Background: Anomalous origin of the coronary artery from the contralateral coronary sinus is a rare coronary anomaly associated with sudden death. The inter-arterial course is most closely associated with sudden death, but it has been suggested that the presence of an intramural segment of a right anomalous coronary is associated with more symptoms and therefore may be an important criterion for intervention in these patients.
Objective: To demonstrate that MR angiography can accurately determine the presence or absence of an intramural segment in an anomalous coronary artery.
Materials and methods: All studies of children who underwent MR angiography for the evaluation of an anomalous coronary artery were retrospectively reviewed by two pediatric radiologists in consensus. Criteria for an intramural anomalous coronary artery were the presence of a small or slit-like ostium and the relative smaller size of the proximal intramural portion of the coronary artery in relation to the more distal epicardial coronary artery. The anomalous coronary artery was classified as not intramural if these two findings were absent. These findings were correlated with operative reports confirming the presence or absence of an intramural segment.
Results: Twelve patients (86%) met MR angiography criteria for the presence of an intramural course. Only 2 patients (14%) met MR angiography criteria for a non-intramural course. When correlating with intraoperative findings, MR angiography was successful in distinguishing between intramural and non-intramural anomalous coronary arteries in all cases (P = 0.01).
Conclusion: MR angiography may be able to reliably identify the intramural segment of an anomalous coronary artery in older children using the imaging criteria of a small or slit-like ostium and relative decrease in size of the proximal portion of the anomalous coronary artery compared to the distal portion of the anomalous coronary artery. Determining the presence of the intramural segment may help with surgical planning and may be an important criterion for the determination of intervention in patients with inter-arterial anomalous right coronary arteries.