ARVD2
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Arrhythmogenic right ventricular dysplasia Microchapters |
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Differentiating Arrhythmogenic right ventricular dysplasia from other Diseases |
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Diagnosis |
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Treatment |
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ARVD2 On the Web |
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American Roentgen Ray Society Images of ARVD2 |
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Directions to Hospitals Treating Arrhythmogenic right ventricular dysplasia |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Arrhythmogenic right ventricular dysplasia type 2;
Overview
This is a "concealed form" of ARVD. There is no change in heart size. There are no EKG changes on the resting electrocardiogram, but there may be exercise induced polymorphic ventricular tachycardia. This variant is associated with premature death.
Pathophysiology
Although the heart is normal in size, on pathologic examination, there are large areas of fibro-fatty replacement in the subepicardial layer of the right ventricle.
There are also abnormalities in calcium hemostasis in the myocytes which may contribute to the occurrence of ventricular arrhythmias.
Genetics
This variant (600996) is associated with a mutation in the RYR2 gene (180902) on chromosome 1q42-q43.
Epidemiology and Demographics
Natural History, Complications, Prognosis
This ARVD variant is associated with premature death.
Diagnosis
Symptoms
Exercise induced polymorphic VT may be present.
Electrocardiogram
There are no EKG changes on the resting electrocardiogram, but there may be exercise induced polymorphic ventricular tachycardia.