CCTA in the clinical workflow of athletes with anomalous origin of coronary arteries from the contralateral valsalva sinus

Abstract Background The anomalous origin of coronary arteries is a rare congenital heart disease, with estimated prevalence of 0.3-5.8% depending on definition, evaluation methods and studied populations, ranging from those considered an incidental finding or just morphologic variants without any clinical implications, to those that may have hemodynamic effects. An early diagnosis of coronary anomalies, especially the so-called "malignant" variants, are a crucial diagnostic challenge, being a relevant cause of sudden cardiac death in young athletes. Purpose To evaluate the role of coronary CT angiography (CCTA) in the workflow of competitive sports eligibility in a cohort of athletes with anomalous origin of the left-coronary artery (AOLCA) or of the right-coronary artery (AORCA) to outline relevant CT findings that may have an impact on diagnostic evaluation and clinical management. Methods Athletes with suspected AOLCA/AORCA at TTE or with inconclusive TTE underwent CCTA to exclude/confirm and characterize specific anatomical findings including partially- or full-interarterial course (INT), high take-off (HTO), acute take-off angle (ATO), slit-like origin, intramural course (IM), interarterial course length, and lumen reduction or hypoplasia (HYPO). Results CCTA identified 28 athletes: 6 AOLCA (3 males, 20.3±11.0) and 22 AORCA (18 males, 29.1±16.5). Symptoms were present only in 13 athletes (46.4%; 10 AORCA). Four patients (3 AORCA) had abnormal rest-ECG, 11 (40.7%; 9 AORCA) had abnormal stress-ECG. The INT course was observed in 15 athletes (53.6%): 6/6 AOLCA and 9/22 AORCA (40.9%). Slit-like origin was present in 7/22 AORCA (31.8%) and never in AOLCA. Suspected IM resulted in 3 AOLCA (50%), always with HYPO/ATO, and in 6/22 AORCA (27.3%) with HYPO. No statistically significant differences were found between asymptomatic/symptomatic patients in the prevalence of partially- o full-INT courses, high-take-off/ATO and slit-like ostium. A slightly significant relationship between suspected proximal-IM (r=0.47, P0.05) and proximal-HYPO of anomalous vessel (r=0.65, P0.01) resulted in AORCA and was confirmed on AOLCA/AORCA pooled analysis (r=0.58, P0.01 for HYPO). All athletes were disqualified from competitive sports and warned to avoid vigorous physical efforts. Surgery was recommended to all AOLCA athletes and to 13 AORCA (3 asymptomatic), but only 6 underwent surgery. No major cardiovascular event/ischemic symptoms/signs developed during a mean follow-up of 49.6±39.5 months. The mean effective radiation dose was 1.64±0.65 mSv. Conclusion CCTA assessment was crucial in the process of detection/characterization of aberrant anatomy and risk stratification, definition of therapeutic strategies and, together with clinical and ECG findings, to propose/decide restriction/suspension of sports activity or permission to return to sports after surgical correction, supporting CCTA as mandatory in the diagnostic workflow together with TTE and stress-ECG.