Guidelines for autopsy investigation of sudden cardiac death: 2017 update from the Association for European Cardiovascular Pathology

Abstract

Although sudden cardiac death (SCD) is one of the most important modes of death in Western countries, pathologists and public health physicians have not given this problem the attention it deserves. New methods of preventing potentially fatal arrhythmias have been developed and the accurate diagnosis of the causes of SCD is now of particular importance. Pathologists are responsible for determining the precise cause and mechanism of sudden death but there is still considerable variation in the way in which they approach this increasingly complex task. The Association for European Cardiovascular Pathology has developed these guidelines, which represent the minimum standard that is required in the routine autopsy practice for the adequate investigation of SCD. The present version is an update of our original article, published 10 years ago. This is necessary because of our increased understanding of the genetics of cardiovascular diseases, the availability of new diagnostic methods, and the experience we have gained from the routine use of the original guidelines. The updated guidelines include a detailed protocol for the examination of the heart and recommendations for the selection of histological blocks and appropriate material for toxicology, microbiology, biochemistry, and molecular investigation. Our recommendations apply to university medical centers, regionals hospitals, and all healthcare professionals practicing pathology and forensic medicine. We believe that their adoption throughout Europe will improve the standards of autopsy practice, allow meaningful comparisons between different communities and regions, and permit the identification of emerging patterns of diseases causing SCD. Finally, we recommend the development of regional multidisciplinary networks of cardiologists, geneticists, and pathologists. Their role will be to facilitate the identification of index cases with a genetic basis, to screen appropriate family members, and ensure that appropriate preventive strategies are implemented.

Sudden cardiac death and valvular pathology

“…With age, degenerative alterations may aggravate the valve deformation, causing stenosis and/or insufficiency. Both the valvulopathy and the aortopathy may lead to unexpected sudden death [1,[13][14][15][16][17].…”

“…Sudden cardiac death (SCD) is defined as "a natural, unexpected fatal event, with a cardiac underlying cause, occurring instantly or within 1 h from the onset of symptoms in an apparently healthy subject or whose disease was not so severe as to predict an abrupt outcome" [1]. When not witnessed, it is considered sudden if "the deceased was in good health 24 h before death" [1]. Statistics on SCD vary with the cohorts/populations studied [2], age, gender, lifestyle and sports activity [1,2].…”

“…When not witnessed, it is considered sudden if "the deceased was in good health 24 h before death" [1]. Statistics on SCD vary with the cohorts/populations studied [2], age, gender, lifestyle and sports activity [1,2]. Subjacent causes are multiple [3,4] and valvular heart disease is reported to range from 1% to 5% [3].…”

Inherited cardiomyopathies

“…All sudden deaths should be considered cardiac after exclusion of non-cardiac causes. Guidelines recommend a full autopsy, including histological examination 22. If the condition is identified at autopsy, notify immediate relatives and offer to refer them to a specialist clinic for evaluation23 and bereavement support.…”

Pacemaker failure as a cause of sudden death

“…I read with interest the Association for European Cardiovascular Pathology's Guidelines for Autopsy Investigation of Sudden Cardiac Death, recently authored by Basso et al [1]. While discussing the value of knowing the decedent's personal history and family history, the authors mention that the presence of cardiac implanted electronic devices (CIEDs; pacemakers and implantable cardioverterdefibrillators [ICDs]) can be especially valuable.…”