Asian Cardiovasc Thorac Ann 2001;9:269-274
© 2001 Asia Publishing EXchange Pte Ltd
Evolution of Surgical Strategies for Anomalous Left Coronary Artery
Smruti Ranjan Mohanty, MCh,
Kona Samba Murthy, MCh,
Roy Varghese, MCh,
Kotturathu Mammen Cherian, FRACS
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Department of Pediatric Cardiac Surgery Institute of Cardiovascular Diseases Madras Medical Mission Chennai, Tamil Nadu, India
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For reprint information contact: Smruti Ranjan Mohanty, MCh Tel: 91 44 656 5961 Fax: 91 44 656 5510 email: mmmbits{at}giasmd01.vsnl.net.in Department of Pediatric Cardiac Surgery, Institute of Cardiovascular Diseases, Madras Medical Mission, 4A Dr. J Jayalalitha Nagar, Mogappair, Chennai, Tamil Nadu 600050, India.
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ABSTRACT
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Between 1980 and 2000, 18 patients aged 3 months to 60 years underwent repair of an anomalous left coronary artery from the pulmonary artery. Up to 1993, 8 patients were managed by subclavian artery turndown (4), coronary artery revascularization with ligation of the anomalous left coronary artery (3), or ligation of the anomalous left coronary artery alone (1). From 1994 to 1999, 6 patients underwent Takeuchi's repair. Great arterial wall tube interposition was used in the latest 4 patients. Concomitant mitral valve procedures were required in 4 patients. There were 3 early postoperative deaths (16.7%), and 1 patient who underwent subclavian artery turndown and mitral valve replacement died of bacterial endocarditis 3 months postoperatively. One patient had a tunnel leak at 6 months postoperatively, which was closed successfully; another developed significant right ventricular outflow obstruction and is awaiting surgery. Early two-coronary repair using the great arterial wall tube interposition technique looks promising.
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INTRODUCTION
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Anomalous left coronary artery from the pulmonary artery (ALCAPA) is an uncommon entity with an incidence of 0.25% to 0.5% among congenital cardiac anomalies. It is often described as the Bland-White-Garland syndrome, and without surgical intervention it carries a mortality rate of as high as 90% in the first year of life.1 Surgical strategies have evolved since the successful ligation of an anomalous left coronary artery by Sabiston and colleagues2 in 1959. Meyer and colleagues3 created a two-coronary artery system by left subclavian artery-to-left coronary artery anastomosis. Cooley and colleagues4 performed saphenous vein grafting to the left main coronary artery after closing the ostium from within the pulmonary artery. Direct implantation of the left coronary artery into the aorta has also been reported.5 Use of a tunnel within the main pulmonary artery to connect the ostium of the left coronary artery to the aorta via an aortopulmonary window with pulmonary arterial wall was described by Takeuchi and colleagues.6 Recently, coronary prolongation using a cuff of aorta and pulmonary artery wall has been undertaken.7–9
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PATIENTS AND METHODS
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From January 1980 to January 2000, 18 patients were treated for ALCAPA by various surgical approaches at our institute. They represented only 0.26% (18/6,841) of congenital heart operations during this period. Ages ranged from 3 months to 60 years (median, 2 years); all but one of the patients were children including 7 infants, and 50% were male. Weights ranged from 3 to 84 kg (median, 7.3 kg). Six children presented with features of congestive heart failure, and 4 complained of angina on exertion. Five children were diagnosed to have ALCAPA while being investigated for an asymptomatic murmur. A 60-year-old man presented with acute myocardial infarction and ventricular fibrillation; he was resuscitated and emergency coronary angiography revealed ALCAPA. He also had lesions in the left anterior descending artery and the 2nd obtuse marginal branch. ALCAPA was discovered incidentally during surgery for coarctation of the aorta in an 8-year-old child, and during intracardiac repair of double-outlet right ventricle and pulmonary atresia in a 14-year-old patient. The mean cardiothoracic ratio was 0.76 (range, 0.6 to 0.8). All patients had evidence of ischemia on an electrocardiogram. Four patients had severe mitral regurgitation, and 4 had moderate mitral regurgi-tation. Severe pulmonary artery hypertension was found in 3 patients who had an associated patent ductus arteriosus with left-to-right shunting. Sixteen patients (89%) had moderate to severe left ventricular dysfunction. The mean left ventricular ejection fraction was 32% ± 12.3%, and the mean left ventricular fractional shortening was 23.6% ± 8.27%. Coronary angiography was performed to diagnose ALCAPA in the first 8 patients; the last 10 were diagnosed by echocardiography and color Doppler only.
The various surgical techniques used throughout this experience are listed in Table 1
. The operations were undertaken through a median sternotomy; anomalous left coronary artery ligation and left subclavian artery-to-left coronary artery (LSA-LCA) turndown were carried out via a left thoracotomy. Cardiopulmonary bypass (CPB) was used in all except the patient who underwent ALCAPA ligation. LSA-LCA turndown was performed as reported previously.10 Intrapulmonary tunnel repair was undertaken as described by Takeuchi and colleagues.6 In the last 4 patients, the anomalous left coronary artery was extended by constructing a tube from flaps of aortic and pulmonary wall (Figures 1A, 1B, and 1C). Of the 4 patients with severe mitral regurgitation, 2 undergoing LSA-LCA turndown had concomitant mitral valve replacement, and 2 undergoing Takeuchi's repair had modified Kay annuloplasty. Cases of associated patent ductus arteriosus were treated by ligation, coarctation was repaired by patch aortoplasty, and the child with double-outlet right ventricle and pulmonary atresia underwent intracardiac repair with a 21-mm pulmonary homograft and revascu-larization of the anomalous coronary artery via a 6-mm polytetrafluoroethylene graft. The patient with associated coronary artery disease received additional bypass grafting of the left anterior descending artery and obtuse marginal artery with segments of saphenous vein. The mean CPB time was 153.9 minutes (range, 72 to 508 minutes), mean aortic crossclamp time was 49.5 minutes (range, 31 to 78 minutes), and the mean period of CPB support after release of the aortic crossclamp was 73.5 minutes (range, 26 to 221 minutes). The last 7 patients underwent hemo-filtration with a mean volume of 528.5 mL (range, 350 to 650 mL). The chest was closed primarily in 10 patients. A child who had undergone Takeuchi's repair had a right ventricular outflow tract gradient of 100 mm Hg subsequently, and the great arterial wall tube (GAWT) interposition technique was used as a bail-out procedure. This patient required CPB support for a long period and the chest was closed with a polytetrafluoroethylene mem-brane because of borderline hemodynamics; delayed sternal closure was carried out on the 12th postoperative day.
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Figure 1. Two-coronary repair of anomalous left coronary artery from the pulmonary artery using the great arterial wall tube interposition technique. (A) The main pulmonary artery is transected and a flap of pulmonary artery wall around the origin of the anomalous left coronary artery is taken. A flap of aorta is raised at the presumed site of the neocoronary artery origin. (B) The aortic and pulmonary artery flaps are sutured to construct a tube. Inset shows the completed great arterial wall tube. (C) Cross-section showing the lie of great arterial wall tube after completion of repair. Inset shows cross-section at points 1 and 2. ALCA = anomalous left coronary artery, Ao = aorta, GAWT = great arterial wall tube, LCA = left coronary artery, PA = pulmonary artery, RCA = right coronary artery.
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RESULTS
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The mean duration of ventilation was 106.2 hours (range, 40 to 228 hours), and inotropic support was required for 154.6 hours (range, 46 to 432 hours). The mean intensive care unit stay was 7 days (range, 2 to 19 days), and mean hospital stay was 15.2 days (range, 7 to 30 days). Two children had ventricular fibrillation in the postoperative period, which was managed successfully. All patients were given decongestive therapy and afterload-reducing agents for 3 months to 1 year postoperatively.
Three patients died in the hospital (early mortality, 16.7%). The 60-year-old man with associated coronary artery disease died of renal failure on the 8th postoperative day. A 15-year-old girl who underwent LSA-LCA turndown could not be weaned from CPB and died immediately after surgery. In a 4-month-old girl who underwent GAWT interposition, the sternum could not be closed due to hemodynamic instability and arrhythmias; a polytetra-fluoroethylene membrane was used to close the chest, but she expired on the 4th postoperative day due to low cardiac output. Of the 15 survivors, one child who under-went LSA-LCA turndown with mitral valve replacement succumbed to subacute bacterial endocarditis at 3 months postoperatively.
One patient with a Takeuchi's repair had a tunnel leak after 12 months of follow-up (Figure 2). This child underwent a successful repair under CPB and did well subsequently. There was no gradient in the neocoronary artery in the cases of GAWT interposition (Figure 3). A patient with LSA-LCA anastomosis and mitral valve replacement underwent follow-up catheterization that showed a patent coronary system.10 Left ventricular function improved in all survivors (Table 2). After a mean follow-up of 6.8 years (range, 6 months to 20 years), all patients were in New York Heart Association functional class I or II and off all medications, except for aspirin in the patient with a polytetrafluoroethylene graft. Of those who had interventions for severe mitral regurgitation, one patient who underwent LSA-LCA anastomosis and valve replacement is now 18 years old and may need a change of valve prosthesis in the future. The 2 patients who underwent mitral valve repair and received an intrapulmonary tunnel had mild mitral regurgitation after 15 months of follow-up. Patients with mild or moderate mitral regurgitation had decreased regurgitation after surgery (Figure 4).
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Figure 2. Postoperative aortic root angiogram in a case of Takeuchi's repair, showing a tunnel leak. There was rapid filling of the branch pulmonary arteries with aortic injection. AO = aorta, LPA = left pulmonary artery, RCA = right coronary artery, T = tunnel leak.
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Figure 3. Two-dimensional echocardiography demonstrating a patent great arterial wall tube. AO = aorta, GAWT = great arterial wall tube, IAS = interatrial septum, LA = left atrium, LCA = left coronary artery, RA = right atrium, RCA = right coronary artery.
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Figure 4. Evolution of mitral regurgitation in patients undergoing repair of anomalous left coronary artery from the pulmonary artery. MR = mitral regurgitation.
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DISCUSSION
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Although rare, ALCAPA is the most common congenital anomaly involving coronary arteries. After a postnatal fall in pulmonary vascular resistance due to significant coronary steal, the myocardium thrives entirely on collaterals from the right coronary artery, and with time, ventricular dysfunction, mitral regurgitation, and con-gestive heart failure ensue. Coronary angiography used to be considered the gold standard for diagnosis; however, this anomaly can be demonstrated by 2-dimensional and pulsed Doppler echocardiography. Ligation of the ALCAPA can stop the steal phenomenon and is favored in critically ill infants.2,11 Although the first patient in this series underwent ligation and is in New York Heart Association functional class I after 20 years, reports of high late mortality (up to 25%) contraindicate the use of this technique in the current era.12
Neovascularization using left subclavian artery, which is of similar caliber to the left coronary artery, is a possible option.3 However, the tendency of the subclavian artery to kink at its origin, inadequate vessel length, potential ischemia of the left upper limb, and a low patency rate (60% at 5 years) are of concern.13 Among the 4 patients with LSA-LCA anastomosis, there was one early death and one late death. One of the 2 survivors has a patent anastomosis demonstrated angiographically after 18 years, and the other is completely asymptomatic after 14 years of follow-up. Saphenous vein grafting has a better patency rate in ALCAPA repair (80% after 14 years) than in athero-sclerotic coronary artery disease.14 One of the 2 patients who had ALCAPA repair with a saphenous vein graft died on the 8th postoperative day, but the other is asymp-tomatic after 16 years. Nevertheless, the use of saphenous vein for ALCAPA repair is unpopular because of questionable conduit patency due to intimal hyperplasia.15
Takeuchi's repair has been shown to have good results, but tunnel obstruction was reported in 1 of 3 patients studied postoperatively at Boston Children's Hospital.16 The problems of supravalvular stenosis, tunnel leak, tunnel obstruction, and impairment of the pulmonary valve are of real concern after Takeuchi's repair.16 There were problems in 3 of the 6 patients in this series treated by this technique: one developed severe right ventricular outflow tract obstruction with a 60-mm Hg pressure gradient and is awaiting surgery; another developed a tunnel leak and required reoperation after 6 months; and the third could not be weaned off bypass because of significant right ventricular outflow tract obstruction, GAWT interposition was used successfully as a bail-out procedure.
Direct reimplantation of the anomalous coronary artery is considered to be an excellent option.16,17 Neirotti and colleagues18 treated 12 patients by direct reimplantation, with no mortality. Alexi-Meskishvili and colleagues19 had no postoperative deaths in 11 children treated by this technique. However, the distance between the midpoint of the left aortic sinus and the anomalous coronary artery origin varies from 2 to 18 mm, and the longer distances preclude direct reimplantation.20 In 17 of our patients, the anomalous artery originated from the left posterior sinus, and mobilization of the proximal branches of the left coronary artery for transfer to the aorta was considered to be unacceptably risky. Hence, the new technique of GAWT interposition was adopted. Extending the coronary artery seems to be a reasonable option. Moreover, the extension tube lies behind the pulmonary artery; it is likely to have a near-natural course, and will not cause right ventricular outflow tract obstruction. As the tube is made of autogenous tissue and only half of its circumference is sutured, it has good potential for growth in the future. A long-term study is required to validate this procedure.
The issue of mitral valve intervention in patients with ALCAPA is controversial. Improved mitral valve function after coronary revascularization has been documented, but the fate of those with severe regurgitation is of concern, and individualized intervention is recommended. Valve repair seems to be logical because annular dilatation persists despite the improvement in myocardial function after revascularization; hence, repair that results in a competent valve should prevent further left ventricular jeopardy. Pulmonary arterial hypertension, although of concern in young infants, usually settles down after the repair and can be managed with vigilant intensive care.
In addition to the need to establish a two-coronary system in patients with ALCAPA, the myocardium requires careful consideration to recover from the chronic ischemic insult, including good myocardial protection, reestablishment of a near-normal coronary supply, repair of a regurgitant mitral valve, and judicious postoperative care. These patients have to be followed up on a regular basis to assess left ventricular function and intervene with appropriate medications, if required, to ensure a better outcome. It was concluded that early two-coronary repair using the GAWT interposition technique looks promising.
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REFERENCES
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Bland EF, White PD, Garland J. Congenital anomalies of the coronary arteries. Am Heart J
1933;8:787–801.
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Sabiston DC Jr, Neil CA, Taussig HB. The direction of blood flow in anomalous left coronary artery arising from the pulmonary artery. Circulation
1960;22:591–7.[Abstract/Free Full Text]
-
Meyer BW, Stefanik G, Stiles QR, Lindesmith GG, Jones JC. A method of definitive surgical treatment of anomalous origin of left coronary artery: a case report. J Thorac Cardiovasc Surg
1968;56:104–7.[Medline]
-
Cooley DA, Hallman GL, Bloodwell RD. Definitive surgical treatment of anomalous origin of left coronary artery from pulmonary artery: indications and results. J Thorac Cardiovasc Surg
1966;52:798–808.[Medline]
-
Neches WH, Mathews RA, Park SC, Lenox CC, Zuberbuhler JR, Siewers RD, et al. Anomalous origin of the left coronary artery from the pulmonary artery. A new method of surgical repair. Circulation
1974;50:582–7.[Abstract/Free Full Text]
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Takeuchi S, Imamura H, Katsumoto K, Hayashi I, Katohgi T, Yozu R, et al. New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg
1979;78:7–11.[Abstract]
-
Tashiro T, Todo K, Haruta Y, Yasunaga H, Nagata M, Nakamura M. Anomalous origin of the left coronary artery from the pulmonary artery. New operative technique. J Thorac Cardiovasc Surg
1993;106:718–22.[Abstract]
-
Sese A, Imoto Y. New technique in the transfer of an anomalously originated left coronary artery to the aorta. Ann Thorac Surg
1992;53:527–9.[Abstract]
-
Katsumata T, Westaby S. Anomalous left coronary artery from the pulmonary artery: a simple method for aortic implantation with autogenous arterial tissue. Ann Thorac Surg
1999;68:1090–1.[Abstract/Free Full Text]
-
Cherian KM, Bharati S, Rao SG. Surgical correction of anomalous origin of left coronary artery from pulmonary artery. J Card Surg
1994;4:386–91.
-
Kirklin JW, Barratt-Boyes BG. Cardiac surgery. New York: Wiley, 1986:945–69.
-
Househam KC, Human DG, Fraser CB, Joffe HS. Anomalous left coronary artery from pulmonary artery: a therapeutic dilemma. S Afr Med J
1983;63:325–7.[Medline]
-
Stephenson LW, Edmunds LH Jr, Friedman S, Meijboom E, Gewitz M, Weinberg P. Subclavian-left coronary artery anastomosis (Meyer operation) for anomalous origin of the left coronary artery from the pulmonary artery. Circulation
1981;64(Suppl II):130–3.
-
Chiariello L, Meyer J, Reul GJ Jr, Hallman GL, Cooley DA. Surgical treatment for anomalous origin of left coronary artery from pulmonary artery. Ann Thorac Surg
1975;19:443–50.[Abstract]
-
El-Said GM, Ruzyllo W, Williams RL, Mullins CE, Hallman GL, Cooley DA, et al. Early and late result of saphenous vein graft for anomalous origin of left coronary artery from pulmonary artery. Circulation
1973;48(Suppl II):12–6.
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Moodie DS, Fyfe D, Gill CC, Cook SA, Lytle BW, Taylor PC, 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;106:381–8.[Medline]
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Laks H, Ardehali A, Grant PW, Allada V. Aortic implantation of anomalous left coronary artery. An improved surgical approach. J Thorac Cardiovasc Surg
1995;109:519–23.[Abstract/Free Full Text]
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Neirotti R, Nijveld A, Ithuralde M, Quaglio M, Seara C, Lubbers L, et al. Anomalous origin of the left coronary artery from the pulmonary artery: repair by aortic reimplantation. Eur J Cardio-thorac Surg
1991;5:368–72.[Abstract]
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Alexi-Meskishvili V, Hetzer R, Weng Y, Lange PE, Jin Z, Berger F, et al. Anomalous origin of the left coronary artery from the pulmonary artery. Early results with direct aortic reimplantation. J Thorac Cardiovasc Surg
1994; 108:354–62.[Abstract/Free Full Text]
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Smith A, Arnold R, Anderson RH, Wilkinson JL, Qureshi SA, Gerlis LM, et al. 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.[Abstract]
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ABSTRACT
INTRODUCTION
