Ann Thorac Surg 2003;76:267-269
© 2003 The Society of Thoracic Surgeons
Case report
A reoperation of Adult-Type Bland-White-Garland syndrome
Tetsuya Niino, MDa*,
Motomi Shiono, MDa,
Tatsuya Inoue, MDa,
Mitsumasa Hata, MDa,
Akira Sezai, MDa,
Nanao Negishi, MDa
a Second Department of Surgery, Nihon University School of Medicine, Tokyo, Japan
Accepted for publication January 17, 2003.
* Address reprint requests to Dr Niino, Second Department of Surgery, Nihon University School of Medicine, 30-1 Ooyaguchi, Kamimachi, Itabashi-ku, Tokyo 173-0081, Japan
e-mail: xj8t-nin{at}asahi-net.or.jp
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Abstract
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We present a 25-year-old female patient with Bland-White-Garland syndrome (anomalous origin of the left coronary artery from the pulmonary artery) who underwent mitral valve replacement (MVR) and coronary artery bypass grafting. She had previously undergone MVR and had been treated by the Takeuchi procedure for mitral valve regurgitation and a coronary artery anomaly, respectively, at 17 years of age. She was hospitalized with shortness of breath within 7 years of surgical intervention. Swan-Ganz catheterization revealed mild pulmonary hypertension and elevated mean pulmonary wedge pressure. Echocardiography revealed marked severe calcification of the bio valve and moderate mitral valve regurgitation. Coronary angiography revealed retrograde collateral blood flow from the right coronary artery to the left coronary artery, but intrapulmonary tunnel was not visualized by pulmonary arteriography. We performed MVR with a mechanical valve and coronary artery bypass grafting between the left internal thoracic artery and the left anterior descending artery. The postoperative course was unremarkable, and no complaints were reported during follow-up at the outpatient clinic.
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Introduction
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Bland-White-Garland (BWG) syndrome is a congenital anomaly rarely seen in adulthood. As the course of this disease in the first year of life is usually very severe, few patients with this anomaly reach adulthood, although various operative procedures for its treatment have been reported. Recently, several investigations have reported the successful surgical treatment of adult-type BWG syndrome. However, mitral valve replacement (MVR) and coronary artery bypass grafting (CABG) have not been performed previously. In this study, we present the first successful case of MVR and CABG for adult-type BWG syndrome.
A 25-year-old female was admitted to our institute after presenting with dyspnea on exertion. The patient had been diagnosed with mitral valve regurgitation in infancy, and she had undergone MVR with a Carpentier-Edwards prosthesis (Edwards Lifesciences LLC, Irvine, CA), and was treated by the Takeuchi procedure for a coronary artery anomaly at another hospital when she was 17 years old. Her immediate postoperative course was unremarkable after the Takeuchi procedure and MVR, but she became symptomatic within 7 years because of worsening mitral valve stenosis and myocardial ischemia. Electrocardiography revealed a normal sinus rhythm, but ST-segment depression was detected in leads V4 to 6. Echocardiography revealed marked mitral valve stenosis, mild insufficiency due to calcification of the bioprosthesis, and enlargement of the left atrium. Radionuclide cardiac imaging revealed myocardial ischemia with infarction of the anteroseptal region, with viable tissue surrounding this area. The Swan-Ganz catheterization (Baxter Healthcare, Deerfield, IL) revealed a pulmonary artery pressure of 68/30 mm Hg, and a pulmonary capillary arterial wedge pressure of 15 mm Hg. Cardiac catheterization was unable to confirm the patency of the left coronary artery, but it did reveal that the intrapulmonary tunnel was not patent. In spite of this, marked retrograde collateral blood flow from the right coronary artery to the left coronary artery was noted (Fig 1).
A ventriculogram of the left ventricle revealed an ejection fraction of 0.69, with moderate mitral regurgitation.
A median sternotomy was performed and revealed marked adhesion, particularly around one of the pulmonary arteries. A cardiopulmonary bypass was established from the superior and inferior vena cava to the ascending aorta. After cross-clamping the ascending aorta, cold crystalloid cardioplegic solution was antegradely administered through the aortic root. After the resection of the prosthetic valve, MVR was performed using an ATS valve (27 mm; ATS Medical, Minneapolis, MN). The left anterior descending (LAD) artery was easily located. The left internal thoracic artery (LITA)was then anastomosed to the LAD artery and a two coronary system was established. The patient was easily weaned from the cardiopulmonary bypass. The resected valve exhibited marked calcification of all cusps. Postoperative cardiac catheterization revealed normal left ventricular function, a successful LITA to LAD bypass (Fig 2), and the absence of mitral regurgitation.
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Fig 2. Postoperative cardiac catheterization revealed satisfactory patency between the left internal thoracic artery and the left anterior descending artery.
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Her postoperative course was unremarkable, and she has remained healthy since then, as assessed by follow-up as an outpatient.
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Comment
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Of the many congenital anomalies of the coronary arterial system, the anomalous origin of the left coronary artery, has the highest mortality rate. This anomaly was first described in 1886 by Brooks [1], and the clinical syndrome was reported by Bland, White, and Garland in 1933 [2]. Agustsson and coworkers [3] were the first to classify this disease into infantile and adult types. Without treatment, the mortality of this anomaly is high in the first year of life, and is usually caused by congestive heart failure, myocardial infarction, or mitral valve insufficiency. Patients with this anomaly occasionally reach adulthood [4]. In patients with the adult type, intracoronary collateral blood flow develops and oxygenated blood flow is supplied from the right coronary. Such patients are usually asymptomatic in infancy and early childhood, but usually manifest symptoms of myocardial ischemia later in life.
Many surgical approaches have been described for the management of Bland-White-Garland syndrome, including ligation of the anomalous left coronary artery, direct aortic reimplantation of the left coronary artery, anastomosis of left subclavian artery to anomalous of the left coronary artery, autologous or venous bypass grafting, and an intrapulmonary tunnel procedure [5]. However surgical ligation has a prohibitively high mortality rate, especially in infants with poorly developed coronary collateral circulation. The standard surgical procedure for this anomaly is the construction of a two coronary system. Autologous or venous bypass grafting and the intrapulmonary tunnel procedure have a higher reported mortality rate than this procedure, because of obstruction of the graft or tunnel. Recently, many authors have recommended coronary artery reimplantation because it can be performed safely and because it yields a satisfactory good arterial patency rate [6]. But reimplantation is difficult in some patients because of the distance from anomalous of the left coronary artery to the aorta. For these patients, intrapulmonary tunnel procedure is preferable. In this patient a Takeuchi procedure was performed using Gore-Tex soft tissue patch (W.L. Gore & Associates, Flagstaff, AZ), but occluded immediately after operation, so we considered that suitable material for intrapulmonary tunnel is autograft rather than prosthetic material. In addition, it is important to keep the pulmonary artery pressure normal level as much as possible. In our patient, we did not perform direct aortic reimplantation of the left coronary artery because she had previously undergone treatment by the Takeuchi procedure, and the tunnel had already occuluded. Instead, we performed a bypass from the left internal thoracic artery to the LAD in addition to the concomitant replacement of the mitral valve.
We present a rare case of adult-type BWG syndrome and its successful surgical management. This report presents a patient with anomalous left coronary artery arising from the pulmonary artery undergoing MVR and coronary artery bypass.
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References
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- Brooks H.StJ Two cases of an abnormal coronary artery arising from the pulmonary artery. J Anat Physiol 1885;20:26-29.[Abstract]
- Bland E.F., White P.D., Garland J. Congenital anomalies of the coronary arteries: report of an usual case associated with cardiac hypertrophy. Am Heart J 1933;8:787-789.[Medline]
- Agustsson M.H., Gasul B.M., Fell W.H., et al. Anomalous origin of the left coronary artery from pulmonary artery. JAMA 1962;180:15-21.[Abstract]
- Wesselhoeft H., Fawcett J.S., Johnson A.L. Anomalous origin of the left coronary artery from the pulmonary trunk. Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. Circulation 1968;38:403-407.[Medline]
- Takeuchi S., Imamura H., Katsumoto K., et al. New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg 1979;78:7-11.
- Backer C.L., Stout M.L., Zales V.R., et al. Anomalous origin of the left coronary artery: a twenty-year review of surgical management. J Thorac Cardiovasc Surg 1992;103:1049-1058.[Medline]
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Bland-White-Garland Syndrome in Pregnancy: Reoperation of ALCAPA With an Internal Thoracic Radial Artery "Y"-Graft
Ann. Thorac. Surg.,
April 1, 2006;
81(4):
1512 - 1514.
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Abstract
Introduction
