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Download Ontario introduces protocol for autopsies in unexplained sudden
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Transcript
Ontario introduces protocol for autopsies in unexplained sudden deaths TORONTO — A new protocol was introduced Tuesday for autopsies that investigate unexplained sudden cardiac death in young people aged two to 40 in Ontario, and it's expected to influence how autopsies are conducted in future across the country. The guidelines from the Office of the Chief Coroner of Ontario were initiated by the University of Ottawa Heart Institute and, in part, are aimed at checking the heart for a commonly overlooked disease that might also afflict family members. Dr. Michael Gollob, director of the Inherited Arrhythmia Clinic and Arrhythmia Research Laboratory at the Heart Institute, said many coroners and pathologists are not familiar with arrhythmogenic right ventricular cardiomyopathy, as the traditional autopsy of older victims of heart disease assesses the left ventricle. "These guidelines now mandate that the right ventricle be assessed in young victims of sudden death, specifically to look for the pathologic characteristics of this condition," he said from Ottawa. The human heart has four chambers. The two lower chambers, the left and right ventricles, are responsible for pumping the blood. ARVC causes electrical signals that drive the heart beat to become irregular (arrhythmia). Gollob said the second major component of the guidelines mandates the storing of tissue for future DNA analysis. There are at least four genetic diseases that affect only the electrical properties of the heart, and there are no structural defects that can be detected in an autopsy, he explained. "So when a young person dies from one of these four purely electrical diseases of the heart, the autopsy will show a perfectly normal appearing heart. And in those situations, it is valuable to put aside tissue that may be used at some point in the future for genetic analyses," he said. Dr. Andrew McCallum, chief coroner of Ontario, said the protocol adds an "ability to do what's called a molecular autopsy, to look at the level of the DNA to see if there's a genetic abnormality." "The goal of testing, obviously, is to help the living. So we're interested in providing the families of these persons with the information they need, and the care they need." The protocol was welcomed by Susan Csatari, a resident of Mississauga, Ont., whose son Stephen died at age 20 during a run when he was a third-year university student studying in the south of England. "It's a key and very welcome change for families like ours, especially those who have suffered the tragedy of not knowing what their child died from," she said in a telephone interview from New Jersey, where she was on vacation. Stephen collapsed and died at the side of the road in 2002, and the family subsequently found out he had ARVC. "We are one of the fortunate families who were given a diagnosis very early," Csatari said, noting that a pathologist insisted on sending the heart to an expert in London who identified ARVC. "We were told we all had to be tested upon our return to Canada and that resulted in finding out that my husband has the disease. Although he had never had any symptoms, he's followed very closely and is now on cardiac medication to protect him from this potentially fatal arrhythmia." In addition, Csatari said her daughter is monitored and takes medication to protect her, because the disease can develop over time. Csatari is involved with the Canadian Sudden Arrhythmia Death Syndromes Foundation, and said she has met several families who lost children or teenagers, but they were never given a diagnosis. It was only through their own efforts or other circumstances that they later found out that their other children were also affected with cardiac disease, she said. The new protocol was announced as the family of a 19-year-old hockey star who died unexpectedly in February issued a statement saying that an autopsy detected an underlying heart condition. Mickey Renaud, captain of the Windsor Spitfires of the Ontario Hockey League, died of hypertrophic cardiomyopathy, the family said. Gollob said that's a structural disease of the heart that would have been detected by the standard autopsy looking at the left ventricle. McCallum said sudden cardiac deaths among young people aren't common - there are three to five cases per million population in Ontario. But that amounts to about 60 to 70 cases per year and "so we certainly want to detect those cases," he said. Gollob said he predicts that every province in Canada will adopt guidelines similar to the Ontario protocol.