Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is probably the second
most common cause of unexpected sudden death in the young.
The incidence of this
condition is now thought to be higher than previously believed, due to the
availability of better diagnostic techniques and general awareness of the
disorder among cardiologists.
It is anticipated that more information
regarding the condition will be available in the coming years, to help to
recognise the pathology.
The disorder is
characterised by a progressive replacement of normal right ventricular muscle
cells by fibrous tissue and fat. Initially this may only involve specific
regions of the right ventricle but later on it becomes global and may even
involve the ventricle as well. ARVC was first recognised in the late 1970’s
and the Cardiovascular Group at the University of Padova in Italy have developed
particular expertise in the condition.
The precise cause of ARVC is not known. The condition is normally familial
and is passed on in the genes from one generation to the next. The pattern of
inheritance is autosomal dominant such that the child of an affected parent will
have 50% chance of inheriting the abnormal gene. The disease affects men and
women equally and has been recognised in people of diverse ethnic origin.
The
Northern region of Italy
seems to have highest prevalence of the disease.
Clinical presentation is usually with symptoms of arrhythmia and occasionally
with sudden death. Typical arrhythmia symptoms are of rapid heart beat associated
with light-headedness or fainting episodes. Unlike most
cardiomyopathies, shortness of breath and chest pains are unusual
symptoms and tend only to occur in older patients.
The diagnosis of ARVC can be
extremely difficult and usually requires specialist expertise. There are no
'gold standard' investigations as there are available for diagnosing HCM.
The investigations are similar to those performed in the diagnosis of hypertrophic and other cardiomyopathies
and include an electrocardiogram and a two dimensional echocardiogram. The
features, however, are usually very subtle, as in
the early stages the condition is often confined only to the right ventricle.
Other methods of imaging the right side of the heart are sometimes useful and
these include magnetic resonance imaging.
Despite all these
investigations, the condition can still be missed.
The majority of patients with this condition are asymptomatic for many years
unless arrhythmias develop. Treatment in the majority then aims to prevent or at
least control arrhythmia with drugs. When drug treatment is unsuccessful,
an implantable cardioverter defibrillator and other specialised treatments may be
necessary, these include:
Catheter Ablation
– This involves the delivery of electrical
energy via a catheter inserted in the groin to the area in the right side of
the heart where the arrhythmia is originating from. The overall effect is to
create a small scar which is incapable of transmitting any arrhythmia.
Surgical Ablation
– In individuals where the arrhythmias are originating from many different
sites in the right side of the heart. Occasionally it is necessary to perform
open-heart surgery. During the
operation, the electrical pathway of the arrhythmia can be mapped,
identified and destroyed.
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