TY - EJOU AU - Cipriani, Alberto AU - Dall’Aglio, Pietro Bernardo AU - Mazzotta, Laura AU - Sirico, Domenico AU - Sarris, George AU - Hazekamp, Mark AU - Carrel, Thierry AU - Frigiola, Alessandro AU - Sojak, Vladimir AU - Rito, Mauro Lo AU - Horer, Jurgen AU - Roussin, Regine AU - Cleuziou, Julie AU - Meyns, Bart AU - Fragata, Jose AU - Telles, Helena AU - Polimenakos, Anastasios C. AU - Francois, Katrien AU - Veshti, Altin AU - Salminen, Jukka AU - Rocafort, Alvaro Gonzalez AU - Nosal, Matej AU - Protopapas, Eleftherios AU - Tumbarello, Roberto AU - Sarto, Patrizio AU - Pegoraro, Cinzia AU - Motta, Raffaella AU - Salvo, Giovanni Di AU - Corrado, Domenico AU - Vida, Vladimiro L. AU - Padalino, Massimo A. TI - Diagnostic Yield of Non-Invasive Testing in Patients with Anomalous Aortic Origin of Coronary Arteries: A Multicentric Experience T2 - Structural and Congenital Heart Disease PY - 2022 VL - 17 IS - 4 SN - 3071-1738 AB - Background: Anomalous aortic origin of a coronary artery (AAOCA) is a congenital heart disease with a 0.3%−0.5% prevalence. Diagnosis is challenging due to nonspecific clinical presentation. Risk stratification and treatment are currently based on expert consensus and single-center case series. Methods: Demographical and clinical data of AAOCA patients from 17 tertiary-care centers were analyzed. Diagnostic imaging studies (Bidimensional echocardiography, coronary computed tomography angiography [CCTA] were collected. Clinical correlations with anomalous coronary course and origin were evaluated. Results: Data from 239 patients (42% males, mean age 15 y) affected by AAOCA were collected; 154 had AAOCA involving the right coronary artery (AAORCA), 62 the left (AAOLCA), 23 other anomalies. 211 (88%) presented with an inter-arterial course. Basal electrocardiogram (ECG) was abnormal in 37 (16%). AAOCA was detected by transthoracic echocardiography and CCTA in 53% and 92% of patients, respectively. Half of the patients reported cardiac symptoms (119/239; 50%), mostly during exercise in 121/178 (68%). An ischemic response was demonstrated in 37/106 (35%) and 16/31 (52%) of patients undergoing ECG stress test and stress-rest single positron emission cardiac tomography. Compared with AAORCA, patients with AAOLCA presented more frequently with syncope (18% vs. 5%, P = 0.002), in particular when associated with inter-arterial course (22% vs. 5%, P < 0.001). Conclusion: Diagnosis of AAOCA is a clinical challenge due to nonspecific clinical presentations and low sensitivity of first-line cardiac screening exams. Syncope seems to be strictly correlated to AAOLCA with inter-arterial course. KW - Anomalous coronary arteries; congenital; echocardiography; coronary computed tomography angiography DO - 10.32604/chd.2022.019385