DIAGNOSIS OF INTRAMURAL CORONARY-ARTERY IN TRANSPOSITION OF THE GREAT-ARTERIES USING 2-DIMENSIONAL ECHOCARDIOGRAPHY

Background. An intramural coronary is an uncommon but potentially sign ificant risk factor for transfer of the coronary arteries as part of t he arterial switch operation for transposition of the great arteries. Preoperative diagnosis is advantageous because it helps prevent accide ntal injury to the intramural coronary artery during transection of th e aortic root and excision of the coronary artery ostium from the aort a. Therefore, we investigated the reliability of two-dimensional echoc ardiography for detecting an intramural coronary artery in infants wit h d-transposition of the great arteries. Methods and Results. All infa nts with d-transposition of the great arteries who underwent echocardi ography and primary surgical repair at this institution between Januar y 1987 and June 1992 were identified by search of the cardiology data base. From this group, all patients diagnosed with an intramural coron ary artery were identified by review of the echocardiographic, surgica l, and autopsy reports. Among 435 infants with transposition, 29 infan ts were diagnosed as having an intramural coronary artery. In 27 cases , the diagnosis was confirmed at surgery or autopsy, and there were tw o false-positive echocardiographic diagnoses (specificity, 99.5%). Twe nty of the 27 patients with an intramural coronary artery were correct ly diagnosed prospectively by echocardiography (sensitivity, 75%), inc luding 17 of 23 patients with an intramural left coronary artery or le ft anterior descending coronary artery and 3 of 4 patients with an int ramural right coronary artery. Two primary diagnostic criteria were id entified: a major coronary artery arising from the contralateral septa l sinus, near the usually intercoronary commissure, and a course for t his vessel within the posterior aortic wall between the great arteries , creating a ''double-border'' appearance. Retrospective review using these criteria identified 26 of the 27 intramural arteries with no fal se-positive diagnoses. Conclusions. We conclude that coronary echocard iography is a very promising technique for detecting an intramural cor onary artery in transposition of the great arteries. Careful prospecti ve application of the identified diagnostic criteria should greatly im prove the diagnostic accuracy.