Childhood Hypertrophic Cardiomyopathy: A new risk stratification tool

Research summary

Childhood cardiomyopathies are associated with an increased risk of arrhythmias,heart failure and sudden death and it remains challenging to identify those at highest risk Principle research question: Can we identify patients with childhood onset dilated cardiomyopathy at risk of adverse events? Methodology: This is an international multicentre study. Collaborating centres will identify eligible patients and collect serial routine anonymised,retrospective clinical and imaging data as assessed at the time of first evaluation,follow up and last evaluation. Anonymised data will be entered into a secure internet-based REDcap database by each collaborating centre. Data will be analysed as part of the international collaboration by the Biostatistics group at University College London/Great Ormond Street Hospital. Standard validation measures will be examined. Patient evaluation: All patients should have: 1. Planned clinical reviews every 6-12 months or earlier if there was a change in symptoms. 2. Clinical assessment and physical examination 3. Pedigree analysis 4. Resting electrocardiography 5. Ambulatory electrocardiography (Holter monitor,minimum duration 24hrs) 6. Transthoracic echocardiography with left ventricular outflow tract gradients For patients with an Implantable Cardioverter Defibrillator (ICD),all patients should additionally have 1. ICD programming details recorded from time of implantation 2. Planned clinical reviews every 6-12 months or earlier if there was a change in symptoms with review of ICD programming details 3. Interrogation of ICD device following any therapy Outcomes: The primary outcomes will be heart failure events (eg cardiac transplantation and heart-failure related death) taken from the last clinic appointment. Outcomes should be assessed by experienced cardiologists using hospital and primary health care records,death certificates,post-mortem reports and interviews with witnesses (relatives and physicians). Secondary outcomes will include other SCD or an equivalent event (eg tachyarrhythmia triggered ICD shock therapy) and all-cause mortality.

Principal Investigator

Dr Satish Sadanand Adwani

Contact us

Email: childrensresearch@ouh.nhs.uk

IRAS number

182354