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Coronary artery anomalies occur in 0.3-0.8% of the population and infer a high risk for sudden cardiac death in young adults. Diagnosis is usually established during coronary angiography, which is hampered by poor spatial visualization. Magnetic resonance imaging is an alternative, but it is not feasible in the presence of metal objects or claustrophobia. In this report, a 15-year-old boy experienced ventricular fibrillation and was successfully resuscitated. Cardiac catheterization was inconclusive, and pacemaker implantation prohibited the use of MR imaging. Multi-slice CT coronary angiography revealed a malignant anomalous right coronary artery.

The anomalous origin of the right coronary artery (RCA) from the left sinus of Valsalva coursing between the aorta and the pulmonary artery or right ventricular outflow tract, is considered a potentially fatal abnormality which may require surgery. However, diagnosing the correct course with coronary arteriography may be difficult. Fast gradient echo magnetic resonance (MR) imaging can be helpful to identify and confirm the course of aberrant coronary arteries and their relationship to the surrounding tissue. In this study, diagnostic procedures and management are described of four patients in whom the RCA originated from the left sinus of Valsalva. Although reported as investigational by the Task Force document on MR imaging by the European Society of Cardiology we are of the opinion that MR coronary angiography may have an important future role in the assessment of anomalous coronary arteries.

We investigated the additional value of magnetic resonance (MR) angiography in 12 patients with clinically significant coronary anomalies. In 5 patients, the referring cardiologist requested additional evaluation because coronary arteriography was inconclusive about the course of the anomaly. For comparison, 7 patients with known anomalous coronary arteries were collected from our database. In these patients, there had been no doubt about the course of the anomalous coronary arteries. MR angiography provided the diagnosis in all 5 patients in whom the diagnosis was inconclusive beforehand. From the 7 patients in whom diagnosis had been established previously by contrast arteriography, the anomaly was confirmed in 5 by MR angiography. In 1 patient, the initial diagnosis at contrast arteriography had to be changed as a result of MR angiography. In 1 patient, the MR image was of insufficient quality to be conclusive. In conclusion, in patients with angiographically suspected coronary anomalies, fast gradient echo MR angiography is a helpful adjunct to coronary arteriography in identifying and confirming the origin and course of clinically significant coronary anomalies. The additional value of fast gradient echo MR coronary angiography is the visualization of both the artery and its surrounding structures.

Background: Current 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. Aim: To provide evidence-substantiated recommendations for diagnostic work-up, treatment and follow-up of patients with anomalous coronary arteries. Methods: A 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. Results: Primary 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. 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. [ABSTRACT FROM AUTHOR]