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Contents


Introduction ............................................................................................................... 1 How the heart works and how it can cause a blackout or cardiac arrest ......................... 2


What is a Sudden Cardiac Death (SCD) & Sudden Arrhythmic Death Syndrome (SADS)? .. 4 Sudden Cardiac Death (SCD)


Sudden Arrhythmic Death Syndrome (SADS)


4 4


How does SADS relate to the cardiac channelopathies/arrhythmia syndromes?............... 4


Ion Channelopathies ................................................................................................... 5 Long QT Syndrome (LQTS) Brugada Syndrome


5 8


Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Progressive Cardiac Conduction Defect (PCCD) Short QT Syndrome (SQTS) Early Repolarisation Syndrome Sodium Channel Disease Familial Atrial Fibrillation


Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Dilated Cardiomyopathy (DCM)


10 11 12 12 13 13


Cardiomyopathies ..................................................................................................... 13 Hypertrophic Cardiomyopathy (HCM)


13 16 17


Other cardiac conditions that can affect young people ................................................. 20 Wolff-Parkinson-White Syndrome (WPW) Marfan Syndrome Myocarditis


Coronary Artery Anomalies Premature Coronary Artery Disease and Familial Hypercholesterolemia


20 21 22 23 24


Tests ....................................................................................................................... 25 Medical and family history Medical examination


Electrocardiogram (ECG) Signal averaged ECG Echocardiogram Exercise test


Cardiopulmonary exercise test Holter


Cardiomemo and event recorder Implantable Loop Recorder (ILR)


Provocation tests (ajmaline, flecainide, adrenaline and adenosine tests) Cardiac magnetic resonance/magnetic resonance imaging (CMR/MRI) scan Computed tomography (CT) Other tests


Coronary angiography


Electrophysiological (EP) study Ablation


Cardiac biopsy Tilt-table testing Genetic testing


Drugs to avoid Low potassium in the blood (hypokalaemia)


25 25 26 26 27 27 28 29 29 29 30 30 30 31 31 31 31 31 31 32


General Lifestyle Advice ............................................................................................ 33 Exercise


Drugs which people with long QT syndrome should avoid.............................................. 35 Drugs which people with Brugada syndrome should avoid............................................. 36 The future................................................................................................................. 37 Technical terms......................................................................................................... 37 Index........................................................................................................................ 46


33 33 34


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