Anomalous Origin of Left Coronary Artery From Pulmonary Artery in Older Children and Adults: Direct Aortic Implantation

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1 Anomalous Origin of Left Coronary Artery From Pulmonary Artery in Older Children and Adults: Direct Aortic Implantation Brijesh P. Kottayil, MS, Karunakaran Jayakumar, MCh, Baiju S. Dharan, MCh, Vivek V. Pillai, MCh, Valaparambil Ajitkumar, MD, Sabarinath Menon, MS, and Ganapathi Sanjay, MD Departments of Cardiovascular and Thoracic Surgery, and Cardiology, Sree Chitra Tirunal Institute For Medical Science and Technology, Trivandrum, India Background. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presents rarely in adulthood and is treated by different surgical techniques with varying results. This study was undertaken to evaluate the feasibility of reestablishment of a dual coronary system in older children and adults with emphasis on direct aortic implantation. Methods. From 2002 to 2010, 10 patients aged 10 to 51 years (median age, 24 years) underwent surgical correction of ALCAPA. Median weight was 44.5 kg. All the patients except 2 were symptomatic. Two patients were in atrial fibrillation. Eight patients had varying degrees of mitral regurgitation, which was severe in 2. In all patients, the left coronary artery was arising from the facing sinus of the pulmonary artery. Direct left main coronary implantation into the aorta was feasible in all patients. Mitral valve replacement was done in 4 patients. Results. At a median follow-up of 5.5 years there was no new angina or infarction. One patient died 3 months postsurgery due to intracerebral bleed. Echocardiography demonstrated normal antegrade flow in the transferred left coronary artery, with 2 patients having persistent moderate mitral regurgitation. Coronary angiography done on follow-up in one patient revealed a patent transferred left coronary artery with distal development of new atherosclerotic lesion. Conclusions. Direct reimplantation of the ALCAPA is considered technically more difficult and hazardous in adults. With increased experience with coronary transfer technique, direct aortic implantation is feasible in adult cases. This provides a more physiologic correction and reestablishment of a dual coronary system with a better outcome. (Ann Thorac Surg 2011;91:549 54) 2011 by The Society of Thoracic Surgeons Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital anomaly. It is usually seen as an isolated lesion and is present in one in 300,000 live births [1]. If left untreated it results in a mortality rate of up to 90% within the first year of life. Ten percent to 15% of patients survive to adulthood aided by a large dominant right coronary artery with extensive intercoronary collaterals [2]. In this subset of patients, there is an estimated 80% to 90% incidence of sudden death at a mean age of 35 years [2 4]. In adults, malignant arrhythmia leading to sudden death may be the first manifestation of the anomaly and they are found to disappear after surgical correction [5]. These findings indicate the severity of the ischemic process in spite of absence of symptoms and justify surgical correction as soon as the diagnosis Accepted for publication Aug 19, Address correspondence to Dr Kottayil, Department of Cardiovascular and Thoracic Surgery, Sree Chitra Tirunal Institute for Medical Science and Technology, Medical College, Trivandrum, Kerala, , India; drbrijeshpk00@gmail.com. is made, regardless of age or degree of intercoronary collateralization. Management of ALCAPA in adults is controversial. Various surgical methods have been attempted, including simple ligation of the anomalous left coronary artery, different types of bypass grafts from the aorta, Takeuchi procedure, and direct reimplantation of the coronary artery into the aorta [4, 6]. It is considered better to have a dual coronary system to avoid the sudden deaths found with other modalities of treatment [7]. Direct reimplantation of the ALCAPA is considered more demanding in adults due to unfavorable anatomy, excessive collateralization, increased coronary artery friability, diminished vessel elasticity for mobilization, and the potential for tearing and resultant catastrophic bleeding [4, 6, 8]. The advantage of direct coronary transfer is that it provides a dual coronary system avoiding the problem of late graft stenosis. Patients and Methods A retrospective analysis of our hospital data revealed 10 older patients who underwent surgical repair of 2011 by The Society of Thoracic Surgeons /$36.00 Published by Elsevier Inc doi: /j.athoracsur

2 550 KOTTAYIL ET AL Ann Thorac Surg ANOMALOUS ORIGIN OF LCA FROM PA 2011;91: ALCAPA between 2002 and The study was approved by the hospital Medical Ethics Review Committee and individual consent for the study was waived. Median age of the patients was 24 years (range, 10 to 51 years). There were 7 males and 3 females. Weight of the patients ranged between 21 and 76 kg (median weight of 44.5 kg). The most common presenting symptom was progressive dyspnea on exertion. Angina on exertion was present in 2 patients. Two patients presented with congestive cardiac failure, which was precipitated by atrial fibrillation (Table 1). Electrocardiography showed evidence of ischemia in the left coronary artery territory in 5 patients. Diagnosis was primarily by transthoracic echocardiography, which showed the anomalous left coronary artery arising from the facing sinus of the pulmonary artery in all the patients (Table 2). Regional wall motion abnormality in the left coronary artery territory was noticed in 4 patients. Two patients had severe mitral regurgitation while moderate regurgitation was present in 4. Six patients underwent coronary angiography, which confirmed the diagnosis (Fig 1). Surgical Technique Cardiopulmonary bypass was established with high ascending aortic and right atrial cannulation. The left atrium was vented through the right superior pulmonary vein. Cardioplegic arrest was achieved by injecting cold cardioplegic solution through the aortic root while simultaneously occluding the main pulmonary artery (MPA) or branch pulmonary arteries individually. The MPA was transected below the bifurcation, the ostium of the left coronary artery identified, and the coronary button developed. Adequate mobilization of the left coronary artery was done with proper control of all the collaterals to facilitate tension-free anastomosis. An opening was made in the corresponding sinus of the aorta at a slightly higher level to avoid the commissures and coronary button transfer was completed using 6-0 polypropylene. The aortic cross-clamp was then removed and end-toend anastomosis of the MPA completed. A tanned pericardial patch was used for MPA augmentation in those Table 2. Echocardiography Details LA left atrium; Mitral Regurgitation Mild 2 Moderate 4 Severe 2 Ejection fraction Giant LA 1 Severe TR 1 TR tricuspid regurgitation. cases where a direct anastomosis would result in excessive tension at the anastomosis site. Four patients underwent concomitant mitral valve replacement with total chordal preservation using mechanical valve prostheses. Among those who underwent mitral valve replacement, two patients had severe mitral regurgitation. In one patient the entire mitral annulus was calcific. One patient who presented with congestive cardiac failure was found to have severe tricuspid regurgitation and underwent tricuspid annuloplasty (DeVega) along with coronary button transfer and mitral valve replacement. A giant left atrium was noticed in one patient who underwent left atrial plication (Kawazoe) and left atrial appendage exclusion. The mean aortic cross-clamp time and cardiopulmonary bypass time were and minutes, respectively. One patient required total circulatory arrest of 20 minutes. Except for one patient in whom delayed sternal closure was done, all the other patients were Table 1. Clinical Profile of Operated Patients Age Sex Duration Presenting Complaint 11 F 2 years DOE 13 M Asymptomatic 35 M 1 year AOE 51 F 1 Year DOE, CCF 34 F Asymptomatic 10 M 1 year DOE 31 M 2 years DOE 10 M 1 year DOE 17 M 2 years DOE, CCF 41 M 6 months AOE AOE angina on exertion; CCF congestive cardiac failure; DOE dyspnea on exertion; F female; M male. Fig 1. Anomalous left coronary artery from the pulmonary artery (arrow).

3 Ann Thorac Surg KOTTAYIL ET AL 2011;91: ANOMALOUS ORIGIN OF LCA FROM PA 551 extubated within 24 hours and inotropic supports weaned off by 48 hours. Data Collection and Statistical Analysis Clinical and operative data of all the patients were obtained from the hospital records and analyzed retrospectively. All patients were followed up yearly with detailed clinical examination, electrocardiography, and complete echocardiography. Descriptive data for continuous variables are presented as mean standard deviation or median with ranges, as appropriate. Results Follow-up was completed in all patients, with a median follow-up of 5.5 years. Echocardiography, done yearly on follow-up, demonstrated antegrade flow in the new coronary artery system without any pulmonary artery obstruction. Electrocardiography did not reveal any new onset ischemic changes. Prosthetic valve function was normal in all four patients who underwent mitral valve replacement. On follow-up, moderate mitral regurgitation was found to persist in two patients in whom coronary button transfer alone was done with conservative management of the mitral valve. Aortic valve function was normal in all patients. One patient, who presented with congestive cardiac failure and underwent direct aortic translocation of ALCAPA with mitral valve replacement, expired 3 months postsurgery due to anticoagulant-related intracerebral bleed. This was the only late mortality in our series. Cardiac catheterization was done 3 years after surgery in one patient who complained of atypical chest pain, and which revealed normal flow across the transferred left coronary button (Figs 2 and 3). The right coronary artery was dilated with regression of most of the collateral vessels. A new atherosclerotic Fig 3. Reconstructed main pulmonary artery using tanned pericardium, postcoronary button transfer. lesion was noticed in the ostium of the posterior descending artery (Fig 4). Comment Adults with ALCAPA may remain asymptomatic despite subclinical myocardial ischemia. Without surgical inter- Fig 2. Patent transferred left main coronary artery with absent stenosis. Fig 4. Coronary angiogram done 3 years after Anomalous left coronary artery from the pulmonary artery correction revealed dilated right coronary artery with regression of most of the collateral vessels. New atherosclerotic lesion noticed in the ostium of posterior descending artery (arrow).

4 552 KOTTAYIL ET AL Ann Thorac Surg ANOMALOUS ORIGIN OF LCA FROM PA 2011;91: vention they are prone for malignant ventricular arrhythmias and sudden death secondary to myocardial ischemia, and global cardiomyopathy, with an estimated incidence of 80% to 90% at a mean age of 35 years [4]. In 1974, Neches and associates [9] were the first to describe the direct reimplantation of the anomalous left coronary artery into the aorta by transferring it with a button of the pulmonary artery. This currently represents the most popular and fully anatomic correction and is considered as the method of choice in infants [10 12]. In adults various techniques are being employed with an aim to establish a dual coronary system. In some adults, with chronic myocardial ischemia and irreversible damage to the heart, cardiac transplantation might be the only option [13]. Simple ligation of ALPACA at its origin was the first accepted definitive surgical correction for this condition in adults [14]. Later in these patients, thallium and stress electrocardiography showed definite evidence of silent myocardial ischemia [15]. Moreover, on follow-up an unacceptable incidence of sudden death was also noticed [16, 17]. Simple ligation results in myocardium dependent on a single coronary artery supply and any significant atherosclerotic change can be fatal. Reduced longterm survival, persistence of left ventricular dilatation, and nonimprovement of ejection fraction on follow-up have all resulted in avoidance of simple ligation as a primary modality of management of ALCAPA in adults [10, 16, 18]. Establishment of a dual coronary system is the accepted norm in the repair of ALCAPA in adults today. The return of antegrade flow in the left coronary artery has been associated with reduction in size of the previously dilated right coronary artery with regression of the intercoronary collateral network. This can be achieved with various techniques as saphenous vein graft, left subclavian artery anastomosis, left internal mammary graft, Takeuchi procedure, or direct implantation [10 12, 19, 20]. Direct aortic reimplantation is the most frequently adopted technique in infants [8], while the technical problems associated with coronary button transfer, presence of extensive intercoronary collaterals, and age of the patient have favored interruption of the fistulous tract with graft to the left coronary artery in adults [8, 20]. Experience with the saphenous vein has resulted in its usage as a conduit for repair of ALCAPA in adults. Moodie and associates [2] reported a graft patency of 80% at a mean follow-up of 5.8 years after its usage in 6 adult patients with ALCAPA. Excellent patency was noticed with the left internal mammary artery as a conduit in coronary artery bypass grafts, and has later resulted in its preference as conduits for repair of ALCAPA. Kitamura and associates [21] successfully used bilateral internal mammary artery grafting to the left anterior descending and circumflex coronary arteries in the repair of an adolescent patient with ALCAPA. Murala and associates [20] repaired 3 adult patients with ALCAPA by transpulmonary pericardial patch closure of the fistula with concomitant left internal mammary artery anastomosis to the left anterior descending artery. Even though left internal mammary artery as conduit is considered as a primary modality of treatment in adults, data regarding graft patency or long-term results after its use as conduits for ALCAPA repair are unavailable [10, 22]. The use of a tunnel within the pulmonary trunk to baffle the anomalous connecting coronary artery to the aorta has been used extensively in children. Takeuchi repair has its own share of complications like supravalvar pulmonary stenosis, baffle obstruction or leakage, and aortic insufficiency [6, 8]. Even though it is an attractive option, very few published reports are available of its usage in adults [4, 23]. Unlike those patients with coronary artery disease who undergo bypass grafts, surgical repair of ALCAPA in adults is done at a younger age and the grafts initially have to deal with competitive flow from the systemic collaterals. Coronary angiogram done 3 years after surgical repair of ALCAPA in a young adult in our series revealed recent significant atherosclerotic change in the coronary artery with regression of intercoronary collaterals. Extrapolating the natural course of bypass grafts for stenosis and the tendency of ALCAPA patients to develop atherosclerosis, it may be presumed that if there is late graft obstruction, it will give rise to equivalent left main disease. Hence, we do feel that, as in infants, direct aortic implantation should be considered as the primary option for repair of ALCAPA in adults. If safely performed, this results in restoration of normal physiologic coronary perfusion and preservation of internal mammary vessels for later usage. Meticulous surgical techniques have to be employed to reduce the complications during coronary button transfer. Over a period of time these patients develop excessive collateralization and hence we systematically clip all tissues with potential for bleeding during coronary mobilization. The coronary ostium is usually excised with a wide cuff of pulmonary arterial wall, which facilitates button anastomosis without coronary injury. When the coronary artery arises from the facing pulmonary sinus, as was the case in all our patients, limited mobilization allows a tension-free anastomosis. In those rare patients when the anomalous coronary artery originates from the nonfacing pulmonary sinus, we feel that a combination of aortic and pulmonary flaps facilitates coronary transfer avoiding extensive coronary mobilization and its inherent complication. Combination of mitral annular dilatation and papillary muscle infarction with regional wall dyskinesia of the left ventricle can lead to progressive mitral regurgitation [20]. Establishment of a dual coronary system with conservative management of even severe mitral regurgitation has yielded good results in infants with ALCAPA [19]. There are practically no fixed guidelines for the management of mitral regurgitation in adult patients with ALCAPA. Given the long-standing effects of severe mitral regurgitation in adults and the lessons learned from dealing ischemic mitral regurgitation, it seems wise to preferably repair or replace the mitral valve whenever these patients have moderate to severe mitral regurgitation. Moreover, on follow-up moderate mitral regurgitation

5 Ann Thorac Surg KOTTAYIL ET AL 2011;91: ANOMALOUS ORIGIN OF LCA FROM PA 553 was found to persist in both the patients in whom ALCAPA alone was surgically addressed. The limitation of the present study is that it is retrospective and performed in a nonrandomized fashion. A longer follow-up to assess the tendency of adult ALCAPA patients for development of atherosclerosis may narrow our options for optimal surgical repair. At present there are no randomized control trials or long-term follow-up studies to compare the different surgical techniques for repair of ALCAPA in adults. It would be appropriate to speculate that successful surgical repair depends on the establishment of a dual coronary system. Direct aortic implantation of the ALCAPA, although technically more challenging is feasible in adults with favorable anatomy and avoids the problem of late graft stenosis. References 1. Keith JD. The anomalous origin of the left coronary artery from the pulmonary artery. Br Heart J 1959;21: Moodie DS, Fyfe D, Gill CC, et al. Anomalous origin of the left coronary artery from the pulmonary artery (Bland- White-Garland syndrome) in adult patients: long-term follow-up after surgery. Am Heart J 1983;106: Arciniegas E, Farooki ZQ, Hakimi M, Green EW. Management of anomalous left coronary artery from the pulmonary artery. Circulation 1980;62(2 Pt 2):I Alexi-Meskishvili V, Berger F, Weng Y, Lange PE, Hetzer R. Anomalous origin of the left coronary artery from the pulmonary artery in adults. J Card Surg 1995;10(4 Pt 1): Frapier JM, Leclercq F, Bodino M, Chaptal PA. Malignant ventricular arrhythmias revealing anomalous origin of the left coronary artery from the pulmonary artery in two adults. Eur J Cardiothorac Surg 1999;15: Chan RK, Hare DL, Buxton BF. Anomalous left main coronary artery arising from the pulmonary artery in an adult: treatment by internal mammary artery grafting. J Thorac Cardiovasc Surg 1995;109: Wilson CL, Dlabal PW, McGuire SA. Surgical treatment of anomalous left coronary artery from pulmonary artery: follow-up in teenagers and adults. Am Heart J 1979;98: Dodge-Khatami A, Mavroudis C, Backer CL. Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy. Ann Thorac Surg 2002;74: Neches WH, Mathews RA, Park SC, et al. Anomalous origin of the left coronary artery from the pulmonary artery. A new method of surgical repair. Circulation 1974;50: Backer CL, Stout MJ, Zales VR, et al. Anomalous origin of the left coronary artery. A twenty-year review of surgical management. J Thorac Cardiovasc Surg 1992;103: Lambert V, Touchot A, Losay J, et al. Midterm results after surgical repair of the anomalous origin of the coronary artery. Circulation 1996;94(9 Suppl): Stern H, Sauer U, Locher D, et al. Left ventricular function assessed with echocardiography and myocardial perfusion assessed with scintigraphy under dipyridamole stress in pediatric patients after repair for anomalous origin of the left coronary artery from the pulmonary artery. J Thorac Cardiovasc Surg 1993;106: Nair KK, Zisman LS, Lader E, Dimova A, Canver CC. Heart transplant for anomalous origin of left coronary artery from pulmonary artery. Ann Thorac Surg 2003;75: Sabiston DC JR, Neill CA, Taussig HB. The direction of blood flow in anomalous left coronary artery arising from the pulmonary artery. Circulation 1960;22: Kececioglu D, Voth E, Morguet A, Munz DL, Vogt J. Myocardial ischemia and left-ventricular function after ligation of left coronary artery (Bland-White-Garland syndrome): a long-term follow-up. Thorac Cardiovasc Surg 1992;40: Wollenek G, Domanig E, Salzer-Muhar U, Havel M, Wimmer M, Wolner E. Anomalous origin of the left coronary artery: a review of surgical management in 13 patients. J Cardiovasc Surg (Torino) 1993;34: 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 system. J Thorac Cardiovasc Surg 1987;93: Gardner TJ, Greene PS, Rykiel MF, et al. Routine use of the left internal mammary artery in the elderly. Ann Thorac Surg 1990;49: Vouhé PR, Tamisier D, Sidi D, et al. Anomalous left coronary artery from the pulmonary artery: results of isolated aortic reimplantation. Ann Thorac Surg 1992;54: Murala JS, Sankar MN, Agarwal R, Golla PN, Nayar PG, Cherian KM. Anomalous origin of left coronary artery from pulmonary artery in adults. Asian Cardiovasc Thorac Ann 2006;14: Kitamura S, Kawachi K, Nishii T, et al. Internal thoracic artery grafting for congenital coronary malformations. Ann Thorac Surg 1992;53: Mavroudis C, Backer CL, Muster AJ, et al. Expanding indications for pediatric coronary bypass. J Thorac Cardiovasc Surg 1996;111: Kattach H, Anastasiadis K, Jin XY, Pillai R. Two-conduit repair for anomalous origin of the left coronary artery from the pulmonary artery in an adult. J Thorac Cardiovasc Surg 2004;128: INVITED COMMENTARY Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is the most common cause of myocardial infarction in children, with a mortality rate as high as 90% in infancy. However, 10% to 15% of patients survive to adulthood because of extensive intercoronary collaterals, but without surgical intervention, they remain prone to malignant ventricular arrhythmias and sudden death. Surgical correction is justified as soon as a diagnosis is made, and restoring a two-coronary system is the current practice in infants and children. Management of ALCAPA in adults is controversial, however. Studies have demonstrated that a dualcoronary system is superior to a single-coronary system and does prevent long-term morbidity and mortality. Direct reimplantation may be technically more hazardous in adults due to extensive collateralization, increased coronary artery friability, decreased vessel elasticity for mobilization, and potential for catastrophic bleeding. Left internal mammary left anterior descending artery bypass with intrapulmonary patch closure of the left coronary ostium is an alternative procedure in this subset of patients by The Society of Thoracic Surgeons /$36.00 Published by Elsevier Inc doi: /j.athoracsur