Arrhythmogenic Right Ventricular Cardiomyopathy: Difference between revisions

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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 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. ]]
[[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. ]]
 
[[Image:epsilon_wave.png|thumb|ECG with an epsilon wave in V1]]
'''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.
'''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.


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


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'''Diagnosis''' ARVC is a difficult diagnosis to make. Therefore, the European Society of Cardiology has created a list of diagnositc criteria for the diagnosis of ARVC<cite>#McKenna1994</cite>




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**anti-arrithmics: Amiodarone, Sotalol
**anti-arrithmics: Amiodarone, Sotalol
**ACE-inhibitors to prevent cardiac remodelling
**ACE-inhibitors to prevent cardiac remodelling
*Preventive [[ICD]] placements in patients in whom optimal medical treatment did not prevent [[Ventricular Tachycardia|ventricular tachycardia]] or [[Ventricular Fibrillation|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.
*[[ICD]] implantation is recommended for the prevention of sudden cardiac death in patients with ARVC with documented sustained VT or VF who are receiving chronic optimal medical therapy.
*Radiofrequency [[ablation]] in patients with recurrent [[Ventricular Tachycardia|ventricular tachycardia]].
*[[ICD]]] implantation can be considered for the prevention of sudden cardiac death in patients with ARVC with extensive disease, including those with left ventricular involvement, 1 or more affected family member with SCD, or undiagnosed syncope when [[Ventricular Tachycardia|ventricular tachycardia]] or [[Ventricular Fibrillation|ventricular Fibrillation]] has not been excluded as the cause of syncope, who are receiving chronic optimal medical therapy, and who have reasonable expectation of survival with a good functional status for more than 1 y.
*Radiofrequency [[ablation]] can be useful as adjunctive therapy in management of patients with ARVC with recurrent [[Ventricular Tachycardia|ventricular tachycardia]], despite optimal antiarrhythmic drug therapy.
 
 


==Referenties==
==Referenties==