Arrhythmogenic right ventricular cardiomyopathy: clinical presentation, diagnosis, and management
S Sen-Chowdhry, MD Lowe, SC Sporton… - The American journal of …, 2004 - Elsevier
Arrhythmogenic right ventricular cardiomyopathy, also known as right ventricular dysplasia,
is a genetically determined heart muscle disease associated with arrhythmia, heart failure,
and sudden death. Autosomal dominant inheritance is typical. The identification of causative
mutations in cell adhesion proteins has shed new light on its pathogenesis. Fibrofatty
replacement of the myocardium, the hallmark pathologic feature, may be a response to
injury caused by myocyte detachment. Sudden death is often the first manifestation in …
is a genetically determined heart muscle disease associated with arrhythmia, heart failure,
and sudden death. Autosomal dominant inheritance is typical. The identification of causative
mutations in cell adhesion proteins has shed new light on its pathogenesis. Fibrofatty
replacement of the myocardium, the hallmark pathologic feature, may be a response to
injury caused by myocyte detachment. Sudden death is often the first manifestation in …