Abstract

BackgroundCurrent guidelines on coronary anomalies are primarily based on expert consensus and a limited number of trials. A gold standard for diagnosis and a consensus on the treatment strategy in this patient group are lacking, especially for patients with an anomalous origin of a coronary artery from the opposite sinus of Valsalva (ACAOS) with an interarterial course.AimTo provide evidence-substantiated recommendations for diagnostic work-up, treatment and follow-up of patients with anomalous coronary arteries.MethodsA clinical care pathway for patients with ACAOS was established by six Dutch centres. Prospectively included patients undergo work-up according to protocol using computed tomography (CT) angiography, ischaemia detection, echocardiography and coronary angiography with intracoronary measurements to assess anatomical and physiological characteristics of the ACAOS. Surgical and functional follow-up results are evaluated by CT angiography, ischaemia detection and a quality-of-life questionnaire. Patient inclusion for the first multicentre study on coronary anomalies in the Netherlands started in 2020 and will continue for at least 3 years with a minimum of 2 years of follow-up. For patients with a right or left coronary artery originating from the pulmonary artery and coronary arteriovenous fistulas a registry is maintained.ResultsPrimary outcomes are: (cardiac) death, myocardial ischaemia attributable to the ACAOS, re-intervention after surgery and intervention after initially conservative treatment. The influence of work-up examinations on treatment choice is also evaluated.ConclusionsStructural evidence for the appropriate management of patients with coronary anomalies, especially (interarterial) ACAOS, is lacking. By means of a structured care pathway in a multicentre setting, we aim to provide an evidence-based strategy for the diagnostic evaluation and treatment of this patient group.Supplementary InformationThe online version of this article (10.1007/s12471-021-01556-9) contains supplementary material, which is available to authorized users.

Highlights

  • Coronary anomalies are rare and often an incidental finding in patients without concomitant congenital heart defects

  • By means of a structured care pathway in a multicentre setting, we aim to provide an evidence-based strategy for the diagnostic evaluation and treatment of this patient group

  • Conflicting reports and lack of evidence-substantiated guidelines lead to challenging treatment decisions, especially for anomalous coronary arteries originating from the opposite sinus of Valsalva or from the opposite coronary artery (ACAOS)

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Summary

Conclusions

Structural evidence for the appropriate management of patients with coronary anomalies, especially (interarterial) ACAOS, is lacking. By means of a structured care pathway in a multicentre setting, we aim to provide an evidence-based strategy for the diagnostic evaluation and treatment of this patient group. Keywords Coronary anomalies · Anomalous coronary artery from the opposite sinus of Valsalva · Coronary arteriovenous fistula · Anomalous coronary artery from the pulmonary artery · Multicentre study circumflex artery, R right, L left, A anterior, P posterior, CT computed tomography

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