Coronary Anomalies

  • Define the most common and clinically significant coronary anomalies.

  • Define common types of coronary fistulae.

  • Define which coronary anomalies are low versus high risk.

  • Outline the advantages of cardiovascular magnetic resonance (CMR) imaging over conventional X-ray angiography.

  • Understand treatment options for common coronary anomalies.


Coronary anomalies are a rare group of congenital disorders with an incidence of approximately 0.6 percent of births. These can broadly be defined as anomalies of origin and course, termination, or intrinsic anatomy ( Table 24-1 ). Patients may be asymptomatic or present with angina, palpitations, or exertional syncope. Although most have a benign course, these conditions can cause sudden cardiac death and myocardial infarction—especially in young adults and athletes. Diagnosis is highly dependent on cardiac imaging. Traditionally, X-ray coronary angiography was used to make the diagnosis. Currently CMR has emerged as a noninvasive method for defining the origin of coronary arteries and their three-dimensional proximal course, without radiation exposure or contrast administration. This makes CMR an ideal choice for young adults with suspected coronary anomaly. Treatment highly depends on the type of anomaly. For those associated with an interarterial course and/or myocardial ischemia, surgical correction is warranted.

TABLE 24-1

Types of Coronary Anomalies

  • Anomalies of origin and course: high risk

    • Anomalous location of coronary ostium outside the aortic root (e.g., pulmonary artery, aortic arch)

    • Anomalous origin of the coronary ostium from the contralateral coronary sinus

      • Interarterial (Preaortic)

        • LCA arising from right sinus

        • RCA arising from left sinus

  • Anomalies of origin and course: low risk

    • Separate origin of the LAD and LCx

    • Anomalous location of the coronary ostium from the proper aortic sinus of Valsalva (e.g., high, low, commissural)

    • Anomalous origin of the coronary ostium from the contralateral coronary sinus

      • Retro-aortic

        • LCA arising from right sinus

        • LCA arising from noncoronary sinus

        • LCx arising from right sinus

      • Prepulmonary

        • LCA arising from right sinus

  • Anomalies of termination

    • Coronary fistulae from coronary arteries to:

      • Pulmonary Artery or Vein

      • Ventricles or Atria

      • SVC or Coronary Sinus

  • Anomalies of intrinsic anatomy

      • Coronary ectasia or hypoplasia

      • Coronary aneurysms

      • Subendocardial course (myocardial bridge)

Adapted from Angelini P: Coronary artery anomalies: An entity in search of an identity. Circulation 2007;115:1296-1305.


Case 1

A 13-year-old boy presented with exertional syncope. CMR was ordered to assess for coronary anomaly. Based on the high-risk anatomy, the patient underwent successful surgery with reimplantation of the LM to the correct sinus ( Figure 24-1 ).

Figure 24-1

A, Three-dimensional (3D) coronary magnetic resonance angiography (MRA) showing the left main (LM) arising from the right coronary sinus and taking an interarterial (preaortic) course between the aorta (Ao) and the pulmonary artery (PA). A multiplanar reformatting (MPR) method is used to display the 3D MRA image in a single plane. B, Schematic diagram of the coronary anatomy in panel A, showing adjacent origins of the LM and the RCA from the right coronary sinus and the interarterial LM course. C, 3D coronary MRA post reimplantation surgery. The LM now arises from the left coronary sinus and follows a normal course. Note the curvilinear structure along the right side of the aorta ( arrowhead ) is postop pericardial fluid. D, Schematic diagram of normal coronary anatomy, consistent with the patient’s postoperative state.

A (Courtesy of G. Greil and R. Botnar.) ; B (Courtesy of G. Greil and R. Botnar.)

Left Coronary Artery from the Right Coronary Sinus with an Interarterial Course

Case 2

An 18-year-old man presented initially with exertional chest tightness and fatigue while playing basketball. A chest X-ray showed cardiomegaly, and an ECG showed nonspecific ST-T wave abnormalities. A transthoracic echocardiogram revealed moderate LV enlargement, a left ventricle ejection fraction (LVEF) of 35% with global hypokinesis, moderate to severe MR, and severe pulmonary HTN estimated at 100 mm Hg. An exercise myocardial perfusion stress test showed an infarct with severe peri-infarct ischemia in the distal anterior and inferolateral walls at 15 METs of exercise. X-ray coronary angiography revealed an anomalous left coronary artery (LCA) originating from the pulmonary artery (PA) and a large right coronary artery (RCA) providing collaterals to the LCA. He was referred for CMR to better delineate the coronary anomaly and to assess myocardial scar ( Figure 24-2 ). Based on the findings on CMR, he was referred for cardiac surgery where a LIMA graft to the LAD was placed as well as an annuloplasty ring to the mitral valve. His symptoms improved after surgery.

Feb 1, 2019 | Posted by in MAGNETIC RESONANCE IMAGING | Comments Off on Coronary Anomalies
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