Idiopathic left ventricular hypertrophy and sudden death
Research type
Research Study
Full title
Significance of idiopathic left ventricular hypertrophy and fibrosis in victims of sudden cardiac death. Familial hypertrophic cardiomyopathy or novel disease entity?
IRAS ID
170406
Contact name
Sanjay Sharma
Contact email
Sponsor organisation
St George's, University of London
Duration of Study in the UK
1 years, 6 months, 1 days
Research summary
Sudden cardiac death (SCD) can be the first manifestation of heart disease, often affecting apparently healthy individuals. The majority of SCDs are attributable to acquired coronary artery disease and manifest in the older population, whereas inherited heart muscle diseases and electrical faults of the heart predominate in the young (<35 years). The inherited nature of these conditions underscores the need for cardiac evaluation of first-degree relatives of the deceased, to detect other affected members and to prevent further tragedies. Postmortem examination is a critical first diagnostic step to guide clinical evaluation of surviving relatives. The interpretation of the results, however, is a complex task because there are uncertainties about the exact significance of certain pathological findings. Idiopathic left ventricular hypertrophy (ILVH) and idiopathic fibrosis (scarring of the heart muscle) are novel entities that are increasingly observed at post-mortem in cases of SCD. Their significance is unclear and the role of family screening in this specific setting has never been analyzed.
The aim of the proposed study is to establish the diagnostic yield of hypertrophic cardiomyopathy or other inherited cardiac conditions during comprehensive evaluation of family members victims of sudden death who demonstrated idiopathic left ventricular hypertrophy (LVH) and/or fibrosis at post-mortem. The study will be based at St George’s University of London and St George’s University Hospitals NHS Foundation Trust. The institutions host a unique service which includes a specialist cardiac pathology unit, inherited cardiac diseases clinics, genetics service and the only sports Cardiology unit in the UK.
REC name
London - Fulham Research Ethics Committee
REC reference
15/LO/2074
Date of REC Opinion
4 Mar 2016
REC opinion
Further Information Favourable Opinion