Echocardiographic screening for the anomalous aortic origin of coronary arteries

Francesco Bianco, Massimo Colaneri, Valentina Bucciarelli, Francesca Chiara Surace, Federica Valentina Iezzi, Martina Primavera, Annaclara Biasi, Giuliano Giusti, Emanuela Berton, Monica Baldoni, Giulia Renda, Alessandra Baldinelli, Sabina Gallina, Marco Pozzi, Francesco Bianco, Massimo Colaneri, Valentina Bucciarelli, Francesca Chiara Surace, Federica Valentina Iezzi, Martina Primavera, Annaclara Biasi, Giuliano Giusti, Emanuela Berton, Monica Baldoni, Giulia Renda, Alessandra Baldinelli, Sabina Gallina, Marco Pozzi

Abstract

Aims: We sought to determine the diagnostic performance, clinical profiles and outcomes of anomalous aortic origin of coronary arteries (AAOCA) using a standardised echocardiographic approach in young adults and athletes.

Methods: In 2015-2019, we screened 5998 outpatients (age 16 years (Q1-Q3: 11, 36)), referred for routine echocardiography, using four specific echocardiographic windows: parasternal short/long axis and apical 4/5-chambers view. Coronary CT confirmed AAOCA. For the performance analysis, 300 coronary-CT scans were available; two independent and double-blinded physicians retrospectively reviewed echocardiographic images.

Results: A total of 47 AAOCA was diagnosed; the overall prevalence was 0.0078%. Over 5 years, we found a significant increment of AAOCA diagnostic rate (P for trend=0.002). Syncope (n=17/47) and palpitations (n=6/47) were prevalent symptoms. All patients suspended sports activity at the diagnosis. Twenty-seven patients underwent surgery, while 20 underwent a conservative medical treatment. All patients are alive at a median follow-up of 3±1.6 years; only surgical repairs restarted their activity. Our method showed better sensitivity than traditional short-axis evaluation: 93% vs 83%, p=0.0030 (AUC 0.96 (95% CI 0.92, 0.99) and AUC 0.89 (95% CI 0.83, 0.95), respectively), with a good interobserver agreement (95%, k=0.83, p<0.001).

Conclusions: The application of a standardised echocardiographic approach for AAOCA detection led to a significantly increased rate of identified anomalies. This approach demonstrated higher sensitivity than the traditional echocardiographic assessment. Implementing this protocol in clinical practice may help improve the AAOCA diagnosis in young adults and athletes.

Trial registration number: NCT04224090.

Keywords: congenital heart disease; coronary artery disease; echocardiography.

Conflict of interest statement

Competing interests: None declared.

© Author(s) (or their employer(s)) 2021. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

Figures

Figure 1
Figure 1
Study design. AAOCA, anomalous aortic origin of coronary arteries; ALCAPA, aortic left coronary artery from the pulmonary artery; ARCAPA, aortic right coronary artery from the pulmonary artery. BAV, biscuspid aortic valve; CHD, congenital heart disease.
Figure 2
Figure 2
Two-dimensional echocardiographic 4-view approach for AAOCA detection: normal examination. Panel A: PSAX view. Panel B: PLAX view. Panel C: Ap-5Ch-view. Panel D: Ap-4Ch-view. AAOCA, anomalous aortic origin of coronary arteries; AO, aorta; Ap-4Ch-view apical four chambers view; Ap-5Ch-view, apical five chambers view; LA, left atrium; LAD, left anterior descending; LV, left ventricle; PLAX, parasternal long-axis view; PSAX, parasternal short-axis view; RA, right atrium; RCA, right coronary artery; RV, right ventricle; RVOT, right ventricular outflow tract. Modified from Lorber, R. et al.JACC Cardiovascular Imaging 2015;8:1239–49.
Figure 3
Figure 3
Two-dimensional echocardiographic 4-view approach for AAOCA detection: abnormal examinations and their anatomic correlates. AO, aorta; AOLCA, anomalous origin of the left coronary artery; AORCA, anomalous origin of the right coronary artery; Ap-4Ch-view, apical four chambers view; Ap-5Ch-view, apical five chambers view; LA, left atrium; LAD, left anterior descending; LCX, left circumflex artery; LV, left ventricle; PLAX, parasternal long-axis view; PSAX, parasternal short-axis view; RCA, right coronary artery; RV, right ventricle; RVOT, right ventricular outflow tract.

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