3. Clinical significance
It is an important cause
of sudden cardiac death
in young adults:
âą11% of all cases and 22% of
cases among athletes
Medscape.com
4. Pathophysiology
fatty
infiltration and
fibrosis of the
myocardium of
the RV
Right ventricle:
progressive RV
dilatation and
dysfunction
Right ventricle:
The prognosis
is worse in
patients with
involvement of
the LV!
Left ventricle:
Medscape.com
5. Mechanisms of myocardial loss are:
apoptosis (programmed cell
death)
inflammation, enhanced
fibrosis and loss of function
fatty replacement of
myocardium
Medscape.com
6. Etiology
âȘ ARVD is an inherited disorder (present in the fetus).
âȘ It may result from an acquired etiology such as viral
infection (myocarditis).
âȘ Patients with a genetic predisposition are more likely to
develop myocarditis.
Disease manifests more frequently in active individuals:
âȘ mechanical sheer stress can cause cell membrane
damage, inflammation, and fibrosis in genetically
predisposed individuals
Medscape.com
7. Genetics
Autosomal dominance
The most common
pattern of inheritance.
Veneto region of Italy:
higher penetrance of
genes.
Autosomal recessive pattern
Naxos disease:
âȘ Greek island of Naxos
âȘ palmoplantar keratosis
âȘ wooly hair
The genetic mutation occurs on
chromosome 17q21.
Penetrance is almost 100%
Medscape.com
11. Clinical presentation
Progressive RV dysfunction results in dyspnea and leg
swelling.
In more severe cases with LV involvement, patients may
present with biventricular congestive heart failure.
Supraventricular arrhythmias (atrial flutter and fibrillation)
may be seen in 25% of cases.
Exercise can induce ventricular arrhythmias and sudden
cardiac death.
Medscape.com
13. ECG findings
âȘ normal in the latent phase of ARVD
âȘ intraventricular conduction
abnormality
âȘ T wave abnormalities
âȘ anteroseptal T wave inversion
âȘ epsilon waves
Medscape.com
14. Major ECG criteria
Inverted T waves in right precordial
leads in individuals older than 14 years
in the absence of complete right
bundle branch block (87% of patients).
Epsilon waves (low-amplitude signals
between end of QRS complex and the
onset of T wave) in leads V1-V3 (33%
of patients).
Medscape.com
15. Inverted T waves in
V1-V2 without
complete RBBB
Medscape.com
Medscape.com
19. Therapy
âȘ The goal of therapy is to reduce the frequency and
severity of arrhythmias.
âȘ Beta-blockers are the first line of drug therapy.
âȘ Sotalol is the most effective drug for inducible or
noninducible ventricular tachycardia.
âȘ An implantable cardioverter-defibrillator (ICD) is
recommended for secondary prevention of sudden
cardiac death in patients with sustained ventricular
tachycardia or ventricular fibrillation, and for primary
prevention in high-risk patients.
Medscape.com
20. Message at the end
Dear GENERAL
PRACTITIONERS be
ready to recognize ARVD
and send the patient to
the cardiologist!
Medscape.com