Clinical Approach to Sudden Cardiac Death Syndromes

Clinical cardiologists are encountering an important challenge in the caring of families with inherited cardiac diseases. The majority of the inherited cardiac diseases causing sudden death express themselves at variable ages in the form of altered muscle function (i.e hypertrophic or dilated cardiomyopathy) or in the form of arrhythmias (i.e. Brugada syndrome, long QT syndrome). However, it is not uncommon that the first sign of the disease may actually be sudden cardiac death, even before the identification of clear clinical abnormalities. In this last decade, with more than 50 new disease-associated genes identified, the possibility of genetic testing has opened a new opportunity to disease diagnosis and prevention. Clinical and genetic research is continuously on-going not only to identify those at risk, but to better define their level or risk still with limited success.