doi:10.1510/mmcts.2008.003285
Anomalous origin of the left coronary artery from the pulmonary artery: surgical treatment Olivier Raisky, Franc¸ois Roubertie, Walid Ben Ali, Pascal R. Vouhe´* Department of Pediatric Cardiac Surgery, Sick Children Hospital, Paris, France Anomalous origin of the left coronary artery from the pulmonary artery is a rare malformation in which the left coronary artery originates from the pulmonary artery. The consequences are variable although, in most cases, this anomaly leads to severe coronary hypoperfusion and left ventricular dysfunction when pulmonary vascular resistances fall in the postnatal period. Surgical correction is indicated as soon as the diagnosis is established. In nearly all cases, the anomalous artery can be excised from its pulmonary origin, mobilized and reimplanted directly into the ascending aorta. In rare circumstances, technical modifications must be used to restore a normal dual coronary perfusion. The operative risk is related mainly to the severity of preoperative left ventricular dysfunction. The current mortality rate is low, but postoperative left ventricular assist device implantation may be necessary in the most severe cases. After successful revascularization, the late results are satisfactory; left ventricular function always recovers; mitral regurgitation, if present, decreases, although reoperation may be necessary for residual ischemic mitral insufficiency.
Keywords: Anomalous origin of left coronary artery from pulmonary artery; Congenital cardiac defect; Coronary anomaly; Surgical correction Introduction Anomalous origin of the left coronary artery from the pulmonary artery is a rare malformation in which the left coronary artery originates from the pulmonary artery. Other, even more uncommon, anomalies include anomalous origin of the right coronary artery, left anterior descending or circumflex artery. Anomalous origin of both the left and right coronary arteries is extremely rare and nearly uniformly lethal. In the absence of surgical correction, the natural prognosis is poor with an 80–90% mortality rate by one year of age. Various surgical techniques have been * Corresponding
author. Service de Chirurgie Cardiaque Pe´diatrique, Hoˆpital Necker, Enfants Malades, 149 rue de Se`vres, 75015 Paris, France. Tel.: q33-1-4438 1867; fax: q33-1-4438 1911 E-mail: [email protected]
䉷 2008 European Association for Cardio-thoracic Surgery
used to create a dual coronary artery system. Direct aortic reimplantation of the anomalous artery has progressively evolved as the procedure of choice w1x.
Surgical anatomy In most cases, the anomalous left coronary ostium originates from the left posterior pulmonary sinus (socalled right-handed sinus); however, its position within the sinus is extremely variable; particularly it may be very close to a valvular commissure. More rarely, the anomalous ostium originates from the right posterior (left-handed) sinus. Even more uncommonly, the left coronary artery may originate from the anterior (nonfacing) pulmonary sinus, from the posterior wall of the main pulmonary trunk or from the posterior wall of one of the main pulmonary arteries (usually the right one) w2x. 1
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2008.003285 Pathophysiology
Surgical technique
Children with anomalous origin of the left coronary artery from the pulmonary artery develop symptoms days to weeks after birth when the ductus arteriosus closes and pulmonary vascular resistances fall. As long as pulmonary vascular resistances are high, coronary perfusion is maintained even if the anomalous artery carries desaturated blood coming from the pulmonary artery. When pulmonary artery pressure falls, perfusion of the left coronary artery becomes inadequate. The ischemic consequences are variable and depend upon two main factors: coronary dominance and development of intercoronary collaterals.
General principles
If the left coronary artery is dominant and if intercoronary collaterals are inadequate, severe left ventricular dysfunction with ischemic mitral regurgitation develops; the prognosis is poor in the absence of early surgical correction. On the contrary, if the right coronary artery is dominant and if intercoronary collaterals develop efficiently, near normal left coronary perfusion may be maintained while left-to-right shunt from the right coronary artery to the left coronary artery and the pulmonary artery progressively increases; the anomaly may then be discovered during childhood or adulthood in patients with relatively preserved ventricular function.
Diagnosis and indications During infancy, the diagnosis is suspected in a child with severe left ventricular dysfunction and mitral regurgitation. Echocardiographic examination usually shows the anomalous coronary ostium in the pulmonary artery with a left-to-right shunt at Doppler evaluation. Rarely, cardiac catheterization or multislice computed tomographic angiography are necessary to ascertain the diagnosis. Surgical correction is indicated as soon as the diagnosis is made to promote earlier and quicker recovery in left ventricular function. Even if mitral regurgitation is present, mitral surgery is not indicated at this stage because mitral regurgitation always decreases when left ventricular function improves. When the child survives past infancy, the diagnosis may be suspected in a patient with moderate left ventricular dysfunction, mitral regurgitation or ischemic symptoms at exercise. Once the diagnosis is established, surgical correction is indicated to restore a normal dual coronary perfusion. 2
In most patients, particularly in infants, preoperative left ventricular function is poor, often extremely poor. Everything should be done to minimize further ischemic damage. Normothermic cardiopulmonary bypass is usually used, although moderate hypothermia may be necessary to allow low flow bypass if needed. Myocardial preservation is achieved using multidose blood cardioplegia. As soon as bypass is started, a left ventricular vent is inserted through the superior right pulmonary vein and both pulmonary arteries are snared to avoid runoff of coronary perfusion into the pulmonary circulation. The first cardioplegic administration is performed in the aortic root (and thus right coronary artery) and it is completed by direct administration into the anomalous left coronary ostium as soon as the pulmonary trunk has been opened. Subsequent cardioplegic administrations are performed in both coronary ostia. It is very useful to leave (or to create) a calibrated small atrial septal defect as a way to unload the failing left ventricle during the early postoperative period. Immediately after aortic unclamping, a left atrial line is inserted and weaning from cardiopulmonary bypass is prepared. In some patients, weaning can be achieved with an acceptably low left atrial pressure and minimal inotropic support. In most cases however, cardiopulmonary bypass must be prolonged for a while until left atrial pressure (which is initially high) reaches an acceptably low level, allowing weaning with a moderate inotropic support. If this is not the case, a left heart assist device must be implanted to allow cardiac assistance for a few days until left ventricular function recovers enough to allow weaning in good hemodynamic conditions. Usual technique of coronary artery reimplantation (Schematic 1) After aortic clamping and induction of cardioplegic arrest, the main pulmonary trunk is opened and the anomalous left coronary ostium (which originates in most cases from the left posterior pulmonary sinus) is identified. Complete transection of the pulmonary trunk is performed, distal to the coronary ostium (Video 1). The coronary ostium is then excised (Video 2). Sometimes, the ostium is very close to a valvular commissure; the commissure should then be taken down on a few millimeters to allow adequate har-
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2008.003285
Video 2. The anomalous coronary ostium is excised with a generous cuff of pulmonary arterial wall around it.
Using a coronary probe, the main trunk and its division into left anterior descending and circumflex arteries are carefully identified. The main coronary trunk is mobilized down to its bifurcation. This step is performed using electrocautery, great care being taken to achieve perfect hemostasis of the numerous small veins which are usually surrounding the coronary arteries (Video 3). The ascending aorta is opened vertically and the ideal site of reimplantation in the left posterior wall of the aorta is determined. A hole is created using a punch, care being taken not to injure the aortic valve (Video 4).
Schematic 1. Usual technique of coronary artery reimplantation. (A) The pulmonary trunk is opened. The anomalous coronary ostium is identified and excised with a generous cuff. (B) The anomalous ostium is excised. The ascending aorta is opened and the ideal site of reimplantation is selected. (C) The anomalous ostium is reimplanted. A piece of fresh autologous pericardium is used to repair the pulmonary trunk. (D) The pulmonary trunk is reconstructed. The aorta is closed. (Reproduced from Raisky O, Vouhe´ P. Chirurgie des anomalies conge´nitales des arte`res coronaires. Techniques chirurgicales. Thorax 2007;42:710, with permission from Elsevier Masson SAS Paris.)
The coronary button is reimplanted using a running suture. Coronary reimplantation must be achieved without torsion or excessive tension of the coronary button. According to anatomic conditions, suturing may be performed either from inside the aorta or from outside the vessel. The ascending aorta is closed (Video 5). Cardioplegic infusion is administrated into the aorta to insure that coronary perfusion is adequate and perfect hemostasis achieved. The ligamentum arteriosum is divided and the main pulmonary arteries are fully mobilized (Video 6). A fresh piece of autologous pericardium is used to close the gap created in the pulmonary root. The pul-
Video 1. The pulmonary trunk is opened and transected after precise visualization of the abnormal coronary ostium.
vesting of the coronary button; the commissure will be reimplanted onto the pericardial patch used for pulmonary artery repair later on.
Video 3. The main left trunk and its branches are carefully identified. The main trunk is dissected free down to its bifurcation.
3
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2008.003285
Video 4. The ascending aorta is opened vertically. The ideal site of reimplantation is determined. A punch hole is created.
Video 5. The anomalous coronary ostium is reimplanted. The aorta is closed.
Video 6. The pulmonary arteries are mobilized after section of the ligamentum arteriosum.
Schematic 2. Anomalous coronary artery arising from the anterior pulmonary sinus. The anomalous coronary ostium is excised with a circular piece of pulmonary trunk. The pulmonary arterial wall is used to create an extension tube of tissue lengthening the coronary artery. (Reproduced from Raisky O, Vouhe´ P. Chirurgie des anomalies conge´nitales des arte`res coronaires. Techniques chirurgicales. Thorax 2007;42:710, with permission from Elsevier Masson SAS Paris.) Video 7. The main pulmonary trunk is reconstructed using a piece of fresh autologous pericardium.
stances in which technical modifications may be necessary.
monary trunk is reconstructed, avoiding any risk of compression of the reimplanted left main coronary artery (Video 7).
Anomalous origin from the anterior pulmonary sinus (Schematic 2).
Technical variants The previously described technique can be used in nearly all cases. There are, however, two rare circum4
The anomalous coronary ostium is harvested with a circular portion of the pulmonary artery wall. The segment of pulmonary artery is used to create a tubular neo-left main trunk thus allowing standard reimplantation into the aorta. A valvular commissure must
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2008.003285
Video 8. The anomalous coronary ostium from the non-facing sinus is excised with the posterior wall of the pulmonary artery. Posterior commissure of the pulmonary valve is taken down.
Video 9. Reimplantation of the coronary starting from the back wall. Reconstruction of the anterior wall with an autologous pericardium patch.
occasionally be taken down and repaired subsequently. Alternatively, a flap of pulmonary wall may be used to create the posterior wall of the new left coronary trunk while the anterior wall is constructed using a patch of fresh autologous pericardium (Videos 8 and 9). Anomalous origin from the right pulmonary artery with an intramural aortic course w3x (Schematic 3). Very rarely, the anomalous coronary artery originates from the posterior wall of the right pulmonary artery and presents an initial intramural segment within the aortic wall. The ascending aorta and the pulmonary artery are opened and the anomalous ostium is identified. Using a coronary probe, the intramural segment is carefully located. The common wall between the aorta and the coronary artery is excised and the intimas of both vessels are approximated using a series of interrupted absorbable sutures, thus creating a neo-coronary ostium within the aortic lumen. The anomalous ostium in the pulmonary artery is then sutured or the artery ligated at its origin.
Results Between 1986 and 2007, 62 consecutive patients with anomalous left coronary artery arising from the pulmonary artery, underwent isolated aortic reimplantation. There were 23 boys and 39 girls. Mean age was 16 months (range 10 days to 11 years).
Schematic 3. Anomalous coronary artery arising from the right pulmonary artery with an intramural aortic course. (A) The initial segment of the anomalous coronary artery is intramural into the aortic wall. (B) The aorta is opened. The intramural segment is identified and unroofed creating a neo-left coronary ostium. The origin of the anomalous artery is ligated. (Reproduced from Raisky O, Vouhe´ P. Chirurgie des anomalies conge´nitales des arte`res coronaires. Techniques chirurgicales. Thorax 2007;42:710, with permission from Elsevier Masson SAS Paris.)
There were six early deaths (9.7%). The causes of death were: arrhythmias in 2, neurologic complication in 1, multiorgan failure in 1 and intractable low cardiac output syndrome in 2 patients. A left heart assist device was implanted in 4 patients. All were weaned from assistance but two died after weaning (neurologic complication in one, multiorgan failure in one). Early mortality was influenced by the severity of preoperative congestive heart failure, the severity of left ventricular dysfunction and the date of operation. Mean follow-up was 116"78 months (range 3 months to 20 years). There were two sudden deaths at three and seven months. Global left ventricular function recovered to normal in all survivors. Five patients underwent reoperation (three for mitral valvuloplasty, one for surgical angioplasty of the reimplanted left coronary artery and one for mammary bypass).
Discussion Surgical technique Simple ligation of the anomalous coronary artery has been the first procedure to be used; this cannot be 5
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2008.003285 recommended because of high early and late mortality rates. Various procedures have been described to revascularize the anomalous artery using saphenous vein, mammary artery or left subclavian artery. They all have been abandoned except the mammary artery which may be used as a bypass in adult patients when direct aortic reimplantation is deemed technically difficult. The Takeuchi procedure involves creation of a fistula between the aorta and the pulmonary artery and construction of a tunnel within the pulmonary artery to direct blood flow from the aorta to the anomalous coronary ostium w4x. Late complications are not uncommon including progressive stenosis of the intrapulmonary coronary tunnel, aortic valve insufficiency, baffle leak creating coronary–pulmonary artery fistula and supravalvar pulmonary stenosis w5–8x. This procedure cannot be recommended anymore. The development of the arterial switch operation for transposition of the great arteries has shown that manipulating the coronary arteries was feasible even in infants and neonates. Therefore, direct aortic reimplantation of the anomalous left coronary artery has become the procedure of choice. In most cases, the anomalous ostium is arising from the left posterior sinus; aortic reimplantation is usually technically easy. It is crucial to select the ideal site of reimplantation in order to avoid any kinking at the anastomotic site. Opening the ascending aorta provides excellent exposure to achieve this goal. In unusual situations (origin from another pulmonary sinus, pulmonary trunk or right pulmonary artery), technical modifications allow aortic reimplantation w9x. The only situation in which coronary transfer is impossible is represented by anomalous origin from the right pulmonary artery with an intramural aortic course; in this condition, creation of a neo-left coronary ostium is recommended w3x.
Indications In infants with severe left ventricular dysfunction, surgical revascularization is indicated as soon as the diagnosis is established. Delaying surgery to decrease the operative risk cannot be recommended. There is a high risk of sudden death during the waiting period. It has been clearly shown that the recovery of left ventricular function is more rapid and more complete when surgery is performed earlier in life. In older children or adults with preserved left ventricular function, surgery should be performed to restore a normal dual coronary perfusion and avoid the risk of sudden death. Results Early mortality is currently low. However, the severity of preoperative left ventricular dysfunction remains an incremental risk factor; this is particularly true when left ventricular impairment is severe enough to warrant preoperative artificial ventilation and inotropic support. In this high risk subset of patients, perioperative mechanical support with left ventricular assist device and extracorporeal membrane oxygenation has improved survival. The full array of circulatory support should always be readily available if required. Late mortality is very rare although there is a risk of sudden death during the first postoperative months until left ventricular function recovers. Global left ventricular function always improves but normalization of left ventricular function may take several months. Recovery is more rapid and more complete when surgery is performed early to reduce the duration of the initial ischemic insult. Although postoperative normalization of left ventricular function is the rule, some degree of chronic impairment may persist definitively (Table 1, w6–8, 10–15x).
Table 1. Results of surgery for anomalous left coronary artery from the pulmonary artery Author (year)
No. of patients
No. of aortic reimplantations
Early mortality (%)
Need for LVAP/ECMO (%)
Mean follow-up (years)
Late mortality (%)
Reoperation for mitral regurgitation (%)
Schwartz et al. (1997) Cochrane et al. (1999) Huddelston et al. (2001) Isomatsu et al. (2001) Azakie et al. (2003) Michielon et al. (2003) Barth et al. (2003) Caspi et al. (2007) Lange et al. (2007) Personnal experience
42 21 17 29 47 31 10 23 56 62
14 21 16 19 47 14 10 23 31 62
14.3 0 5.8 6.7 8.5 16.1 0 0 14.2 9.7
19 24 18 0 11 0 0 8.6 0 6.5
3.7 6.5 6.9 8.3 4.7 8.8 4.3 6.5 11 9.7
0 0 0 0 0 0 0 0 4.2 3.6
2.8 4.8 12.5 3.7 2.3 3.8 10 0 6.5 5.6
6
(33%) (100%) (94%) (66%) (100%) (45%) (100%) (100%) (55%) (100%)
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2008.003285 Along with left ventricular function improvement, mitral regurgitation, if present, always decreases. In many cases, it even disappears completely. This is the reason why attempting to repair the mitral valve at the time of the initial operation cannot be recommended. However, recovery of normal mitral valve function is usually less rapid than that of left ventricular function. It may even be incomplete and significant mitral regurgitation due to irreversible ischemic damage may persist and need reoperation. When recovery of left ventricular function or improvement in mitral valve regurgitation are slow and incomplete, the suspicion of inadequate coronary revascularization should be raised, which needs to be confirmed by coronary angiography. Stenosis or occlusion of the reimplanted coronary artery may then lead to reoperation.
w7x
w8x
w9x
w10x
References w1x Dodge-Khatami A, Mavroudis C, Backer L. Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy. Ann Thorac Surg 2002;74:946–955. w2x Smith A, Arnold R, Anderson RH, Wilkinson JL, Qureshi SA, Gerlis LM, McKay R. Anomalous origin of the left coronary artery from the pulmonary trunk. Anatomic findings in relation to pathophysiology and surgical repair. J Thorac Cardiovasc Surg 1989;98:16–24. w3x Barbero-Marcial M, Tanamati C, Atik E, Ebaid M, Jatene A. Anomalous origin of the left coronary artery from the pulmonary artery with intramural aortic route: diagnosis and surgical treatment. J Thorac Cardiovasc Surg 1999;117:823–824. w4x Takeuchi S, Imamura H, Katsumoto K, Hayashi I, Katoghi T, Yosu R, Ohkura M, Inoue T. New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg 1979;78:7–11. w5x Bunton R, Jonas RA, Lang P, Rein AJ, Castaneda AR. Anomalous origin of left coronary artery from pulmonary artery. Ligation versus establishment of a two coronary artery system. J Thorac Cardiovasc Surg 1987;93:103–108. w6x Schwartz ML, Jonas RA, Colan SD. Anomalous origin of left coronary artery from pulmonary artery: recovery of left ventricular function after
w11x
w12x
w13x
w14x
w15x
dual coronary repair. J Am Coll Cardiol 1997;30: 547–553. Cochrane AD, Coleman DM, Davis AM, Brizard CP, Wolfe R, Karl TR. Excellent long-term functional outcome after an operation for anomalous left coronary artery from the pulmonary artery. J Thorac Cardiovasc Surg 1999; 117:332–342. Isomatsu Y, Imai Y, Shin’oka T, Aoki M, Iwata Y. Surgical intervention for anomalous origin of the left coronary artery from the pulmonary artery: the Tokyo experience. J Thorac Cardiovasc Surg 2001;121:792–797. Turley K, Szarnicki RJ, Flachsbart KD, Richter RC, Popper RW, Tarnoff H. Aortic implantation is possible in all cases of anomalous origin of the left coronary artery from the pulmonary artery. Ann Thorac Surg 1995;60:84–89. Huddleston CB, Balzer DT, Mendeloff EN. Repair of anomalous left main coronary artery arising from the pulmonary artery in infants: long-term impact on the mitral valve. Ann Thorac Surg 2001;71:1985–1989. Azakie A, Russell JL, McCrindle BW, Van Arsdell GS, Benson LN, Coles JG, Williams WG. Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome. Ann Thorac Surg 2003;75:1535–1541. Michielon G, Di Carlo D, Brancaccio G, Guccione P, Mazzera E, Toscano A, Di Donato RM. Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery. Ann Thorac Surg 2003;76:581–588. Barth MJ, Allen BS, Gulecyuz M, Chiemmongkoltip P, Cuneo B, Ilbawi MN. Experience with an alternative technique for the management of anomalous left coronary artery from the pulmonary artery. Ann Thorac Surg 2003; 76:1429–1434. Caspi J, Pettitt TW, Sperrazza C, Mulder T, Stopa A. Reimplantation of anomalous left coronary artery from the pulmonary artery without mitral valve repair. Ann Thorac Surg 2007;84:619–623. Lange R, Vogt M, Ho¨rer J, Cleuziou J, Menzel A, Holper K, Hess J, Schreiber C. Long-term results of repair of anomalous origin of the left coronary artery from the pulmonary artery. Ann Thorac Surg 2007;83:1463–1471.
7
Anomalous origin of the left coronary artery from the pulmonary artery: surgical treatment Olivier Raisky, Franc¸ois Roubertie, Walid Ben Ali, Pascal R. Vouhe´* Department of Pediatric Cardiac Surgery, Sick Children Hospital, Paris, France Anomalous origin of the left coronary artery from the pulmonary artery is a rare malformation in which the left coronary artery originates from the pulmonary artery. The consequences are variable although, in most cases, this anomaly leads to severe coronary hypoperfusion and left ventricular dysfunction when pulmonary vascular resistances fall in the postnatal period. Surgical correction is indicated as soon as the diagnosis is established. In nearly all cases, the anomalous artery can be excised from its pulmonary origin, mobilized and reimplanted directly into the ascending aorta. In rare circumstances, technical modifications must be used to restore a normal dual coronary perfusion. The operative risk is related mainly to the severity of preoperative left ventricular dysfunction. The current mortality rate is low, but postoperative left ventricular assist device implantation may be necessary in the most severe cases. After successful revascularization, the late results are satisfactory; left ventricular function always recovers; mitral regurgitation, if present, decreases, although reoperation may be necessary for residual ischemic mitral insufficiency.
Keywords: Anomalous origin of left coronary artery from pulmonary artery; Congenital cardiac defect; Coronary anomaly; Surgical correction Introduction Anomalous origin of the left coronary artery from the pulmonary artery is a rare malformation in which the left coronary artery originates from the pulmonary artery. Other, even more uncommon, anomalies include anomalous origin of the right coronary artery, left anterior descending or circumflex artery. Anomalous origin of both the left and right coronary arteries is extremely rare and nearly uniformly lethal. In the absence of surgical correction, the natural prognosis is poor with an 80–90% mortality rate by one year of age. Various surgical techniques have been * Corresponding
author. Service de Chirurgie Cardiaque Pe´diatrique, Hoˆpital Necker, Enfants Malades, 149 rue de Se`vres, 75015 Paris, France. Tel.: q33-1-4438 1867; fax: q33-1-4438 1911 E-mail: [email protected]
䉷 2008 European Association for Cardio-thoracic Surgery
used to create a dual coronary artery system. Direct aortic reimplantation of the anomalous artery has progressively evolved as the procedure of choice w1x.
Surgical anatomy In most cases, the anomalous left coronary ostium originates from the left posterior pulmonary sinus (socalled right-handed sinus); however, its position within the sinus is extremely variable; particularly it may be very close to a valvular commissure. More rarely, the anomalous ostium originates from the right posterior (left-handed) sinus. Even more uncommonly, the left coronary artery may originate from the anterior (nonfacing) pulmonary sinus, from the posterior wall of the main pulmonary trunk or from the posterior wall of one of the main pulmonary arteries (usually the right one) w2x. 1
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2008.003285 Pathophysiology
Surgical technique
Children with anomalous origin of the left coronary artery from the pulmonary artery develop symptoms days to weeks after birth when the ductus arteriosus closes and pulmonary vascular resistances fall. As long as pulmonary vascular resistances are high, coronary perfusion is maintained even if the anomalous artery carries desaturated blood coming from the pulmonary artery. When pulmonary artery pressure falls, perfusion of the left coronary artery becomes inadequate. The ischemic consequences are variable and depend upon two main factors: coronary dominance and development of intercoronary collaterals.
General principles
If the left coronary artery is dominant and if intercoronary collaterals are inadequate, severe left ventricular dysfunction with ischemic mitral regurgitation develops; the prognosis is poor in the absence of early surgical correction. On the contrary, if the right coronary artery is dominant and if intercoronary collaterals develop efficiently, near normal left coronary perfusion may be maintained while left-to-right shunt from the right coronary artery to the left coronary artery and the pulmonary artery progressively increases; the anomaly may then be discovered during childhood or adulthood in patients with relatively preserved ventricular function.
Diagnosis and indications During infancy, the diagnosis is suspected in a child with severe left ventricular dysfunction and mitral regurgitation. Echocardiographic examination usually shows the anomalous coronary ostium in the pulmonary artery with a left-to-right shunt at Doppler evaluation. Rarely, cardiac catheterization or multislice computed tomographic angiography are necessary to ascertain the diagnosis. Surgical correction is indicated as soon as the diagnosis is made to promote earlier and quicker recovery in left ventricular function. Even if mitral regurgitation is present, mitral surgery is not indicated at this stage because mitral regurgitation always decreases when left ventricular function improves. When the child survives past infancy, the diagnosis may be suspected in a patient with moderate left ventricular dysfunction, mitral regurgitation or ischemic symptoms at exercise. Once the diagnosis is established, surgical correction is indicated to restore a normal dual coronary perfusion. 2
In most patients, particularly in infants, preoperative left ventricular function is poor, often extremely poor. Everything should be done to minimize further ischemic damage. Normothermic cardiopulmonary bypass is usually used, although moderate hypothermia may be necessary to allow low flow bypass if needed. Myocardial preservation is achieved using multidose blood cardioplegia. As soon as bypass is started, a left ventricular vent is inserted through the superior right pulmonary vein and both pulmonary arteries are snared to avoid runoff of coronary perfusion into the pulmonary circulation. The first cardioplegic administration is performed in the aortic root (and thus right coronary artery) and it is completed by direct administration into the anomalous left coronary ostium as soon as the pulmonary trunk has been opened. Subsequent cardioplegic administrations are performed in both coronary ostia. It is very useful to leave (or to create) a calibrated small atrial septal defect as a way to unload the failing left ventricle during the early postoperative period. Immediately after aortic unclamping, a left atrial line is inserted and weaning from cardiopulmonary bypass is prepared. In some patients, weaning can be achieved with an acceptably low left atrial pressure and minimal inotropic support. In most cases however, cardiopulmonary bypass must be prolonged for a while until left atrial pressure (which is initially high) reaches an acceptably low level, allowing weaning with a moderate inotropic support. If this is not the case, a left heart assist device must be implanted to allow cardiac assistance for a few days until left ventricular function recovers enough to allow weaning in good hemodynamic conditions. Usual technique of coronary artery reimplantation (Schematic 1) After aortic clamping and induction of cardioplegic arrest, the main pulmonary trunk is opened and the anomalous left coronary ostium (which originates in most cases from the left posterior pulmonary sinus) is identified. Complete transection of the pulmonary trunk is performed, distal to the coronary ostium (Video 1). The coronary ostium is then excised (Video 2). Sometimes, the ostium is very close to a valvular commissure; the commissure should then be taken down on a few millimeters to allow adequate har-
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2008.003285
Video 2. The anomalous coronary ostium is excised with a generous cuff of pulmonary arterial wall around it.
Using a coronary probe, the main trunk and its division into left anterior descending and circumflex arteries are carefully identified. The main coronary trunk is mobilized down to its bifurcation. This step is performed using electrocautery, great care being taken to achieve perfect hemostasis of the numerous small veins which are usually surrounding the coronary arteries (Video 3). The ascending aorta is opened vertically and the ideal site of reimplantation in the left posterior wall of the aorta is determined. A hole is created using a punch, care being taken not to injure the aortic valve (Video 4).
Schematic 1. Usual technique of coronary artery reimplantation. (A) The pulmonary trunk is opened. The anomalous coronary ostium is identified and excised with a generous cuff. (B) The anomalous ostium is excised. The ascending aorta is opened and the ideal site of reimplantation is selected. (C) The anomalous ostium is reimplanted. A piece of fresh autologous pericardium is used to repair the pulmonary trunk. (D) The pulmonary trunk is reconstructed. The aorta is closed. (Reproduced from Raisky O, Vouhe´ P. Chirurgie des anomalies conge´nitales des arte`res coronaires. Techniques chirurgicales. Thorax 2007;42:710, with permission from Elsevier Masson SAS Paris.)
The coronary button is reimplanted using a running suture. Coronary reimplantation must be achieved without torsion or excessive tension of the coronary button. According to anatomic conditions, suturing may be performed either from inside the aorta or from outside the vessel. The ascending aorta is closed (Video 5). Cardioplegic infusion is administrated into the aorta to insure that coronary perfusion is adequate and perfect hemostasis achieved. The ligamentum arteriosum is divided and the main pulmonary arteries are fully mobilized (Video 6). A fresh piece of autologous pericardium is used to close the gap created in the pulmonary root. The pul-
Video 1. The pulmonary trunk is opened and transected after precise visualization of the abnormal coronary ostium.
vesting of the coronary button; the commissure will be reimplanted onto the pericardial patch used for pulmonary artery repair later on.
Video 3. The main left trunk and its branches are carefully identified. The main trunk is dissected free down to its bifurcation.
3
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2008.003285
Video 4. The ascending aorta is opened vertically. The ideal site of reimplantation is determined. A punch hole is created.
Video 5. The anomalous coronary ostium is reimplanted. The aorta is closed.
Video 6. The pulmonary arteries are mobilized after section of the ligamentum arteriosum.
Schematic 2. Anomalous coronary artery arising from the anterior pulmonary sinus. The anomalous coronary ostium is excised with a circular piece of pulmonary trunk. The pulmonary arterial wall is used to create an extension tube of tissue lengthening the coronary artery. (Reproduced from Raisky O, Vouhe´ P. Chirurgie des anomalies conge´nitales des arte`res coronaires. Techniques chirurgicales. Thorax 2007;42:710, with permission from Elsevier Masson SAS Paris.) Video 7. The main pulmonary trunk is reconstructed using a piece of fresh autologous pericardium.
stances in which technical modifications may be necessary.
monary trunk is reconstructed, avoiding any risk of compression of the reimplanted left main coronary artery (Video 7).
Anomalous origin from the anterior pulmonary sinus (Schematic 2).
Technical variants The previously described technique can be used in nearly all cases. There are, however, two rare circum4
The anomalous coronary ostium is harvested with a circular portion of the pulmonary artery wall. The segment of pulmonary artery is used to create a tubular neo-left main trunk thus allowing standard reimplantation into the aorta. A valvular commissure must
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2008.003285
Video 8. The anomalous coronary ostium from the non-facing sinus is excised with the posterior wall of the pulmonary artery. Posterior commissure of the pulmonary valve is taken down.
Video 9. Reimplantation of the coronary starting from the back wall. Reconstruction of the anterior wall with an autologous pericardium patch.
occasionally be taken down and repaired subsequently. Alternatively, a flap of pulmonary wall may be used to create the posterior wall of the new left coronary trunk while the anterior wall is constructed using a patch of fresh autologous pericardium (Videos 8 and 9). Anomalous origin from the right pulmonary artery with an intramural aortic course w3x (Schematic 3). Very rarely, the anomalous coronary artery originates from the posterior wall of the right pulmonary artery and presents an initial intramural segment within the aortic wall. The ascending aorta and the pulmonary artery are opened and the anomalous ostium is identified. Using a coronary probe, the intramural segment is carefully located. The common wall between the aorta and the coronary artery is excised and the intimas of both vessels are approximated using a series of interrupted absorbable sutures, thus creating a neo-coronary ostium within the aortic lumen. The anomalous ostium in the pulmonary artery is then sutured or the artery ligated at its origin.
Results Between 1986 and 2007, 62 consecutive patients with anomalous left coronary artery arising from the pulmonary artery, underwent isolated aortic reimplantation. There were 23 boys and 39 girls. Mean age was 16 months (range 10 days to 11 years).
Schematic 3. Anomalous coronary artery arising from the right pulmonary artery with an intramural aortic course. (A) The initial segment of the anomalous coronary artery is intramural into the aortic wall. (B) The aorta is opened. The intramural segment is identified and unroofed creating a neo-left coronary ostium. The origin of the anomalous artery is ligated. (Reproduced from Raisky O, Vouhe´ P. Chirurgie des anomalies conge´nitales des arte`res coronaires. Techniques chirurgicales. Thorax 2007;42:710, with permission from Elsevier Masson SAS Paris.)
There were six early deaths (9.7%). The causes of death were: arrhythmias in 2, neurologic complication in 1, multiorgan failure in 1 and intractable low cardiac output syndrome in 2 patients. A left heart assist device was implanted in 4 patients. All were weaned from assistance but two died after weaning (neurologic complication in one, multiorgan failure in one). Early mortality was influenced by the severity of preoperative congestive heart failure, the severity of left ventricular dysfunction and the date of operation. Mean follow-up was 116"78 months (range 3 months to 20 years). There were two sudden deaths at three and seven months. Global left ventricular function recovered to normal in all survivors. Five patients underwent reoperation (three for mitral valvuloplasty, one for surgical angioplasty of the reimplanted left coronary artery and one for mammary bypass).
Discussion Surgical technique Simple ligation of the anomalous coronary artery has been the first procedure to be used; this cannot be 5
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2008.003285 recommended because of high early and late mortality rates. Various procedures have been described to revascularize the anomalous artery using saphenous vein, mammary artery or left subclavian artery. They all have been abandoned except the mammary artery which may be used as a bypass in adult patients when direct aortic reimplantation is deemed technically difficult. The Takeuchi procedure involves creation of a fistula between the aorta and the pulmonary artery and construction of a tunnel within the pulmonary artery to direct blood flow from the aorta to the anomalous coronary ostium w4x. Late complications are not uncommon including progressive stenosis of the intrapulmonary coronary tunnel, aortic valve insufficiency, baffle leak creating coronary–pulmonary artery fistula and supravalvar pulmonary stenosis w5–8x. This procedure cannot be recommended anymore. The development of the arterial switch operation for transposition of the great arteries has shown that manipulating the coronary arteries was feasible even in infants and neonates. Therefore, direct aortic reimplantation of the anomalous left coronary artery has become the procedure of choice. In most cases, the anomalous ostium is arising from the left posterior sinus; aortic reimplantation is usually technically easy. It is crucial to select the ideal site of reimplantation in order to avoid any kinking at the anastomotic site. Opening the ascending aorta provides excellent exposure to achieve this goal. In unusual situations (origin from another pulmonary sinus, pulmonary trunk or right pulmonary artery), technical modifications allow aortic reimplantation w9x. The only situation in which coronary transfer is impossible is represented by anomalous origin from the right pulmonary artery with an intramural aortic course; in this condition, creation of a neo-left coronary ostium is recommended w3x.
Indications In infants with severe left ventricular dysfunction, surgical revascularization is indicated as soon as the diagnosis is established. Delaying surgery to decrease the operative risk cannot be recommended. There is a high risk of sudden death during the waiting period. It has been clearly shown that the recovery of left ventricular function is more rapid and more complete when surgery is performed earlier in life. In older children or adults with preserved left ventricular function, surgery should be performed to restore a normal dual coronary perfusion and avoid the risk of sudden death. Results Early mortality is currently low. However, the severity of preoperative left ventricular dysfunction remains an incremental risk factor; this is particularly true when left ventricular impairment is severe enough to warrant preoperative artificial ventilation and inotropic support. In this high risk subset of patients, perioperative mechanical support with left ventricular assist device and extracorporeal membrane oxygenation has improved survival. The full array of circulatory support should always be readily available if required. Late mortality is very rare although there is a risk of sudden death during the first postoperative months until left ventricular function recovers. Global left ventricular function always improves but normalization of left ventricular function may take several months. Recovery is more rapid and more complete when surgery is performed early to reduce the duration of the initial ischemic insult. Although postoperative normalization of left ventricular function is the rule, some degree of chronic impairment may persist definitively (Table 1, w6–8, 10–15x).
Table 1. Results of surgery for anomalous left coronary artery from the pulmonary artery Author (year)
No. of patients
No. of aortic reimplantations
Early mortality (%)
Need for LVAP/ECMO (%)
Mean follow-up (years)
Late mortality (%)
Reoperation for mitral regurgitation (%)
Schwartz et al. (1997) Cochrane et al. (1999) Huddelston et al. (2001) Isomatsu et al. (2001) Azakie et al. (2003) Michielon et al. (2003) Barth et al. (2003) Caspi et al. (2007) Lange et al. (2007) Personnal experience
42 21 17 29 47 31 10 23 56 62
14 21 16 19 47 14 10 23 31 62
14.3 0 5.8 6.7 8.5 16.1 0 0 14.2 9.7
19 24 18 0 11 0 0 8.6 0 6.5
3.7 6.5 6.9 8.3 4.7 8.8 4.3 6.5 11 9.7
0 0 0 0 0 0 0 0 4.2 3.6
2.8 4.8 12.5 3.7 2.3 3.8 10 0 6.5 5.6
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(33%) (100%) (94%) (66%) (100%) (45%) (100%) (100%) (55%) (100%)
O. Raisky et al. / Multimedia Manual of Cardiothoracic Surgery / doi:10.1510/mmcts.2008.003285 Along with left ventricular function improvement, mitral regurgitation, if present, always decreases. In many cases, it even disappears completely. This is the reason why attempting to repair the mitral valve at the time of the initial operation cannot be recommended. However, recovery of normal mitral valve function is usually less rapid than that of left ventricular function. It may even be incomplete and significant mitral regurgitation due to irreversible ischemic damage may persist and need reoperation. When recovery of left ventricular function or improvement in mitral valve regurgitation are slow and incomplete, the suspicion of inadequate coronary revascularization should be raised, which needs to be confirmed by coronary angiography. Stenosis or occlusion of the reimplanted coronary artery may then lead to reoperation.
w7x
w8x
w9x
w10x
References w1x Dodge-Khatami A, Mavroudis C, Backer L. Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy. Ann Thorac Surg 2002;74:946–955. w2x Smith A, Arnold R, Anderson RH, Wilkinson JL, Qureshi SA, Gerlis LM, McKay R. Anomalous origin of the left coronary artery from the pulmonary trunk. Anatomic findings in relation to pathophysiology and surgical repair. J Thorac Cardiovasc Surg 1989;98:16–24. w3x Barbero-Marcial M, Tanamati C, Atik E, Ebaid M, Jatene A. Anomalous origin of the left coronary artery from the pulmonary artery with intramural aortic route: diagnosis and surgical treatment. J Thorac Cardiovasc Surg 1999;117:823–824. w4x Takeuchi S, Imamura H, Katsumoto K, Hayashi I, Katoghi T, Yosu R, Ohkura M, Inoue T. New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg 1979;78:7–11. w5x Bunton R, Jonas RA, Lang P, Rein AJ, Castaneda AR. Anomalous origin of left coronary artery from pulmonary artery. Ligation versus establishment of a two coronary artery system. J Thorac Cardiovasc Surg 1987;93:103–108. w6x Schwartz ML, Jonas RA, Colan SD. Anomalous origin of left coronary artery from pulmonary artery: recovery of left ventricular function after
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dual coronary repair. J Am Coll Cardiol 1997;30: 547–553. Cochrane AD, Coleman DM, Davis AM, Brizard CP, Wolfe R, Karl TR. Excellent long-term functional outcome after an operation for anomalous left coronary artery from the pulmonary artery. J Thorac Cardiovasc Surg 1999; 117:332–342. Isomatsu Y, Imai Y, Shin’oka T, Aoki M, Iwata Y. Surgical intervention for anomalous origin of the left coronary artery from the pulmonary artery: the Tokyo experience. J Thorac Cardiovasc Surg 2001;121:792–797. Turley K, Szarnicki RJ, Flachsbart KD, Richter RC, Popper RW, Tarnoff H. Aortic implantation is possible in all cases of anomalous origin of the left coronary artery from the pulmonary artery. Ann Thorac Surg 1995;60:84–89. Huddleston CB, Balzer DT, Mendeloff EN. Repair of anomalous left main coronary artery arising from the pulmonary artery in infants: long-term impact on the mitral valve. Ann Thorac Surg 2001;71:1985–1989. Azakie A, Russell JL, McCrindle BW, Van Arsdell GS, Benson LN, Coles JG, Williams WG. Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: early survival, patterns of ventricular recovery and late outcome. Ann Thorac Surg 2003;75:1535–1541. Michielon G, Di Carlo D, Brancaccio G, Guccione P, Mazzera E, Toscano A, Di Donato RM. Anomalous coronary artery origin from the pulmonary artery: correlation between surgical timing and left ventricular function recovery. Ann Thorac Surg 2003;76:581–588. Barth MJ, Allen BS, Gulecyuz M, Chiemmongkoltip P, Cuneo B, Ilbawi MN. Experience with an alternative technique for the management of anomalous left coronary artery from the pulmonary artery. Ann Thorac Surg 2003; 76:1429–1434. Caspi J, Pettitt TW, Sperrazza C, Mulder T, Stopa A. Reimplantation of anomalous left coronary artery from the pulmonary artery without mitral valve repair. Ann Thorac Surg 2007;84:619–623. Lange R, Vogt M, Ho¨rer J, Cleuziou J, Menzel A, Holper K, Hess J, Schreiber C. Long-term results of repair of anomalous origin of the left coronary artery from the pulmonary artery. Ann Thorac Surg 2007;83:1463–1471.
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