Ann Thorac Surg 1996;61:1008-1011
© 1996 The Society of Thoracic Surgeons
Case Reports
Maze Procedure and Anomalous Coronary Artery Repair
Junjiro Kobayashi, MD,
Yoshio Kosakai, MD,
Yasunaru Kawashima, MD
Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
Accepted for publication September 7, 1995.
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Abstract
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A 60-year-old woman had anomalous origin of the left coronary artery from the pulmonary artery, mitral regurgitation with left ventricular dysfunction, and atrial fibrillation. We performed mitral valve annuloplasty, maze procedure, and intrapulmonary tunnel repair of anomalous origin of the left coronary artery. The patient regained normal sinus rhythm and showed improved left ventricular function and no mitral regurgitation.
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Introduction
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Most patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) show the symptom of congestive heart failure with mitral regurgitation (MR) in infancy [1]. However, a few patients survive until the fifth or sixth decade of life and have severe MR with depressed left ventricular function and ischemia [24]. We successfully treated a 60-year-old woman with ALCAPA, MR with left ventricular dysfunction, and atrial fibrillation (AF).
A 60-year-old woman was referred to our institution with the diagnosis of MR. Systolic murmurs were indicated since childhood. The electrocardiogram showed AF, left ventricular hypertrophy, and strain (Fig 1A
). Echocardiography revealed a dilated left ventricle with poor contraction and a dilated left atrium. Left ventricular end-diastolic and end-systolic dimensions were 72 mm and 62 mm, respectively, and the left atrial dimension was 62 mm. There was calcified thickening of the mitral valve apparatus but no major stenosis. Doppler examination showed severe MR and tricuspid regurgitation.


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Fig 1. . (A) Preoperative and (B) postoperative electrocardiograms. The former shows left ventricular hypertrophy and strain. Atrial fibrillation was converted to normal sinus rhythm by the maze procedure.
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Cardiac catheterization was performed. Pulmonary artery pressure was 73/40 mm Hg with a mean pressure of 52 mm Hg. Wedge pressure was 15 mm Hg. Left ventriculography showed severe MR. The right coronary artery was dilated and tortuous, and the injected contrast medium passed into the left coronary arterial system through the numerous collateral channels and finally drained retrogradely into the pulmonary artery (Fig 2
). The patient was diagnosed with ALCAPA, severe MR and tricuspid regurgitation, poor left ventricular function, pulmonary hypertension, and AF.

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Fig 2. . Right coronary arteriogram in the posteroanterior projection. The tortuous and dilated right coronary artery (RCA) was opacified initially. Injected contrast medium passed into the left coronary artery and the main pulmonary artery (PA) through numerous small collaterals. (LAD = left anterior descending coronary artery.)
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A median sternotomy was performed. Extracorporeal circulation was established by ascending aortic and bicaval cannulation. The operation was done under moderate hypothermia and cardioplegic arrest with St. Thomas' solution. The right atrium and the superior vena cava were incised for the maze procedure as reported previously [5]. Antegrade and retrograde injection of the cardioplegic solution was performed with compression of the pulmonary artery to obliterate the ostium of the left coronary artery. The left atrium was incised in front of the right superior pulmonary vein. The left atriotomy was extended to encircle the orifices of the pulmonary veins. Cryoablation was carried out by the previously reported method [5]. The mitral valve was thickened, especially in the posterior leaflet, but no commissural fusion was seen. Subvalvar abnormalities were minimal. Because the mitral annulus was dilated, it was plicated by a modified Kay procedure at both commissures, reducing the diameter from 36 to 27 mm. The tricuspid valve was also dilated and plicated by the De Vega method from 42 to 27 mm in diameter. A test injection of cardioplegic solution into the left ventricle showed no MR and trivial tricuspid regurgitation. The pulmonary artery was opened longitudinally to expose the large left coronary ostium. After a 6-mm aortopulmonary window was established, an intrapulmonary tunnel was made using a bovine pericardial patch [6].
The patient's postoperative course was uncomplicated, and the cardiac rhythm was a normal sinus rhythm (Fig 1B
). Three weeks after the operation, an echocardiographic study was performed. Left ventricular end-diastolic and end-systolic dimensions were 58 mm and 47 mm, respectively, and the left atrial dimension was 35 mm. Color Doppler study showed no MR and no tricuspid regurgitation.
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Comment
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This report concerns a maze procedure performed for AF in an elderly patient with ALCAPA. Only 9 patients older than 50 years with ALCAPA have been described in previous reports [24]. Mitral regurgitation is a common complication of ALCAPA and in older adults, is attributed to long-standing ischemia. Depressed ventricular function is probably due to the combination of ischemia and severe MR. Atrial fibrillation is caused by the dilated left and right atrial walls.
The condition of most relatively young patients with MR can be improved with reconstruction of the two coronary systems without a direct mitral valve procedure [7]. However, in older patients, MR is more or less attributed to organic changes secondary to long-standing ischemia and sometimes is associated with AF [24]. In a previous report [4], AF was left untreated, and mitral valve replacement was performed. The left coronary ostium was simply closed.
In our patient, normal sinus rhythm was restored by the maze procedure, and mitral valve replacement was avoided. Therefore, antithrombotic therapy was unnecessary, and thromboembolism and bleeding complications were not observed. Atrial contraction is very important in the failing heart to increase the cardiac output. In addition, because early and late results of mitral valve replacement for ischemic MR are less satisfactory than mitral valve repair, as reported by Kay and associates [8], mitral valve repair is preferred.
Options for surgical treatment of anomalous left coronary artery are simple ligation, transfer, rerouting in the pulmonary artery, and ligation with bypass grafting. Although reconstruction of the two coronary systems may be more difficult than ligation of the anomalous coronary artery in older patients, late sudden death was reported after ligation in a follow-up study [7].
To date, we have performed about 200 maze operations on patients with cardiac diseases. About 75% of patients regained sinus rhythm, and complications of the maze procedure during and after the operation were minimal. We consider the maze procedure reasonable for AF in patients with ALCAPA.
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Footnotes
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Address reprint request to Dr Kobayashi, Department of Cardiovascular Surgery, National Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita-city, Osaka 565, Japan.
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References
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- Wesselhoeft H, Fawcett JS, Johnson AL. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, pathophysiology based on a review of 140 cases with seven further cases. Circulation 1968;38:40325.[Abstract/Free Full Text]
- Purut CM, Sabiston DC Jr. Origin of the left coronary artery from the pulmonary artery. J Thorac Cardiovasc Surg 1991;102:56670.[Abstract]
- Letcher JR, McCormick D, Tendler S, Ross J, Chandrasekaran K, Brockman S. Left main coronary artery arising from the pulmonary trunk in a 56-year-old patient presenting with acute myocardial infarction. Am J Cardiol 1991;68:12579.[Medline]
- Saeed BT, Rosin MD, Murray RG. Successful operation in an old survivor of anomalous origin of the left coronary artery from the pulmonary trunk (Bland-White-Garland syndrome). Br Heart J 1994;71:1935.[Abstract/Free Full Text]
- Kosakai Y, Kawaguchi A, Isobe F, et al. Cox maze procedure for chronic atrial fibrillation associated with mitral valve disease. J Thorac Cardiovasc Surg 1994;108:104955.[Abstract/Free Full Text]
- Hamilton DI, Ghosh PK, Donnelly RJ. An operation for anomalous origin of left coronary artery. Br Heart J 1979;41:1214.[Abstract/Free Full Text]
- 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;103:104958.[Abstract]
- Kay GL, Kay JH, Zubiate P, Yokoyama T, Mandez M. Mitral valve repair for mitral regurgitation secondary to coronary artery disease. Circulation 1986;74(Suppl 1):8898.
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