摘要
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.
