Aim To raise awareness of congenital coronary artery anomalies (CCAAs) as an important cause of sudden cardiac death (SCD) in athletes, we describe a cohort of the malignant subset. Defining the key anatomical features for the cardiologist and pathologist to be aware of and detailing a systematic approach to examining the coronary arteries at autopsy.

Methods Retrospective non-case-controlled analysis of 2304 cases of SCD referred by pathologists between 1994 and January 2012.

Results 31 (1.3%) of the 2304 cases of SCD had CCAAs; 24 men (77%) and 7 women (23%), mean age 28 years (range 16 months–63 years). In 15 cases (48%), SCD occurred during or immediately after physical exertion. Cardiac symptoms were documented to have occurred in only seven patients (23%) prior to SCD. The anomaly had been identified by the referring pathologist in only 11 of the 31 cases (35%).

Conclusions CCAAs are a rare and mostly benign entity, but a subset has the potential to be fatal without any forewarning. In a significant proportion of cases identified in this large cohort, the victim was under exertion at the time of death, highlighting the relevance of this anomaly to the sports and exercise medicine community.