Sudden cardiac death and cardiovascular pathology: from anatomic theater to double helix

Abstract

The experience as a cardiovascular pathologist on sudden cardiac death (SCD) in the young and the impact that the findings had on in vivo diagnosis, treatment, and prevention are herein reported. The story dates back in the late 70s, when a series of juvenile sudden deaths occurred in the Veneto Region, North East of Italy. A successful application for a prospective study on young people dying suddenly (<35 years old, sudden infant death syndrome excluded) was submitted to the regional health authorities, thus implementing a network of collaboration with anatomic and forensic pathologists, to collect all such events and to gather epidemiological data. The project is still in progress, and since then we studied >650 hundreds consecutive juvenile SCD cases, allowing to identify the culprit diseases with abnormalities in the various cardiac structures (aorta, coronary arteries, myocardium, valves, and conduction system). The long standing Veneto Region experience clearly shows that autopsy still plays a pivotal role in the study and prevention of SCD and should be carried out regularly in the young. With time, the investigation of SCD necessarily moved from the classic postmortem study to molecular autopsy. In conclusion, SCD prevention in the young has to be faced by an interdisciplinary team, including pathologists, cardiologists, sport physicians, and geneticists, with a translational approach; the clinicopathologic correlation method still being the polar-star. In other words, the game in the fight against SCD is still played in the anatomical theater, the place where "death enjoys to save lives."