Asian Cardiovasc Thorac Ann 2006;14:38-42
© 2006 Asia Publishing EXchange Ltd
Anomalous Origin of Left Coronary Artery from Pulmonary Artery in Adults
John S. K. Murala, MCh,
Madhu N Sankar, PhD,
Ravi Agarwal, MCh,
Prasad N Golla, DNB1,
Pradeep G Nayar, DNB1,
Kotturathu M Cherian, FRACS
Department of Cardiothoracic Surgery
1 Department of Cardiology, International Centre for Cardiothoracic and Vascular Diseases, Chennai, India
For reprint information contact: Kotturathu M Cherian, FRACS, Tel: 91 44 2656 7200, Fax: 91 44 2656 5150, Email: drkmc{at}md3.vsnl.net.in, International Centre for Cardiothoracic and Vascular Diseases (A Unit of Frontier LifeLine), R 30 C Ambattur Industrial Estate Road, Mogappair, Chennai 600 101, India.
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ABSTRACT
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Various techniques have been described for management of anomalous origin of the left coronary artery from the pulmonary artery presenting in adults. Three patients, 1 male and 2 females, aged 27–37 years, underwent transpulmonary pericardial patch closure with concomitant left internal thoracic artery anastomosis to the left anterior descending artery, under standard cardiopulmonary bypass, thus creating a two-coronary system. One patient had concomitant mitral valve repair. All 3 survived the operation. Postoperative angiography in 2 patients revealed good antegrade flow with decreased collaterals in one and competitive inhibition with increased collaterals in the other. This procedure is considered to be the safest and simplest in this subset of patients.
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INTRODUCTION
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Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect with an incidence of 1 in 300,000 live births, comprising 0.24%–0.46% of all congenital cardiac anomalies. It is the most common cause of myocardial infarction in children, with a mortality of 90% in infancy. However, 10%–15% of patients survive to adulthood because of extensive intercoronary collaterals. Restrictive opening between the anomalous coronary artery and the pulmonary artery may be an additional factor in the severity of ischemia and infarction.1,2 We have previously reported our experience with ALCAPA in children; this report concerns treatment of ALCAPA in adults.3
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PATIENTS AND METHODS
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Three adults (1 male and 2 females) with ages ranging from 27 to 37 years were treated for ALCAPA in our institute from February to July 2004. All 3 presented with exertional dyspnea, and in one, a stress thallium scan showed evidence of reversible ischemia in the left anterior descending artery (LAD) territory. The diagnosis in all was made by 2-dimentional echocardiography (Figure 1A
) showing a severely dilated right coronary system with continuous flow into the main pulmonary artery (MPA). In one patient, there was a regional wall abnormality involving the anterior and apical areas, with a global ejection fraction of 35% and grade III central mitral regurgitation. The diagnosis was confirmed by coronary angiography (Figures 2A and 3A). In one patient, the circumflex arose anomalously from the right coronary artery (Figure 1B).
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Figure 1. (A) Echocardiogram in parasternal short-axis view with color Doppler of the second patient, showing the left anterior descending coronary artery (LAD) draining into the main pulmonary artery (MPA). (B) Angiography showing the anomalous origin of the circumflex artery (CX) from the right coronary artery (RCA) sinus
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Figure 2. (A) Preoperative angiogram of the first patient, showing the tortuous right coronary artery (RCA) connecting to the left anterior descending coronary artery (LAD) through the collaterals and draining into the main pulmonary artery (MPA). (B) Postoperative angiogram showing good antegrade flow from the left internal mammary artery (LIMA) to the LAD.
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Figure 3. (A) Preoperative angiogram showing a tortuous right coronary artery (RCA) with extensive collaterals draining into the left anterior descending coronary artery (LAD) which opens into the main pulmonary artery (MPA). (B) Postoperative angiogram showing the left internal mammary artery (LIMA)-to-LAD anastomosis with poor antegrade flow due to highly competitive flow from extensive collaterals.
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All 3 patients underwent transpulmonary pericardial patch closure of the ALCAPA, with concomitant left internal thoracic artery (LITA) anastomosis to the LAD under standard cardiopulmonary bypass with aortic-bicaval cannulation and root cardioplegia after crossclamping the aorta and MPA. The coronary arteries were grossly dilated. A continuous thrill was palpable at the root of the MPA. On opening the MPA, the anomalous coronary artery orifice was observed to vary from 0.5 to 1.5 cm in diameter. The orifice was located posterolaterally on the left side in 2 patients and on the posteromedial aspect in the other. After closure of the ALCAPA with a pericardial patch, the MPA was closed and an additional dose of cardioplegia was given. In one patient, the left atrium was opened and repair of the mitral valve (commissural plication) was achieved. Thereafter, routine LITA-to-LAD anastomosis was performed.
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RESULTS
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All patients survived the operation and after an uneventful postoperative course they were discharged on the 8th postoperative day. Two patients underwent repeat angiography before discharge; the 3rd did not consent to another angiogram. In the first patient, the collaterals had decreased in number and there was good antegrade flow in the LAD (Figure 2B
). In the second patient, there was an increase in collaterals with competitive inhibition of antegrade flow from the LITA to the LAD (Figure 3B). Pre-discharge 2-dimensional echocardiography revealed good ventricular function in all 3 patients. There was trivial mitral regurgitation in the patient who had undergone mitral valve repair.
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DISCUSSION
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Adults with ALCAPA may remain asymptomatic despite subclinical myocardial ischemia, and they will become symptomatic only when myocardial steal continues.2 Without surgical intervention, they are prone to malignant ventricular arrhythmias and sudden death secondary to myocardial ischemia, and global cardiomyopathy with an estimated incidence of 80%–90% at a mean age of 35 years.3,4 Surgical correction is justified as soon as a diagnosis is made, except in a few asymptomatic patients at the extremes of age.5 Restoring a 2-coronary system is the current practice in infants and children; however, the management in adults is controversial. The various options are medical management, simple ligation, or establishing a 2-coronary system.2,5–7 All of these techniques are effective so long as there is potential for myocardial recovery. However, in some long-term survivors with chronic myocardial ischemia and irreversible damage to the heart, these choices may be suboptimal. Hence, cardiac transplantation has been performed in a few cases.8
In the long-term follow-up of patients who have been treated for ALCAPA by establishing a 2-coronary system, a return to normal size of the previously dilated right coronary artery with regression of the intercoronary collateral network has been observed. However, no difference in long-term left ventricular function or late mortality has been demonstrated among the various surgical techniques used, such as direct reimplantation, LITA graft, left subclavian artery anastomosis, saphenous vein graft, or the Takeuchi procedure.9–11 Wilson and colleagues12 compared long-term follow-up in the literature among those who underwent only ligation of ALCAPA and others who had ligation and saphenous vein bypass surgery. They concluded that it is better to have a dual coronary system to avoid the sudden deaths found in the ligation-only group.12 It was also noted that there was reduced long-term survival in the patients who underwent ligation only, and their left ventricular volume and ejection fraction were not normalized.9,13,14 The proponents of a single-coronary system argue that it is safe, can be performed off-pump, and that the extensive collaterals will sustain the myocardium.6 Extrapolating the natural course of saphenous grafts, it may be presumed that if there is late obstruction, it will give rise to equivalent left main disease.2 Kececioglu and colleagues15 studied myocardial perfusion and left ventricular function in 8 patients following simple ligation of ALCAPA with a mean follow-up of 16 years. Four patients had abnormal ST-segment changes on exercise, and 5 had evidence of ventricular dysfunction. Hence they recommended elective revascularization with bypass grafting, thus establishing a 2-coronary system. Backer and colleagues,9 in their 20-year follow-up of 20 patients operated on for ALCAPA, reported 3 deaths in the ligation-only group and none in patients who had a 2-coronary artery system or transplantation. They concluded that those who survived ligation should be considered for elective establishment of a 2-coronary artery system because of the risk of late sudden death. Alexi and colleagues4 described 4 adults who underwent surgery for ALCAPA; all had a 2-coronary system established and on follow-up there was clinical improvement in all 4. They concluded that despite having this long-standing congenital heart defect, adults and elderly patients with ALCAPA might benefit from surgical intervention to establish a 2-coronary system.
Direct reimplantation of the ALCAPA may be technically more difficult and hazardous in adults due to unfavorable anatomy, increased coronary artery friability, diminished vessel elasticity for mobilization, and the potential for tearing and resultant catastrophic bleeding.2,4,7,11 On the other hand, some authors have reported successful coronary reimplantation.16,17 Intrapulmonary tunneling has been associated with pulmonary valvular dysfunction, baffle obstruction, and bleeding.7,11 Although it has been extensively used in children, there are only a few reported cases of this procedure in adults.4,18 Kattach and colleagues18 performed Takeuchi repair on an adult but also carried out LITA-to-LAD anastomosis to ensure adequate left coronary circulation should the tunnel fail. Although saphenous vein grafts have been used before, we would not recommend them because of their poor patency rates. Moodie and colleagues2 reported a saphenous vein graft patency rate of only 80% at a mean follow-up of 5.8 years in adults with ALCAPA.
While no randomized trials or long-term follow-up studies comparing the options in adults are available, it would be appropriate to speculate that successful surgical repair depends on the establishment of a left-sided 2-coronary system. As to the procedure of choice, we have already discussed the technical difficulties and complexity of direct reimplantation of the coronary artery or the intrapulmonary tunnel procedure. With the poor patency of the saphenous vein graft, and extrapolating from the excellent long-term patency of internal thoracic arteries for coronary surgical procedures in general, we would agree with Chan and colleagues7 that coronary artery bypass grafting with the LITA may be the most judicious, simple, and safe option in adults with ALCAPA.
Maintenance of cardioplegic arrest is usually difficult in adults with ALCAPA because of the presence of profuse noncoronary collateral blood flow and run-off of the cardioplegic solution into the MPA. This can be achieved most often by injecting cardioplegic solution through the aortic root while simultaneously occluding the MPA or branch pulmonary arteries individually, or by simultaneous retrograde continuous cold blood cardioplegia or simultaneous administration into both great vessels after crossclamping.11 In all of our patients, we used the method of simultaneous clamping of the aorta and MPA with topical cooling to moderate hypothermia, expeditious opening of the MPA, and pericardial patch closure of the ALCAPA. We then delivered an extra dose of cardioplegia before the LITA-to-LAD anastomosis. We believe that this is safe and quick and gives adequate myocardial protection.
The etiology of mitral regurgitation in ALCAPA is likely to be a combination of left ventricular dilatation and consequent mitral annular dilatation (as seen in one of our patients), papillary muscle ischemia or infarction, and left ventricular free wall dyskinesis. It was believed that even severe mitral insufficiency fully regressed after reperfusion alone in the majority of cases, and immediate postoperative mild to moderate regurgitation is acceptable and can be tackled at a later date.4,10,11,19 Some have advocated mitral valve repair in selected cases, and others still advocate mitral valve repair in all patients with any degree of regurgitation at the time of presentation.4,19 However, there are no guidelines for mitral valve repair in adults. It would be right to assume that in our patient with severe mitral regurgitation it was unlikely that revascularization alone would have had any impact on the degree of regurgitation, given what appeared intraoperatively to be fixed changes in the mitral valve structure. In the patient with poor antegrade flow in our series we anticipate that the collaterals will close over a period of time and the LAD territory will be adequately revascularized as the pressure head from the LITA to the LAD is higher than that from the collaterals to the LAD. Although there was no deterioration of ventricular function in the immediate postoperative period, we would like to follow up with stress thallium and coronary angiogram at a later date.
We subscribe to the view that it is always safer to create a 2-coronary system. We recommend regular follow-up in patients who previously underwent only ligation of ALCAPA. Stress thallium studies should be performed, and elective coronary artery bypass should be attempted to prevent sudden death. Use of the LITA gives good results without adding to the complexity or morbidity of this procedure.
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ACKNOWLEDGMENTS
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The authors would like to thank Dr. BR Jeganath, Dr. Pankaj and Dr. Smartin for their contributions. We thank Ms. Meena for the echo pictures, Mrs. Revathy and Mr. Ramesh for preparing the manuscript.
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REFERENCES
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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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ABSTRACT
INTRODUCTION
