Anomalous Coronary Artery Origin and Sudden Cardiac Death

Study Questions:

What are the clinical and pathological features of anomalous origin of a coronary artery (AOCA) in sudden cardiac death (SCD) victims?


The investigators reviewed a database of 5,100 consecutive cases of SCD referred to a specialist cardiac pathology center between January 1994 and March 2017, and identified a subgroup of 30 cases (0.6%) with AOCA. All cases underwent detailed post-mortem evaluation including histological analysis by an expert cardiac pathologist. Clinical information was obtained from referring coroners. Comparison of groups was performed using Student’s t-test for continuous variables with correction for unequal variance when necessary and chi-square test or Fisher exact test, as appropriate for categorical variables.


The mean age was 28 ± 16 years and 23 individuals were male (77%). In eight cases (27%), SCD occurred before 18 years of age. Cardiac symptoms were present in 11 individuals (37%), and syncope was the most common (n = 6, 20%). Anomalous left coronary artery arising from the right sinus of Valsalva (ALCA) with interarterial course (n = 11) and anomalous right coronary artery arising from the left sinus of Valsalva (ARCA) with interarterial course (n = 11) were the most common found. ALCA arising from the pulmonary artery was present in seven cases, whereas in one case, the left coronary artery arose from the noncoronary cusp. Left ventricular fibrosis was reported in 11 cases (37%) and was mainly subendocardial. There was evidence of acute infarction in two cases. Death occurred during exercise or emotional stress in 15 (50%) cases. The AOCA variant where death occurred more frequently during physical activity was ALCA (8 of 11, 73%), followed by ALCA arising from the pulmonary artery (4 of 7, 57%) and ARCA (2 of 11, 18%).


The authors concluded that ALCA and ARCA with interarterial course are the most common anatomical variants recognized at the post-mortem of SCD victims.


This study reports that anomalous left or right coronary artery arising from the wrong sinus of Valsalva with interarterial course was the most common anatomical variant followed by anomalous coronary artery arising from the pulmonary artery. Of note, in ALCA, death occurred commonly during exercise, whereas in ARCA, death occurred usually during rest or sleep. Since a myocardial substrate for arrhythmias is identified in only a minority of individuals with sudden death diagnosed with a coronary anomaly at post-mortem, a single episode of acute ischemia may lead to sudden death in most of the patients.

Clinical Topics: Acute Coronary Syndromes, Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Prevention, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Aortic Surgery, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Prevention, Exercise, Stress

Keywords: Acute Coronary Syndrome, Arrhythmias, Cardiac, Coronary Vessel Anomalies, Death, Sudden, Cardiac, Exercise, Fibrosis, Myocardial Infarction, Myocardial Ischemia, Pulmonary Artery, Sinus of Valsalva, Stress, Psychological, Syncope

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