Published online Apr 26, 2014. doi: 10.4330/wjc.v6.i4.196
Revised: November 6, 2013
Accepted: March 13, 2014
Published online: April 26, 2014
Processing time: 221 Days and 3.8 Hours
AIM: To assess the current diagnostic and therapeutic management and the clinical implications of congenital single coronary artery (SCA) in adults.
METHODS: We identified 15 patients with a SCA detected from four Dutch angiography centers in the period between 2010 and 2013. Symptomatic patients who underwent routine diagnostic coronary angiography (CAG) for suspected coronary artery disease and who incidentally were found to have isolated SCA were analyzed.
RESULTS: Fifteen (7 females) with a mean age of 58.5 ± 13.78 years (range 43-86) had a SCA. Conventional CAG demonstrated congenital isolated SCA originating as a single ostium from the right sinus of Valsalva in 6 patients and originating from the left in 9 patients. Minimal to moderate coronary atherosclerotic changes were found in 4, and severe stenotic lesions in another 4 patients. Seven patients were free of coronary atherosclerosis. Runs of non-sustained ventricular tachycardia were documented in 2 patients, one of whom demonstrated transmural ischemic changes on presentation. Myocardial perfusion scintigraphic evidence of transmural myocardial ischemia was found in 1 patient due to kinking and squeezing of the SCA with an interarterial course between the aorta and pulmonary artery. Multi-slice computed tomography (MSCT) was helpful to delineate the course of the anomalous artery relative to the aorta and pulmonary artery. Percutaneous coronary intervention was successfully performed in 3 patients. Eight patients were managed medically. Arterial bypass graft was performed in 4 patients with the squeezed SCA.
CONCLUSION: SCA may be associated with transient transmural myocardial ischemia and aborted sudden death in the absence of coronary atherosclerosis. The availability and sophistication of MSCT facilitates the delineation of the course of a SCA. We present a Dutch case series and review of the literature.
Core tip: A Dutch case series of 15 adult patients with congenital isolated single coronary artery (SCA) are presented. Conventional coronary angiography demonstrated congenital isolated SCA originating as a single ostium from the right sinus of Valsalva in 6 patients and originating from the left in 9 patients. SCA may be associated with symptomatic transient transmural myocardial ischemia, non-sustained ventricular tachycardia, and aborted sudden death in the absence or presence of coronary atherosclerosis. The availability of multi-slice computed tomography (MSCT) and cardiovascular magnetic resonance imaging facilitates the delineation of the course of the anomalous vessel. MSCT was helpful to delineate the course of the anomalous artery relative to the aorta and pulmonary artery. Percutaneous coronary intervention was successfully performed in 3 patients. Eight patients were managed medically. Arterial bypass graft was performed in 4 patients with the squeezed SCA. The literature addressing SCA is reviewed.