Registry for Invasive and Non-invasive Anatomical Assessment and Outcome of Coronary Artery Anomalies

Anomalous coronary arteries (CAA) represent a congenital disorder hallmarked by an anomalous location of the coronary ostium and/or vessel course. Based on the largest study assessing the prevalence and characteristics of CAA detected by coronary computer tomography angiography (CCTA) in Switzerland, the overall prevalence of CAA is 2.6%. However, depending of the type of CAA and its course in relation to the big vessels (aorta and pulmonary artery), not every CAA is accompanied by an increased cardiovascular risk.

Of particular interests are anomalous coronary arteries from the opposite sinus of Valsalva with an interarterial course (ACAOS-IC). Those CAAs represents a congenital disorder with an anomalous location of the coronary ostium and a course of the anomalous vessel between the aorta and the pulmonary artery. The prevalence of ACAOS-IC in the general population is around 1 % and even they are mostly clinically insignificant and remain often undetected.

However, some variants of ACAOS-IC are associated with adverse cardiac events (e.g. sudden cardiac death in young athletes). The possible presence of an intramural course, a course within the aortic wall is the primary cause for an oval proximal vessel shape and proximal vessel narrowing and is suggested to be the primary cause for ischemia. These features may lead under stress conditions to a "dynamic compression" of the vessel (compared to "fixed" stenosis in coronary artery disease). Therefore, to mimic these conditions, dobutamine and volume challenge is used to invasively measure fractional flow reserve (FFRDobutamine) during coronary angiography and is seen as the gold standard in assessing the hemodynamic relevance of ACAOS-IC. Presence of an abnormal FFRDobutamine is one of the most important factors in the decision-making towards surgical repair in ACAOS-IC.

With the frequent use of invasive and non-invasive imaging to rule out coronary artery disease, an increase in absolute numbers of ACAOS-IC is seen and physicians are more confronted with the dilemma of how to manage these patients. Usually the question is whether the ACAOS-IC is a coincidental finding or if the anomaly is causative for the patients' symptoms. Thus, systematic acquisition of all available diagnostic methods (invasive and non-invasive) is required to assess the optimal diagnostic procedure for this patients.

The presented project will help to understand the pathophysiology of CAAs with particular focus on ACAOS-IC and improve risk stratification based on non-invasive imaging.

• Quantitative assessment (in SI-Units) of the anatomical features in the coronary computed tomography angiography (CCTA) [Time frame: 5 years]
• Invasive assessment of hemodynamic relevance [Time frame: Through clinically indicated diagnostic evaluation, an average of 2 months]
• Computational fluid dynamics [Time frame: Through clinically indicated diagnostic evaluation, an average of 2 months]
• IVUS [Time frame: Through clinically indicated diagnostic evaluation, an average of 2 months]
• Sports [Time frame: at baseline, one year and 5 year after diagnosis]