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Cipriani, A.; Dall'Aglio, P.B.; Mazzotta, L.; Sirico, D.; Sarris, G.; Hazekamp, M.; ... ; Padalino, M.A. (2022)

Diagnostic Yield of Non-Invasive Testing in Patients with Anomalous Aortic Origin of Coronary Arteries: A Multicentric Experience

Article / Letter to editor
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,...Show moreBackground: 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.Show less
Anomalous coronary arteries
congenital
echocardiography
coronary computed tomography angiography
All authors
Cipriani, A.; Dall'Aglio, P.B.; Mazzotta, L.; Sirico, D.; Sarris, G.; Hazekamp, M.; Carrel, T.; Frigiola, A.; Sojak, V.; Rito, M. lo; Horer, J.; Roussin, R.; Cleuziou, J.; Meyns, B.; Fragata, J.; Telles, H.; Polimenakos, A.C.; Francois, K.; Veshti, A.; Salminen, J.; Rocafort, A.G.; Nosal, M.; Protopapas, E.; Tumbarello, R.; Sarto, P.; Pegoraro, C.; Motta, R.; Salvo, G. di; Corrado, D.; Vida, V.L.; Padalino, M.A.
Date
2022-01-01
Journal
Congenital Heart Disease
Volume
17
Issue
4
Pages
375 - 385
DOI
doi:10.32604/chd.2022.019385