Massive pericardial effusion with left-to-right intracardiac shunt

We describe a 31-year-old man with Down's syndrome who presented with sever e chronic hypothyroidism and a massive pericardial effusion. Following part ial aspiration of this effusion, he rapidly deteriorated and died. Findings at autopsy revealed him to have an atrioventricular septal defect with shu nting at the atrial level. We postulate that, by releasing extrinsic pressu re on his right heart by the aspiration, there was sudden shunting of blood from left-to-right, resulting in hypotension, shock, and subsequent death. We highlight the difficulties in management: of such a case, and suggest a lternative strategies.