Multi-Modality Imaging in Coronary Anomalies: Focus on Anomalous Aortic Origin of Coronary Arteries

Abstract

Coronary artery anomalies represent a series of congenital heart diseases characterized by the abnormal circulation of coronary arteries. Of particular interest is the anomalous aortic origin of coronary arteries (AAOCA), an underdiagnosed but potentially fatal anomaly, specifically relevant in young adult athletes. During the last decades, knowledge of the pathophysiology of AAOCA-related ischemic mechanisms has increased, and major risk conditions have been discovered. Unfortunately, a universally shared approach is still lacking, and clinicians often follow the policy of their own centers. Most of the population with AAOCA is asymptomatic or complains of minor symptoms but is among them that there is much more uncertainty about the correct strategy to perform a risk stratification process, and consequently for providing indications on exercise restriction or surgery. This review focuses on the diagnostic approach to coronary anomalies and discusses the diagnostic workflow in these patients. Finally, we perform an in-depth analysis of novel angiographic invasive functional methods and how they contribute to AAOCA in selected cases.

Keywords: angiography; coronary arteries anomalies; flow‐chart; imaging; physical activity.

FIGURE 1

Anomalous origin of the left main coronary artery from the pulmonary artery. (A) Left ventricle was severely dilated. (B) Right coronary artery was dilated and well visible from a short‐axis view. (C) CT scan showed drainage of left coronary artery into pulmonary artery (inverted flow across the vessel). (D) 3D reconstruction of the heart. Both the coronaries were dilated. Several collateral vessels can be seen. LCA drainage in main pulmonary artery can be easily appreciated. Ao, aorta; LA, left atrium; LCA, left coronary artery; LV, left ventricle; PA, pulmonary artery; RCA, right coronary artery.

FIGURE 2

A 13‐years‐old male patient with cardiac arrest during training before a football match. Echo imagines (on the left) acquired when he was in Venoarterial—ExtraCorporeal Membrane Oxygenation seem to capture a normal origin of left main coronary artery in the left Valsalva sinus (yellow arrow). CT (on the right) diagnosed an anomalous origin of left coronary artery from opposite sinus (red arrow) with inter‐arterial course and acute take‐off. * Pneumopericardium (due to resuscitation maneuvers).

FIGURE 3

The same patient shown in Figure 2. (A) After a short, slit‐like and intramural course, left coronary artery showed a long interarterial course (arrow). (B) In that tract, the vessel was significantly eccentric (arrow). (C) 3D reconstruction of aorta and left coronary artery. The elliptic shape of left coronary artery along the right coronary sinus can be easily seen (interarterial course, arrow). * Pneumopericardium; Ao, aorta; PA, pulmonary artery.

FIGURE 4

Angiography confirmed the anomalous origin of left coronary artery. (A) Left anterior caudal view, (B) right anterior caudal view. The MLCA showed an intramural course (corresponding to a thinned calibre of the first part of the left coronary artery, yellow arrow). Normal arborization of the peripheral branches of the left coronary artery was also found. Cx, circumflex artery, LCA, left coronary artery; MLCA, main left coronary artery.

FIGURE 5

Anomalous origin of right coronary artery (RCA) from the left aortic sinus. (A) CT scan showed a long intramural course behind the right ventricular infundibulum. (B) Selective right coronary angiography showed an irregular shape of the proximal tract (red arrow). The tip of pressure guidewire (yellow arrows) was distal in the vessel. However, iFR plot (C) showed values within normal limits (> 0.93).

FIGURE 6

Diagnostic management in patients with AAOCA with symptoms.

FIGURE 7

Diagnostic management in patients with AAOCA without symptoms. SCA, sudden cardiac arrest.