What is aortic disease? The aorta is the largest tube (artery) that carries blood cells containing oxygen around the body to your vital organs. The blood cells are pumped from the main pumping chamber called the left ventricle in to the aorta. The aorta can also be affected by disease and can either generally enlarge (dilate)

WPW – Wolff (Wolfe)-Parkinson-White Syndrome WPW – Wolff-Parkinson-White results from an additional electrical connection between the upper chambers of the heart (atria) and the lower chambers of the heart (ventricles). This extra or ‘accessory’ pathway is seen in approximately 1 in every 300-500 people and sometimes it allows conduction of the electrical pulse at high speed,

Idiopathic left ventricular hypertrophy (LVH) is a fairly novel entity that is increasingly reported following post-mortem examination in cases of sudden cardiac death (SCD)9,10. Studies from our group and others reveal that in selective cohorts idiopathic LVH may be reported in up to 30% of sudden cardiac deaths. Professor Sheppard, one of our senior investigators, was

What is hypertrophic cardiomyopathy? Hypertrophic cardiomyopathy (HCM) is a condition where the heart muscle becomes thickened. Although HCM is a relatively rare heart disease, it is the commonest of the cardiomyopathies, affecting 1 in every 500 people. https://www.youtube.com/embed/FfWnCtmp9IQ Traditionally, the term HCM was used for disease caused by abnormalities in genes which make the proteins responsible

Short QT syndrome (SQTS) is a very rare and poorly understood condition with less than 200 cases of the condition worldwide to date. As the name suggests the QT interval in carriers is shorter than normal, which means that the heart takes a short time to repolarise or reset itself. People with this condition can sometimes

Progressive cardiac conduction defect (PCCD) (also known as Lev-Lenegre’s Syndrome) 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)

Idiopathic ventricular fibrillation (IVF) describes the group of conditions responsible for life-threatening, rapid rhythm disturbances without any signs of the heart diseases described above. In some of these patients, changes on their ECG known as early repolarisation, have been seen. This has become known as early repolarisation syndrome and in a few cases mutations of potassium

Cardiac ion channelopathies (also sometimes referred to as inherited arrhythmic syndromes) 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

Catecholaminergic polymorphic ventricular tachycardia is a rare condition mainly 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. If these do not

Brugada syndrome is a cardiac condition which causes a disruption to the heart’s normal rhythm. Specifically, this disorder can lead to irregular heartbeats in the heart’s lower chambers (ventricles), which is an abnormality called ventricular arrhythmia. Brugada syndrome was first described in the early 1990s. It is an uncommon condition in the western world but seems to be much