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

Alberto Cipriani, Pietro Bernardo Dall’aglio, Laura Mazzotta, Domenico Sirico, George Sarris, Mark Hazekamp, Thierry Carrel, Alessandro Frigiola, Vladimir Sojak, Mauro Lo Rito, Jurgen Horer, Regine Roussin, Julie Cleuziou, Bart Meyns, Jose Fragata, Helena Telles, Anastasios C. Polimenakos, Katrien Francois, Altin Veshti, Jukka SalminenAlvaro Gonzalez Rocafort, Matej Nosal, Eleftherios Protopapas, Roberto Tumbarello, Patrizio Sarto, Cinzia Pegoraro, Raffaella Motta, Giovanni Di Salvo, Domenico Corrado, Vladimiro L. Vida, Massimo A. Padalino

Research output: Contribution to journalArticlepeer-review

3 Scopus citations


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 electrocar-diogram (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. Syn-cope seems to be strictly correlated to AAOLCA with inter-arterial course.

Original languageEnglish
Pages (from-to)375-385
Number of pages11
JournalCongenital Heart Disease
Issue number4
StatePublished - 2022
Externally publishedYes


  • Anomalous coronary arteries
  • congenital
  • coronary computed tomography angiography
  • echocardiography


Dive into the research topics of 'Diagnostic Yield of Non-Invasive Testing in Patients with Anomalous Aortic Origin of Coronary Arteries: A Multicentric Experience'. Together they form a unique fingerprint.

Cite this