Abstract
Congenital heart defects (CHDs) have undergone a large change in epidemiology due to prenatal screening and improved outcomes with surgery and percutaneous procedures. In patients with complex CHD there is an increased risk of sudden cardiac death (SCD) and up to 11% of all SCDs in the young occur in people with CHD. It is essential for clinicians to be aware of the risk factors, and for all patients to be followed up in specialised centres. When an SCD occurs, it is important that an autopsy is done and for the pathologist to have an in-depth knowledge of the particular defect and the corrective surgical techniques employed, as well as any complications due to these procedures. Both pathologist and cardiologist should work closely together to explain the cause of death to the family. A terminal cardiac arrhythmia explains many of the SCD cases, often with underlying cardiac fibrosis due to previous procedures. SCD may also be the first presentation of CHD, so great care is required when examining such cases and referral for a detailed expert opinion is recommended in all CHD-SCD cases.