| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
J Thorac Cardiovasc Surg 1994;107:1538-1539
© 1994 Mosby, Inc.
LETTERS TO THE EDITOR |
Division of Thoracic and Cardiovascular Surgery
Division of Anatomic Pathology
Mayo Clinic
Rochester, MN 55905
To the Editor:
The arterial switch procedure is the treatment of choice for complete transposition of the great arteries Although the various coronary artery patterns in transposition and their influence on outcome of the arterial switch operation have been studied extensively,
1-7 congenital causes of coronary insufficiency have not been emphasized. We report the case history of a patient with transposition of the great arteries and a congenital ostial membrane of the right coronary artery.
In 1974, a 30-month-old white girl with transposition of the great arteries {S,D,D} underwent a Mustard procedure at the Mayo Clinic. The initial postoperative course was uneventful and the patient was discharged on the tenth day after the operation. Twelve days later, however, fever and bilateral chylothorax developed. Cardiac catheterization showed the intraatrial baffle to be intact. Through a right thoracotomy the thoracic duct was ligated. Unfortunately, progressive cachexia and sepsis developed despite adequate nutritional support and treatment with broad-spectrum antibiotics. The child died of sepsis 30 days after the operation.
At autopsy, the cardiac repair was found to be intact without signs of obstruction at the atrial level or endocarditis. The left coronary artery originated from the commissure between the right and left sinuses of Valsalva. Histologic sections of the origins of both coronary arteries showed that the proximal left coronary artery had an aortic intramural course and that the right coronary artery had a congenital ostial membrane that was unrelated to an aortic valve cusp (Fig. 1). No evidence of myocardial infarction was detected.
|
1-8 In certain patterns performance of coronary artery transfer without tension and kinking may be technically more demanding. 3,4,6 Other causes that may result in impaired coronary perfusion in patients with transposition have not been reported.
Classic (or "forme fruste") supravalvular aortic stenosis has been reported in association with occlusion of the ostium of a coronary artery; the occlusion is due to adhesion of the free edge of an aortic cusp to the area of aortic narrowing distal to the ostium of the involved coronary artery. 9-11 Coronary artery stenosis caused by a congenital ostial membrane in a patient without supravalvular aortic stenosis, as presented in this report, is an extremely uncommon anomaly. Such a membrane at the ostium of the left coronary artery has previously been reported in two patients from our institution, one with valvular aortic stenosis and the other with truncus arteriosus; both patients were successfully treated by operation. 12 In the latter patient, severe impairment of ventricular function developed after termination of extracorporeal circulation, and secondary excision of the obstructing membrane resulted in full recovery of ventricular function.
To our knowledge, this is the first report of congenital ostial membrane of the right coronary artery in a patient without features of supravalvular aortic stenosis. During life, the diagnosis is extremely difficult to establish because an aortic root injection often fails to visualize the coronary arteries satisfactorily, while selective ostial injection displaces the obstructing membrane resulting in a normal appearance of the injected vessel. Establishing the diagnosis of congenital ostial membrane requires a high index of suspicion, and the diagnosis is best made during the operation. Although the reported anomaly is rare (with only one case in 255 autopsied hearts with transposition of the great arteries at our institution), the reported case serves as a reminder that this unusual cause of coronary artery obstruction may occur. Therefore, in patients with myocardial ischemia after an arterial switch procedure with coronary transfer, after exclusion of more common causes of coronary insufficiency, the possibility of a congenital coronary ostial membrane should be evaluated.
References
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| 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 |
