Abstract

<h3>Key points</h3> 1. Most severe congenital cardiac disease is diagnosed prenatally. 2. Most autopsies in CHD are on patients who have had palliative procedures often in stages with evolution of procedures over time. 3. Check whole body for dysmorphic features linked to increased risk of CHD. 4. Thorough autopsy with detailed sequential segmental analysis of the heart and vessels. 5. Keep heart and lungs intact in order to see the relationship between the hearts and pulmonary circulation. 6. Detailed operative notes are essential for post-operative deaths. Autopsy in the context of CHD usually is due to severe conditions leading to death <i>in utero</i> or shortly after delivery and is in the remit of the paediatric pathologist. Most who survive will have complex operative procedures with which the pathologist should become familiar. In later life death usually follows surgical interventions but can also be sudden in the context of stable disease. Detailed history with previous procedures and detailed surgical notes are essential. Detailed case history, meticulous post-mortem examination and painstaking cardiac dissection are essential. Examination of the fixed heart is best and detailed dissection after 24 hours fixation is much easier than on the fresh heart.

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