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Sudden Cardiac Death The AECVP Protocol Patrick J Gallagher Southampton University Hospitals The Investigation of Sudden Death • Practice in the EU is highly variable, even within the same country • There are no international guidelines on which cases should have autopsies • The proportion of sudden deaths that have autopsies is not known • The care with which these autopsies are performed is variable Why Perform an Autopsy? • To determine whether death was of cardiac or non cardiac origin • What is the exact nature of the cardiac disease? • Is the cardiac disease inherited? Do relatives require screening or treatment? • Did drugs of any sort play a role in death? Southampton University Hospitals 900 adult autopsies 600 sudden deaths 350 sudden cardiac deaths 60 50 40 Male Female 30 20 10 0 40s 50s 60s 70s 80s 90s Age of sudden cardiac death patients in Southampton Why another Protocol? • After all there are excellent chapters in forensic and autopsy textbooks • Cardiologists are unhappy about the standard of post mortem reports • This is especially important in young sudden deaths • In England an independent audit has shown that 25% of reports are poor Virchows Archiv 2008;452:11-18 Four Essential Steps Four Aspects of the Protocol • Clinical information is valuable and desirable • All post mortems should be full sequential structured examinations. The exact nature of the underlying cardiac disease should be determined • Histology and other laboratory investigations are essential • Pathologists must accept that there are grades of diagnostic certainty and gray areas. They must take great care in formulating a diagnosis Dissection of the Heart • We have written a detailed method • But attention to detail is more important than precise methodology! • In everyday practice there are technical problems such as calcification of the coronary arteries and decomposition • Most patients die of ischaemia or heart failure BUT always think of the certainty scale 40 35 30 25 Male Female 20 15 10 5 0 Grade 1 Grade 2 Grade 3 HF Causes of sudden cardiac death ? First degree of certainty of IHD Second and third degrees of certainty Heart Failure First Level of Certainty • With very detailed histology acute coronary thrombosis was identified in 47% of 202 out of hospital sudden cardiac deaths in Nottingham UK. • Last year we identified 70 patients who died at home with coronary thrombosis or acute infarction. What prevents these patients getting to hospital? Leach et al Eur Heart J 1995;16:1181-5 Second degree of certainty Third degree of certainty • Coronary artery narrowing • No acute or healed infarct • Did the narrowing cause death? Diagnosis of Heart Failure • The jugular venous pulse and an echo are useful for diagnosis in life • Symmetrical pleural effusions and hepatic congestion are the best autopsy signs • Good treatment prevents decompensation and the associated pathological signs • Sudden death can occur in the early stages of heart failure even when symptoms are well controlled Cardiomyopathies • Dilated cardiomyopathy is the commonest type in autopsy practice • HOCM affects patients of all ages • Unexplained left ventricular hypertrophy is a real entity • Arrhythmogenic right ventricular cardiomyopathy (index cardiomyopathy) Unexplained Cardiac Deaths • Sudden arrhythmic death syndrome • Not only a disease of the young: 25F,26M,37F,38F,40M,41F,45M,46M, 46F,64M • Pathologists must consider and exclude other important cardiac disorders • Unexplained deaths also occur in anorexia, obesity, alcohol abuse and diabetes (dead in bed in diabetes) The Gray Zones • Coronary artery narrowing without thrombosis, old or recent infarction • Hypertensive heart disease, unexplained left ventricular hypertrophy and HOCM • Focal interstitial fibrosis and lymphocytic inflammation • Degenerative disease of the mitral valve • Adipose tissue infiltration of the right ventricle The Final Diagnosis • State clearly the most likely cause of death • List the contributory causes • Write a clinical comment indicating the degree of diagnostic certainty • Provide a recommendation for further study of the patients records and for screening of relatives • Make a data base entry We hope … • That you agree that a full protocol driven autopsy is essential in all sudden deaths in patients less than 50 years • That histology, biochemistry and toxicology are essential and valuable • That you will read our paper and provide us with your comments • That you will join us for our next meeting in Lisbon Fourth Biennial Meeting Lisbon 21-23 October 2010 Local Organiser Rosa Henriques de Gouveia MD PhD