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Clinical relevance of right ventricular dysplasia/cardiomyopathy
  1. Divisione di Cardiologia, Ospedale Maggiore
  2. piazza Ospedale 1, 34129 Trieste, Italy

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(Arrhythmogenic) right ventricular dysplasia, also called (arrhythmogenic) right ventricular cardiomyopathy (RVD/C) is a recently defined heart muscle disease of unknown origin that predominantly, although not exclusively, affects the right ventricular myocardium.

Its pathological hallmark is the atrophy of myocytes with fatty or fibro-fatty infiltration of the right ventricle.1 The typical clinical picture is related to ventricular tachyarrhythmias with left bundle branch block morphology, which may cause sudden death,1 ,2 while a less frequent presentation is “right” heart failure. The disease may be localised or widespread, with biventricular involvement in some cases.3

In the past decade several investigators have contributed to the knowledge of this “new” cardiomyopathy4; nevertheless, there are still many uncertainties that deserve further studies. This editorial is mainly oriented to some still unsolved problems of RVD/C.

Aetiologic theories

Several hypotheses have been advocated to explain the cause and pathogenesis of RVD/C. The first, that explains the term “dysplasia” (from the ancient Greek, abnormality of development) considered the disease a congenital or disontogenetic disorder caused by an abnormal development of right ventricular myocardium.2 However, clinical and pathological observations favour the concept of a degenerative process (“atrophy” or “dystrophy”) caused by progressive death of myocardial cells.5 Recent reports suggest that programmed myocyte death (apoptosis) may play an important role, and Mallatet al observed this process in the right ventricular myocardium of patients with RVD/C.6 Moreover, James et al demonstrated massive apoptosis at the level of the right ventricle and conductive system in an infant with Uhl's disease.7

Several factors, known or suspected to be present in the failing myocardium (cytokines, oxidants, nitric oxide, growth factors), can stimulate apoptosis in a variety of cells including cardiac myocytes. However, it is speculative whether apoptosis reflects a primary hereditary abnormality that determines an imbalance …

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