Arrhythmogenic Right Ventricular Dysplasia
ARVD
Nicole Rice RN, BSN
March 7,2012
MSN 675
Microsoft Cipart
Case Study
Lorenzo Guiterrez, 39, was driving when he experienced sudden
onset of chest pains, dizziness, diaphoresis. He happened to be
driving by a local emergency department. As he entered, he
collapsed. He was revived after CPR and defibrillation, restoring
his ventricular tachycardia (VT) to normal sinus rhythm (NSR).
He reported having feelings like this before, and two of his family
members had died of sudden cardiac death. His past medical
history was significant for mild hypertension.
“ARVD/C is a leading cause of sudden death among young athletes,
although people within a broad range of ages and activity levels
have this condition. It affects men and women of all races, and
approximately 1 out of every 5,000 is diagnosed with ARVD/C. It’s
possible to have this disease without knowing it. These silent
carriers may not recognize its symptoms when—or if –they occur.
Palpitations, fainting, chest pain, and rapid heartbeat are some
warning signals of ARVD/C.” (2010 Johns Hopkins Patient
Education Brochure, p. 1)
Back to
question
Tutorial Objectives
Define Arrhythmogenic Right Ventricular
Dysplasia (ARVD)
 Identify common signs and symptoms in
the physical exam of the ARVD patient
 Outline commonly used diagnostic tests to
confirm a ARVD diagnosis
 Describe treatments used to help those
who suffer from ARVD
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What is ARVD?
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ARVD is a dangerous cardiac condition that can
cause irregular heart rhythms and sudden death.
It is characterized by a thinned and dilated right
ventricle (RV) from a progressive loss of
myocytes which are replaced with fibrous and
fatty tissue in the RV myocardium.
The fatty tissue disrupts electrical pathways.
Fatty tissue in RV causes abnormal contractions
like ventricular tachycardia (VT) thus abnormal
ejection of blood.
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Microsoft Clipart
The Johns Hopkins Arrhythmogenic
Right Ventricular Dysplasia Program has
described that it is widely recognized
that ARVD is often caused by mutations
in proteins called desmosomal proteins.
These proteins are like a bridge that
links one heart cell to the next. Major
components of desmosomes are
plakophilin-2, desmoglein -2,
desmocollin-2, desmoplakin, and
plakoglobin.
It has been discovered that those who
are afflicted with ARVD have genetic
abnormalities in the genes encoding for
these desmosomal proteins.
Since the mechanical bonds are weak
and faulty, over time and exertion, the
process of pulling apart causes
formation of scarring and fat
replacement. We would see the pulling
action when the heart is being worked,
like during exercise.
What is ARVD?
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The condition is precipitated by
a release of catecholamine
surges like exercise,
dehydration, stress, cocaine or
electrolyte imbalances.
Clinical manifestations include
palpitations, syncope, cardiac
arrest, mental confusion,
anxiety. In progressive phases
with left ventricular
involvement dyspnea on
exertion and orthopnea are
present.
Four ARVD Stages
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Concealed phase: Pt usually asymptomatic, may have minor
VT, RV is only seen with subtle changes. These clients are at high
risk of sudden death.
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Overt phase: Noted structural and functional changes in the
RV. Symptoms seen are ventricular dysrhythmias, presyncope,
syncope, and palpitiations.
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Weakening of RV: The RV has dilated and weakened. Client
exhibits symptoms like RV failure including edema of lower
extremities, abdominal distention, dyspepsia, and anorexia.
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Weakening of the LV:
The LV dilates and weakens. Now see
symptoms of heart failure which include dyspnea on exertion,
orthopnea, and breathlessness.
Source:
Andrews, T., Cook, S., Baumeister, M., Hickey, K., (2010). Sudden Killer: Arrhythmogenic right ventricular cardiomyopathy.
Nursing, 40 (7) p. 7-11.
Question about topic
 After
reviewing the case study, what
are the early signs and symptoms of
ARVD? Click here to see case study.
Note: there is more than one right answer
Palpitations, chest
pain, irregular heart
rhythm
Yes that’s it!
Mental confusion,
anxiety
You bet!
Lower extremity
edema, large
abdominal girth
Sorry, those are later
signs
Testing your knowledge so far
 Knowing
what you have already
seen, what are the characteristics of
ARVD?
Note: there is more than one right answer
Happens to young
athletes
Clients can present
at different stages
of ARVD
Fatty fibrous tissue
in RV
Yes, you are correct
Yes, four stages of
ARVD
Yes, that is right
ECG abnormalities:
• 90% of patients have some abnormality, typically T wave
inversions. Also noted is a right bundle branch block.
• “In addition, an epsilon wave secondary to slowing of
intraventricular conduction. This wave is seen at the terminal
notch in the QRS complex, and most often seen on signalaveraged ECG, (Andrews,Cook,Baumeister,Hickey (2010), p.
4.)”. The epsilon wave is also described as “distinct waves of
small amplitude present in the ST segment in right precordial
leads, (Yerra,Caskey,Modi,Reddy (2008), p. 4)”.
• Also noted are a wide QRS greater than 110 milliseconds
and a prolonged S wave in leads V1 to V3 due to delayed RV
repolarization.
Illustration of epsilon wave
Red triangles mark epsilon wave, also note inverted T wave.
wikipedia image
used under creative
commons license
Cross section of heart showing pronounced adipose infiltration of right
ventricular free wall and nearly normal left ventricle and ventricular
septum. Image used with permission from Johns Hopkins ARVD Dysplasia Program.
http://www.ncbi.nlm.nih.gov/sites/ppmc/articles/PMC483655
Fatty
infiltrate
Diagnostic Testing
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ECG, Holter monitoring,
Echocardiograph, Cardiac
MRI, and RV angiography,
cardiac biopsy.
Additional test that is helpful
is ECG with exercise stress
testing. This helps to see
with stress on the heart the
frequency and duration of
ectopic beats or ventricular
dysrhythmias.
Diagnostic Findings
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Echocardiographic abnormalities: RV dilation, hypokinesis, and very
thin RV free wall. Tricuspid regurgitation may also be noted.
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MRI: RV-free wall changes. Fatty infiltration, thinning, and akinesis of RV
free-wall are commonly noted.
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Angiography: “akinetic or dyskenetic bulging may be localized in the
triad of dysplasia areas (infundibular, apical, and subtricuspid) this test
has 90% specificity (Andrews,Cook,Baumeister,Hickey (2010), p. 4.) “.
“For a clinician to arrive at a definitive diagnosis of ARVD, two major criteria
or one major and two minor critieria must be present, or four minor
criteria from different groups (Andrews,Cook,Baumeister,Hickey (2010), p. 4.)”.
Review
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Thinking back to the case study, what kinds of
ECG findings might we see in diagnostic studies
after he has stabilized?
Note: there is more than one right answer
the epsilon wave
T wave inversion
Prolonged S wave
definitely, that is
a hallmark sign
yes!
Yes! Also wide QRS
Genetic Testing
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ARVD is an inherited disease with
variable presentation within families.
Studies about the genetic possibilities
of the disease found that nearly 40%
of people with familial tendencies have
ARVD. It is primarily an autosomal
dominant genetic disorder, or a 50%
chance that offspring will develop
ARVD or its symptoms.
A genetic mutation in the protein
plakophilin-2 (PKP2) is associated with
up to 45% of ARVD patients.
Patients with PKP2 are also more likely
to develop ARVD at an early age.
Further testing has been taking place
to isolate more genes that may identify
a person at risk for developing ARVD.
(Yerra,Caskey,Modi,Reddy (2008), p.6),
(Andrews,Cook,Baumeister,Hickey (2010), p.4)
http://www.hopkinsmedicine.org/hmn/F07/medical.
cfm
Microsoft Clipart
Treatment of ARVD
Medications:
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Sotalol (beta-adrenergic blocker)
Amiodarone (anti-arrhythmic)
Warfarin – “prevents embolic development
secondary to high residual blood volume in
RV (Andrews,Cook,Baumeister,Hickey (2010), p. 4.)”
Treatment of ARVD
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ICD placement – commonly performed, with
caution due to thinning of RV wall causing the
risk of perforation.
Radio Frequency Ablation - for those with less
risk of perforation it is a therapeutic option in
patients unresponsive or intolerant to
antiarrhythmic drugs. In recent research,
(Arbelo, 2010) catheter ablation should be the
first line approach in patient with recurrent
monomorphic ventricular tachycardia. This will
disable the malfunctioning circuits and reduce
incidence of arrhythmia occurring.
Now that I know about ARVD…
It is very important that a thorough physical assessment is
performed, especially for young adults undergoing physicals
for competitive or contact sports.
The assessment should include asking questions of parents about:
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family history of palpitations
lightheadedness
blackouts or syncopal events
evaluation of ECG if history or symptoms are
present
● If there are concerns or a strong history of sudden
cardiac events (arrhythmias, arrest, MI) in close
relatives a family pedigree should be obtained with at
least three generations.
Literature Cited
Andrews, T., Cook, S., Baumeister, M., Hickey, K., (2010). Sudden Killer:
Arrhythmogenic right ventricular cardiomyopathy. Nursing, 40 (7) p. 7-11.
Arbelo, E., Josephson, M., (2010). Ablation of ventricular arrhythmias in
arrhythmogenic right ventricular dysphasia. Journal of Cardiovascular
Electrophysiology, 21 (4): 473-86.
Porth, C. M., & Matfin, G., (2009). Pathophysiology: Concepts of altered
health states (8th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.
http://en.wikipedia.org/wiki/ARVD retrieved March 29, 2012.
Yerra, L., Caskey, D., Modi, K., Reddy, P., (2008). Arrhythmogenic Right
Ventricular Dysplasia/Cardiomyopathy: Cinical Profile of Four Patients and
Review. Southern Medical Journal, 101 (3) p. 309-316.
Johns Hopkins Medicine, ARVD Program Patient Brochure, January 2010,
Authored by Johns Hopkins Medical Professionals. Images utilized with
permission from Johns Hopkins ARVD Program official.
http://www.arvd.com