Russell Goodman
Living with an implantable cardioverter defibrillator (ICD) and an undiagnosed condition A healthy way to start the weekend, get up Saturday morning and go for a run. This was something my fiancée Charlotte and I enjoy regularly, sick this one however was very different. After being out for just over an hour running the streets […]
Implantable cardioverter defibrillator (ICD)
Read the ICD Special Issue myheart newsletter If the bottom chambers (ventricles) of your heart are prone to going into either a dangerously fast or a chaotic heart rhythm (called ventricular tachycardia and ventricular fibrillation respectively), or if you are thought to be at risk of your heart going into these dangerous rhythms, then an implantable […]
Pacemakers
Pacemakers electrically stimulate your heart to make it contract and produce a heartbeat. They differ from implantable cardioverter defibrillators (ICD) in that they do not have the ability to give your heart an electric shock. Pacemakers can be helpful if you have problems with the electrical activity in your heart which causes it to beat […]
Arrhythmogenic right ventricular cardiomyopathy (ARVC)
The incidence of arrhythmogenic right ventricular cardiomyopathy (ARVC) is now thought to be higher than previously believed (affecting 1 in every 1000 individuals), due to the availability of better diagnostic techniques and general awareness of the disorder amongst the medical profession. ARVC was first recognised in the late 1970’s. It is anticipated that even more information regarding ARVC will be available in the coming years, to help us understand the natural history of the condition.
Brugada syndrome
In the western world it affects mainly young and middle-aged adult men. It has been associated with mutations in the same sodium channel that is affected in long QT syndrome, but this appears to account for only 1 in every 5 people with the condition. The sodium channel behaves abnormally in that movement of sodium ions into the cells is restricted. This results in particular changes on the ECG but no abnormalities in the structure of the heart. Other genes have been described as being involved in Brugada syndrome that produce calcium ion channels and a protein in the cell surface (membrane) that interacts with the sodium channel. They have, however, only been detected in a small number of carriers.
Catecholaminergic polymorphic ventricular tachycardia (CPVT)
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare condition that has been associated with two genes that make proteins found inside the cell – the human ryanodine receptor (a calcium ion channel) and calsequestrin (a protein that interacts with the calcium channel). These regulate the release of calcium ions into the rest of the cell. […]
Hypertrophic cardiomyopathy (HCM)
Read personal stories from myheart members with hypertrophic cardiomyopathy here. Hypertrophic cardiomyopathy (HCM) is a condition where the heart muscle becomes thickened. Traditionally, the term HCM was used for disease caused by abnormalities in genes which make the proteins responsible for contraction of the heart (sarcomeric contractile proteins). More recently the definition of HCM has […]
Long QT syndrome (LQTS)
Long QT syndrome (LQTS) is an ion channelopathy. Ion channelopathies (also sometimes referred to as arrhythmia syndromes or cardiac channelopathies)affect the electrical functioning of the heart without affecting the heart’s structure. They are a group of rare genetic conditions that are caused by abnormalities of the DNA known as mutations. They are usually inherited from parents although they can occur for the first time in a person. (If they occur for the first time they are described as sporadic.)
Progressive cardiac conduction defect (PCCD)
Progressive cardiac conduction defect (PCCD) is a rare condition. In people with PCCD, the heart’s electrical impulses are conducted very slowly and this results in the gradual development over time of heart block. (Heart block is a failure of the heart’s electrical impulse to conduct properly from the top chambers [the atria] to the bottom […]
Short QT syndrome (SQTS)
This rare condition is similar to but distinct from long QT syndrome (LQTS). As the name suggests the QT interval in carriers is shorter than in normal people. This means that the heart takes a shorter time to repolarise or reset itself,making it prone to ventricular arrhythmias. There is also an increased risk of a […]