14th International Congress
THE "NEW FRONTIERS"
OF ARRHYTHMIAS 2000

Jan. 29 - Feb. 5, 2000
Marilleva, Trento, Italy

RT-20

Hypertrophic cardiomyopathy and sudden death in the young

Cristina Basso, Domenico Corrado, Gaetano Thiene.
Department of Pathology, University of Padua Medical School, Padua, Italy

Hypertrophic cardiomyopathy (HCM) is a highly arrhythmogenic primary heart muscle disease. Clinical manifestations are variable ranging from a benign asymptomatic course to severe heart failure and cardiac arrest. Ventricular arrhythmias, palpitations and syncope are frequent signs and symptoms and sudden, unexpected cardiac arrest is a pending catastrophe along with the natural history. Molecular genetic studies demonstrated that HCM is a heterogeneous disease, with several missense mutations in genes encoding for 7 proteins of the cardiac sarcomere¹, including beta-myosin heavy chain on chromosome 14, cardiac essential myosin light chain on chromosome 3, cardiac regulatory myosin light chain on chromosome 12, cardiac troponin T on chromosome 1, cardiac troponin I on chromosome 19, alfa-tropomyosin on chromosome 15 and cardiac myosin-binding protein C on chromosome 11.

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