Results from a Specialist Cardiac Pathology Service for Sudden Cardiac Death in the United Kingdom (UK).
Sofia V de Noronha, Keiko Ogo, Jemma Wells, Mary N Sheppard. Imperial College, London, United Kingdom; Royal Brompton and Harefield NHS Foundation Trust, London, United Kingdom
Background: Sudden, unexplained death is usually due to cardiac disease and its incidence is unknown in the UK. A unique fast track cardiac pathology service, the CRY Centre for Cardiac Pathology, for sudden cardiac death (SCD) in the UK was launched in March 2007 with the generous support of a family who experienced sudden death in two generations. This service was established with the aim of offering a free specialist cardiac pathology diagnosis quickly (two week turn around) to pathologists nationwide.
Design: A prospective non-case control observational study.
Results: A total of 726 cases of SCD were referred from March 2007 to December 2009 consisting predominately of males (60%), median age 31 years. Most deaths took place at home, at rest (56%) but 16% occurred during exertion. Figure 1 shows the main cardiac diagnoses including normal hearts (n=337) (suggesting channelopathy), cardiomyopathy (n=212) and coronary artery pathology (n=71). In the cardiomyopathy group idiopathic left ventricular hypertrophy was most common (n=98), followed by hypertrophic cardiomyopathy (n=41) and arrhythmogenic ventricular cardiomyopathy (ARVC) (n=30). When comparing the referring pathologist's opinion with ours, in a sample of 200 cases, we found that 27% of the cardiac causes were mismatched and the major discrepancy was an overdiagnosis in ARVC.
Abbreviations: AD: aortic disease, CAP: coronary artery pathology, CHD: congenital heart disease, CM: cardiomyopathy, VD: valve disease.
Conclusions: This large prospective study highlights the importance of inherited cardiac disease as a cause of SCD as at least 75% of our referrals are of potentially genetic origin. Our service is important as families are becoming increasingly aware of SCD and wish to establish a specific cardiac cause of death quickly to ensure an early cardiological screening of the remaining family members.
Monday, February 28, 2011 1:15 PM
Platform Session: Section G, Monday Afternoon