Asian Cardiovasc Thorac Ann 2000;8:27-31
© 2000 Asia Publishing EXchange Pte Ltd
Two-Coronary Repair for Anomalous Left Coronary Artery from Pulmonary Artery
Sachin Talwar, MS,
Anil Bhan, MCh,
Rajesh Sharma, MCh,
Shiv Kumar Choudhary, MCh,
Balram Airan, MCh,
Anita Saxena, DM,
Shyam Sunder Kothari, DM,
Rajnish Juneja, DM,
Panangipalli Venugopal, MCh
Department of Cardiothoracic and Vascular Surgery
Cardiothoracic Sciences Centre
All India Institute of Medical Sciences
New Delhi, India
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For reprint information contact: Anil Bhan, MCh Tel: 91 11 686 4851 or 656 1123 Fax: 91 11 686 2663 email: anil_bhan{at}hotmail.com Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Sciences Centre, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India.
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Abstract
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Between January 1989 and December 1998, 13 patients (7 males) aged 3 months to 32 years, underwent surgery for anomalous left coronary artery from the pulmonary artery. Eight presented with congestive cardiac failure and all had evidence of left ventricular dysfunction. One patient had associated tetralogy of Fallot. Preoperative diagnosis was established by echocardiography and cineangiography. Nine patients underwent Takeuchi repair and 4 had direct implantation of the anomalous artery into the aorta. There were 2 postoperative deaths due to low cardiac output. In survivors, serial echocardiograms demonstrated significant improvement in left ventricular function 3 months to 10 years after surgery. Postoperative angiograms in 4 patients showed a patent aortocoronary tunnel in 3 who underwent Takeuchi repair and a patent aortocoronary anastomosis in one who had direct implantation of the anomalous artery into the aorta. It was concluded that early establishment of a two-coronary system gave gratifying short-term and long-term results.
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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 cardiac anomaly occurring in 0.24% to 0.46% of all patients with congenital heart disease.1 Left untreated, it leads to myocardial infarction, left ventricular dysfunction, congestive heart failure, and death in more than 80% of such infants in their first year of life.2 Since the first description of ALCAPA in 1886 by Brooks3 and the first successful treatment in 1959 by Sabiston and colleagues,4 surgical treatment has evolved from simple ligation to establishment of a two-coronary system.5–8 We reviewed our experience of surgical treatment of ALCAPA and the early and long-term follow-up.
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Patients and Methods
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Between January 1989 and December 1998, 13 patients, 7 males and 6 females, underwent surgery for ALCAPA at the All India Institute of Medical Sciences, New Delhi, India. Patient details are summarized in Table 1
. Age at the time of operation ranged from 3 months to 32 years (median, 6 months). Seven patients were 6 months of age or less, 8 presented with congestive heart failure in the first year of life, 2 presented with recurrent cough and fever (a preoperative diagnosis of tetralogy of Fallot with ALCAPA was made in one of these), 1 presented with abdominal colic and was found to have a cardiac murmur and subsequently diagnosed with ALCAPA. An apical systolic murmur was present on physical examination in 12 patients; 2 also had a systolic murmur in the pulmonary area. Twelve patients had cardiac enlargement on preoperative chest radiographs. Ischemic ST-segment changes were present in 12 patients; one of these had features suggestive of acute myocardial infarction. One patient had evidence of right ventricular hypertrophy without significant evidence of ischemia.
Echocardiography using two-dimensional pulsed and color-flow Doppler techniques was diagnostic in all patients. Echocardiographic findings included an enlarged right coronary artery, dilatation and hypokinesia of the left ventricle, enlarged end-diastolic dimensions of the left ventricle, and direct visualization of the left coronary artery arising from the pulmonary artery. Six patients had mild and 5 had moderate mitral insufficiency. Echo-cardiographically determined left ventricular ejection fractions in these patients ranged from less than 10% to 50% (median, 30%). However, for correct anatomic diagnosis, cardiac catheterization and cineangiography was undertaken in all patients. In all cases, the right coronary artery was seen to be enlarged with collaterals providing retrograde filling of the anomalous left coronary artery with a blush of opacification in the pulmonary artery (Figure 1) along with a dilated hypokinetic left ventricle.
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Figure 1. Selective right coronary injection angiogram (right anterior oblique view) of patient no. 13 showing enlarged right coronary artery (straight arrow), intercoronary collaterals (curved arrows), and left coronary artery (arrowheads) arising from the pulmonary artery (PA).
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The patients were operated on soon after diagnosis on an urgent basis. Surgical procedures were carried out via a median sternotomy using standard hypothermic cardio-pulmonary bypass. For myocardial protection, intermittent antegrade hyperkalemic cold blood cardioplegia was delivered into the aortic root while the pulmonary artery was occluded digitally or with forceps. Topical ice slush was also applied. In 5 patients, the anomalous left coronary artery (ALCA) originated from the posterior part of the main pulmonary artery, it originated from the left posterior sinus in 6, and from the left lateral pulmonary artery in 2. One patient had associated tetralogy of Fallot.
The choice of surgical procedure depended upon the distance between the ALCA and the aorta. Direct implantation was performed when the distance between the two arteries permitted easy transfer of the ALCA to the aorta. However, when it originated from the lateral aspect of the pulmonary artery or when the distance did not permit easy transfer, the repair described by Takeuchi and colleagues8 was performed. Nine patients underwent the Takeuchi procedure that consists of creation of an aortopulmonary window and a tunnel within the pulmonary artery from the aortopulmonary window to the coronary artery with autogenous pulmonary artery, and reconstruction of the pulmonary artery with peri-cardium.8 One patient (no. 9) underwent Dacron patch (CR Bard, Inc., Haverhill, MA, USA) closure of a ventricular septal defect and right ventricular outflow tract reconstruction using autogenous pericardium for associated tetralogy of Fallot. Another patient (no. 13) required a pericardial patch to augment the right ventricular outflow tract for suspected narrowing, following the Takeuchi procedure. Four patients underwent direct implantation of the ALCA into the ascending aorta.
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Results
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All patients required mechanical ventilatory support for 36 to 168 hours (mean, 72 hours). Mean duration of inotropic support was 96 hours (range, 72 to 210 hours). Two patients died within 24 hours postoperatively due to low cardiac output; one (no. 8) had presented with severe congestive heart failure at 3 months of age and underwent the Takeuchi procedure, the other (no. 9) was 6 months old and had undergone ventricular septal defect closure and right ventricular outflow tract reconstruction using a pericardial patch for associated tetralogy of Fallot in addition to the Takeuchi repair. Both of these patients had preoperative moderate mitral regurgitation that did not improve following surgery. One patient (no. 12) required prolonged mechanical ventilatory support for 7 days before recovery. Postoperative recovery was uneventful in the other patients. Mean hospital stay in the survivors was 12.1 days (range, 7 to 25 days).
Follow-up ranged from 3 months to 10 years (mean, 4.8 years) and was complete. All survivors underwent clinical assessment, serial chest radiographs, electrocardiograms, and serial echocardiography. Follow-up angiograms could be performed in 4 patients. All survivors are currently in New York Heart Association functional class I with minimal or no cardiomegaly on chest radiographs. Electrocardiographic changes resolved in all patients and in the patient who sustained a previous myocardial infarction, no new changes appeared. Serial echocardio-grams demonstrated marked improvement in left ventricular function. Overall improvement was 15% to 40% above the preoperative ejection fraction. Patients who were operated on below 1 year of age had a 20% to 45% increase in ejection fraction, whereas those operated beyond 1 year of age had only 15% to 20% improvement. There was trivial or no postoperative mitral regurgitation in 6 patients who had mild mitral regurgitation pre-operatively. In 3 patients with moderate mitral regurgi-tation, it had reduced to trivial or none at the 1-year follow-up. Presently, no patient has residual mitral regurgitation. No patient has aortic regurgitation, supravalvular pulmonary stenosis, or baffle obstruction, which are known long-term complications of Takeuchi repair.9 Postoperative angiograms in 3 patients who underwent Takeuchi repair demonstrated a patent aortocoronary tunnel at 6 months, 1 year, and 10 years after surgery (Figure 2). In 1 patient who underwent direct implantation of the ALCA into the aorta, angio-graphy revealed a patent aortocoronary anastomosis 6 years after the operation.
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Figure 2. Selective injection into the aortocoronary tunnel of patient no. 13, showing the patent tunnel (T) and filling of the left anterior descending (straight arrow) and left circumflex (curved arrow) arteries.
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Discussion
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ALCAPA is a rare congenital cardiac anomaly that usually occurs as an isolated lesion.1 At birth, because of the high pulmonary vascular resistance and high pulmonary artery pressures, there is flow of desaturated blood into the left coronary artery. However, as the pulmonary artery pressure gradually declines, there is reversal of flow in the left coronary artery and myocardial ischemia develops if collateral flow from the right coronary artery is inadequate. This leads to development of myocardial infarction, left ventricular dysfunction, congestive heart failure, and death if not treated early.
Definitive diagnosis is easily established by cardiac catheterization and angiography although echocardio-grams are also sensitive. In our series, all patients underwent echocardiograms and angiography prior to surgery although for postoperative follow-up, serial echocardiography was carried out. As reported previously, serial echocardiography was a useful tool for assessment of coronary patency, left ventricular function, and residual mitral regurgitation in such patients.10
Medical management of ALCAPA is limited to temporary control of congestive cardiac failure. With medical therapy alone, the mortality ranges from 45% to 100%, 90% die before 1 year of age.2,11 Without surgery, only patients who have minimal deterioration of left ventricular function survive late, as evident in 4 of our patients. Also, with progression of the disease, there is gradual deterioration of left ventricular function manifesting as increased morbidity and mortality with surgery. In these patients, postoperative recovery of left ventricular function is limited in comparison to those operated early in life. Therefore, early surgery should be considered in these patients.
Surgical treatment options for ALCAPA vary from simple ligation of the ALCA to the establishment of a two-coronary system. Simple ligation carries a mortality rate of 20% to 50% and a 25% risk of sudden death but may be required in critically ill infants who may not withstand other major surgical procedures.9 Establishment of a two-coronary system is the best form of treatment for ALCAPA.8,12,13 The options are saphenous vein bypass to the ALCA, left subclavian artery-to-ALCA anastomosis, internal mammary artery grafting to the ALCA, direct implantation of the ALCA into the aorta, and intra-pulmonary artery aortocoronary tunnel repair as described by Takeuchi and colleagues.5–8,14,15 Saphenous vein bypass grafting to the ALCA is technically difficult in younger children, has poor long-term graft patency, and carries a mortality rate of 0% to 38%.16 It may be carried out mainly in older children and young adults. Homograft saphenous veins have been used but there is a high incidence of graft occlusion.14 In young patients, anas-tomosis of the left subclavian artery to the ALCA has a long-term patency rate of 60% to 80%.6,13 It can be carried out without cardiopulmonary bypass. Concerns have been kinking of the subclavian artery at its origin, inadequate length of the subclavian artery, and upper limb ischemia.16 Ventricular fibrillation may occur in young infants during anastomosis on a beating heart.9 The internal mammary artery may be a better conduit than saphenous vein or subclavian artery in these patients.15 However, long-term results are still awaited.
Direct implantation of the ALCA into the aorta appears to be the most attractive treatment option. It avoids the use of a conduit, offers a high patency rate, and is particularly suitable if the ALCA originates from the right posterior pulmonary sinus or the right pulmonary artery.16,17 Mortality ranges from 0% to 23%.7,11,17 We performed this procedure in 4 patients and the long-term results were excellent with a patent aortocoronary anastomosis on angiography in one patient and good left ventricular function as assessed by echocardiography in the others. However, the procedure may be technically difficult if the ALCA originates from a left lateral position where the distance between the ALCA and the aorta makes implantation nearly impossible.16 In these patients, the transfer may be performed with the aid of a coronary prolongation technique using the cuff of the pulmonary trunk and an aortic flap.18
Takeuchi repair avoids many of the technical difficulties associated with other procedures. It can be carried out regardless of the distance between the coronary ostium in the pulmonary artery and the aorta, avoids the need for a conduit, and has good early and long-term results.16 On long-term follow-up, some patients may have moderate aortic regurgitation, supravalvular pulmonary stenosis, or obstructed baffle.9 We performed this procedure in 9 patients and the 7 survivors had patent aortocoronary tunnels with marked improvement in left ventricular function and no complications. Though mortality was higher (2/9) in patients undergoing Takeuchi repair, this is not significant due to the small number of patients in our series.
There is great potential for recovery of myocardial function following either direct implantation of the ALCA into the aorta or the Takeuchi procedure. However, some patients may require prolonged inotropic support (seen in patient no. 12) and some may require a left heart assist device.19 However, severe myocardial dysfunction sometimes persists and for these patients, the only hope for survival is orthotopic cardiac transplantation.20 On the other hand, in our experience, even patients with severe left ventricular dysfunction had a good long-term prognosis with early surgery using direct implantation into the aorta or Takeuchi repair. We feel that as more are diagnosed and treated early in life, the need for cardiac transplantation will be significantly reduced. We recommend direct implantation of the ALCA into the aorta or the Takeuchi procedure at the time of diagnosis in all patients with ALCAPA, irrespective of ventricular function.
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References
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Abstract
Introduction
