| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
J Thorac Cardiovasc Surg 1994;108:17-20
© 1994 Mosby, Inc.
SURGERY FOR CONGENITAL HEART DISEASE |
Los Angeles, Calif.
Received for publication June 21, 1993. Accepted for publication Dec. 14, 1993. Address for reprints: Farhouch Berdjis, MD, Division of Pediatric Cardiology, University of California Irvine Medical Center, P.O. Box 8119, Orange, CA 92664-8119.
Abstract
Seventeen patients with an anomalous left coronary artery from the pulmonary trunk underwent surgical treatment and were evaluated, with a mean follow-up of 9.5 years. Analysis has included clinical symptoms cardiac laboratory tests, angiographic grading of intercoronary collaterals, echocardiographic and angiographic measurement of left ventricular function, surgical methods, and the postoperative outcome. Ten patients (59%) survived, and there were seven early deaths. The only significant variable relating to survival was the extent of preoperative intercoronary connections. In survivors, the postoperative left ventricular ejection fraction increased from 45% ± 25% to 64% ± 14% (p < 0.01). Nine of ten survivors are free of symptoms and have normal left ventricular function. Nevertheless, half of all created anastomoses were occluded within 2 years of the operation. We speculate that even if temporarily constructed, a dual coronary supply allows for left ventricular recovery and normalization of function. (J THORAC CARDIOVASC SURG 1994;108:17-20)
Anomalous origin of the left coronary artery from the main pulmonary artery (ALCA) is a rare disorder elegantly described by Bland, White, and Garland. 
1 Several surgical procedures have been described to improve myocardial perfusion, 2-5 but few reports include significant numbers of patients followed up to the intermediate and long term, 6 particularly with several surgical modalities having been used. 7,8 To our knowledge no study has specifically examined intercoronary collaterals and their influence on outcome in patients with ALCA. This lack led us to review patients with an emphasis on longer-term follow-up and the relationship of outcome to intercoronary collaterals.
PATIENTS AND METHODS
In the 15-year period between 1967 and 1982, 17 patients underwent surgical treatment at Childrens Hospital of Los Angeles for ALCA. Their clinical records have been retrospectively reviewed and preoperative demographic data, symptoms, and electrocardiographic (ECG), echocardiographic, and catheterization findings were tabulated. Left ventricular function was assessed by measuring ejection fraction by a single plane area-length method. 9 Coronary collaterals were quantitated from the aortogram and classified into three groups: 0, no visible collaterals with retrograde opacification of the left coronary artery; 1+, meager (fewer than three) collaterals and small collaterals (diameter less than that of the distal third of the right coronary artery); and 2+, extensive (more than three) and/or large collaterals (diameter bigger than the distal third of the right coronary artery) (Fig.1).
|
RESULTS
Demographics.
The patients were divided into three groups on the basis of age at operation. Fifteen of our 17 patients (88%) were younger than 1 year of age, and 41% were less than 4 months old. The remaining two patients were 4 and 8 years of age. The patients were nearly equally divided between boys and girls.
Preoperative status and testing
Symptoms.
Fifteen of the 17 patients (88%) had symptoms of heart failure, most commonly characterized as shortness of breath or failure to thrive. The two remaining patients were referred for evaluation of a murmur.
ECG.
The ECG was abnormal in all patients and, in 82% (14/17), retrospectively showed signs of anterolateral myocardial infarction, although this pattern was recognized at presentation in only nine. Three patients had ST/T wave changes only, including the two symptom-free children.
Clinic diagnosis before cardiac catheterization.
Before catheterization the correct diagnosis of ALCA was made in nine of the seventeen patients (53%). Six patients were thought to have a cardiomyopathy; one patient had a murmur suggestive of a ductus versus a small ventricular septal defect. A final patient was referred at 2 weeks of age after a cardiac arrest during resection of a liver hamartoma.
Echocardiography.
Preoperative two-dimensional echocardiography was available for diagnostic work-up in only five of the patients and suggested the diagnosis in two.
Cardiac catheterization and angiography.
Angiography demonstrated the abnormal coronary pattern in all patients. One aortogram is shown in Fig. 1. Ejection fraction was compared among the three age groupings. No difference in ventricular function was detected among those presenting between 1 month and 3 months, 3 months and 1 year, and greater than 1 year.
Intercoronary collaterals.
Collaterals were assessed from the preoperative angiogram and graded from 0 to 2+ as previously described. The majority of patients, 59% (10/17) had extensive collaterals, whereas a smaller group, 23% (4/17), had meager connections between the right and left coronary systems. A minority, 18% (3/17), had no significant collateral channels identified.
Surgical procedures.
Four surgical techniques were used in our series of 17 patients with ALCA as summarized in Table I.
|
Survival.
Overall, 10 of 17 patients survived (59%). All deaths occurred during the initial hospitalization, most during or immediately after the operation. The survivors had a significantly larger number of intercoronary collaterals than the nonsurvivors (p = 0.025). Eighty percent of them had collaterals of type 2+ and only 20% were type 1+. In contrast, only two of the seven nonsurvivors had type 2+ collaterals, two had mediocre collaterals (type 1+), and three had poor collaterals. Other variables, such as preoperative ventricular function, age at presentation or operation, or type of operation did not correlate with survival.
Postoperative evaluation.
All ten hospital survivors have been followed up for a mean of 9.5 years (range 4 to 17 years). There have been no late deaths. Testing has been performed on these patients as summarized herein:
Symptomatic status.
At last follow-up, nine of the ten survivors were symptom free and unrestricted in the activities of daily life. One patient had mild exercise intolerance, although the left ventricular ejection fraction was 54%.
ECG.
A resting ECG obtained 5 to 17 years after the operation (mean 10.4 ± 3.8 years) was available in seven of the ten survivors. The ECG showed diffuse residual ST/T wave changes in five and signs of new myocardial injury in only one. The latter patient subsequently had abnormalities on a thallium perfusion scan and an akinetic anterior segment on left ventriculography.
Stress testing.
Seven surviving patients underwent maximal stress testing 1 to 17 years after the operation (mean 8.7 ± 5.4 years), and five of them had normal results. Both children with abnormal exercise performance were subsequently shown by angiography to have an obstructed coronary connection.
Echocardiogram.
Six patients had measurements of left ventricular function by two-dimensional echocardiography 5 to 13 years after the operation (mean 9.3 ± 3.0 years) and all measurements were within normal limits. Mild mitral insufficiency was evident in two.
Thallium 201 myocardial perfusion scan.
A thallium scan was done in three of the ten survivors 2 to 8 years after the operation (mean 5.3 ± 2.5 years) and showed abnormalities in only one, who had ECG and wall motion evidence of infarction.
Cardiac catheterization.
Repeat cardiac catheterization was performed in nine of the ten survivors (90%) at an average of 2.1 years after the operation (range 1 to 6.5 years). Patency of the coronary bypass graft or coronary connection was shown in five of the nine (56%). A statistically significant difference between those having a graft versus those with direct reimplantation could not be demonstrated (p = 0.6), although the small number of patients available for review limited the value of statistical analysis. Ventricular function improved after the operation from a mean of 45% ± 25% to 61% ± 14%. This improvement reached statistical significance (p = 0.01) and the function was considered to be normal in seven of the nine recatheterized children. One child, who did not have ventriculography, had normal function by echocardiogram. Thus 80% (8/10) of operative survivors after repair of ALCA had normally contracting left ventricles.
Wall motion.
Left ventricular wall motion was evaluated before the operation in all patients. In six patients data were available for postoperative comparison. Five patients improved with regard to the amount of segmental dyskinesis. In three, wall motion became entirely normal. Two patients had a marked increase in collaterals; both had obstructed anastomoses. In one child the left anterior wall akinesis did not improve.
Patency of the anastomosis.
Patency was not correlated to the age or type of operation. We grouped the patients into those undergoing reimplantation versus those having other types of surgical procedures. No statistically significant difference in patency existed between the two groups, although because the number of patients for review was small the value of statistical analysis was limited (p = 0.6).
DISCUSSION
This series examines intermediate to long-term results of patients treated in an "earlier era." The patients in this report underwent operation from 13 to 26 years ago, and substantial diagnostic and surgical advances have been made since that time. However, useful information can be gained from the long-term follow-up of these patients.
The most important variable in regard to survival was the extent of the preoperative intercoronary connections. Although the ejection fraction of survivors was better than that of nonsurvivors, the difference did not reach statistical significance. This is in contrast to the findings of Vouhe, Baillot-Vernant, and Trinquet. 8
Half of all survivors had a thrombosed anastomosis, but all but one were free of symptoms. In most patients wall motion improved markedly, even in those with occluded grafts. Additional collaterals developed in some. We speculate that the construction of a two coronary system, even if temporary, provides the patient with a "honeymoon" period in which the left ventricle recovers despite possible later occlusion of the anastomosis. Recruitment of additional collaterals seems to be possible. These speculations are in agreement with the observed normalization of left ventricular function. It is also possible that occlusion of the anastomosis may have occurred early and created the equivalent of coronary ligation. This, in and of itself, might improve left ventricular function by preventing a "steal" into the pulmonary artery.
We favor reimplantation even though we could not supply statistical evidence of superiority of this method. With the establishment of the arterial switch operation for D-transposition of the great arteries, the experience in coronary reimplantation increased tremendously, and surgical results for ALCA with this method will improve.
The retrospective investigation demonstrated that some cases were not identified immediately. A meticulous echocardiographic examination including color Doppler flow mapping is mandatory. 10-12 If the diagnosis cannot be made with certainty, catheterization and angiography are mandatory.
Almost all patients have a good clinical status approximately a decade later. Further follow-up examination is necessary to delineate the cardiovascular status of these patients once they reach adolescence and adulthood.
Footnotes
From the Divisions of Pediatric Cardiology a and Cardiothoracic Surgery, b Childrens Hospital Los Angeles, University of Southern California School of Medicine, Los Angeles, Calif. 
References
This article has been cited by other articles:
|
|
 |
|
  C. Kampmann, W. Kuroczynski, H. Trubel, M. Knuf, M. Schneider, and M. K. Heinemann Late Results After PTCA for Coronary Stenosis After the Arterial Switch Procedure for Transposition of the Great Arteries Ann. Thorac. Surg., November 1, 2005; 80(5): 1641 - 1646. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 M. Ando, R. B.B. Mee, B. W. Duncan, J. J. Drummond-Webb, S. G. Seshadri, and C. I. Mesia Creation of a dual-coronary system for anomalous origin of the left coronary artery from the pulmonary artery utilizing the trapdoor flap method Eur. J. Cardiothorac. Surg., October 1, 2002; 22(4): 576 - 581. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Dodge-Khatami, C. Mavroudis, and C. L. Backer Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy Ann. Thorac. Surg., September 1, 2002; 74(3): 946 - 955. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. Turley, R. J. Szarnicki, K. D. Flachsbart, R. C. Richter, R. W. Popper, and H. Tarnoff Aortic Implantation Is Possible in All Cases of Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery Ann. Thorac. Surg., July 1, 1995; 60(1): 84 - 89. [Abstract] [Full Text]
|
|
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| ANN THORAC SURG | ASIAN CARDIOVASC THORAC ANN | EUR J CARDIOTHORAC SURG |
| J THORAC CARDIOVASC SURG | ICVTS | ALL CTSNet JOURNALS |
