Medical Information - ARVC to WPW

Sudden death syndrome (SDS) is an umbrella term used for the many different causes of cardiac arrest in young people. CRY provides medical information on the most common causes of unexpected sudden cardiac death – sometimes referred to as SADS – in the young (under 35)

In the UK, unexplained sudden death is frequently recorded as due to death from natural causes. Until the law is changed and coroners have to refer hearts on to specialists we will not know the true figures. CRY’s fast track coroner / pathology service enables the cause of death in a sudden death case to be established more quickly and accurately than might otherwise happen if left to a local coroner lacking expertise in cardiac pathology.

  • Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

    Arrhythmogenic right ventricular cardiomyopathy (ARVC; also known as arrhythmogenic right ventricular dysplasia – ARVD) is a heart muscle disease that can cause life-threatening heart rhythm abnormalities and, rarely, significant weakening of the right side of the heart. ARVC is caused by a defect in the ‘glue’ that holds the muscle cells of the heart together. As the heart muscle is …

  • Churg-Strauss Syndrome

    What is Churg-Strauss syndrome? Churg-Strauss syndrome is a rare disease characterised by generalised inflammation of small to medium blood vessels in the body. The syndrome was described by 2 doctors – Churg and Strauss – in 1951. Increased levels of certain cells in the blood (eosinophils) are seen. The etiology of the syndrome remains unknown and it is thought to …

  • Coronary Artery Anomalies (CAAs)

    What are coronary artery anomalies? The heart muscle requires a regular supply of oxygen and food. This is supplied by blood carried by two blood vessels known as the left and right coronary arteries which come from the left and right side of the body’s main artery, the aorta. In 1 in 100 people both coronary arteries come from the …

  • Coronary Artery Disease (CAD)

    What are premature coronary artery disease and familial hypercholesterolemia? The heart muscle requires a regular supply of oxygen and food. This is supplied by blood carried by a network of blood vessels known as the coronary arteries. The coronary arteries can become narrowed by ‘furring’ of the artery wall with cholesterol, called atherosclerotic plaques. Blood clots can form on these …

  • Dilated Cardiomyopathy (DCM)

    What is dilated cardiomyopathy? In dilated cardiomyopathy (DCM) the main pumping chambers of the heart are enlarged (dilated) and contract poorly. This results in less blood being pumped around the body which fails to meet the body’s demand. This is known as heart failure. There can be a build up of fluid in the lungs and under the skin, which …

  • Endocardial Fibroelastosis (EFE)

    What is EFE? The term Endocardial Fibroelastosis (EFE) refers to the thickening of and the replacement of the heart muscle with fibrous tissue. It may be related to parasitic infections and viruses such as the Coxsackie virus (a form of common cold); or rarely, may have an inherited nature. Unfortunately, it is seen to frequently be fatal in affected children …

  • Hypertrophic cardiomyopathy (HCM)

    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. Traditionally, the term HCM was used for disease caused by abnormalities in genes which make the proteins responsible for contraction of the heart, …

  • Kawasaki Disease

    What is Kawasaki disease? Kawasaki disease is a rare disease that mainly affects children. It is also known as mucocutaneous lymph node syndrome. In developed countries, it is the commonest cause of acquired heart disease in children.   Who gets Kawasaki disease? About 85 percent of the people with Kawasaki disease are under age five, most commonly between the ages …

  • Long QT Syndrome, Brugada & Ion Channelopathies

    Long QT syndrome, Brugada syndrome and ion channelopathies Cardiac ion channelopathies (also sometimes referred to as arrhythmia 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 …

  • Marfan Syndrome

    Information last reviewed: January 2013 What is Marfan Syndrome? Marfan syndrome affects many parts of the body, including the heart, blood vessels, skeleton, and eyes. 1 in 5,000 people in the United Kingdom have Marfan syndrome and both men and women can be affected. People with Marfan syndrome produce abnormal ‘connective tissue’. Connective tissue helps hold the body together, binding …

  • Myocarditis

    What is myocarditis? Myocarditis refers to inflammation of the heart muscle. It is most often due to a virus. Other causes include drug abuse and conditions such as systemic lupus erythematosus (SLE). Myocarditis due to a virus is relatively common, but most cases are very mild and are never seen by a doctor. However, some cases are severe and can …

  • Patent Foramen Ovale (PFO)

    What is a patent foramen ovale (PFO) and what causes it? A patent foramen ovale (or PFO for short) is a flap between the top 2 chambers of the heart which has not closed the way it should do at birth. During a baby’s development in the womb, this flap is fully open as a hole called the foramen ovale, …

  • Premature Coronary Artery Disease and Familial Hypercholesterolemia

    The heart muscle requires a regular supply of oxygen and food. This is supplied by blood carried by a network of blood vessels known as the coronary arteries. The coronary arteries can become narrowed by ‘furring’ of the artery wall with cholesterol, called atherosclerotic plaques. Blood clots can form on these plaques and block the artery, causing chest pains. The …

  • Restrictive Cardiomyopathy

    What is restrictive cardiomyopathy? Restrictive cardiomyopathy is the rarest of the cardiomyopathies. It is characterised by increased stiffness of the heart muscle, usually due to scar tissue, which prevents adequate filling of the chambers of the heart. The actual pumping action of the heart is usually normal. The cause of restrictive cardiomyopathy is unknown in the majority of cases. In …

  • Right Bundle Branch Block (RBBB)

    What is right bundle branch block? Every normal heart is gifted with a natural in-built electrical system. The electrical impulse generated from this system results in beating of the heart. The electrical system divides into 2 branches (called right and left bundle) at the level of the ventricle (bottom chambers of the heart). The right bundle stimulates the right ventricle …

  • Tachycardia

    Definition A tachycardia is a heart rate exceeding 100 beats per minute. Classification A tachycardia can be either physiological (normal response) or pathological. Examples of a physiological tachycardia are during exercise, in pregnancy or in situations of anxiety or excitement (“flight, fright, fight”). A pathological tachycardia can be due to either a primary cardiac abnormality or a non-cardiac factor. Non-cardiac …

  • Wolff (Wolfe)-Parkinson-White Syndrome (WPW)

    What is Wolff (Wolfe)-Parkinson-White Syndrome? Wolff-Parkinson-White (WPW) 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, generating an electrical short circuit, …

Cardiac Conditions in the Young: From ARVC to WPW

Information on these medical conditions is also available in this CRY booklet - Cardiac Conditions in the Young: from ARVC to WPW

Information for the family and relatives of a young person who has died of Sudden Arrhythmic Death Syndrome - SADS sometimes called sudden adult death syndrome

by Dr Elijah R Behr MD