A5063976169- Cardiac Arrhythmias and Treatments
- Cardiac pacing and defibrillation studies
- Cardiac electrophysiology and arrhythmias
- Cardiovascular Syncope and Autonomic Disorders
- Congenital Heart Disease Studies
- Atrial Fibrillation Management and Outcomes
- Ion channel regulation and function
- Cardiovascular Effects of Exercise
- Mitochondrial Function and Pathology
- Metabolism and Genetic Disorders
- Cardiomyopathy and Myosin Studies
- Cardiac Structural Anomalies and Repair
- Congenital heart defects research
- Cardiovascular Function and Risk Factors
- Neonatal Health and Biochemistry
- RNA regulation and disease
- Cardiac Valve Diseases and Treatments
- Hemoglobinopathies and Related Disorders
- Folate and B Vitamins Research
- Cardiac Fibrosis and Remodeling
- ATP Synthase and ATPases Research
- Pharmaceutical studies and practices
- Heart rate and cardiovascular health
- Erythropoietin and Anemia Treatment
- Heart Rate Variability and Autonomic Control
Royal Brompton Hospital
2013-2024
Guy's and St Thomas' NHS Foundation Trust
2022-2023
Harefield Hospital
2017-2023
Royal Brompton & Harefield NHS Foundation Trust
2012-2022
Imperial College London
1997-2021
National Health Service
2021
United Nations Industrial Development Organization
2019
St George's, University of London
1993-2017
St George’s University Hospitals NHS Foundation Trust
2017
Bradford Royal Infirmary
2016
Life-threatening ventricular arrhythmia and sudden death remain serious late complications after tetralogy of Fallot repair. Nevertheless, there remains no clear way predicting which patients are at risk.The study population included a total 178 adult survivors (mean follow-up, 21.4 years) repair who were currently attending our clinic. Mechano-electrical relations sought in 41 the 23.6 operated on by one surgeon prospectively studied with 12-lead ECG, chest radiography, two-dimensional...
Background— Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder causing life-threatening arrhythmias whenever sympathetic activity increases. β-Βlockers are the mainstay of therapy; when they fail, implantable cardioverter-defibrillators (ICDs) used but often cause multiple shocks. Preliminary results with flecainide appear encouraging. We proposed left cardiac denervation (LCSD) as useful additional therapy, evidence remains anecdotal. Methods and Results—...
Sudden arrhythmic death syndrome (SADS) describes a sudden with negative autopsy and toxicological analysis. Cardiac genetic disease is likely etiology.
Abstract Aims Calmodulinopathy due to mutations in any of the three CALM genes (CALM1–3) causes life-threatening arrhythmia syndromes, especially young individuals. The International Registry (ICalmR) aims define and link increasing complexity clinical presentation underlying molecular mechanisms. Methods results ICalmR is an international, collaborative, observational study, assembling analysing genetic data on CALM-positive patients. has enrolled 140 subjects (median age 10.8 years...
We have previously shown that QRS prolongation (> or = 180 ms) is a risk marker for sustained ventricular tachycardia (VT) late after repair of tetralogy Fallot (rTOF). now examined the dispersion QT and its components JT, in an attempt to determine whether any association exists between these measurements presence VT patients.QRS duration QT/QRS/JT were measured manually from standard ECGs 10 syncopal rTOF patients (21.4 +/- 4.6 years repair; group 1) with > ms documented compared 9 no...
One hundred and seventeen episodes of supraventricular tachycardia in 50 children, including 28 infants, were treated with intravenous adenosine. Adenosine was prepared a sterile solution 0.9% saline (1 mg/ml) given incremental doses 0.05 mg/kg every two minutes to maximum 0.25 mg/kg. Ninety the 117 terminated. This included 88 102 junctional (79 92 atrioventricular reentry tachycardia, seven eight nodal both long R-P' tachycardia). Only one four His bundle ectopic atrial None three flutter...
Genetic variants in calsequestrin-2 (
Background: Symptomatic children with catecholaminergic polymorphic ventricular tachycardia (CPVT) are at risk for recurrent arrhythmic events. β-Blockers decrease this risk, but studies comparing individual β-blockers in sizeable cohorts lacking. We aimed to assess the association between events and type of β-blocker a large cohort symptomatic CPVT. Methods: From 2 international registries patients CPVT, RYR2 variant–carrying (defined as syncope or sudden cardiac arrest before initiation...
<h3>Importance</h3> Calcium-release deficiency syndrome (CRDS), which is caused by loss-of-function variants in cardiac ryanodine receptor 2 (RyR2), an emerging cause of ventricular fibrillation. However, the lack complex polymorphic/bidirectional tachyarrhythmias during exercise stress testing (EST) may distinguish it from catecholaminergic polymorphic tachycardia (CPVT). Recently, first clinical series describing condition, mouse and human studies showed that long-burst, long-pause,...
Twenty three children with recurrent supraventricular tachycardia were treated flecainide. one of these received intravenous treatment during an attack (2 mg/kg over 10 minutes). The was terminated in 17. After bolus flecainide, blood samples drawn at regular intervals for analysis flecainide concentration 48 hours. Pharmacokinetic variables calculated--median terminal half life 7.5 hours, median volume distribution 6.2 l/kg, and plasma clearance 7.2 ml/min/kg. There a significant...
Variants in NDUFB11, which encodes a structural component of complex I the mitochondrial respiratory chain (MRC), were recently independently reported to cause histiocytoid cardiomyopathy (histiocytoid CM) and microphthalmia with linear skin defects syndrome (MLS syndrome). Here we report an additional case CM, carries de novo nonsense variant NDUFB11 (ENST00000276062.8: c.262C > T; p.[Arg88*]) identified using whole-exome sequencing (WES) family trio. An identical has been previously...
Abstract Aims Sudden cardiac death (SCD) is the most common mode of in paediatric hypertrophic cardiomyopathy (HCM). This study describes implant and programming strategies with clinical outcomes following implantable cardioverter-defibrillator (ICD) insertion a well-characterized national HCM cohort. Methods results Data from 90 patients undergoing ICD at median age 13 (±3.5) for primary (n = 67, 74%) or secondary prevention 23, 26%) were collected retrospective, longitudinal multi-centre...
To examine the benefits of restoring atrioventricular synchrony to children with His bundle tachycardia after operation for congenital heart disease.Review clinical outcome adopting technique R wave synchronised atrial pacing as an adjunct management from September 1987 till June 1990.Eleven consecutive (aged between 3 days and 13 years) haemodynamically significant cardiopulmonary bypass surgery.Atrial either manually or automatically was implemented so that depolarisation preceded...
This report concerns three children with His bundle tachycardia who died following cardiac surgery. At autopsy the conduction system was examined in detail. In all three, sinus node intact and supplied by a well-formed artery. Haemorrhagic tracks were identified invading penetrating atrioventricular bundle. The originated from stitches placed close to tissue. hypothesis that disruption of tissue results an arrhythmogenic focus is discussed.
PurposeBiallelic hypomorphic variants in PPA2, encoding the mitochondrial inorganic pyrophosphatase 2 protein, have been recently identified individuals presenting with sudden cardiac death, occasionally triggered by alcohol intake or a viral infection. Here we report 20 new families harboring PPA2 variants.MethodsSynthesis of clinical and molecular data concerning 34 five previously reported 12 novel variants, 11 which were functionally characterized.ResultsAmong individuals, only 6 remain...