Chapter 6: Clinical Presentation, Spectrum of Disease, and Natural History

Dilated cardiomyopathy (DCM) represents the third most common cause of heart failure (HF) and the most frequent cause of heart transplantation in the western world. DCM aetiologies can be classified as genetic or nongenetic. Genetic causes account for 30–40% of DCMs and involve genes that encode cytoskeletal, sarcomere, and nuclear envelope proteins among others. Transmission is variable but mostly with an autosomal dominant pattern. Acquired causes include myocarditis, tachyarrhythmias, alcohol abuse, drugs, catecholamines, toxins, and metabolic or endocrine disturbances. Before diagnosing DCM, it is necessary to exclude conditions with phenotypic overlap. A comprehensive integrated approach to patients with a newly diagnosed DCM is essential in order to achieve an accurate early prognostic stratification. Thus, the aim of this chapter is to discuss about the clinical features, the natural history, and the spectrum of such a heterogeneous disease.