Abstract

BackgroundAnomalous coronary artery originating from the opposite sinus of Vasalva with interarterial course (ACAOS-IAC) is associated with sudden cardiac death (SCD) in young athletes. If identified in adulthood prognosis is usually more benign, resulting in a dilemma regarding revascularization. MethodsThis is a retrospective observational single-center study, including adults with ACAOS-IAC. Medical records between 2012 and 2019 were reviewed for management approach, mortality, cardiac death and coronary related adverse events. Coronary computed tomographic angiography (CCTA) were reviewed. We provide a literature review in regard to clinical outcome. ResultsWe identified 40 patients with ACAOS-IAC (mean age 51). Presentation was acute in 7/40 (18%). Ischemia detection with single photon emission tomography (SPECT), cardiac magnetic resonance (CMR) or dobutamine stress echocardiography were performed in 25/40 (63%) patients. Ischemia in the vascular territory of the anomaly was present in 2/25 (8%). In 39/40 (98%) patients were treated expectative. During median follow-up of 2.7 years (IQR 1.5–5.3) no cardiovascular death was observed. Mortality occurred in 1/40 (3%) and coronary related adverse events in 2/40 (5%). We identified 20 studies describing 1194 patients. Revascularization was performed in 376/1154 (32.6%) patients. Mortality stratified for clinical management was 23/431 (5.3%) in the non-revascularization versus 16/253 (6.3%) in the revascularization group during 4.0 years follow-up (weighted median). Cause of death was cardiovascular in 10/596 (1.7%) in 4.2 years (weighted median) follow up. ConclusionsBoth revascularization and non-invasive management have good prognosis in adults with ACAOS-IAC during early follow up. There is need for guidelines and long-term surveillance.

Highlights

  • Anomalous origin of the coronary artery originating from the opposite sinus of Valsalva with an interarterial course (ACAOS-IAC) between the aorta and pulmonary artery is a rare congenital cardiac abnormality

  • Emergency ICA was performed which showed a left ACAOS-IAC and suspicion of a dissected interarterial segment which was treated with PCI

  • Post-procedure Coronary computed tomographic angiography (CCTA) showed proximal from the coronary stent tapering of the anomalous coronary artery either due to residual dissection or proximal intramural course

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Summary

Introduction

Anomalous origin of the coronary artery originating from the opposite sinus of Valsalva with an interarterial course (ACAOS-IAC) between the aorta and pulmonary artery is a rare congenital cardiac abnormality. ACAOS-IAC is often classified as malignant because it is associated with sudden cardiac death (SCD) in young athletes [2]. Identification of this coronary anomaly in adults is believed to be usually more benign but long-term follow-up is missing. Criteria for the necessity of surgical treatment in adults remain controversial and existing guidelines are not always being followed [3,4,5] This is a rare congenital anatomic variant, findings are likely to increase due to a more widespread use of coronary computed tomographic angiography (CCTA). Anomalous coronary artery originating from the opposite sinus of Vasalva with interarterial course (ACAOS-IAC) is associated with sudden cardiac death (SCD) in young athletes.

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