Clinical and cardiac CT characteristics of congenital coronary abnormalities occasionally detected in a middle-aged population: A long-term follow-up study

Introduction: Congenital coronary artery anomalies (CCAA) represent one of the most challenging conditions as their clinical presentation may range from sudden cardiac death to a complete subclinical form. The aim of the present study was to evaluate the diagnostic and prognostic role of cardiac CT (CCT) evaluation in patients with CCAA, focusing on anomalies of origin. Material and methods: The present is a retrospective analysis of a prospective clinical registry including a consecutive cohort of patients who underwent clinically indicated CCT from January 2007 to October 2015 for suspected but unknown coronary artery disease (CAD) and were diagnosed for having a congenital coronary abnormality compared to a control group matched for age, sex and segment stenosis score (SSS). Dedicated analysis of all CCT was performed for the present study and only coronary anomalies of origin were considered and included in the study. Two different composite end-points were identified for the present analysis: major cardiovascular events (MACE) and all-cause of death. Results: Among the 81 patients with CCAAs enrolled the most frequent anomaly was left main artery absence, which was identified in 41 individuals (50.6%). Forty-five subjects (55.5%) have an anomalous origin of the coronary artery from a different sinus of Valsalva and 45 subjects had also an anomalous course with the retro-aortic being the most common (32%). Eleven participants (13.6%) displayed also an intramural segment, while 10 (10.3%) had a slit-like ostial morphology. At multivariate analysis CT identification of ARCA, anomalous inter-arterial course and abnormal ostial morphology were significantly associated with MACE even when adjusted for age and SSS, without any differences in all-cause mortality between the two groups (6.2% vs 2.4% p 1/4 0.2478). Conclusion: The result of the present study is that CCT can be successfully used to define the anatomy and features of CAA. It suggested that in middle-aged patients, the identification of high risk characteristics at CT may have a prognostic value in term of cardiovascular events occurrence at follow-up even if the rate of events strictly linked to CCAA is low.