A New Standard for Determining the Cause of Unexplained Sudden Death: Interview with Dr. Michael H. Gollob

There is a new protocol of conducting autopsies for cases of unexplained cardiac death; the new standard was developed by the Office of the Chief Coroner for Ontario and the University of Ottawa Heart Institute. Dr. Michael Gollob is the director of the Inherited Arrhythmia Clinic and Arrhythmia Research Laboratory, located at The University of Ottawa Heart Institute in Canada.

What brought about this new standard of conducting autopsies in cases of unexplained cardiac death? At the Inherited Arrhythmia Clinic at The University of Ottawa Heart Institute, we routinely evaluate surviving family members who have lost a loved one prematurely to sudden cardiac death (SCD). We observed many autopsy reports that did not comment on the evaluation of the right ventricle, leaving open the possibility of arrhythmogenic right ventricular cardiomyopathy (ARVC). A thorough and comprehensive autopsy is essential to facilitate clinical investigations in surviving family members, and we recognized the need for a standardized approach in Ontario. How do these types of autopsies differ from “standard” autopsies? How is the heart examined during this type of autopsy? How long does the whole procedure take? The standard autopsy of the heart focuses on the left ventricle, as the most common cause of SCD in individuals over age 40 involve coronary disease and infarcts within the left ventricle that may create arrhythmias. However, in young individuals (age 40), and therefore, special considerations for the autopsy procedure in the young is necessary. How many of these autopsies have been completed so far? The issue was the inconsistency of the autopsy, depending on the pathologist. Some pathologists have been aware that RV assessment is important, while others have not been. The guidelines ensure that all pathologists/coroners will perform a comprehensive autopsy and store tissue for the consideration of DNA testing. The clinical utility of DNA testing on stored tissue is demonstrated in a recent publication in the Canadian Journal of Cardiology (Rutberg J et al), where clinical evaluation of a young lady whose sister experienced SCD produced equivocal and non-diagnostic results. We performed DNA testing on stored tissue of the deceased SCD victim, identifying a genetic defect responsible for LQTS as the cause of death. The genetic information was used to rule out the disease in the surviving sister who suffered from significant anxiety. Is this being done anywhere else? When do you think other institutions will begin to participate in similar programs? Ontario is the first province in Canada to have implemented guidelines on a standardized autopsy in the young. I predict this will be adopted nationwide. I am unaware if any states have a standardized autopsy approach. Why was it important to offer this type of specialized autopsy? Since these are genetic conditions, multiple family members may be at risk of a similar tragedy. These guidelines, by assisting us in reaching a diagnosis for the cause of SCD, will greatly facilitate our clinical evaluation and management strategies in surviving family members. Often, family members may appear clinically normal, but still harbor the gene defect responsible for the disease and therefore pass it on to the offspring who will be at risk. Does the University of Ottawa Heart Institute also offer separate family screening for genetic heart rhythm disorders? In addition, if the autopsy shows that there is a genetic link to a certain heart rhythm disorder, are the relatives referred to or treated at the University of Ottawa Heart Institute? Yes, the Inherited Arrhythmia Clinic at the Ottawa Heart Institute is staffed to offer social support, genetic counseling, and expert clinical evaluation of individuals who may be at risk of harboring a genetic heart rhythm disorder. We routinely evaluate and offer second opinions on patients from across North America. Tell us about the Inherited Arrhythmia Clinic and Arrhythmia Research Laboratory. How long have you worked there? What other types of procedures or research initiatives are done there? I am currently the Director of the Inherited Arrhythmia Clinic and Research Laboratory, which was established in 2004. In addition to our clinical care of patients, we are focused on many research initiatives to further our knowledge in the field of cardiac arrhythmias. We are currently focusing on a genetic basis for atrial fibrillation. We have collected numerous families where this arrhythmia appears to be prevalent. Our laboratory is also focused on understanding the electrical and biochemical abnormalities that are created by genetic defects that predispose to arrhythmias. The Inherited Arrhythmia Clinic may be contacted by fax (613-761-5060) or telephone (613-761-5016).