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Anaesthetic implications of arrhythmogenic right ventricular dysplasia/cardiomyopathy
Author(s) -
Alexoudis A. K.,
Spyridonidou A. G.,
Vogiatzaki T. D.,
Iatrou C. A.
Publication year - 2009
Publication title -
anaesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.839
H-Index - 117
eISSN - 1365-2044
pISSN - 0003-2409
DOI - 10.1111/j.1365-2044.2008.05660.x
Subject(s) - medicine , arrhythmogenic right ventricular dysplasia , cardiomyopathy , cardiology , dysplasia , heart failure
Summary Arrhythmogenic right ventricular dysplasia, also called right ventricular cardiomyopathy, is a genetically determined heart muscle disease, characterised by life‐threatening ventricular arrhythmias in apparently healthy young people. The primary myocardial pathology is that the myocardium of the right ventricular free wall is replaced by fibrous or fibrofatty tissue, with scattered residual myocardial cells. Right ventricular function is abnormal and in severe cases is associated with global right ventricular dilation and overt biventricular heart failure. Although still relatively rare, arrhythmogenic right ventricular cardiomyopathy is a well recognised cause of sudden unexpected peri‐operative death. In this review, we describe the basic characteristics of this disease, emphasising the diagnosis and we offer some suggestions for the anaesthetic management of these patients in the peri‐operative period.