Arrhythmogenic right @entity296 / XXXX : a review and update.

@entity1164 @entity1 @entity296 @entity1165 @entity712 @entity8259 @entity1163 @entity2778 @entity74 @entity14106 @entity67 @entity943 @entity23661 @entity204 @entity1141 @entity710
Arrhythmogenic right @entity296 / @entity1141 (ARVC/D) is a predominantly genetically determined and heritable form of @entity296 that is characterized pathologically by the replacement of myocytes by adipose and fibrous tissue and leads to @entity67 , @entity943 , and @entity1164 . The estimated prevalence of ARVC/D in the general population ranges from 1 in 2,000 to 1 in 5,000, @entity1 are more frequently affected than @entity1 , with an approximate ratio of 3:1. ARVC/D can be inherited as an @entity74 with reduced penetrance and variable expression, autosomal recessive inheritance is also described. There have been 12 genes identified which are linked to ARVC/D, encoding several components of the cardiac desmosome. Dysfunctional desmosomes resulting in defective cell adhesion proteins, such as plakoglobin ( @entity23661 ), @entity710 (DSP), @entity14106 ( @entity14106 ), and @entity8259 ( @entity8259 ) consequently cause loss of electrical coupling between cardiac myocytes, leading to myocyte cell death, fibrofatty replacement and @entity943 . Diagnosis is based on the finding a combination of characteristic abnormalities in family history, electrocardiography, cardiac imaging as well as endomyocardial biopsy (original task force criteria). Therapeutic options remain limited because of the progressive nature of ARVC/D. Competitive athletics should be avoided. @entity1 with ARVC/D with a history of having been resuscitated from @entity1164 , @entity1 with @entity1163 , very young @entity1 , and those who have marked right ventricular involvement are at the highest risk for @entity204 and also, the presence of left ventricular involvement is a risk factor. Several authors concluded that @entity1 who meet the Task Force criteria for ARVC/D are at high risk for @entity1164 and should undergo ICD placement for primary and secondary prevention, regardless of electrophysiologic testing results. The role of electrophysiologic study and VT catheter ablation in ARVC/D remains poorly defined, and is frequently used as a palliative measure for @entity1 with refractory VT. The progressive nature of ARVC/D suggests that catheter ablation would not be a long-term curative procedure. @entity2778 proved to be highly effective in @entity1 with ARVC/D and inducible as well as non-inducible @entity1165 ; if it is ineffective in inducible @entity1165 response to other antiarrhythmic drugs is unlikely and therefore non-pharmacological therapy without further drug testing should be considered. Orthotopic heart transplantation is considered in @entity1 with progressive @entity712 and intractable recurrent @entity943 .
