Italian Registry of Cardiovascular Magnetic Resonance on Congenital Anomalies of the Coronary Arteries (ITACA-Registry)

Congenital coronary anomalies (ACC) are relatively rare heart disease, in which a congenital defect of origin, course, and term, of one or more epicardial cardiac coronary arteries, is recognized. The global prevalence is estimated at around 0.6-0.7% in the general population, where the most frequent ACCs consist of the anomalous origin of the right coronary artery from the left breast (ARCA), with a prevalence of 0.23%; while the anomalous origin from the Sn coronary artery from the right breast (ALCA) would seem to have a prevalence of 0.03%.

ACCs have often been associated with an increased risk of Sudden Cardiac Death (SCD), especially in athletes. In fact, within this population, ACCs are counted as the second most frequent cause of SCD after hypertrophic cardiomyopathy.

In the general population, the risk of sudden death from ALCA is estimated at around 6.3% at 20 years, while for ARCA it is estimated at around 0.2% at 20 years. The estimated incidence in athletes instead recognizes a range that varies between 0.5 and 13 deaths per 100,000.

Although ACC are therefore recognized as a frequent cause of sudden cardiac death, a recent English study, conducted on a population of 11,168 young footballers, showed a significantly lower incidence than expected: only two coronary anomalies, an ALCA and an ARCA, respectively; the prevalence was therefore 0.002%, at least 10 times lower than expected.

The most common manifestations of ACC are chest pain, palpitations, dizziness and syncope, although more than 50% of patients with ACC are asymptomatic. In the past, these anomalies could only be described at autopsy, while thanks to the enormous developments that the different cardiac imaging modalities have undergone in recent years, ACCs can now also be detected non-invasively. The initial diagnosis is echocardiography, but a confirmation method is necessary (CT, MRI, or coronary angiography), moreover most of the time the finding can be completely incidental and found in the course of examinations conducted for another reason.