Left main coronary artery atresia: literature review and therapeutical considerations

Left main coronary artery atresia is a rare coronary anomaly in which there is no left coronary ostium, the proximal left main trunk ends blindly, blood flows from the right coronary artery to the left via small collateral arteries and retrogradely in at least one of the left-sided arteries. Since published case reports are few and rather scattered, no comprehensive information about this uncommon anomaly is available.

A through search for published cases of left main coronary atresia was performed in the major medical journals by electronic (MEDLINE and INTERNET) and hand-scanning.

The authors found 28 cases of left main atresia (including two from their own experience), 15 of which were pediatric; five of these cases had associated cardiac anomalies. While pediatric patients were usually overtly symptomatic early in their life (syncope, dyspnea, sudden death, failure to thrive, infarction, ventricular tachycardia), adult patients began showing symptoms (angina, dyspnea, sudden death) only at an advanced age; associated coronary atherosclerosis seemed to be uncommon, though (2/13 adult patients, 15%). We know of only one asymptomatic patient, a 76-year old lady who had died of unrelated causes; in her case left main atresia was an unforeseen autopsy finding. Four untreated patients had died suddenly; most of the others were highly symptomatic and required surgical therapy, usually as direct coronary artery revascularization via one or more saphenous vein or mammary artery grafts to the left-sided arteries; all revascularized patients were reported to be alive and well; in one pediatric case the left main coronary artery was reconstructed using an aortic wall baffle, with a good result. In contrast, the outcome of patients who did not receive revascularization has been poor.

In light of the favorable results obtained by surgical therapy, the authors endorse prompt coronary artery revascularization for all patients with left main coronary artery atresia.