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Title: Sudden arrhythmic death syndrome in the UK : the experience of a tertiary cardiogenetics centre in South London
Author: Papadakis, Michael
ISNI:       0000 0004 5368 1954
Awarding Body: King's College London
Current Institution: King's College London (University of London)
Date of Award: 2014
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Background: In a proportion of sudden cardiac deaths (SCD), no structural pathology can be identified and a diagnosis of sudden arrhythmic death syndrome (SADS) is advocated. Recognition of SADS is important since a significant proportion is attributed to inherited cardiac conditions, and evaluation of relatives may identify individuals at risk. Aims: The aims of the thesis are: To investigate the magnitude of SADS in young (≤35 years) and athletic individuals; To investigate the impact of expert cardiac pathology; To evaluate a large cohort of SADS families to ascertain: 1.The diagnostic yield of familial evaluation, 2.Short-term outcomes, 3.The implications of autopsy findings of uncertain significance, 4.The impact of higher intercostal leads, 5.The limitations of current risk stratification protocols in Brugada syndrome (BrS). Methods and Results: Appraisal of the Office of National Statistics data revealed an incidence of young SADS of 0.24/100,000/year. Histopathological evaluation of hearts of athletic individuals who experienced SCD (n=118), identified a morphologically normal heart in 23% of cases. A study of 158 SCD cases where a pathological evaluation was performed by both the referring and an expert cardiac pathologist identified a disparity in the diagnosis in 41% of cases. Evaluation of 83 families of victims of SADS demonstrated a diagnostic yield of 48%, with BrS being the predominant familial diagnosis. A quarter of the relativesevaluated) were diagnosed with an inherited condition. During 25.5±16.8 months of followup,1 patient died, despite being cleared after comprehensive clinical evaluation. A review of 50 SADS victims with a familial diagnosis of BrS identified high-risk features in only 20%, highlighting the limitations of current risk stratification protocols. Utilising higher intercostal V1 and V2 leads during Ajmaline testing doubled the diagnostic yield of BrS. Finally, familial evaluation following SCD with autopsy findings of uncertain significance identified a similar proportion of primary arrhythmogenic syndromes to “true” SADS. Conclusions: After a suspected SADS death expert cardiac pathology evaluation is necessary to ensure accurate diagnosis. First-degree relatives should be referred for comprehensive cardiac screening in an expert setting. Higher intercostal leads should be used when BrS is suspected and there is a need for improved risk stratification protocols in BrS.
Supervisor: Shah, Ajay Manmohan Sponsor: Not available
Qualification Name: Thesis (Ph.D.) Qualification Level: Doctoral
EThOS ID:  DOI: Not available