Ann Thorac Surg 1998;66:1819-1820
© 1998 The Society of Thoracic Surgeons
Case Reports
Delayed diagnosis of anomalous origin of the left coronary artery 16 years after mitral valve replacement
Akihiko Ohkado, MDa,
Masafumi Yashima, MDa,
Masakuni Ishiyama, MDa,
Shigehiro Morishima, MDa,
Eiichi Tei, MDa
a Department of Cardiovascular Surgery, Ayase Heart Hospital, Tokyo, Japan
Accepted for publication April 29, 1998.
Address reprint requests to Dr Ohkado, Department of Cardiovascular Surgery, Heart Institute of Japan, Tokyo Women’s Medical College, 8-1 Kawada-cho, Shinjuku-ku, Tokyo, Japan 162
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Abstract
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Mitral insufficiency caused by ischemia is frequently found in anomalous origin of the left coronary artery from the pulmonary artery. We report a case of a 25-year-old woman who was diagnosed to have anomalous origin of the left coronary artery from the pulmonary artery and had successful left internal mammary artery bypass grafting 16 years after mitral valve replacement for mitral insufficiency of an unknown cause in her childhood.
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Introduction
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A nomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) or Bland-White-Garland syndrome, a rare congenital anomaly, is frequently associated with mitral insufficiency (MI) caused by ischemia. We report a case in which ALCAPA was diagnosed and surgically treated 16 years after mitral valve replacement was performed for MI at the age of 9 years.
In 1981, a 9-year-old girl with a diagnosis of severe MI and pulmonary hypertension (main pulmonary artery pressure, 39/20 mm Hg) underwent mitral valve replacement with a Björk-Shiley 27-mm mechanical valve. Preoperatively, MI was evaluated by echocardiography and cardiac catheterization. An aortogram was performed, but a coronary angiogram was not performed. The operation was performed on an empty, beating heart. There were clear clefts in the posterior mitral leaflet, forming a developed commissure cusp in the posterior medial commissure. The posterior papillary muscle was bifurcated into two parts, each reaching the anterior leaflet and commissural cusp. Regurgitation was observed through both commissures. The cause of the valvular malformation was possibly congenital but could not be determined.
The patient had been followed up thereafter at our outpatient clinic. However, at the age of 25 years, in March 1997, she started to feel easy fatiguability and was admitted to our hospital for further examination. A grade 3 systolic murmur was heard in the middle left sternal border. No rales were audible over either lung fields. The electrocardiogram showed a normal sinus rhythm at a rate of 80 beats/min, with normal axis deviation. A chest x-ray film showed the prominence of the pulmonary artery and increased pulmonary vascularity. Cardiac catheterization revealed a step-up of oxygen saturation from 72.9% to 79.1% at the level of the main pulmonary artery. The left-to-right shunt ratio was 0.54, and the ratio of pulmonary to body blood flow was 2.17. A coronary angiogram showed that the right coronary artery arising from the ascending aorta was enormously dilated and tortuous, retrogradely filling the left coronary artery and pulmonary trunk through collaterals. The left circumflex artery was hypoplastic (Fig 1). Thallium scintigram showed hypoperfusion in the left anterior and apical wall. She was diagnosed as having ALCAPA.
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Fig 1. Preoperative right coronary angiography. Note that the right coronary artery arising from the ascending aorta is enormously dilated and tortuous, retrogradely filling the left coronary artery (arrow) and main pulmonary artery (not shown here) through collaterals.
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An operation was performed on June 10, 1997. The heart was exposed through a median sternotomy. The left coronary artery originated from the nonfacing sinus of the main pulmonary artery. There was a palpable thrill over the main pulmonary artery. The left main coronary trunk was too short to translocate to the aorta. The left internal mammary artery was dissected to the length of approximately 10 cm. After the institution of the cardiopulmonary bypass, the heart was arrested with induction of cardioplegia. The left main coronary trunk was doubly ligated with 4-0 polypropylene, and the left internal mammary artery was anastomosed to the left anterior descending artery with 8-0 polypropylene.
Her postoperative course was uneventful. Postoperative coronary angiography showed excellent patency of the graft. A postoperative thallium scintigram on the 72nd postoperative day showed remarkable recovery of perfusion in the left anterior and apical wall.
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Comment
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Anomalous origin of the left coronary artery from the pulmonary artery is an extremely rare congenital anomaly. It is reported that most patients, if untreated, die in infancy of myocardial ischemia [1]. Mitral insufficiency, which is frequently associated with this disease, suggests the existence of ischemic dysfunction of papillary muscles [2, 3]. Because of the distribution of the left coronary artery, the anterior papillary muscle usually shows dysfunction. In our patient, it is not clear whether the cause of the malformated leaflet and papillary muscle was a true congenital defect [4] or ischemia caused by ALCAPA. It is interesting that our patient never showed ischemic symptoms until the age of 25 years. We think that there are two possible reasons. First, the disease appeared as mitral insufficiency in her childhood. Moreover, mitral valve replacement was performed on an empty, beating heart. Therefore, although this is pure speculation, infusion of cardioplegic solution from the aortic root might have been able to suggest the coronary anomalies because of hardly attainable cardiac arrest. Second, remarkable development of collateral vessels effectively prevented acute aggravation of ischemia [5].
Several surgical strategies have been reported for this disease [6]. We think our method was simple and reasonable in that reestablishment of two coronary systems is recommended to achieve sufficient coronary perfusion, and a reimplantation technique is unavailable for the anomalous coronary artery originating from the nonfacing sinus of the main pulmonary artery [3].
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References
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- Moodie D.S., Fyfe D., Gill C.C., 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;106:381-388.[Medline]
- Bojar R.M., Ilbawi M.N., DeLeon S.Y., Riggs T.W., Idriss F.S. Surgical management of anomalous left coronary artery with mitral insufficiency in infancy: contribution of echocardiography. Pediatr Cardiol 1984;5:35-38.[Medline]
- Ortiz De Salazar A., Juanena C., Aramendi J.I., Castellanos E., Cabrera A., Agosti J. Anomalous origin of the left coronary artery from the pulmonary artery. Surgical alternatives depending of the age of the patient. J Cardiovasc Surg 1990;31:801-804.[Medline]
- Carpentier A., Branchini B., Cour J.C., et al. Congenital malformations of the mitral valve in children. Pathology and surgical treatment. J Thorac Cardiovasc Surg 1976;72:854-866.[Abstract]
- Askenazi J., Nadas A.S. Anomalous left coronary artery originating from the pulmonary artery: report on 15 cases. Circulation 1975;51:976-987.[Abstract/Free Full Text]
- Backer C.L., Stout M.J., 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.[Abstract]
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