Difference between revisions of "Arrhythmogenic Right Ventricular Cardiomyopathy"

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===What is ARVC?===
 
===What is ARVC?===
[[Image:arvdhart.png|thumb| A section throught the heart of a ARVC patient. (A) Transmural vervetting van de vrije wand van de rechter kamer. (B) De myocardiale atrofie is beperkt tot de rechter kamer, de linker kamer en het interventriculaire septum zijn gespaard gebleven. <cite>Corrado</cite> Reproduced with permission from BMJ Publishing Group Ltd. ]]
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[[Image:arvdhart.png|thumb| A section throught the heart of a ARVC patient. (A) Transmural fatty replacement of the right ventricular free wall. (B) Myocardial atrophy is confined to the right ventricle and substantially spares the interventricular septum as well as the left ventricular free wall. <cite>Corrado</cite> Reproduced with permission from BMJ Publishing Group Ltd. ]]
Aritmogene Rechter Ventrikel Dysplasie, ARVD of ARVC (cardiomyopathie) is een ziekte dat gekenmerkt wordt door vervetting en fibrosering van het hart. Met name de rechter ventrikel (apex), de rechter ventrikel uitstroombaan en de rechter ventrikel vrije wand , net onder de triscupidalis kleppen zijn het meest aangedaan. Meestal is de rechter ventrikel aangedaan, maar de linker kamer kan ook betrokken zijn.
 
  
Door de vervetting en fibrosering ontstaan er makkelijk kamerritmestoornissen, die zich kunnen uiten als palpitaties, duizeligheid, syncope (flauwvallen) of plotselinge dood. Op latere leeftijd kan ook rechterkamerfalen optreden door een verminderde pompkracht.
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'''Arrythmogenic Right Ventricular Cardiomyopathy''', (ARVC, or ARVD: Arrythmogenic Right Ventricular Disease) is characterized by fatty replacement and fibrosis of the heart. Especially the right ventricle apex and outflow tract are involved. However the left ventricle can be affected to.
  
De diagnose wordt gesteld op basis van major en minor criteria van de European Society of Cardiology.<cite>McKenna1994</cite>
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As a result of the fatty replacement and fibrosis, ventricular arrhythmias are common in this disease and can lead to palpitations, syncope and sudden death. At older age right ventricular pump failure can occur.
  
ARVD is een progressieve ziekte. De incidentie wordt geschat op 1:3.000-1:10.000. De ziekte presenteert zich vooral in de tienerleeftijd. Hoewel de ziekte vaker bij atleten wordt gezien, wordt het echter niet door sport veroorzaakt. ARVD kan familiair voorkomen; er zijn inmiddels 9 chromosomale afwijkingen gevonden in verschillende families. De ziekte lijkt autosomaal dominant over te erven.  
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The diagnosis is based on major and minor criteria, as published by the European Society of Cardiology.<cite>McKenna1994</cite>
Hiernaast is er ook tenminste één duidelijk onderkende variant van ARVD (De ziekte van Naxos, naar het Griekse eiland waar een familie gevonden is met deze variant) die met een autosomaal recessief patroon overerft.
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ARVC is a progressive disease. The '''incidence''' is estimated to be 1:3.000-1:10.000. Manifestations is usually during teenage. Although the diagnosis is more often made in athletes, sports are not thought to have a causative relationship with the disease. ARVD can occur in families; more than 9 different chromosomal defects have been described, most often with autosomal dominant inheritance.  
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One unique form of ARVD, called Naxos disease (after the Greek island where it was first diagnosed), has an autosomal recessive pattern of inheritance.  
  
 
{{clr}}
 
{{clr}}
  
===Diagnose===
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===Diagnosis===
 
[[Image:epsilon_wave.png|thumb|Een epsilon golf in V1]]
 
[[Image:epsilon_wave.png|thumb|Een epsilon golf in V1]]
De diagnose ARVD is niet makkelijk te stellen, behalve bij obductie. Op het ECG zijn soms een aantal karakteristieke afwijkingen te zien, namelijk:
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ARVC is a difficult diagnosis to make. ECG characteristics that can occur in ARVC are:
* Epsilon golven
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* Epsilon waves
* Verlengde QRS duur (>110ms) in de precordiale afleidingen (V1–V3)
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* QRS prolongation (>110ms) in the precordial leads (V1–V3)
* Late potentialen op een [[signal averaged ECG]]
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* Late potentials on a [[signal averaged ECG]]
  
 
The European Society of Cardiology has created a list of diagnositc criteria for the diagnosis of ARVC.
 
The European Society of Cardiology has created a list of diagnositc criteria for the diagnosis of ARVC.
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{{clr}}
 
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===Behandeling===
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===Treatment===
De therapie bestaat vooralsnog uit preventie van complicaties.<cite>ACC2006</cite>
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Treatment focusses on avoiding complications.<cite>ACC2006</cite>
 
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*Medication:
*Medicatie: anti-arritmetica: Amiodarone, Sotalol; verder ace-remmers ter voorkoming van remodeling van het hart.
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**anti-arrithmics: Amiodarone, Sotalol
*Preventieve [[:w:nl:Internal_Cardiac_Defibrillator|ICD]] plaatsing bij patienten die ondanks optimale medicamenteuze behandeling een sustained [[Ritmestoornissen#Ventrikeltachycardie|ventriculaire tachycardie]] of [[Ritmestoornissen#Ventrikelfibrilleren|ventrikelfibrilleren]] ontwikkelen. Ook komen in aanmerking voor ICD: patiënten met een uitgebreid aangedaan hart waarbij bijvoorbeeld ook de linker kamer aangedaan is, patiënten met meerdere familie-leden die overleden zijn aan ARVD, patiënten die ondanks medicatie een syncope hebben waarbij ventrikelfibrilleren of ventrikeltachycardie niet kan worden uitgesloten.
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**ACE-inhibitors to prevent cardiac remodelling
*[[Ablatie]] kan toegepast worden bij patienten met recidiverende ventrikeltachycardiën ondanks medicamenteuze behandeling.
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*Preventive [[ICD]] placements in patients in whom optimal medical treatment did not prevent [[ventricular tachycardia]] or [[ventricular fibrillation]]. Other patients who qualify for ICD placement: patients with severe disease with left ventricular involvement, patients with more family members who died of ARVD, patients who had a syncope while on medication and in whom arrhythmias cannot be excluded as a cause.
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*[[Ablation]] in patients with recurrent [[ventricular tachycardia]].
  
==Externe links==
 
* [http://www.cardiogenetica.nl Cardiogenetica.nl]
 
 
==Referenties==
 
==Referenties==
 
<biblio>
 
<biblio>

Revision as of 15:56, 1 August 2007

Author(s) J.S.S.G. de Jong, MD
Moderator J.S.S.G. de Jong, MD
Supervisor
some notes about authorship

What is ARVC?

A section throught the heart of a ARVC patient. (A) Transmural fatty replacement of the right ventricular free wall. (B) Myocardial atrophy is confined to the right ventricle and substantially spares the interventricular septum as well as the left ventricular free wall. [1] Reproduced with permission from BMJ Publishing Group Ltd.

Arrythmogenic Right Ventricular Cardiomyopathy, (ARVC, or ARVD: Arrythmogenic Right Ventricular Disease) is characterized by fatty replacement and fibrosis of the heart. Especially the right ventricle apex and outflow tract are involved. However the left ventricle can be affected to.

As a result of the fatty replacement and fibrosis, ventricular arrhythmias are common in this disease and can lead to palpitations, syncope and sudden death. At older age right ventricular pump failure can occur.

The diagnosis is based on major and minor criteria, as published by the European Society of Cardiology.[2]

ARVC is a progressive disease. The incidence is estimated to be 1:3.000-1:10.000. Manifestations is usually during teenage. Although the diagnosis is more often made in athletes, sports are not thought to have a causative relationship with the disease. ARVD can occur in families; more than 9 different chromosomal defects have been described, most often with autosomal dominant inheritance.

One unique form of ARVD, called Naxos disease (after the Greek island where it was first diagnosed), has an autosomal recessive pattern of inheritance.


Diagnosis

Een epsilon golf in V1

ARVC is a difficult diagnosis to make. ECG characteristics that can occur in ARVC are:

  • Epsilon waves
  • QRS prolongation (>110ms) in the precordial leads (V1–V3)
  • Late potentials on a signal averaged ECG

The European Society of Cardiology has created a list of diagnositc criteria for the diagnosis of ARVC.

Major diagnostic criteria for Aritmogenic Right Ventricular Cardiomyopathy[2]
  • Familial disease confirmed at necroscopy or surgery
  • Severe dilatation and reduciton of right ventricular ejection fraction with no (or only mild) LV impairment
  • Localised irhgt ventricular aneurysms (akinetic or diskinetic areas with diastolic bulging)
  • Severe segmental dilataion of the right ventricle
  • Fibrofatty replacement of myocardium on endomyocardial biopsy
Diagnostic criteria that can be diagnosed on the ECG
  • (major) Epsilon wave or localised prolongation (>110ms) of the QRS complex in right precordial leads (V1-V3)
  • (minor) Inverted T waves in right precordial leads (V2 and V3) (people aged more than 12 yr; in absence of RBBB
  • (minor) Late potentials (signal averaged ECG)
  • (minor) Left bundle branch block type ventricular tachycardia (sutained and non-sustained) (ECG, Holter, exercise testing
  • (minor) Frequent ventricular extrasystoles (more than 1000/24h) (Holter)


Treatment

Treatment focusses on avoiding complications.[3]

  • Medication:
    • anti-arrithmics: Amiodarone, Sotalol
    • ACE-inhibitors to prevent cardiac remodelling
  • Preventive ICD placements in patients in whom optimal medical treatment did not prevent ventricular tachycardia or ventricular fibrillation. Other patients who qualify for ICD placement: patients with severe disease with left ventricular involvement, patients with more family members who died of ARVD, patients who had a syncope while on medication and in whom arrhythmias cannot be excluded as a cause.
  • Ablation in patients with recurrent ventricular tachycardia.

Referenties

Error fetching PMID 8142187:
Error fetching PMID 10768917:
Error fetching PMID 16949478:
  1. Error fetching PMID 10768917: [Corrado]
  2. Error fetching PMID 8142187: [McKenna1994]
  3. Error fetching PMID 16949478: [ACC2006]

All Medline abstracts: PubMed | HubMed