National Heart & Lung Institute (NHLI)

Sudden death overall analysis

CRY unit sudden cardiac results (SCD)  2007- 2009
Setting up a pathology laboratory to investigate sudden cardiac death. Results from the first 2 years of CRY centre for cardiac pathology
Sofia V de Noronha, Keiko Ogo, Jemma Wells and Mary N Sheppard
Cardiac Risk in the Young (CRY) Centre for Cardiac Pathology (CCP) was launched in March 2007 as a fast track pathology service of young sudden cardiac death in the United Kingdom with funding from the charity CRY. We report retrospectively the results of 600 sudden cardiac deaths over a 24 month period since the establishment of the Unit.

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Whole SCD database results 1994-2009
Sudden cardiac from predominately non-atherosclerotic causes: a pathological study in the UK, Sofia V de Noronha, Keiko Ogo, Jemma Wells and Mary N Sheppard
We have assembled a large database of 1899 cases of sudden cardiac death throughout the United Kingdom from 1994 to 2009. We aim to determine the demographics and aetiology of SCD within the context of a predominately non-atherosclerotic cohort.

Arrhythmogenic right ventricular cardiomyopathy (ARVC)

Variation in Both Macroscopic and Microscopic Features of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)
K Patel, SV Noronha, MN Sheppard
Seventy-three cases of ARVC were retrieved from our large SCD database of 1645 cases (between 1994 and early 2009) an incidence of 4.4%. Our series shows that SCD with ARVC is common as a first presentation and prevalent in young male athletes typically under exertion (n=27) but also at rest (n=22). Nine cases had a family history of cardiac disease and only 4 had received a clinical diagnosis of ARVC during life. Therefore it is undiagnosed in life. Our study showed that even with a specialist examination the spectrum of macroscopic findings in ARVC included, classic features of ARVC, cardiac hypertrophy, scarring resembling DCM and normal features. Therefore, a detailed histological analysis is essential to confirm the diagnosis. Our histological analysis detected a variable ventricular involvement that was predominately biventricular followed by right and left dominance, there was also an association with inflammation. These findings raise questions regarding the pathogenesis of ARVC. A correct diagnosis is important for screening family members as this is a genetically inherited disease.

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Genetics of ARVC
A collaborative project with Dr Dominic Abrams (Consultant electrophysiologist at St Barts Hospital London) and the department of dermatology with Professor John Kessel to investigate the genetics of four cases with ARVC in formalin fixed heart tissue. Four paraffin blocks have been sent for genetic analysis.

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Idiopathic Myocardial Fibrosis in Sudden Cardiac Death Patients with and without left ventricular hypertrophy
Sofia V de Noronha, Declan McGuone and Mary N Sheppard
Seventy-seven cases of idiopathic myocardial fibrosis were reported over a 13 year period (1995 to June 2008). The aim of this study was to determine the demographics including any associations to past medical history as well as to characterize/quantify the histological pattern and distribution of myocardial fibrosis in the left ventricle.

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Obesity and sudden cardiac death. Does a specific obesity cardiomyopathy exist? A study of predominantly non- ischaemic causes of sudden death.
Agata M Plonczak, Sofia V de Noronha, H Purcell and Mary N Sheppard
Obesity is a major health concern in the modern world and it is associated with an increased risk of sudden cardiac death. We investigated the cardiac pathology in 57 cases with history of sudden death and with BMI above 30.

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Non-Atherosclerotic coronary artery pathology responsible for sudden cardiac death.
Mary N Sheppard and Sharleen F Hill
We sought to determine the incidence of non-ischaemic coronary artery pathology in a retrospective study. Fifty cases were identified from our database.

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Sudden Cardiac Death in Young Adults: Look more closely at the Coronaries. Spontaneous Coronary Dissection can be missed: A study of 7 cases.
Saral Desai and Mary N Sheppard
Spontaneous coronary artery dissection is a rare cause of death seen largely in young women, with many cases occurring in the early post-partum period. A retrospective study showed a  total of 7 cases with this condition  from our histopathology records.

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Cardiopulmonary Causes of Maternal Deaths from 1994 to 2009 in the United Kingdom
K Ogo, SV Noronha, E Shamil, S Lucas, MN Sheppard
Cardiopulmonary disease has become increasingly important in maternal mortality in the United Kingdom (UK). The aim of our study was to investigate the cardiopulmonary causes of 82 maternal deaths referred to our specialist tertiary centres, Royal Brompton Hospital/CRY centre for cardiac pathology (CCP) and St Thomas’s hospital, over a 16 year period.

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Sudden Cardiac Death After Emotional Stress Associated with Physical Restraint, Assault and/or Altercation.
R Georgiou, SV Noronha, MN Sheppard
We retrospectively investigated 33 cases of sudden cardiac death (SCD) in relation to an emotionally stressful event such as physical restraint (n=11; 3 in police custody, 4 in mental health institution, 4 other), assault (n=9), altercation (n=4), fight (n=3) and chase with restraint (n=1). The aim of this study was to highlight deaths during emotional stress and the fact that the diagnoses were mainly of non-atherosclerotic origin (n=23, 82%). A thorough autopsy is essential in such cases where medicolegal issues are important.

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Sudden death following an acute or chronic episode/s of alcohol intake
Junaid P, S Noronha, MN Sheppard
Sudden death with alcohol is often linked with liver disease, we aim to demonstrate the importance of this substrate in cardiac death particularly in the young. Alcohol may trigger a fatal arrhythmia in vulnerable individuals with underlying inherited cardiac disease. We aim to investigate a retrospective group of 160 cases.

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Sarcoid and sudden cardiac death
I Bagwan, Lucy V B Hooper and MN Sheppard
Sarcoidosis is a non-necrotizing granulomatous inflammation wherein cardiac involvement, isolated or as a part of multi-systemic involvement has been described. Sixteen cases of cardiac sarcoidosis were identified over a period of 15 years in our database (1995-2008).

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Mitral valve disease and sudden cardiac death
D. Baskaran, A Plonczak, SV Noronha, MN Sheppard
The risk of sudden death in mitral valve prolapse (MVP) is very rare, however, it is still twice as in the general population which is a source of concern because of the very high prevalence of this valvular abnormality in the industrialised world. We aim to investigate the frequency and aetiology of MVP in our database.

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