A silent cause of sudden cardiac death especially in sport: congenital coronary artery anomalies.

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.

Non-atherosclerotic coronary artery disease associated with sudden cardiac death.

BACKGROUND: The concept of non-atherosclerotic coronary artery pathology in sudden cardiac death (SCD) has not been given the attention it deserves. OBJECTIVE: To determine the incidence of non-atherosclerotic coronary artery pathology in SCD and raise awareness among cardiologists and pathologists alike. DESIGN: Retrospective non-case-controlled analysis. Setting Cardiac pathology centre at the National Heart and Lung Institute and Royal Brompton Hospital. SUBJECTS: Between 1994 and 2008, the hearts of 1647 people undergoing SCD were referred for pathological assessment to ascertain the precise aetiology of SCD. RESULTS: Fifty (3.0%) of the 1647 cases of SCD were associated with non-atherosclerotic coronary pathology (31 male subjects (62%) and 19 female subjects (38%, age range (8 weeks-71 years)). Twenty four of the 50 cases had anomalous coronary arteries (48%); eight cases had coronary artery dissection (16%); six cases had coronary artery vasculitis (12%); six cases had coronary artery spasm (12%); three cases had idiopathic arterial calcification of infancy (6%); two cases had fibromuscular dysplasia (4%) and one case had a benign tumour occluding the left coronary ostium (2%). Only 20 of the 50 patients (40%) were documented to have experienced cardiac symptoms such as syncope, chest pain on exertion or breathlessness before their SCD. Twelve of the patients (24%) died during or immediately after physical exertion. CONCLUSION: Non-atherosclerotic coronary disease is associated with sudden death in all age groups, particularly younger, male patients. Cardiologists need to be aware of these entities and investigate any patient who has cardiac symptoms especially with exertion.