35-39Abstract Full Text Full Text PDF Scopus (6) Google Scholar, 9Karl T. Provenzano M.C. Nunn G.R. Anomalous aortic origin of a coronary artery: An universally surgical strategy.Cardiol Young. 2010; 20: 44-49Crossref PubMed Scopus (12) Google Scholar; this represents an ideal approach for an LMCA originating from the right sinus (A). The aorta is transected. An incision is made into the ostium of the anomalous coronary artery beginning from the cut edge of the aorta. The incision extends close on the bifurcation of the left main coronary artery. The pericardial patch is sutured into this incision to enlarge the proximal segment of the anomalous coronary and create, thereby, an ostium of 5 mm or more. Thereafter, the ascending aorta is re-anastomosed, incorporating the base of the pericardial patch into the anastomotic suture line (B). This procedure is almost physiological because it allows enlargement of a slit-like ostium, augmentation of the diameter of the proximal coronary segment, and improvement of the acute angulation at the take-off site. In case the anomalous coronary artery runs between the great arteries, the pulmonary artery is transected just under the bifurcation. The incision is continued into the left branch, and thereafter pulmonary translocation (the main pulmonary artery is anastomosed to the left branch, whereas the right pulmonary artery is closed with a pericardial patch to avoid stenosis) helps to resolve compression of the coronary artery between the great vessels.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 10Modified reimplantation of an anomalous left coronary artery with intramural course between the great vessels. For this special malformation, the technique of Pascal Vouhé, which is a further modification of Karl et al's technique, is useful.10Vouhé P. Anomalous origin of a coronary artery is always a surgical disease.Pediatr Cardiac Surg Annu. 2016; 19: 30-36Google Scholar This technique is specially indicated for an anomalous left coronary artery originating from the right sinus of Valsalva and with an intramural course between the great arteries. In such cases, it is recommended to divide the aorta at the level of the sinotubular junction and the main pulmonary artery just above the pulmonary valve commissures. Both vessels are separated from each other as low as possible, close to the annular plane. The most proximal epicardial course of the left coronary artery is incised in a longitudinal fashion. Then a vertical incision is performed into the left sinus in the direction of the incised left coronary artery (A). Both incisions are joined together and may be approximated at the level where the intramural part of the coronary artery becomes extramural (B). A patch of fresh autologous or xenopericardium is used to enlarge and close the aortocoronary incision and therefore create the neo-ostium in the left sinus. The aortic re-anastomosis is performed, including the top edge of the pericardial patch into the aortic anastomotic suture line (C).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 10Modified reimplantation of an anomalous left coronary artery with intramural course between the great vessels. For this special malformation, the technique of Pascal Vouhé, which is a further modification of Karl et al's technique, is useful.10Vouhé P. Anomalous origin of a coronary artery is always a surgical disease.Pediatr Cardiac Surg Annu. 2016; 19: 30-36Google Scholar This technique is specially indicated for an anomalous left coronary artery originating from the right sinus of Valsalva and with an intramural course between the great arteries. In such cases, it is recommended to divide the aorta at the level of the sinotubular junction and the main pulmonary artery just above the pulmonary valve commissures. Both vessels are separated from each other as low as possible, close to the annular plane. The most proximal epicardial course of the left coronary artery is incised in a longitudinal fashion. Then a vertical incision is performed into the left sinus in the direction of the incised left coronary artery (A). Both incisions are joined together and may be approximated at the level where the intramural part of the coronary artery becomes extramural (B). A patch of fresh autologous or xenopericardium is used to enlarge and close the aortocoronary incision and therefore create the neo-ostium in the left sinus. The aortic re-anastomosis is performed, including the top edge of the pericardial patch into the aortic anastomotic suture line (C).View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 10Modified reimplantation of an anomalous left coronary artery with intramural course between the great vessels. For this special malformation, the technique of Pascal Vouhé, which is a further modification of Karl et al's technique, is useful.10Vouhé P. Anomalous origin of a coronary artery is always a surgical disease.Pediatr Cardiac Surg Annu. 2016; 19: 30-36Google Scholar This technique is specially indicated for an anomalous left coronary artery originating from the right sinus of Valsalva and with an intramural course between the great arteries. In such cases, it is recommended to divide the aorta at the level of the sinotubular junction and the main pulmonary artery just above the pulmonary valve commissures. Both vessels are separated from each other as low as possible, close to the annular plane. The most proximal epicardial course of the left coronary artery is incised in a longitudinal fashion. Then a vertical incision is performed into the left sinus in the direction of the incised left coronary artery (A). Both incisions are joined together and may be approximated at the level where the intramural part of the coronary artery becomes extramural (B). A patch of fresh autologous or xenopericardium is used to enlarge and close the aortocoronary incision and therefore create the neo-ostium in the left sinus. The aortic re-anastomosis is performed, including the top edge of the pericardial patch into the aortic anastomotic suture line (C).View Large Image Figure ViewerDownload Hi-res image Download (PPT) Following warm reperfusion blood cardioplegia, the cross-clamp is removed. The suture lines are inspected, and fibrin glue may be applied to improve hemostasis. Atrial and ventricular pacing wires are placed, and the patient is weaned from cardiopulmonary bypass under atrial stimulation 90 beats per minute. Out of our prospective institutional database, we were able to identify 35 patients with anomalous aortic origin of a coronary artery. In 11 patients, the malformation was left without treatment; in 7 patients (mean age: 47 ± 9 years), no surgical intervention was recommended because the patients were asymptomatic: the anomalous coronary did not demonstrate compression or distortion or MRI did not demonstrate exercise-induced ischemia. In 4 patients (mean age 69 ± 11 years), the anomalous origin was discovered incidentally during preoperative assessment before aortic valve replacement, and intraoperative inspection did not justify, in our eyes, a surgical approach.